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Wu YS, Garcia J, Schmoke N, Nemeh C, Liszewski MC, Duron V. Rare Congenital Lung Lesions in Even Rarer Locations: A Single-Institution Experience with Extrathoracic Congenital Lung Lesions. J Laparoendosc Adv Surg Tech A 2025. [PMID: 40014420 DOI: 10.1089/lap.2024.0384] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/01/2025] Open
Abstract
Background: Extrathoracic congenital lung lesions (CLLs) are a rare anomaly of lung development. Management and surgical approach are variable. We present our institutional experience with extrathoracic CLLs for the past 18 years. Methods: We retrospectively reviewed all patients younger than 18 years diagnosed with a CLL from January 2005 to June 2023. Only patients with suspected extrathoracic CLLs on prenatal imaging were included. Results: A CLL was identified in 381 patients and was extrathoracic in 25 patients (6.6%). Six patients (24%) with other congenital anomalies were excluded, and 3 were lost to follow-up. The prenatal course and delivery of the 16 study patients were unremarkable and all were asymptomatic. Larger lesions, both prenatally and postnatally, were more likely to undergo surgical resection (11.8 ± 8.2 cm3 versus 3.9 ± 3.3 cm3, P = .05; 6.1 ± 5.1 cm3 versus 2.5 ± 2.5 cm3, P = .10, respectively). Seven patients (7/16; 44%) underwent resection-5 thoracoscopic and 2 laparoscopic. Thoracoscopic transdiaphragmatic approach was favored for lesions extending >50% above the diaphragm. Five lesions were intradiaphragmatic, one infradiaphragmatic, and one intrathoracic. Four lesions (4/7; 57%) were hybrid lesions on pathology. No malignancy was identified. Nine patients (9/16; 56%) underwent surveillance. Imaging modality for surveillance varied. Duration of follow-up ranged from 0.5 to 83 months. Conclusions: We provide the largest case series to date describing both operative and nonoperative management of extrathoracic CLLs. Surveillance appears to be appropriate for small lesions. If surgical excision is pursued, the thoracoscopic approach is safe and preferred if diaphragmatic involvement is suspected.
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Affiliation(s)
- Yeu Sanz Wu
- Division of Pediatric Surgery, Columbia University Irving Medical Center/New York Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA
| | - Juan Garcia
- Department of Radiology, Columbia University Irving Medical Center/New York Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA
| | - Nicholas Schmoke
- Division of Pediatric Surgery, Columbia University Irving Medical Center/New York Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA
| | - Christopher Nemeh
- Division of Pediatric Surgery, Columbia University Irving Medical Center/New York Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA
| | - Mark C Liszewski
- Department of Radiology, Columbia University Irving Medical Center/New York Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA
| | - Vincent Duron
- Division of Pediatric Surgery, Columbia University Irving Medical Center/New York Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA
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Alberti P, Ade-Ajayi N, Greenough A. Respiratory Support Strategies for Surgical Neonates: A Review. CHILDREN (BASEL, SWITZERLAND) 2025; 12:273. [PMID: 40150556 PMCID: PMC11941308 DOI: 10.3390/children12030273] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/31/2025] [Revised: 02/16/2025] [Accepted: 02/22/2025] [Indexed: 03/29/2025]
Abstract
Neonates with congenital conditions which require surgical management frequently experience respiratory distress. This review discusses the management of pulmonary complications and the respiratory support strategies for four conditions: oesophageal atresia-tracheoesophageal fistula (OA-TOF), congenital diaphragmatic hernia (CDH), congenital lung malformations (CLM), and anterior abdominal wall defects (AWD). Mechanical ventilation techniques which can reduce the risk of ventilator-induced lung injury (VILI) are discussed, as well as the use of non-invasive respiratory support modes. While advances in perioperative respiratory support have improved outcomes in infants with OA-TOF, managing respiratory distress in premature OA-TOF neonates remains a challenge. In CDH infants, a randomised trial has suggested that conventional ventilation may improve outcomes compared to high-frequency ventilation. Echocardiographic assessment is essential in the management of CDH infants with pulmonary hypertension. Lung-protective ventilation settings may lower the rate of postoperative complications in symptomatic CLM infants, but there remains debate regarding the choice of expectant versus surgical management in neonates with asymptomatic CLMs. Infants with AWDs can require ventilation due to pulmonary hypoplasia, but the effects of this on their long-term respiratory health are poorly understood. As surgical techniques continue to evolve and novel ventilation techniques become available, prospective multi-centre studies will be required to define the optimal respiratory support strategies for neonatal surgical conditions that affect lung function.
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Affiliation(s)
- Piero Alberti
- Department of Women and Children’s Health, School of Life Course Sciences, Faculty of Life Sciences and Medicine, King’s College London, London SE5 9RS, UK; (P.A.); (N.A.-A.)
| | - Niyi Ade-Ajayi
- Department of Women and Children’s Health, School of Life Course Sciences, Faculty of Life Sciences and Medicine, King’s College London, London SE5 9RS, UK; (P.A.); (N.A.-A.)
- Department of Paediatric Surgery, King’s College Hospital, Denmark Hill, London SE5 9RS, UK
| | - Anne Greenough
- Department of Women and Children’s Health, School of Life Course Sciences, Faculty of Life Sciences and Medicine, King’s College London, London SE5 9RS, UK; (P.A.); (N.A.-A.)
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Kambe M, Oki T, Iizuka S, Otsuki Y, Nakamura T. Confronting Upside-Down Video-Assisted Thoracic Surgery Approach for Hemorrhagic Bronchogenic Cyst Manifested by Sudden Back Pain. Surg Case Rep 2025; 11:24-0126. [PMID: 40012964 PMCID: PMC11861584 DOI: 10.70352/scrj.cr.24-0126] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/09/2024] [Accepted: 01/30/2025] [Indexed: 02/28/2025] Open
Abstract
INTRODUCTION Bronchogenic cysts, arising from an aberrant bronchial primordium inclusion during the fetal period, are typically located in the mediastinum but can develop in ectopic regions. While generally asymptomatic, these cysts may become symptomatic due to infection or, rarely, hemorrhage. This report details a case of a hemorrhagic bronchogenic cyst in the supradiaphragmatic region, successfully resected using video-assisted thoracic surgery (VATS) with a confronting upside-down monitor setting. CASE PRESENTATION An 18-year-old female presented with a fever and sudden left-sided back pain. Blood tests revealed leukocytosis and an elevated C-reactive protein. Imaging studies identified a well-circumscribed cyst along the left diaphragm, suspected to be an infected bronchogenic cyst. Magnetic resonance imaging 2 days later indicated disease progression with concomitant empyema, prompting emergency surgery. Using the confronting upside-down monitor setting, the cyst was resected. Thoracoscopic findings revealed a dark red cyst and bloody pleural effusion. The surgery was uneventful, and the patient was discharged on postoperative day 2. Bacterial cultures of the pleural effusion and cystic content were negative, and histopathological analysis confirmed the diagnosis of a hemorrhagic bronchogenic cyst. CONCLUSIONS Hemorrhagic bronchogenic cysts should be considered in the differential diagnosis of intrathoracic cysts presenting with sudden pain. Upfront surgery is recommended for symptomatic bronchogenic cysts, irrespective of the location or etiology. VATS via the confronting upside-down monitor setting is the feasible option alongside the conventional approach.
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Affiliation(s)
- Masato Kambe
- Department of General Thoracic Surgery, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
| | - Tomonari Oki
- Department of General Thoracic Surgery, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
| | - Shuhei Iizuka
- Department of General Thoracic Surgery, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
| | - Yoshiro Otsuki
- Department of Pathology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
| | - Toru Nakamura
- Department of General Thoracic Surgery, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan
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Tolekova N, Antonova Z, Kartulev N, Gabrovska N, Shivachev H. A Rare Case of Extralobar Bronchopulmonary Sequestration. Cureus 2024; 16:e73330. [PMID: 39655139 PMCID: PMC11626991 DOI: 10.7759/cureus.73330] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/09/2024] [Indexed: 12/12/2024] Open
Abstract
Bronchopulmonary sequestration is a rare developmental abnormality of the pulmonary system. It is a pulmonary malformation and is defined as lung tissue without connection to the tracheobronchial tree with a systemic blood supply. We present a case of an eight-month-old infant with prenatally diagnosed bronchopulmonary sequestration. At six months of age, an extralobar sequestration with a feeding vessel from truncus coeliacus and venous drainage to vena thoracica interna sinistra was diagnosed from a CT scan of the lung. Thoracoscopy was performed with the identification of intradiaphragmatic sequestration, conversion to thoracotomy with resection of the lesion, and repair of the diaphragm. Postoperative follow-up revealed no complications. Intradiaphragmatic bronchopulmonary sequestration is an extremely rare congenital anomaly. The gold standard for diagnostic clarification is computed tomography. The preferred approach for surgical treatment is transthoracic.
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Affiliation(s)
- Nadezhda Tolekova
- Pediatric Surgery, University Multi-Profile Hospital for Active Treatment and Emergency Medicine (UMHATEM) "N.I. Pirogov", Sofia, BGR
| | - Zdravka Antonova
- Pediatric Surgery, University Multi-Profile Hospital for Active Treatment and Emergency Medicine (UMHATEM) "N.I. Pirogov", Sofia, BGR
| | - Nikola Kartulev
- Pediatric Surgery, University Multi-Profile Hospital for Active Treatment and Emergency Medicine (UMHATEM) "N.I. Pirogov", Sofia, BGR
| | - Natalia Gabrovska
- Pediatric Pulmology, Specialized Hospital for Active Treatment of Children's Diseases "Prof. Ivan Mitev", Sofia, BGR
| | - Hristo Shivachev
- Pediatric Surgery, University Multi-Profile Hospital for Active Treatment and Emergency Medicine (UMHATEM) "N.I. Pirogov", Sofia, BGR
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di Natale A, Flohr S, Mathew L, Katterman C, Gallagher C, Reynolds TA, Gebb JS, Panitch HB, Oliver ER, Rintoul NE, Peranteau WH, Flake AW, Adzick NS, Hedrick HL. Neonatal Outcomes of Multiple Congenital Thoracic Lesions. Fetal Diagn Ther 2024:1-13. [PMID: 39245038 DOI: 10.1159/000541319] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2024] [Accepted: 08/28/2024] [Indexed: 09/10/2024]
Abstract
INTRODUCTION Congenital pulmonary airway malformations (CPAM), intra- and extralobar bronchopulmonary sequestrations (iBPS/eBPS), CPAM-BPS hybrid lesions (HL), congenital lobar emphysema (CLE), bronchial atresia (BA), and foregut duplication cysts (FDC), collectively referred to as congenital thoracic lesions (CTL), are mostly solitary. Patients with multiple CTL are rare, and reports on such cases are scarce. To address this dearth, we analyzed a large multifocal CTL patient cohort. METHODS Retrospective chart review of patients born between September 1, 2013, and March 31, 2023, who underwent surgery for a CTL at our tertiary center. Patients with radiological and surgical diagnosis of multifocal CTL, defined as ≥2 CTL present in more than one lobe were included to record pre-, peri-, and postnatal patient characteristics. RESULTS Among 701 CTL patients, 74 (10.5%) had multiple CTL. CTL multifocality was prenatally recognized correctly in 8 (12.9%) patients. Most multiple CTL were right-sided, unilateral multilobar lesions (n = 33, 44%). Bilateral CTL were found in 9 (12.1%) patients. CPAM-CPAM lesions were the most prevalent CTL types (n = 36, 49%). Genetic syndromes were confirmed in 3 (4%) and additional congenital anomalies in 9 (12.9%) patients, 5 of those had multiple congenital anomalies. Of 49 (65%) patients with multilobar CTL, 25 (51%) underwent bilobectomy and 24 (49%) lung-sparing surgery. Length of stay was similar. Mortality was 5.4%. CONCLUSION We report on the largest patient cohort with multiple CTL to date. Multiple CTL occurred in 1/10 patients with CTL, and only 12.9% were recognized prenatally. Lung-sparing surgery can be considered. Multiple additional congenital anomalies and genetic syndromes may be more common and genetic testing should be considered. Overall, outcomes in this patient population are favorable.
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Affiliation(s)
- Anthony di Natale
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Sabrina Flohr
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Leny Mathew
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Cara Katterman
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Colby Gallagher
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Thomas A Reynolds
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Juliana S Gebb
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Howard B Panitch
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Edward R Oliver
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Natalie E Rintoul
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - William H Peranteau
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Alan W Flake
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - N Scott Adzick
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
| | - Holly L Hedrick
- Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
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6
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Breckenfelder C, Dawson-Gore CC, Galambos C, Corkum KS, Partrick D, Derderian SC. Congenital Pulmonary Airway Malformation Associated With Papillary Adenocarcinoma. ANNALS OF THORACIC SURGERY SHORT REPORTS 2024; 2:400-403. [PMID: 39790383 PMCID: PMC11708395 DOI: 10.1016/j.atssr.2024.04.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Accepted: 04/01/2024] [Indexed: 01/12/2025]
Abstract
Congenital pulmonary airway malformations (CPAMs) are cystic lung lesions often detected prenatally. Resection is often recommended for potential recurrent infections and malignancy. This report describes a case of a 14-year-old female patient who presented with abdominal pain. A computed tomographic scan of the abdomen revealed a cystic lesion at the base of her right lung. Consequently, a computed tomographic arteriogram of the chest demonstrated a right lower lobe lesion concerning for type I CPAM. After thoracoscopic segmentectomy, histopathologic examination revealed papillary adenocarcinoma with a KRAS mutation. Residual CPAM prompted a lobectomy, emphasizing the importance of surgical intervention for cystic lesions.
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Affiliation(s)
| | | | - Csaba Galambos
- Department of Pathology, University of Colorado School of Medicine and Children’s Hospital Colorado, Aurora, Colorado
| | - Kristine S. Corkum
- Department of Surgery, University of Colorado School of Medicine, Aurora, Colorado
- Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine and Children’s Hospital Colorado, Aurora, Colorado
| | - David Partrick
- Department of Surgery, University of Colorado School of Medicine, Aurora, Colorado
- Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine and Children’s Hospital Colorado, Aurora, Colorado
| | - S. Christopher Derderian
- Department of Surgery, University of Colorado School of Medicine, Aurora, Colorado
- Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine and Children’s Hospital Colorado, Aurora, Colorado
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7
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Khan AA, Agrawal M, Ahuja S. Clinical and histopathological spectrum of congenital pulmonary airway malformations: A case series. Int J Surg Case Rep 2024; 122:110096. [PMID: 39088970 PMCID: PMC11342210 DOI: 10.1016/j.ijscr.2024.110096] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2024] [Revised: 07/21/2024] [Accepted: 07/26/2024] [Indexed: 08/03/2024] Open
Abstract
BACKGROUND Congenital pulmonary airway malformations (CPAM) are a spectrum of cystic and non-cystic anomalies arising from abnormal airway development in utero, with an incidence of 1 in 25,000 to 35,000 births. CPAM can present prenatally or postnatally with respiratory distress, recurrent infections, or occasionally as an incidental finding. This case series aims to highlight the clinical, radiological, and histopathological characteristics of CPAM through three pediatric cases, which include types 1, 2, and 3 CPAM. CASE PRESENTATION Case 1: A four-month-old male presented with cough, cold, and respiratory distress. Imaging revealed hypoattenuation and overinflation of the left upper lobe with mediastinal shift. Left upper lobectomy confirmed CPAM type 2. Case 2: A one-month-old female presented with recurrent respiratory distress and infections. Imaging showed a large cystic lesion in the right middle lobe. Right middle lobectomy confirmed CPAM type 3. Case 3: A two-month-old male presented with dyspnea. Imaging showed a large bullae in the right chest with mediastinal shift. Right upper lobectomy confirmed CPAM type 1. CLINICAL DISCUSSION CPAM is a rare congenital lung malformation characterized by abnormal bronchial development and localized glandular overgrowth. Management involves surgical resection, with the timing of surgery dependent on symptomatology. Early identification and intervention are crucial for preventing complications such as pulmonary hypoplasia and recurrent infections. Histopathological examination post-resection is essential for accurate classification and management of CPAM. CONCLUSION This case series provides valuable insights into the clinical, radiological, and histopathological features of CPAM, including the types encountered in each case. It underscores the importance of early diagnosis and timely surgical intervention, advocating for routine prenatal and postnatal screenings to effectively identify and manage CPAM.
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Affiliation(s)
- Adil Aziz Khan
- Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
| | - Meetu Agrawal
- Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
| | - Sana Ahuja
- Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
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8
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Plut D, Bauer M, Mikić A, Winant AJ, Park H, Lee EY. Pediatric Congenital Lung Malformations: Contemporary Perspectives on Imaging Characteristics. Semin Roentgenol 2024; 59:249-266. [PMID: 38997180 DOI: 10.1053/j.ro.2024.03.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2024] [Revised: 03/13/2024] [Accepted: 03/15/2024] [Indexed: 07/14/2024]
Affiliation(s)
- Domen Plut
- Clinical Radiology Institute, University Medical Centre Ljubljana, Ljubljana, Slovenia; Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
| | - Matthew Bauer
- Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
| | - Aleksandra Mikić
- Clinical Radiology Institute, University Medical Centre Ljubljana, Ljubljana, Slovenia; Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Abbey J Winant
- Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
| | - Halley Park
- Department of Radiology, Nicklaus Children's Hospital, Miami, FL
| | - Edward Y Lee
- Department of Radiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
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Pandey A, Pandey A, Keshari S, Dulal A, Acharya S, Chaudhary A, Chaudhary A, Pande P, Bhardwaj S. Intralobar bronchopulmonary sequestration in an adult: a case report. Ann Med Surg (Lond) 2024; 86:4143-4145. [PMID: 38989173 PMCID: PMC11230738 DOI: 10.1097/ms9.0000000000001969] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2023] [Accepted: 03/06/2024] [Indexed: 07/12/2024] Open
Abstract
Introduction Bronchopulmonary sequestration (BPS) is typically a rare congenital disorder characterized by the presence of non-functioning lung tissue. There are two types of BPS: intralobar and extralobar sequestration, where extralobar sequestration can either be intrathoracic or sub-diaphragmatic. Case presentation In this case report, we present the case of a 70-year-old male with intralobar BPS who presented with recurrent chest infections, and a diagnosis of intralobar pulmonary sequestration was made based on a computed tomography (CT) scan. Discussion The diagnosis of intralobar pulmonary sequestration can be delayed as the intralobar type can present with varying imaging findings. A diagnosis can be made based on CT or MRI findings. A CT scan or MRI can show mass or consolidation with or without a cyst. Both CT and MRI can be reliable modalities to identify the arterial supply of the sequestered lung tissue, which is commonly a branch of the descending aorta. Conclusion Sequestration should be suspected when a posterobasal lung abnormality is supplied by an abnormal artery from the aorta or another systemic artery.
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Affiliation(s)
- Abhishek Pandey
- Kathmandu University School of Medical Sciences, Dhulikhel, Kavre
| | - Archana Pandey
- Kathmandu University School of Medical Sciences, Dhulikhel, Kavre
| | - Suraj Keshari
- Kathmandu University School of Medical Sciences, Dhulikhel, Kavre
| | - Aliza Dulal
- Kathmandu University School of Medical Sciences, Dhulikhel, Kavre
| | - Suyash Acharya
- Kathmandu University School of Medical Sciences, Dhulikhel, Kavre
| | | | | | | | - Sushant Bhardwaj
- Kathmandu University School of Medical Sciences, Dhulikhel, Kavre
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10
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Chataut D, Katwal S, Suwal S, Thapa A, Bhusal A. Endovascular embolization for massive hemoptysis in intralobar pulmonary sequestration with celiac artery supply: A comprehensive case report. Radiol Case Rep 2024; 19:2239-2244. [PMID: 38523721 PMCID: PMC10959639 DOI: 10.1016/j.radcr.2024.02.069] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2023] [Revised: 02/16/2024] [Accepted: 02/18/2024] [Indexed: 03/26/2024] Open
Abstract
Pulmonary sequestration (PS) is a rare congenital anomaly characterized by noncommunicative lung tissue supplied by an abnormal systemic vessel. We present a case of a 30-year-old male with intralobar PS, receiving arterial supply from the celiac artery, manifesting as massive hemoptysis. After urgent stabilization, endovascular embolization using polyvinyl alcohol particles was successfully employed. The patient's symptoms resolved, and follow-up confirmed satisfactory recovery. Our case underscores the diverse arterial origins of PS and the efficacy of endovascular embolization as a minimally invasive treatment. The complexity of PS, its diagnostic imaging, and alternative therapeutic options are discussed, emphasizing tailored approaches for optimal outcomes in managing this uncommon congenital anomaly.
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Affiliation(s)
- Dinesh Chataut
- Department of Radiology, Maharajgunj Medical Campus, Kathmandu, Nepal
| | - Shailendra Katwal
- Department of Radiology, Dadeldhura Subregional Hospital, Dadeldhura, Nepal
| | - Sundar Suwal
- Department of Radiology, Maharajgunj Medical Campus, Kathmandu, Nepal
| | - Ajit Thapa
- Department of Radiology, Maharajgunj Medical Campus, Kathmandu, Nepal
| | - Amrit Bhusal
- Department of Radiology, BP Koirala Institute of Health Science, Sunsari, Nepal
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11
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Rahman ST, Islam MM, Akhter KM, Islam MZ, Hossain M. Bronchogenic cyst at unusual location. Respir Med Case Rep 2023; 46:101947. [PMID: 38058403 PMCID: PMC10696229 DOI: 10.1016/j.rmcr.2023.101947] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2023] [Revised: 11/03/2023] [Accepted: 11/08/2023] [Indexed: 12/08/2023] Open
Abstract
Bronchogenic cyst results from abnormal bronchial budding. Thin-stalked mobile bronchogenic cysts are rare and sometimes radiologically mimic mass lesion, making preoperative diagnosis difficult. We present a 12-year-old boy with a preoperative diagnosis of intraparenchymal cystic lung lesion misled by radiology. We performed a mini-thoracotomy revealing a thin stalked mobile elongated cyst that arose from the right inferior pulmonary ligament, confirmed as a bronchogenic cyst in histopathology.
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Affiliation(s)
- S.M. Tajdit Rahman
- Department of Thoracic Surgery, National Institute of Diseases of the Chest & Hospital, Dhaka, Bangladesh
| | - Md. Morsedul Islam
- Department of Thoracic Surgery, National Institute of Diseases of the Chest & Hospital, Dhaka, Bangladesh
| | - Kazi Munzerin Akhter
- Department of Thoracic Surgery, National Institute of Diseases of the Chest & Hospital, Dhaka, Bangladesh
| | - Md. Zahidul Islam
- Department of Thoracic Surgery, National Institute of Diseases of the Chest & Hospital, Dhaka, Bangladesh
| | - Mosharraf Hossain
- Department of Thoracic Surgery, National Institute of Diseases of the Chest & Hospital, Dhaka, Bangladesh
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12
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Pederiva F, Rothenberg SS, Hall N, Ijsselstijn H, Wong KKY, von der Thüsen J, Ciet P, Achiron R, Pio d'Adamo A, Schnater JM. Congenital lung malformations. Nat Rev Dis Primers 2023; 9:60. [PMID: 37919294 DOI: 10.1038/s41572-023-00470-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/03/2023] [Indexed: 11/04/2023]
Abstract
Congenital lung malformations (CLMs) are rare developmental anomalies of the lung, including congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and isolated congenital bronchial atresia. CLMs occur in 4 out of 10,000 live births. Postnatal presentation ranges from an asymptomatic infant to respiratory failure. CLMs are typically diagnosed with antenatal ultrasonography and confirmed by chest CT angiography in the first few months of life. Although surgical treatment is the gold standard for symptomatic CLMs, a consensus on asymptomatic cases has not been reached. Resection, either thoracoscopically or through thoracotomy, minimizes the risk of local morbidity, including recurrent infections and pneumothorax, and avoids the risk of malignancies that have been associated with CPAM, bronchopulmonary sequestration and bronchogenic cyst. However, some surgeons suggest expectant management as the incidence of adverse outcomes, including malignancy, remains unknown. In either case, a planned follow-up and a proper transition to adult care are needed. The biological mechanisms through which some CLMs may trigger malignant transformation are under investigation. KRAS has already been confirmed to be somatically mutated in CPAM and other genetic susceptibilities linked to tumour development have been explored. By summarizing current progress in CLM diagnosis, management and molecular understanding we hope to highlight open questions that require urgent attention.
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Affiliation(s)
- Federica Pederiva
- Paediatric Surgery, "F. Del Ponte" Hospital, ASST Settelaghi, Varese, Italy.
| | - Steven S Rothenberg
- Department of Paediatric Surgery, Rocky Mountain Hospital for Children, Denver, CO, USA
| | - Nigel Hall
- University Surgery Unit, Faculty of Medicine, University of Southampton, Southampton, UK
| | - Hanneke Ijsselstijn
- Department of Paediatric Surgery and Intensive Care, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands
| | - Kenneth K Y Wong
- Department of Surgery, University of Hong Kong, Queen Mary Hospital, Hong Kong, China
| | - Jan von der Thüsen
- Department of Pathology and Clinical Bioinformatics, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands
| | - Pierluigi Ciet
- Departments of Radiology and Nuclear Medicine and Respiratory Medicine and Allergology, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands
- Department of Radiology, University of Cagliari, Cagliari, Italy
| | - Reuven Achiron
- Department of Obstetrics and Gynecology, Fetal Medicine Unit, The Chaim Sheba Medical Center Tel-Hashomer, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Adamo Pio d'Adamo
- Laboratory of Medical Genetics, Institute for Maternal and Child Health - IRCCS "Burlo Garofolo", Trieste, Italy
- Department of Medical, Surgical and Health Sciences, University of Trieste, Trieste, Italy
| | - J Marco Schnater
- Department of Paediatric Surgery, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands
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13
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Huerta CT, Ramsey WA, Courel SC, Gilna GP, Saberi RA, Ribieras AJ, Perez EA, Sola JE, Thorson CM. Nationwide Outcomes After Thoracoscopic Versus Open Resection of Congenital Pulmonary Airway Malformations in Newborns. J Laparoendosc Adv Surg Tech A 2023; 33:897-903. [PMID: 37406288 DOI: 10.1089/lap.2023.0010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/07/2023] Open
Abstract
Purpose: Elective resection of congenital pulmonary airway malformations (CPAM) has been debated for decades and varies significantly between individual surgeons. However, few studies have compared outcomes and costs associated with thoracoscopic and open thoracotomy approaches on a national level. This study sought to compare nationwide outcomes and resource utilization in infants undergoing elective lung resection for CPAM. Materials and Methods: The Nationwide Readmission Database was queried from 2010 to 2014 for newborns who underwent elective surgical resection of CPAM. Patients were stratified by operative approach (thoracoscopic versus open). Demographics, hospital characteristics, and outcomes were analyzed using standard statistical tests. Results: A total of 1716 newborns with CPAM were identified. Elective readmission for pulmonary resection was performed in 12% (n = 198), with 63% of resections completed at a different hospital than the newborn stay. Most resections were thoracoscopic (75%), compared to only 25% via thoracotomy. Infants treated with thoracoscopic resection were more often male (78% versus 62% open, P = .040) and were older at the time of resection. Patients who had an open thoracotomy experienced a higher rate of serious complications (40% versus 10% thoracoscopic, P < .001), including postoperative hemorrhage, tension pneumothorax, and pulmonary collapse. Readmission costs were higher for infants treated via thoracotomy (P < .001). Conclusion: Thoracoscopic lung resection for CPAM is associated with lower cost and fewer postoperative complications than thoracotomy. Most resections are performed at different hospitals than the place of birth, which may affect long-term outcomes from single institutional studies. These findings may be used to address costs and improve future evaluations of elective CPAM resections.
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Affiliation(s)
- Carlos Theodore Huerta
- Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, Florida, USA
| | - Walter A Ramsey
- Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, Florida, USA
| | - Steve C Courel
- University of Miami Miller School of Medicine, Miami, Florida, USA
| | - Gareth P Gilna
- Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, Florida, USA
| | - Rebecca A Saberi
- Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, Florida, USA
| | - Antoine J Ribieras
- Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, Florida, USA
| | - Eduardo A Perez
- Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, Florida, USA
| | - Juan E Sola
- Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, Florida, USA
| | - Chad M Thorson
- Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, Miami, Florida, USA
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14
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Joo S, Yun T, Kang CH, Na KJ, Park S, Park IK, Kim YT. Thoracoscopic segmentectomy in children with congenital lung malformation. Sci Rep 2023; 13:9640. [PMID: 37316608 DOI: 10.1038/s41598-023-36700-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2022] [Accepted: 06/08/2023] [Indexed: 06/16/2023] Open
Abstract
Congenital lung malformations (CLM) are most commonly treated with a pulmonary lobectomy. However, due to technological advancement, video-assisted thoracoscopic surgery (VATS) segmentectomy is becoming an attractive alternative to VATS lobectomy. This study aimed to evaluate the safety, feasibility, and efficacy of VATS segmentectomy as a lung parenchyma-saving strategy in children with CLM. A retrospective analysis was performed on 85 children, for whom VATS segmentectomy was tried for CLM between January 2010 and July 2020. We compared the surgical outcomes of VATS segmentectomy with the outcomes of 465 patients who underwent VATS lobectomy. Eighty-four patients received VATS segmentectomy and thoracotomy conversion was necessary for one patient for CLM. The mean age was 3.2 ± 2.5 (range 1.2-11.6) years. The mean operative time was 91.4 ± 35.6 (range 40-200) minutes. The median duration of chest tube drainage was 1 (range 1-21) day, and the median length of postoperative hospital stay was 4 (range 3-23) days. There were no postoperative mortality and postoperative complications developed in 7 patients (8.2%), including persistent air leakage in 6 patients (7.1%) and postoperative pneumonia in 1 patient (1.2%). The median follow-up period was 33.5 (interquartile range 31-57) months and there were no patients requiring re-intervention or reoperation during the follow-up period. In the VATS segmentectomy group, the persistent air leakage rate was higher than in the VATS lobectomy group (7.1 vs. 1.1%, p = 0.003). Otherwise, postoperative outcomes were comparable between the two groups. VATS segmentectomy in children with CLM is a technically feasible alternative to VATS lobectomy with acceptable early and mid-term outcomes. However, the persistent air-leakage rate was higher in VATS segmentectomy.
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Affiliation(s)
- Seohee Joo
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Korea
| | - Taeyoung Yun
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Korea
| | - Chang Hyun Kang
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Korea.
| | - Kwon Joong Na
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Korea
| | - Samina Park
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Korea
| | - In Kyu Park
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Korea
| | - Young Tae Kim
- Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Korea
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15
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Oreglio C, Tocchioni F, Ghionzoli M, Buccoliero A, Morabito A, Morini F. Intradiaphragmatic pulmonary sequestrations: a surgical challenge. Case series. Front Surg 2023; 10:1181007. [PMID: 37304185 PMCID: PMC10248170 DOI: 10.3389/fsurg.2023.1181007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2023] [Accepted: 05/05/2023] [Indexed: 06/13/2023] Open
Abstract
Bronchopulmonary sequestrations (BPSs) are rare congenital anomalies characterized by non-functioning embryonic lung tissue receiving anomalous blood supply. They are most commonly located within the thorax (supradiaphragmatic) or into the abdominal cavity (infradiaphragmatic). Intradiaphragmatic extralobar BPs (IDEPS) are an exceptionally rare finding, representing a diagnostic and operative challenge. We report three cases of IDEPS and their surgical management, describing our experience and approach to such rare clinical entities. From 2016 to 2022, we treated 3 cases of IDEPS. Surgical techniques, histopathological findings and clinical outcomes were retrospectively evaluated for each case and compared. Three different surgical techniques were used to approach each lesion, from open thoracotomy to a combined laparoscopic and thoracoscopic approach. Histopathological analysis of the specimens revealed hybrid pathological features, proper of both congenital pulmonary airway malformation (CPAM) and extralobar pulmonary sequestration. IDEPS represent a surgical challenge for pediatric surgeons, given their complex surgical planning. In our experience, the thoracoscopic approach is safe and feasible when performed by trained surgeons, even though a combined thoracoscopic-laparoscopic approach allows for optimal vessels control. The presence of CPAM elements within the lesions supports their surgical removal. Additional studies are required to better characterize IDEPS and their management.
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Affiliation(s)
- Chiara Oreglio
- Department of Neuroscience, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy
- Department of Pediatric and Neonatal Surgery, Meyer Children’s Hospital IRCCS, Florence, Italy
| | - Francesca Tocchioni
- Department of Pediatric and Neonatal Surgery, Meyer Children’s Hospital IRCCS, Florence, Italy
| | - Marco Ghionzoli
- Department of Pediatric and Adolescent Surgery, University of Pisa, Pisa, Italy
- Department of Surgical, Medical, Molecular Pathology and of the Critic Area, University of Pisa, Pisa, Italy
| | | | - Antonino Morabito
- Department of Neuroscience, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy
- Department of Pediatric and Neonatal Surgery, Meyer Children’s Hospital IRCCS, Florence, Italy
| | - Francesco Morini
- Department of Neuroscience, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy
- Department of Pediatric and Neonatal Surgery, Meyer Children’s Hospital IRCCS, Florence, Italy
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16
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Yang W, Gao Y, Li P, Eckman MH. Should asymptomatic patients with congenital lung malformations undergo surgery? A decision analysis. Pediatr Pulmonol 2023; 58:449-456. [PMID: 36251577 PMCID: PMC10092826 DOI: 10.1002/ppul.26206] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/28/2022] [Revised: 09/22/2022] [Accepted: 10/12/2022] [Indexed: 01/25/2023]
Abstract
BACKGROUND Congenital lung malformations (CLMs) are being detected more frequently during pregnancy. There is controversy regarding the optimal treatment for an asymptomatic child with prenatally diagnosed CLMs. Due to the paucity of information from clinical trials, we developed decision analytic models to compare two treatment strategies for such patients-elective surgical resection versus expectant management. METHODS We built decision analytic models stratified by lesion size. We used data from English language literature identified through PubMed searches along with estimates from expert opinions of surgical colleagues. We analyzed results for two hypothetical asymptomatic 6-month-old children with CLMs; one has a large lesion occupying more than 50% of the involved lobe, while the other has a small lesion occupying less than 50% of the involved lobe. We used quality-adjusted life years (QALYs) to measure effectiveness. RESULTS For an asymptomatic child with a small or large lesion, expectant management resulted in a small gain of 0.09 or 0.15 QALYs, respectively. Sensitivity analyses showed that surgical resection would be preferred if the probability of remaining asymptomatic was low. CONCLUSIONS In contrast to current practice, expectant management may be a better alternative for asymptomatic children born with CLMs. More longitudinal studies are required to improve the accuracy of the model.
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Affiliation(s)
- Weili Yang
- Department of Environmental and Public Health Sciences, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.,Department of Pediatric Surgery, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China
| | - Ya Gao
- Department of Pediatric Surgery, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China
| | - Peng Li
- Department of Pediatric Surgery, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China
| | - Mark H Eckman
- Division of General Internal Medicine, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
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17
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Gupta A, Chetty G, Hopkinson D, Rao J, Chetty G. Right upper lobe pulmonary sequestration masquerading clinically and radiologically as malignancy: a case report. J Surg Case Rep 2023; 2023:rjad022. [PMID: 36741081 PMCID: PMC9890208 DOI: 10.1093/jscr/rjad022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/14/2022] [Revised: 12/06/2022] [Indexed: 02/04/2023] Open
Abstract
Bronchopulmonary sequestration is a rare disease in which a non-functional region of pulmonary tissue receives an aberrant vascular supply and lacks normal communication with the tracheobronchial tree. We present the case of a 30-year-old female with a primary complaint of unexplained weight loss and no other additional signs or symptoms. In view of this, computed tomography imaging was ordered, showing a 33HU mass in the right upper lobe. A specialist radiologist reviewed the images and concluded that the most likely differentials were mediastinal lymphoma or thymic malignancy. Video-assisted thoracoscopic surgery was performed, when it was seen that no malignancy was present, but rather a bronchopulmonary sequestration. Histology confirmed the diagnosis; the patient fared well post-operatively. Bronchopulmonary sequestration is a rare pathology, with most cases occurring in the lower lung lobes. This case is highly atypical, due to the lack of clinical features and the lesion radiologically mimicking the appearance of malignancy.
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Affiliation(s)
- Ankit Gupta
- School of Medicine, Worsley Building, University of Leeds, Woodhouse, Leeds, LS2 9JT, UK
| | - Gavin Chetty
- Northern General Hospital, Herries Road, Sheffield S5 7AU, UK
| | - David Hopkinson
- Northern General Hospital, Herries Road, Sheffield S5 7AU, UK
| | - Jagan Rao
- Northern General Hospital, Herries Road, Sheffield S5 7AU, UK
| | - Govind Chetty
- Correspondence address. Northern General Hospital, Herries Road, Sheffield S5 7AU, UK. Tel: (+44)114 2714833; Fax: (+44)114 2610350; E-mail:
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18
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Cheng K, Liu X, Yuan M, Yang G, He T, Luo D, Liu C, Xu C. Thoracoscopic anatomic pulmonary segmentectomy for the treatment of congenital lung malformation in children. Asian J Surg 2023; 46:532-538. [PMID: 35780025 DOI: 10.1016/j.asjsur.2022.06.040] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2022] [Revised: 04/28/2022] [Accepted: 06/10/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Lung-sparing surgery has been used to treat congenital lung malformation in children, and segmentectomy has been advocated as a lung-preservation strategy. However, thoracoscopic pulmonary segmentectomy has gained limited popularity considering the complications, the potential for residual lesions, and the technical difficulties associated with this procedure. Therefore, this study aimed to investigate the safety and feasibility of pediatric thoracoscopic anatomic pulmonary segmentectomy for the treatment of congenital lung malformations. METHODS We conducted a retrospective review of the medical records of 568 patients who were treated at West China Hospital, Sichuan University, from January 2014 to January 2020. The patients were divided into segmentectomy and lobectomy groups according to the surgical procedures they underwent. Clinical and follow-up outcomes were compared between the two groups. RESULTS The segmentectomy and lobectomy groups included 206 and 361 cases, respectively. The mean intraoperative blood loss was significantly higher in the segmentectomy group (6.9 mL vs. 4.5 mL; p = 0.03). The mean surgical time was also significantly longer in the segmentectomy group, (55.6 min vs. 41.5 min; p = 0.018). However, the incidence of complications did not differ significantly between the two groups (2.9% vs. 1.1%, p = 0.21). Patients in both groups did not require reoperation or show residual lesions during hospitalization and follow-up. CONCLUSIONS Thoracoscopic anatomic pulmonary segmentectomy is a safe and feasible definitive lung-sparing treatment for specific cases of congenital lung malformation, and has a complication rate comparable to that of thoracoscopic lobectomy.
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Affiliation(s)
- Kaisheng Cheng
- Department of Pediatric Surgery, West China Hospital, Sichuan University, China
| | - Xiaojuan Liu
- Department of Laboratory Medicine, West China Second University Hospital, Sichuan University, China
| | - Miao Yuan
- Department of Pediatric Surgery, West China Hospital, Sichuan University, China
| | - Gang Yang
- Department of Pediatric Surgery, West China Hospital, Sichuan University, China
| | - Taozhen He
- Department of Pediatric Surgery, West China Hospital, Sichuan University, China
| | - Dengke Luo
- Department of Pediatric Surgery, West China Hospital, Sichuan University, China
| | - Chenyu Liu
- Department of Pediatric Surgery, West China Hospital, Sichuan University, China
| | - Chang Xu
- Department of Pediatric Surgery, West China Hospital, Sichuan University, China.
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19
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Qiu M, Lin DR. Buccal carcinoma associated with adult congenital pulmonary hypoplasia: A case report and literature review. Medicine (Baltimore) 2022; 101:e32142. [PMID: 36482601 PMCID: PMC9726420 DOI: 10.1097/md.0000000000032142] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
RATIONALE Congenital pulmonary hypoplasia is a relatively rare lung malformation. The disease is usually identified in the neonatal period or early childhood. Most affected patients die of respiratory insufficiency after birth. Mild congenital dysplasia is asymptomatic, while moderate congenital dysplasia manifests as respiratory distress or does not become evident until adulthood. Cases of congenital pulmonary hypoplasia in adults are rare, especially in combination with malignant tumors. PATIENT CONCERNS We report the case of a 64-year-old man with buccal carcinoma who was accidentally found to have hypoplasia of the left lung during treatment. DIAGNOSES After chest computed tomography and chest radiograph, the diagnosis of adult congenital pulmonary hypoplasia was confirmed. INTERVENTIONS Since the patient had a history of bronchiectasis for more than 30 years and only had 1 healthy lung, the cardiopulmonary compensatory function was poor and the patient could not tolerate surgery, he was given radiotherapy and chemotherapy. OUTCOMES Radiotherapy and chemotherapy were successfully completed, and within the patient's tolerance, there was no serious adverse reaction of respiratory system. CONCLUSION Congenital pulmonary hypoplasia's diagnosis is challenging in adults because the condition can easily be mistaken for a more common disease. However, early diagnosis is very important to enable prompt therapy and ensure proper follow-up to detect and treat complications such as pulmonary infection and pulmonary hypertension in a timely manner.
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Affiliation(s)
- Miao Qiu
- Department of Tumor Radiotherapy, Shaoxing Second Hospital, Shaoxing, China
- *Correspondence: Miao Qiu, Department of Tumor Radiotherapy, Shaoxing Second Hospital, Shaoxing, Zhejiang 312000, China (e-mail: )
| | - De-Rong Lin
- Department of Tumor Radiotherapy, Shaoxing Second Hospital, Shaoxing, China
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20
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Karlsson M, Conner P, Ehren H, Bitkover C, Burgos CM. The natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations. J Pediatr Surg 2022; 57:282-287. [PMID: 35431039 DOI: 10.1016/j.jpedsurg.2022.03.021] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/27/2021] [Revised: 03/21/2022] [Accepted: 03/22/2022] [Indexed: 02/04/2023]
Abstract
BACKGROUND The natural history of congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestrations (BPS) is not fully understood, and the management of the newborn with an asymptomatic lesion is a controversial issue. We aimed to study the natural history and outcome of CPAM/BPS at our institution with a policy of watchful waiting, and to investigate if any prognostic factors in the pre- and/or postnatal- period may predict the need for surgery. MATERIAL AND METHODS A retrospective review study was conducted of children prenatally diagnosed with CPAM and/or BPS during the 18-year period, from 2002 to 2020. Data from the pre and postnatal period was collected and analysed. RESULTS Sixty- six patients with prenatally observed lung lesions were entered in the study, with an overall survival rate of 94%. Fifty-six percent of the lesions decreased in size during gestation. Thirty-one percent had surgery and 69% could be managed conservatively with a median follow-up of 4 years. Nineteen percent developed symptoms after the neonatal period. Children with a presence of mediastinal shift on postnatal imaging (p = 0.003), with a high CVR (p = 0.005) and a large lesion size during gestation (p = 0.014) were significantly more likely to require surgery. CONCLUSION Prenatal regression is common among prenatally diagnosed CPAM/BPS and the majority of children that are asymptomatic beyond the neonatal period will remain asymptomatic throughout their childhood. Future analysis with a longer follow-up might give new insights in order to identify children at risk of developing symptoms. LEVEL OF EVIDENCE III.
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Affiliation(s)
- Matilda Karlsson
- Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden; Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.
| | - Peter Conner
- Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Center for Fetal Medicine Karolinska University Hospital, Stockholm, Sweden
| | - Henrik Ehren
- Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden; Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
| | - Catarina Bitkover
- Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden; Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
| | - Carmen Mesas Burgos
- Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden; Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
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21
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Hermelijn SM, Mackenbach MJ, van Horik C, Ciet P, Wolf JL, von der Thüsen JH, Wijnen RMH, Tiddens HAWM, Schnater JM. Quantitative CT imaging analysis to predict pathology features in patients with a congenital pulmonary airway malformation. J Pediatr Surg 2022; 57:1567-1572. [PMID: 34809963 DOI: 10.1016/j.jpedsurg.2021.10.008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/14/2021] [Revised: 09/23/2021] [Accepted: 10/10/2021] [Indexed: 11/25/2022]
Abstract
BACKGROUND Risk for infection and potential malignant degeneration are the most common arguments for resecting asymptomatic Congenital Pulmonary Airway Malformations (CPAM). We aimed to investigate if CT- imaging characteristics can be used to predict histopathological features, by using an objective quantitative CT scoring method. METHODS Archival CPAM tissue samples were histologically re-assessed and patients who had a pre-operative volumetric CT-scan were included. Lung disease was quantified using the newly-developed congenital lung abnormality quantification(CLAQ) scoring method and obtained percentages were used to predict histopathological signs of inflammation and presence of mucinous proliferation (MP). Because MP is presumed a precursor for mucinous adenocarcinoma in situ (AIS) this method was also used to compare CT-scans of patients with AIS to those with only CPAM. RESULTS Thirty-three CPAM patients were included of which 13(39%) had histological signs of inflammation and 8(24%) had a MP. Patients with inflammation had a significantly smaller lesion (14% vs 38%) while those with MP had more extensive disease (54%vs17%). Patients with AIS had a significantly smaller lesion compared to CPAM patients (5%vs29%). Significant predictors for inflammation were smaller lesion size and percentage hypodensity within lesions while a larger lesion size and percentage parenchymal hyperdensity (solid lung tissue components) were predictors for MP as well as AIS. CONCLUSIONS Smaller CPAM lesions may be more susceptible to inflammation while larger lesions may be associated with the presence of MP. Parenchymal hyperdensity is found as a predictor for MP as well as AIS and should therefore elicit more extensive gross sampling. LEVEL OF EVIDENCE Level III.
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Affiliation(s)
- Sergei M Hermelijn
- Department of Pediatric Surgery, Erasmus Medical Center, Sophia Children's Hospital, Molewaterplein 40. 3015 GD Rotterdam, Mailing address: Postbus 2060, Rotterdam 3000 CB, the Netherlands
| | - Maarten J Mackenbach
- Department of Pediatric Metabolic Diseases, Erasmus Medical Center, Sophia Children's Hospital, Rotterdam, the Netherlands
| | - Cathy van Horik
- Department of Pediatric Surgery, Erasmus Medical Center, Sophia Children's Hospital, Molewaterplein 40. 3015 GD Rotterdam, Mailing address: Postbus 2060, Rotterdam 3000 CB, the Netherlands
| | - Pierluigi Ciet
- Department of Pediatric Pulmonology, Erasmus Medical Center, Sophia Children's Hospital, Rotterdam, the Netherlands; Department of Radiology, Erasmus University Medical Center, Rotterdam, the Netherlands
| | - Janina L Wolf
- Department of Pathology, Erasmus University Medical Center, Rotterdam, the Netherlands
| | - Jan H von der Thüsen
- Department of Pathology, Erasmus University Medical Center, Rotterdam, the Netherlands
| | - René M H Wijnen
- Department of Pediatric Surgery, Erasmus Medical Center, Sophia Children's Hospital, Molewaterplein 40. 3015 GD Rotterdam, Mailing address: Postbus 2060, Rotterdam 3000 CB, the Netherlands
| | - Harm A W M Tiddens
- Department of Pediatric Pulmonology, Erasmus Medical Center, Sophia Children's Hospital, Rotterdam, the Netherlands; Department of Radiology, Erasmus University Medical Center, Rotterdam, the Netherlands
| | - J Marco Schnater
- Department of Pediatric Surgery, Erasmus Medical Center, Sophia Children's Hospital, Molewaterplein 40. 3015 GD Rotterdam, Mailing address: Postbus 2060, Rotterdam 3000 CB, the Netherlands.
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22
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Lopyan NM, Perrone EE, VanHulle R, Bloom DA, Mychaliska GB, Speck KE. A single institution's experience with the management of peripheral bronchial atresia. Pediatr Surg Int 2022; 38:853-860. [PMID: 35229175 DOI: 10.1007/s00383-022-05089-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 02/16/2022] [Indexed: 11/27/2022]
Abstract
PURPOSE Peripheral bronchial atresia is a pulmonary abnormality diagnosed on postnatal computed tomography after prenatal imaging reveals a congenital lung lesion. Debate regarding management of this abnormality prompted us to review our institution's practice patterns and outcomes. METHODS All patients diagnosed with bronchial atresia were assessed from 6/2014 to 7/2020. Pediatric radiologists were surveyed to delineate computed tomography criteria used to diagnose peripheral bronchial atresia. Criteria were applied in an independent blinded review of postnatal imaging. Data for patients determined to have peripheral bronchial atresia and at least an initial pediatric surgical evaluation were analyzed. RESULTS Twenty-eight patients with bronchial atresia received at least an initial pediatric surgical evaluation. Expectant management was planned for 22/28 (79%) patients. Two patients transitioned from an expectant management strategy to an operative strategy for recurrent respiratory infections; final pathology revealed bronchial atresia in both. Six patients were initially managed operatively; final pathology revealed bronchial atresia (n = 3) or congenital lobar overinflation (n = 3). CONCLUSIONS Peripheral bronchial atresia can be safely managed expectantly. A change in symptoms is suspicious for alternate lung pathology, warranting further workup and consideration for resection. LEVEL OF EVIDENCE Level IV.
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Affiliation(s)
- Natalie M Lopyan
- Division of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, 1540 E. Hospital Dr., Ann Arbor, MI, USA.
| | - Erin E Perrone
- Division of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, 1540 E. Hospital Dr., Ann Arbor, MI, USA
- Fetal Diagnosis and Treatment Center, University of Michigan, Michigan Medicine, Ann Arbor, MI, USA
| | - Rachel VanHulle
- Division of Pediatric Radiology, Department of Radiology, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA
| | - David A Bloom
- Division of Pediatric Radiology, Department of Radiology, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA
| | - George B Mychaliska
- Division of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, 1540 E. Hospital Dr., Ann Arbor, MI, USA
- Fetal Diagnosis and Treatment Center, University of Michigan, Michigan Medicine, Ann Arbor, MI, USA
| | - K Elizabeth Speck
- Division of Pediatric Surgery, Department of Surgery, C.S. Mott Children's Hospital, University of Michigan, 1540 E. Hospital Dr., Ann Arbor, MI, USA
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Vinayak TP, Mohanty S, Das K. 'Hybrid' Bronchopulmonary Malformation - Lobar Emphysema and Extra Lobar Sequestration. Fetal Pediatr Pathol 2022; 41:505-510. [PMID: 33252288 DOI: 10.1080/15513815.2020.1849474] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
Background: Idiopathic lobar emphysema (ILE) and bronchopulmonary sequestration (BPS) are two of the well-characterized pulmonary malformations. Case report: An antenatally detected case of a left bronchopulmonary malformation (BPM) was clinicoradiologically diagnosed to be a left upper lobar emphysema with isolated dextrocardia in the neonatal period. Besides the emphysematous left upper lobe, an accessory lobe akin to an extra lobar BPS was an operative surprise. Histopathological examination of both excised lobes led to a revised diagnosis of a 'hybrid' malformation comprising lobar emphysema and extra lobar BPS. The postoperative recovery was uneventful. Discussion/conclusion: The observations suggest that BPS and ILE may be interim entities in a continuum of abnormal embryogenesis. Such a hybrid malformation has not been hitherto reported.
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Affiliation(s)
| | - Suravi Mohanty
- Department of Pathology, St. John's Medical College, Bangalore, India
| | - Kanishka Das
- Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, India
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van den Beukel - Bakker EM, Pijnenburg MW. An infant with fever and tachypnoea: diagnosis at first sight? Breathe (Sheff) 2022; 18:220002. [DOI: 10.1183/20734735.0002-2022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2022] [Accepted: 03/22/2022] [Indexed: 11/05/2022] Open
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Gao Y, Han X, Jin J, Tan Z. Ten cases of intradiaphragmatic extralobar pulmonary sequestration: a single-center experience. WORLD JOURNAL OF PEDIATRIC SURGERY 2022; 5:e000334. [DOI: 10.1136/wjps-2021-000334] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2021] [Accepted: 01/14/2022] [Indexed: 11/04/2022] Open
Abstract
BackgroundIntradiaphragmatic extralobar pulmonary sequestration (IDEPS) is a rare type of pulmonary sequestration (PS). The purpose of this study is to assess diagnosis and operative treatment of IDEPS.MethodsPatients with PS who were diagnosed and treated in our center from January 2015 to December 2020 were analyzed retrospectively to identify patients with IDEPS.ResultsTotally, 215 patients with PS were treated surgically, including 10 cases with IDEPS. Prenatal ultrasounds and postnatal-enhanced CT showed the presence of IDEPS in four cases and in seven cases, respectively. The three-dimensional (3D) reconstruction software was performed perfectly to identify the location of the lesions in 10 cases. The surgeries were performed smoothly by laparoscopic surgery in one case, video-assisted thoracic surgery (VATS) in five cases and Da Vinci robot-assisted thoracoscopic surgery (DVRATS) in four cases. In the VATS group, the average operative duration, intraoperative blood loss volume, length of stay after operation, and postoperative thoracic catheter indwelling duration were 48 min, 3.8 mL, 6.4 days and 2.2 days, respectively. That of the DVRATS group were 80 min, 3.5 mL, 4.3 days and 1.5 days, respectively. No side effects had appeared.ConclusionsThe 3D reconstruction software was proven to be capable in assisting the assessment of IDEPS. We suggested early surgery to treat IDEPS, and the best path was accessing the mass from the chest. Both DVRATS and VATS for the treatment of an IDEPS are safe, feasible, and effective. Furthermore, DVRATS provides a 3D magnified view, more flexibility and precision.
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Johansen M, Veyckemans F, Engelhardt T. Congenital anomalies of the large intrathoracic airways. Paediatr Anaesth 2022; 32:126-137. [PMID: 34797930 DOI: 10.1111/pan.14339] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/21/2021] [Revised: 11/03/2021] [Accepted: 11/15/2021] [Indexed: 11/29/2022]
Abstract
Congenital lung lesions are numerous but rare in individual clinical practice. They do require close multidisciplinary collaboration between health care professionals. This educational review will focus on the pathophysiology, clinical manifestations, surgical approaches, and anesthetic management of congenital anomalies of the large intrathoracic airways: congenital tracheal stenosis, tracheal agenesis, tracheal diverticulum, bronchial anomalies (tracheal, esophageal, or bridging bronchus), congenital lung malformations, lung sequestrations and Scimitar syndrome, lobar emphysema, Williams-Campbell syndrome, and pleuropulmonary blastoma. In addition, this review will illustrate common pitfalls and challenges related to the anesthesia management with emphasis on ventilation and correct endotracheal tube positioning.
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Affiliation(s)
- Mathias Johansen
- Department of Paediatric Anaesthesiology, Montreal Children's Hospital, Montreal, Quebec, Canada
| | - Francis Veyckemans
- Clinique d'Anesthésie Pédiatrique, Hôpital Jeanne de Flandre, CHU de Lille, Lille, France
| | - Thomas Engelhardt
- Department of Paediatric Anaesthesiology, Montreal Children's Hospital, Montreal, Quebec, Canada
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Ohm B, Jungraithmayr W. Angeborene Fehlbildungen der Lunge – eine Übersicht. Zentralbl Chir 2022; 147:90-97. [DOI: 10.1055/a-1669-9574] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Abstract
ZusammenfassungKongenitale pulmonale Malformationen stellen eine heterogene Gruppe seltener
Erkrankungen dar, die auf Fehlentwicklungen während der embryonalen und fetalen
Wachstumsphase basieren. Zu ihnen gehören der Trachealbronchus, die bronchiale
Atresie, die bronchogene Zyste, die Lungensequestration, das kongenitale lobäre
Emphysem sowie die sogenannte Congenital pulmonary Airway Malformation. Eines
der Leitsymptome dieser Malformationen ist die durch ihren verdrängenden Effekt
bedingte postnatale respiratorische Insuffizienz, welche eine rasche operative
Versorgung erfordert. Auch bei asymptomatischen Malformationen wird aufgrund des
erhöhten Infektrisikos die Resektion empfohlen.In der folgenden Übersicht wird auf die Ursachen, das klinische Bild und die
therapeutischen Optionen dieser angeborenen Fehlbildungen der Lunge und des
Bronchialsystems eingegangen.
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Affiliation(s)
- Birte Ohm
- Klinik für Thoraxchirurgie, Universitätsklinikum Freiburg, Medizinische
Fakultät, Albert-Ludwigs-Universität Freiburg, Freiburg,
Deutschland
| | - Wolfgang Jungraithmayr
- Klinik für Thoraxchirurgie, Universitätsklinikum Freiburg, Medizinische
Fakultät, Albert-Ludwigs-Universität Freiburg, Freiburg,
Deutschland
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28
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Chen T, Yu J, Zhang N, Chen C, Fu L, Zeng Q. Intralobar Pulmonary Sequestration Presenting as Hemothorax Secondary to Spontaneous Pneumothorax: Case Report and Literature Review. Front Pediatr 2022; 10:937563. [PMID: 35844764 PMCID: PMC9279614 DOI: 10.3389/fped.2022.937563] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2022] [Accepted: 06/13/2022] [Indexed: 11/13/2022] Open
Abstract
INTRODUCTION Patients with pulmonary sequestration (PS), a rare congenital lung malformation, are mostly asymptomatic. Recurrent localized infection is a major complication, while sudden hemothorax is extremely rare. We present a case of intralobar PS presenting as hemothorax secondary to spontaneous pneumothorax and comprehensively review the relevant literature. CASE REPORT A 16-year-old male presented with chest pain after strenuous exercise. Chest X-ray showed a moderate pneumothorax. After admission and conservative treatment, he developed dizziness, amaurosis, and urinary incontinence. Bedside chest X-ray suggested a massive pleural effusion, and hemothorax was further identified via catheter drainage. Contrast-enhanced computed tomography was performed, and no abnormal blood vessels or leakage of contrast agent were observed. As the hemoglobin level continued to drop, exploratory thoracoscopic surgery was performed immediately. The abnormal systemic artery supplying the lung tissue was found to be ruptured; therefore, ligation of the abnormal artery with resection of the diseased lung tissue was performed. Pathological examination revealed non-specific manifestations of PS. He was followed up for 1 year without related complications. CONCLUSION Our case suggests that the abnormal supply vessels of PS are unstable, which may cause sudden hemothorax. Therefore, patients with PS should undergo surgery promptly after diagnosis. In patients with hemothorax, we should consider the diagnosis of PS; however, contrast-enhanced computed tomography or angiography cannot confirm the diagnosis in all cases. Surgical intervention is recommended in emergency settings.
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Affiliation(s)
- Tian Chen
- Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
| | - Jie Yu
- Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
| | - Na Zhang
- Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
| | - Chenghao Chen
- Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
| | - Libing Fu
- Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
| | - Qi Zeng
- Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China
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Lv L, Zhai Y, Zhao H, Guo R, Xu H, Zhang S. Case Report: An extremely rare case of double extralobar pulmonary sequestration with anomalous supplying arteries originating from the abdominal aorta in the left thoracic cavity. Front Pediatr 2022; 10:926942. [PMID: 35935357 PMCID: PMC9354599 DOI: 10.3389/fped.2022.926942] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2022] [Accepted: 06/30/2022] [Indexed: 11/13/2022] Open
Abstract
To the best of our knowledge, double or multiple extralobar pulmonary sequestrations (PSs) with anomalous arterial supply in the ipsilateral thoracic cavity have rarely been reported before. PS can be divided into two types: intralobar sequestration (ILS) and extralobar sequestration (ELS). We encountered a 5-month-old infant with double ELS in the left thoracic cavity that was incidentally detected during thoracoscopic surgery. Surgical exploration revealed two separate, well-circumscribed abnormal masses in the left thoracic cavity, and the patient was successfully treated using thoracoscopic surgery. Postoperative pathology confirmed that both masses were PS tissues. Accurate preoperative diagnosis using CT alone may be inadequate in this type of case. Therefore, thoracoscopy may be more suitable for diagnosing and treating unusual ELS.
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Affiliation(s)
- Longfei Lv
- Department of Thoracic and Oncological Surgery, Children's Hospital Affiliated to Shandong University, Jinan, China.,Department of Thoracic and Oncological Surgery, Jinan Children's Hospital, Jinan, China
| | - Yunpeng Zhai
- Department of Thoracic and Oncological Surgery, Children's Hospital Affiliated to Shandong University, Jinan, China.,Department of Thoracic and Oncological Surgery, Jinan Children's Hospital, Jinan, China
| | - Huashan Zhao
- Department of Thoracic and Oncological Surgery, Children's Hospital Affiliated to Shandong University, Jinan, China.,Department of Thoracic and Oncological Surgery, Jinan Children's Hospital, Jinan, China
| | - Rui Guo
- Department of Thoracic and Oncological Surgery, Children's Hospital Affiliated to Shandong University, Jinan, China.,Department of Thoracic and Oncological Surgery, Jinan Children's Hospital, Jinan, China
| | - Hongxiu Xu
- Department of Thoracic and Oncological Surgery, Children's Hospital Affiliated to Shandong University, Jinan, China.,Department of Thoracic and Oncological Surgery, Jinan Children's Hospital, Jinan, China
| | - Shisong Zhang
- Department of Thoracic and Oncological Surgery, Children's Hospital Affiliated to Shandong University, Jinan, China.,Department of Thoracic and Oncological Surgery, Jinan Children's Hospital, Jinan, China
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Willers C, Maager L, Bauman G, Cholewa D, Stranzinger E, Raio L, Casaulta C, Latzin P. School-age structural and functional MRI and lung function in children following lung resection for congenital lung malformation in infancy. Pediatr Radiol 2022; 52:1255-1265. [PMID: 35305121 PMCID: PMC9192451 DOI: 10.1007/s00247-022-05317-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/23/2021] [Revised: 10/21/2021] [Accepted: 02/03/2022] [Indexed: 10/31/2022]
Abstract
BACKGROUND The management of asymptomatic congenital lung malformations is debated. Particularly, there is a lack of information regarding long-term growth and development of the remaining lung in children following lung resection for congenital lung malformations. In addition to conventional pulmonary function tests, we used novel functional magnetic resonance imaging (MRI) methods to measure perfusion and ventilation. OBJECTIVE To assess functionality of the remaining lung expanded into the thoracic cavity after resection of congenital lung malformations. MATERIALS AND METHODS A prospective, cross-sectional pilot study in five children who had surgery for congenital lung malformations during infancy. Participants had structural and functional MRI as well as spirometry, body plethysmography and multiple breath washout at school age. RESULTS Structural MRI showed an expansion of the remaining lung in all cases. Fractional ventilation and relative perfusion of the expanded lung were locally decreased in functional MRI. In all other parts of the lungs, fractional ventilation and relative perfusion were normal in all children. There was an association between overall impairment of perfusion and elevated lung clearance index. The results of spirometry and body plethysmography varied between patients, including normal lung function, restriction and obstruction. CONCLUSION Fractional ventilation and relative perfusion maps from functional MRI specifically locate impairment of the remaining lung after lung resection. These changes are not captured by conventional measures such as structural MRI and standard pulmonary function tests. Therefore, following lung resection for congenital lung malformation, children should be investigated more systematically with functional lung MRI.
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Affiliation(s)
- Corin Willers
- grid.5734.50000 0001 0726 5157Division of Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse 8, 3010 Bern, Switzerland
| | - Lukas Maager
- grid.5734.50000 0001 0726 5157Division of Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse 8, 3010 Bern, Switzerland
| | - Grzegorz Bauman
- grid.410567.1Division of Radiological Physics, Department of Radiology, University of Basel Hospital, Basel, Switzerland ,grid.6612.30000 0004 1937 0642Department of Biomedical Engineering, University of Basel, Allschwil, Switzerland
| | - Dietmar Cholewa
- grid.5734.50000 0001 0726 5157Department of Pediatric Surgery, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Enno Stranzinger
- grid.5734.50000 0001 0726 5157Institute of Diagnostic, Interventional and Pediatric Radiology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Luigi Raio
- grid.5734.50000 0001 0726 5157Department of Obstetrics and Gynecology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Carmen Casaulta
- grid.5734.50000 0001 0726 5157Division of Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse 8, 3010 Bern, Switzerland
| | - Philipp Latzin
- Division of Pediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Freiburgstrasse 8, 3010, Bern, Switzerland.
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Weller JH, Peter SDS, Fallat ME, Saito JM, Burns CR, Deans KJ, Fraser JD, Gadepalli SK, Helmrath MA, Hirschl RB, Kabre R, Lal DR, Landman MP, Leys CM, Mak GZ, Minneci PC, Wright TN, Kunisaki SM. Thoracoscopic versus open lobectomy in infants with congenital lung malformations: A multi-institutional propensity score analysis. J Pediatr Surg 2021; 56:2148-2156. [PMID: 34030879 DOI: 10.1016/j.jpedsurg.2021.04.013] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/22/2020] [Revised: 03/18/2021] [Accepted: 04/15/2021] [Indexed: 11/26/2022]
Abstract
PURPOSE The impact of thoracoscopic surgery on outcomes in children with congenital lung malformations (CLM) remains controversial. The purpose of this study was to determine the effect of operative approach on perioperative outcomes in infants undergoing lobectomy for an asymptomatic CLM. METHODS After IRB approval, a retrospective cohort study was conducted on 506 children with a CLM resected at one of eleven children's hospitals over a seven-year period. Infants undergoing elective lobectomy were identified, and covariates were balanced based on operative approach using propensity scores with full matching. Outcomes were analyzed based on intention to treat with weighted conditional regression. RESULTS One hundred seventy-five infants met inclusion criteria. There were 67 (38.3%) open, 89 (50.9%) thoracoscopic, and 19 (10.9%) thoracoscopic-converted-to-open lobectomies. Thoracoscopic lobectomy was associated with significantly longer operative times (26 min, 95% CI 6-47 min, p = 0.012) but used less epidural anesthesia (OR 0.02, 95% CI 0.004-0.11, p<0.001) when compared to open lobectomy. There were no significant differences in intraoperative blood loss, postoperative complications, chest tube duration, or length of stay. CONCLUSIONS Thoracoscopy has become the most common operative approach for elective lobectomy in infants with asymptomatic CLMs. The non-inferiority of thoracoscopic lobectomy in postoperative outcomes supports its continued use as an alternative to open lobectomy. LEVEL OF EVIDENCE Treatment study, Level III.
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Affiliation(s)
- Jennine H Weller
- Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Johns Hopkins Children's Center, Baltimore, MD, United States
| | | | - Mary E Fallat
- Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Norton Children's Hospital, Louisville, KY, United States
| | - Jacqueline M Saito
- Division of Pediatric Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, MO, United States
| | - Cartland R Burns
- Division of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, United States
| | - Katherine J Deans
- Center for Surgical Outcomes Research, the Research Institute and Department of Surgery, Nationwide Children's Hospital, Ohio State University College of Medicine, Columbus, OH, United States
| | - Jason D Fraser
- Children's Mercy Hospital, Kansas City, MO, United States
| | - Samir K Gadepalli
- University of Michigan and Michigan Medicine, C.S. Mott Children's Hospital, Ann Arbor, MI, United States
| | - Michael A Helmrath
- Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States
| | - Ronald B Hirschl
- University of Michigan and Michigan Medicine, C.S. Mott Children's Hospital, Ann Arbor, MI, United States
| | - Rashmi Kabre
- Division of Pediatric Surgery, Department of Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, United States
| | - Dave R Lal
- Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, United States
| | - Matthew P Landman
- Division of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, United States
| | - Charles M Leys
- Division of Pediatric Surgery, Department of Surgery, University of Wisconsin, Madison, WI, United States
| | - Grace Z Mak
- Section of Pediatric Surgery, Department of Surgery, Comer Children's Hospital, University of Chicago Medicine and Biological Sciences, Chicago, IL, United States
| | - Peter C Minneci
- Center for Surgical Outcomes Research, the Research Institute and Department of Surgery, Nationwide Children's Hospital, Ohio State University College of Medicine, Columbus, OH, United States
| | - Tiffany N Wright
- Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Norton Children's Hospital, Louisville, KY, United States
| | - Shaun M Kunisaki
- Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Johns Hopkins Children's Center, Baltimore, MD, United States.
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Elhattab A, Elsaied A, Wafa T, Jugie M, Delacourt C, Sarnacki S, Aly K, Khen-Dunlop N. Thoracoscopic surgery for congenital lung malformations: Does previous infection really matter? J Pediatr Surg 2021; 56:1982-1987. [PMID: 33573805 DOI: 10.1016/j.jpedsurg.2021.01.036] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/22/2020] [Revised: 01/13/2021] [Accepted: 01/19/2021] [Indexed: 12/15/2022]
Abstract
BACKGROUND/ PURPOSE Elective resection of congenital lung malformations (CLMs) is still debatable. The two main risks are malignant transformation and recurrent pulmonary infections. Our study aimed to assess the effect of previous pulmonary infection on the intraoperative and postoperative courses of thoracoscopic surgery for CLMs. METHODS This is a retrospective study including all thoracoscopic lung resections for CLMs between 2010 and 2019. Ninety patients were included. There was a history of previous pulmonary infection in 28 patients (group A) and no such history in 62 patients (group B). RESULTS The median age at operation for group A was 20.4 months (IQR:14.9-41.4) versus 15.1 months (IQR:9.7-20.8) in group B (p = 0.006). There were 10 conversions (35.7%) in group A and 8 (12.9%) in group B (p = 0.02). The operative time was significantly shorter in group B (p<0.002). In group A, 32.1% of patients experienced postoperative fever versus 11.3% of group B (p = 0.03), with higher antibiotics requirement (28.6% versus 6.5% respectively, p = 0.007). However, no significant differences were found in terms of postoperative complications (p = 0.99). CONCLUSION Earlier intervention for CLMs before the development of pulmonary infection carries higher chances for the success of the thoracoscopic approach with shorter operative time and more uneventful postoperative courses.
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Affiliation(s)
- Ahmad Elhattab
- Department of Pediatric Surgery, AP-HP, Necker-Enfants malades Hospital, Paris, France; Department of Pediatric Surgery, Mansoura University Children's Hospital, Mansoura, Egypt
| | - Adham Elsaied
- Department of Pediatric Surgery, Mansoura University Children's Hospital, Mansoura, Egypt
| | - Tamer Wafa
- Department of Pediatric Surgery, Mansoura University Children's Hospital, Mansoura, Egypt
| | - Myriam Jugie
- Intensive care Unit, AP-HP, Necker-Enfants malades Hospital, Paris, France
| | - Christophe Delacourt
- Department of Pediatric Pulmonology, AP-HP, Necker-Enfants malades Hospital, Paris, France; Université de Paris, Paris, France
| | - Sabine Sarnacki
- Department of Pediatric Surgery, AP-HP, Necker-Enfants malades Hospital, Paris, France; Université de Paris, Paris, France
| | - Kamal Aly
- Department of Pediatric Surgery, Mansoura University Children's Hospital, Mansoura, Egypt
| | - Naziha Khen-Dunlop
- Department of Pediatric Surgery, AP-HP, Necker-Enfants malades Hospital, Paris, France; Université de Paris, Paris, France
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Pairawan SS, Tagge EP, Sacks MA, Hashmi A, Radulescu A, Khan FA. Thoracoscopic segmentectomy for a large previously undiagnosed CPAM presenting as a spontaneous pneumothorax: A case report. Int J Surg Case Rep 2021; 87:106412. [PMID: 34560589 PMCID: PMC8473762 DOI: 10.1016/j.ijscr.2021.106412] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2021] [Revised: 09/11/2021] [Accepted: 09/12/2021] [Indexed: 11/30/2022] Open
Abstract
Introduction Incidentally found congenital pulmonary airway malformations (CPAM) in older children are extremely rare and have traditionally been managed with minimally invasive versus open lobectomy of the affected lobe. Presentation of case In this report, we present a 11-year-old male who presented with a recurrent spontaneous pneumothorax and was found to have a large symptomatic CPAM confined to a single segment of the right lower lobe. The patient was successfully treated with thoracoscopic segmentectomy without any residual disease seen on follow up imaging. Discussion Minimally invasive thoracoscopic approach has many advantages over open approach including better pain control, reduced hospital length of stay, and decreased intraoperative blood loss. With increasing use of minimally invasive approaches, lung-sparing surgery has demonstrated to be a viable and an attractive option for definitive resection of CPAM, without compromising resection margins and/or future lung function. Conclusion This report demonstrates that minimally invasive lung-sparing surgical treatment of a large CPAM is feasible in older children.
Older children with CPAM can present with a spontaneous pneumothorax. CPAM confined to a segment of a single lobe should be managed with segmentectomy. VATS segmentectomy for CPAM in older children can be successful.
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Affiliation(s)
- Seyed S Pairawan
- Division of Pediatric Surgery, Department of Surgery, Loma Linda University Children's Hospital, Loma Linda, CA, United States of America
| | - Edward P Tagge
- Division of Pediatric Surgery, Department of Surgery, Loma Linda University Children's Hospital, Loma Linda, CA, United States of America
| | - Marla A Sacks
- Division of Pediatric Surgery, Department of Surgery, Loma Linda University Children's Hospital, Loma Linda, CA, United States of America
| | - Asra Hashmi
- Department of Plastic Surgery, Loma Linda University Medical Center, Loma Linda, CA, United States of America
| | - Andrei Radulescu
- Division of Pediatric Surgery, Department of Surgery, Loma Linda University Children's Hospital, Loma Linda, CA, United States of America
| | - Faraz A Khan
- Division of Pediatric Surgery, Department of Surgery, Loma Linda University Children's Hospital, Loma Linda, CA, United States of America.
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Al Jadaan SA, Alaqeel SM, Alsadoun N, Allababidi NH, Alharbi M, Almuzaini AS, Alsuhaibani M, Alsulaiman A. Clinicopathological features of resected pediatric congenital lung and mediastinal lesions: A single institution experience. Saudi Med J 2021; 42:209-212. [PMID: 33563741 PMCID: PMC7989286 DOI: 10.15537/smj.2021.2.25705] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/21/2021] [Accepted: 01/14/2021] [Indexed: 11/24/2022] Open
Abstract
Objectives: To evaluate resected congenital lung and mediastinal lesions among children and their characteristics in a single tertiary hospital. Methods: A retrospective chart review analysis of all patients under 14 years of age who underwent congenital lung and mediastinal lesion resection in a single tertiary center from June 1997 to June 2018 was performed. Results: In total, 108 cases of resected lung and mediastinal lesions were performed from June 1997 to June 2018. Congenital lung and mediastinal lesions were found in 52 (48%) cases. Overall, 23 cases were males (44%) and 29 (56%) were females. The most common histopathology was congenital lobar emphysema. Conclusion: This study provides a 21-year review of the clinical and histopathological features of resected congenital lung and mediastinal lesions in a single center. Congenital lung and mediastinal lesions represented 48% of all resected lesions.
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Affiliation(s)
- Saud A. Al Jadaan
- From the Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
| | - Suliaman M. Alaqeel
- From the Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
- Address correspondence and reprint request to: Dr. Suliaman M. Alaqeel, Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia. E-mail: ORCID ID: https://orcid.org/0000-0003-2778-9710
| | - Nouf Alsadoun
- From the Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
| | - Noor H. Allababidi
- From the Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
| | - Majed Alharbi
- From the Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
| | - Alaa S. Almuzaini
- From the Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
| | - Marya Alsuhaibani
- From the Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
| | - Abdulrahman Alsulaiman
- From the Department of Pediatric Surgery, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
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Patrizi S, Pederiva F, d'Adamo AP. Whole-Genome Methylation Study of Congenital Lung Malformations in Children. Front Oncol 2021; 11:689833. [PMID: 34262872 PMCID: PMC8273538 DOI: 10.3389/fonc.2021.689833] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/01/2021] [Accepted: 06/07/2021] [Indexed: 11/30/2022] Open
Abstract
Background and Objectives The treatment of asymptomatic patients with congenital pulmonary malformations (CPMs) remains controversial, partially because the relationship between congenital lung malformations and malignancy is still undefined. Change in methylation pattern is a crucial event in human cancer, including lung cancer. We therefore studied all differentially methylated regions (DMRs) in a series of CPMs in an attempt to find methylation anomalies in genes already described in association with malignancy. Methods The DNA extracted from resected congenital lung malformations and control lung tissue was screened using Illumina MethylationEPIC arrays. Comparisons between the group of malformed samples or the malformed samples of same histology or each malformed sample and the controls and between a pleuropulmonary blastoma (PPB) and controls were performed. Moreover, each malformed sample was pairwise compared with its respective control. All differentially methylated regions (DMRs) with an adjusted p-value <0,05 were studied. Results Every comparison highlighted a number of DMRs closed to genes involved either in cell proliferation or in embryonic development or included in the Cancer Gene Census. Their abnormal methylation had been already described in lung tumors. Conclusions Methylation anomalies already described in lung tumors and also shared by the PPB were found in congenital lung malformations, regardless the histology. The presence of methylation abnormalities is suggestive of a correlation between congenital lung malformations and some step of malignant transformation.
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Affiliation(s)
- Sara Patrizi
- Medical, Surgical and Health Sciences Department, University of Trieste, Trieste, Italy
| | - Federica Pederiva
- Pediatric Surgery, Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy
| | - Adamo Pio d'Adamo
- Medical, Surgical and Health Sciences Department, University of Trieste, Trieste, Italy.,Laboratory of Medical Genetics, Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Trieste, Italy
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Morbidity after thoracoscopic resection of congenital pulmonary airway malformations (CPAM): single center experience over a decade. Pediatr Surg Int 2021; 37:549-554. [PMID: 33388955 DOI: 10.1007/s00383-020-04801-1] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 12/02/2020] [Indexed: 10/22/2022]
Abstract
PURPOSE Video-assisted thoracoscopic (VATS) resection of CPAM in children is an established, albeit controversial strategy for its management. We report a 10-year single center experience. METHODS All children underwent VATS (2008-2017) and their current status was reviewed. Patients were grouped: 'symptomatic-P' (if parents reported recurrent lower respiratory tract infections etc.) or 'symptomatic-S' (neonates presenting with respiratory distress/difficulty) or 'asymptomatic'. RESULTS 73 children, aged 10 m (4d-14yrs) underwent VATS; a neonate as an emergency ('symptomatic-S') and all others electively. The lesion was unilateral in all but one case. Histologically none were malignant. Of the elective 72 cases, 7 (10%) required conversion to open thoracotomy. Twenty (27.7%) were 'symptomatic-P' and the duration of surgery when compared to 'asymptomatic' children was longer 269 (range 129-689) versus 178 (range 69-575) minutes (P = 0.01). Post operatively, 8 children (11%) had a grade III/IV (Clavien-Dindo) complication; persistent air leak/pneumothorax (n = 5), chylothorax (n = 1), pleural effusion (n = 1) and seizure/middle cerebral artery thrombosis (n = 1). There was no mortality. Twenty-four children (33.3%) were reported 'symptomatic-P' post-surgery after a median follow up of 2.18 years. The surgical intervention had no impact on 'symptomatic-P' status (P = 0.46). CONCLUSION The risks of surgery may outweigh benefit in asymptomatic children. CLINICALTRIALS. GOV IDENTIFIER NCT04449614.
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Zheng J, Tang H, Xu H, Li J, Mao X, Liu G. Thoracoscopic versus open resection for symptomatic congenital pulmonary airway malformations in neonates: a decade-long retrospective study. BMC Pulm Med 2021; 21:82. [PMID: 33706735 PMCID: PMC7953538 DOI: 10.1186/s12890-021-01445-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/15/2020] [Accepted: 02/23/2021] [Indexed: 11/10/2022] Open
Abstract
PURPOSE The purpose of this study is to evaluate the potential advantages of thoracoscopic versus open resection for symptomatic congenital pulmonary airway malformation (CPAM) in neonates. METHODS A retrospective review of the medical records of neonates (age ≤ 28 days) who underwent surgery for symptomatic CPAM from 2010 to 2020. RESULTS Of the 24 patients, 14 patients underwent thoracoscopic resection and 10 patients underwent open resection. 4 patients with CPAM located in the upper or middle lobes underwent lobectomy, and 20 underwent lung-preserving wedge resection in the lower lobe. Between the two groups, there were no statistically significant differences in related preoperative variables, including gestational age at birth, body weight, head circumference, lesion size, cystic adenomatoid malformation volume ratio (CVR), and age at operation (P > .05). The differences in intraoperative variables were statistically significant. The length of the surgical incision was significantly shorter in thoracoscopic resection group than in open resection group (1.4 cm [1.3-1.8] vs. 6.0 cm [5.0-8.0], P = .000), along with significantly less operative blood loss (3 ml [1-6] vs. 5 ml [2-10], P = .030) but significantly longer operation time (159 min [100-220] vs. 110 min [70-170], P = .003). Regarding postoperative variables, ventilator days, duration of chest tube use and length of hospital stay were not statistically significant (P > .05). CONCLUSION Both thoracoscopic and open resection for symptomatic CPAM achieve good clinical outcomes, even in neonates. Thoracoscopic resection has minimal aesthetic effects and does not increase the risk of surgical or postoperative complications. Lung-preserving resection may be feasible for neonatal CPAM surgery.
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Affiliation(s)
- Jintao Zheng
- Department of Neonatal and Pediatric Surgery, Chancheng District, Foshan Women and Children Hospital Affiliated to Southern Medical University, No. 11 West Renmin Rd, Foshan City, 528000, Guangdong, China.
| | - Huajian Tang
- Department of Neonatal and Pediatric Surgery, Chancheng District, Foshan Women and Children Hospital Affiliated to Southern Medical University, No. 11 West Renmin Rd, Foshan City, 528000, Guangdong, China
| | - Huiyu Xu
- Department of Neonatal and Pediatric Surgery, Chancheng District, Foshan Women and Children Hospital Affiliated to Southern Medical University, No. 11 West Renmin Rd, Foshan City, 528000, Guangdong, China
| | - Jiequan Li
- Department of Neonatal and Pediatric Surgery, Chancheng District, Foshan Women and Children Hospital Affiliated to Southern Medical University, No. 11 West Renmin Rd, Foshan City, 528000, Guangdong, China
| | - Xiangming Mao
- Zhujiang Hospital, Southern Medical University, Guangzhou, 510282, Guangdong, China.
| | - Guoqing Liu
- Department of Neonatal and Pediatric Surgery, Chancheng District, Foshan Women and Children Hospital Affiliated to Southern Medical University, No. 11 West Renmin Rd, Foshan City, 528000, Guangdong, China.
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Frick AE, Decaluwé H, Weynand B, Proesmans M, Van Raemdonck D. Invasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report. Acta Chir Belg 2021; 121:55-60. [PMID: 31262240 DOI: 10.1080/00015458.2019.1634926] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Abstract
INTRODUCTION Congenital pulmonary airway malformation (CPAM), previously described as congenital cystic adenomatoid malformation (CCAM), is a congenital disorder of lung parenchyma. The association with the presence of a malignant transformation like rhabdomyosarcoma, pleuropulmonary blastoma, and most common invasive mucinous adenocarcinoma (IMA) is a rare development described in patients with CPAM. PATIENTS AND METHODS Here, we report the case of a 68-year-old male patient who underwent a right lower lobectomy for a mass in the right pulmonary lobe. From his clinical history, we noted a recurrent pulmonary infection of a bullous malformation in the right lower lobe treated with antibiotics. RESULTS The histopathological finding showed an invasive mucinous adenocarcinoma arising in a type 1 CPAM in the right lower lobe. A review of presentation, diagnosis, and treatment of this association is described in a case report. CONCLUSIONS Surgical resection should be considered in adults with asymptomatic cysts to prevent malignant transformation. For further analysis, histopathological examination of specimen is essential for a proper diagnosis and eventually further postoperative treatment.
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Affiliation(s)
- A. E. Frick
- Department of Thoracic Surgery, University Hospitals Leuven, Leuven, Belgium
| | - H. Decaluwé
- Department of Thoracic Surgery, University Hospitals Leuven, Leuven, Belgium
| | - B. Weynand
- Department of Pathology, University Hospitals Leuven, Leuven, Belgium
| | - M. Proesmans
- Department of Pediatrics, University Hospitals Leuven, Leuven, Belgium
| | - D. Van Raemdonck
- Department of Thoracic Surgery, University Hospitals Leuven, Leuven, Belgium
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Qingyu J, Xiaolong L, Ruohan Z, Licong M, Zhichao T, Qingwei C, Yuan W, Ying Z. Computed tomography helps pre-operative evaluation before laparoscopic resection of retroperitoneal bronchogenic cyst: A case report. J Minim Access Surg 2021; 17:95-97. [PMID: 32964892 PMCID: PMC7945650 DOI: 10.4103/jmas.jmas_72_20] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2020] [Revised: 03/21/2020] [Accepted: 03/26/2020] [Indexed: 01/01/2023] Open
Abstract
Bronchogenic cysts are congenital foregut dysplasia that occur mostly in the lungs and mediastinum. Here, we report a rare case of retroperitoneal bronchogenic cyst, the location, relationship to adjacent structures and blood supply of which were determined by computed tomography (CT) recombination technology and resected by laparoscope. The case was a 41-year-old female patient. The patient came to the hospital because of intermittent lumbar back discomfort for 1 month. CT scanning revealed a cystic mass of 3.9 cm × 3.2 cm × 3.0 cm behind the left peritoneum. The mass was close to the left adrenal gland, and a branch artery from the left renal artery was revealed to supply the mass. The cystic mass was excised by laparoscopy and confirmed as bronchogenic cyst on histopathology.
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Affiliation(s)
- Ji Qingyu
- Department of Radiology, The Second Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou, China
| | - Li Xiaolong
- Surgical Minimally Invasive Center, The Second Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou, China
| | - Zhu Ruohan
- Department of Ultrasound, The Second Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou, China
| | - Ma Licong
- Department of Pathology, The Second Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou, China
| | - Tang Zhichao
- Department of Radiology, The Second Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou, China
| | - Chen Qingwei
- Department of Radiology, The Second Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou, China
| | - Wang Yuan
- Department of Ultrasound, The Maternal and Child Health Center of Baotou City, Baotou, China
| | - Zhao Ying
- Department of Radiology, The Second Affiliated Hospital of Baotou Medical College, Inner Mongolia University of Science and Technology, Baotou, China
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Birkemeier KL. Imaging of solid congenital abdominal masses: a review of the literature and practical approach to image interpretation. Pediatr Radiol 2020; 50:1907-1920. [PMID: 33252758 DOI: 10.1007/s00247-020-04678-1] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2019] [Revised: 02/27/2020] [Accepted: 04/13/2020] [Indexed: 12/12/2022]
Abstract
Fetal abdominal tumors are rare, usually benign, and cause a great deal of anxiety for expectant parents and the physicians counseling them. In this paper the author reviews the most common fetal abdominal tumors in the liver (hemangioma, mesenchymal hamartoma, hepatoblastoma, metastases) and the kidney (congenital mesoblastic nephroma, Wilms tumor, malignant rhabdoid tumor, and clear cell sarcoma), and suprarenal mass lesions (adrenal neuroblastoma, adrenal hemorrhage, and subdiaphragmatic extralobar pulmonary sequestration). The author describes the imaging approach, imaging appearance and differentiating features of tumors, and differences between fetal and childhood appearances of tumors.
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Affiliation(s)
- Krista L Birkemeier
- Department of Radiology, Pediatric Section, Baylor Scott and White Health-Temple, McLane Children's Medical Center, Texas A&M Health Science Center, 2401 S. 31st St., MS-01-W256, Temple, TX, 76508, USA.
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Sharma D, Tsibizova VI. Current perspective and scope of fetal therapy: part 1. J Matern Fetal Neonatal Med 2020; 35:3783-3811. [PMID: 33135508 DOI: 10.1080/14767058.2020.1839880] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
Fetal therapy term has been described for any therapeutic intervention either invasive or noninvasive for the purpose of correcting or treating any fetal malformation or condition. Fetal therapy is a rapidly evolving specialty and has gained pace in last two decades and now fetal intervention is being tried in many malformations with rate of success varying with the type of different fetal conditions. The advances in imaging techniques have allowed fetal medicine persons to make earlier and accurate diagnosis of numerous fetal anomalies. Still many fetal anomalies are managed postnatally because the fetal outcomes have not changed significantly with the use of fetal therapy and this approach avoids unnecessary maternal risk secondary to inutero intervention. The short-term maternal risk associated with fetal surgery includes preterm labor, premature rupture of membranes, uterine wall bleeding, chorioamniotic separation, placental abruption, chorioamnionitis, and anesthesia risk. Whereas, maternal long-term complications include risk of infertility, uterine rupture, and need for cesarean section in future pregnancies. The decision for invasive fetal therapy should be taken after discussion with parents about the various aspects like postnatal fetal outcome without fetal intervention, possible outcome if the fetal intervention is done, available postnatal intervention for the fetal condition, and possible short-term and long-term maternal complications. The center where fetal intervention is done should have facility of multi-disciplinary team to manage both maternal and fetal complications. The major issues in the development of fetal surgery include selection of patient for intervention, crafting effective fetal surgical skills, requirement of regular fetal and uterine monitoring, effective tocolysis, and minimizing fetal and maternal fetal risks. This review will cover the surgical or invasive aspect of fetal therapy with available evidence and will highlight the progress made in the management of fetal malformations in last two decades.
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Affiliation(s)
- Deepak Sharma
- Department of Neonatology, National Institute of Medical Science, Jaipur, India
| | - Valentina I Tsibizova
- Almazov National Medical Research Centre, Health Ministry of Russian Federation, Saint Petersburg, Russia
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Koo BS, Lee SH, Lee SJ, Jung WH, Chung YH, Lee JH, Cho SH, Kim SH. A case of one-lung ventilation using a single-lumen tube placed under fiberoptic bronchoscopic guidance in a 4-year-old child: A case report. Medicine (Baltimore) 2020; 99:e21737. [PMID: 32846795 PMCID: PMC7447431 DOI: 10.1097/md.0000000000021737] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
RATIONALE One-lung ventilation (OLV) is essential for adequate visualization and exposure of the surgical site via a videoscopic approach. Although many instruments facilitating OLV are available, the choice is limited in pediatric patients. PATIENT CONCERNS A 4-year-old female (weight: 18.6 kg, height: 100 cm) was admitted via our pediatric outpatient clinic because of recurrent hemoptysis, 2 weeks in duration. She had no medical or surgical history. DIAGNOSIS Contrast-enhanced computed tomography (CT) revealed a 4.5-cm-diameter mass in the left, lower lung lobe. She was diagnosed with a congenital pulmonary airway malformation (CPAM). INTERVENTIONS She was scheduled for emergency lobectomy via video-assisted thoracoscopic surgery (VATS). To ensure successful VATS, OLV was essential. As our hospital lacked a small-diameter fiberoptic bronchoscope and a proper bronchial blocker, we decided to use single-lumen tube (SLT) with adult fiberoptic bronchoscope. OUTCOMES We performed successful bronchoscopic-guided OLV using a SLT. We aligned the tube to the right upper lobar bronchus and Murphy eye to prevent obstruction of the right upper lobe bronchus. At the end of surgery, the endotracheal tube lumen had been narrowed by blood clots, we decided to exchange the tracheal tube. The tube was immediately exchanged. After re-intubation, the pulse oximetry (SpO2) then gradually increased. LESSONS Appropriate preparation and careful management should be considered to perform OLV in pediatric patients without significant complications.
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Fievet L, Gossot D, de Lesquen H, Calabre C, Merrot T, Thomas P, Becmeur F, Grigoroiu M. Resection of Bronchogenic Cysts in Symptomatic Versus Asymptomatic Patients: An Outcome Analysis. Ann Thorac Surg 2020; 112:1553-1558. [PMID: 32599038 DOI: 10.1016/j.athoracsur.2020.05.031] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2019] [Revised: 04/19/2020] [Accepted: 05/01/2020] [Indexed: 12/16/2022]
Abstract
BACKGROUND The natural evolution of bronchogenic cysts (BCs) is unpredictable. Although most surgeons agree that symptomatic BCs should be resected, questions remain regarding the optimal management of asymptomatic mediastinal cysts. We present a case series of BCs to compare patients who underwent preventive operation with those who underwent surgical procedure after symptom onset. METHODS This 15-year multicenter retrospective study included 114 patients (32 children and 82 adults). Data on clinical history, pathology, mean hospital stay, intraoperative and postoperative complications, and associated intraoperative procedures were analyzed separately for symptomatic and asymptomatic patients. RESULTS A total of 53 asymptomatic patients (46.5%) were compared with 61 symptomatic patients (53.5%). There were significantly more adults in the symptomatic group than in the asymptomatic group (48 vs 34 patients, P < .05). A thoracoscopic approach was used in 88 patients (77%), with 7 conversions to thoracotomy (9%), all in symptomatic patients. There were significantly more additional procedures (20% vs 4%, P = .01) and more intraoperative complications (20% vs 4%, P = .01) in symptomatic patients, but postoperative complications between symptomatic and asymptomatic patients were similar. The postoperative length of stay was significantly longer in symptomatic patients (5.71 days vs 4 days, P < .001). Pathologic examination found significantly more inflammatory reactions in symptomatic patients. CONCLUSION Early surgical management of BCs may be recommended to prevent symptomatic complications, which are unpredictable and whose management is more complicated in advanced BCs. Surgery can be performed with a thoracoscopic approach, which is easier and safer when the cyst is small and uncomplicated.
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Affiliation(s)
- Lucile Fievet
- Department of Pediatric Surgery, Centre Hospitalier Régional Henri Duffaut, Provence-Alpes-Côte d'Azur, Avignon, France.
| | - Dominique Gossot
- Department of Thoracic Surgery, Institut Mutaliste Montsouris, Île de France, Paris, France
| | - Henri de Lesquen
- Department of Thoracic Surgery, North Hospital, Assistance Publique-Hôpitaux de Marseille, Provence-Alpes-Côte d'Azur, Marseille, France
| | - Charline Calabre
- Department of Pediatric Surgery, Centre Hospitalier Régional Universitaire Strasbourg, Alsace, France
| | - Thierry Merrot
- Department of Pediatric Surgery, La Timone Hospital, Assistance Publique-Hôpitaux de Marseille, Provence-Alpes-Côte d'Azur, Marseille, France
| | - Pascal Thomas
- Department of Thoracic Surgery, North Hospital, Assistance Publique-Hôpitaux de Marseille, Provence-Alpes-Côte d'Azur, Marseille, France
| | - François Becmeur
- Department of Pediatric Surgery, Centre Hospitalier Régional Universitaire Strasbourg, Alsace, France
| | - Madalina Grigoroiu
- Department of Thoracic Surgery, Institut Mutaliste Montsouris, Île de France, Paris, France
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Computed tomography features can distinguish type 4 congenital pulmonary airway malformation from other cystic congenital pulmonary airway malformations. Eur J Radiol 2020; 126:108964. [DOI: 10.1016/j.ejrad.2020.108964] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/09/2020] [Revised: 03/09/2020] [Accepted: 03/10/2020] [Indexed: 11/20/2022]
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Robson VK, Shieh HF, Wilson JM, Buchmiller TL. Non-operative management of extralobar pulmonary sequestration: a safe alternative to resection? Pediatr Surg Int 2020; 36:325-331. [PMID: 31707604 DOI: 10.1007/s00383-019-04590-2] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/13/2019] [Indexed: 12/17/2022]
Abstract
PURPOSE This retrospective cohort study compares the natural history of patients with extralobar sequestrations (ELS) who do not undergo intervention with those who undergo resection to assess the safety of non-operative management. METHODS 126 patients with pulmonary sequestrations or congenital pulmonary airway malformations born between 1999 and 2016 were identified. 49 patients had ELS on postnatal imaging, but two were excluded for associated congenital diaphragmatic hernia. Demographic and clinical data were retrospectively reviewed, with phone follow-up for non-operative patients with no records for > 1 year. Statistical analysis was by Fisher's exact test or Wilcoxon signed-rank test (two-tailed p < 0.05). RESULTS 40% (19/47) were managed non-operatively and 60% (28/47) underwent resection. Non-operative patients were less likely to have an intrathoracic ELS: 47% (9/19) vs. 75% (21/28), p = 0.07. No symptoms were attributable directly to the ELS. Non-operative patients had median follow-up 3.2 years, during which time 88% (15/17) of ELS decreased in size on serial imaging. For patients who underwent resection, there was 100% concordance between imaging and intraoperative findings. There was no evidence of inflammation, infection or malignancy on final pathology, though 57% (16/28) of resected lesions had foci of non-aerated cysts. CONCLUSIONS Although further longitudinal study is required, this study supports the safety of non-operative ELS management.
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Affiliation(s)
- Victoria K Robson
- Department of Medicine and Pediatrics, Boston Children's Hospital and Harvard Medical School, Boston, MA, 02115, USA
| | - Hester F Shieh
- Department of Surgery, Boston Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Fegan 3rd Floor, Boston, MA, 02115, USA
| | - Jay M Wilson
- Department of Surgery, Boston Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Fegan 3rd Floor, Boston, MA, 02115, USA
| | - Terry L Buchmiller
- Department of Surgery, Boston Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Fegan 3rd Floor, Boston, MA, 02115, USA.
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Den J, Nunez Lopez O, Garza Serna U, Tran S, Radhakrishnan RS, Bowen-Jallow KA. Laparoscopic excision of intra-abdominal extralobar bronchopulmonary sequestration. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2019. [DOI: 10.1016/j.epsc.2019.101327] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
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47
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Cherian SV, Kumar A, Ocazionez D, Estrada -Y- Martin RM, Restrepo CS. Developmental lung anomalies in adults: A pictorial review. Respir Med 2019; 155:86-96. [PMID: 31326738 DOI: 10.1016/j.rmed.2019.07.011] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/09/2019] [Revised: 06/03/2019] [Accepted: 07/05/2019] [Indexed: 11/16/2022]
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Suzuki T, Kamata S, Sato N. A case of massive hemothorax associated with an intrathoracic bronchogenic cyst. Gen Thorac Cardiovasc Surg 2019; 68:396-398. [PMID: 31134529 DOI: 10.1007/s11748-019-01145-z] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2018] [Accepted: 05/20/2019] [Indexed: 11/30/2022]
Abstract
A 34-year-old man presented with sudden back pain and dyspnea. Chest X-ray showed left-sided massive pleural effusion. Chest computed tomography revealed an intrathoracic mass sized 9 cm. Hemorrhagic effusion was achieved with thoracic drainage on admission. Diagnostic video-assisted surgery was indicated, and an unexpected cyst with bloody content was observed. The cyst was bluntly dissected from the pleura and removed from the diaphragm. The patient discharged uneventfully and there were no significant postoperative complications including bleeding or pneumothorax. Pathological observation of the cyst revealed pseudostratified ciliated epithelial cells, mucinous glands, and cartilage compatible with the diagnostic criteria for a bronchogenic cyst. Malignant transformation was not observed. Common clinical presentations of bronchogenic cysts include pain, dyspnea, and cough. Although rare, the risk of hemorrhage from bronchogenic cysts and subsequent development of hemothorax should not be underestimated.
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Affiliation(s)
- Takaya Suzuki
- Department of Respiratory Medicine, Aomori Prefectural Center Hospital, 2-1-1, Higashitsukurimichi, Aomori, 030-0913, Japan.
| | - Satoshi Kamata
- Department of Respiratory Medicine, Aomori Prefectural Center Hospital, 2-1-1, Higashitsukurimichi, Aomori, 030-0913, Japan
| | - Nobuyuki Sato
- Department of Respiratory Medicine, Aomori Prefectural Center Hospital, 2-1-1, Higashitsukurimichi, Aomori, 030-0913, Japan
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Cystic and pseudocystic pulmonary malformations in children: Clinico-pathological correlation. Ann Diagn Pathol 2019; 39:78-85. [PMID: 30798075 DOI: 10.1016/j.anndiagpath.2019.02.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2019] [Accepted: 02/08/2019] [Indexed: 11/21/2022]
Abstract
Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1 year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8 months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia.
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Annunziata F, Bush A, Borgia F, Raimondi F, Montella S, Poeta M, Borrelli M, Santamaria F. Congenital Lung Malformations: Unresolved Issues and Unanswered Questions. Front Pediatr 2019; 7:239. [PMID: 31249823 PMCID: PMC6584787 DOI: 10.3389/fped.2019.00239] [Citation(s) in RCA: 32] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/18/2019] [Accepted: 05/28/2019] [Indexed: 12/30/2022] Open
Abstract
Advances in prenatal and postnatal diagnosis, perioperative management, and postoperative care have dramatically increased the number of scientific reports on congenital thoracic malformations (CTM). Nearly all CTM are detected prior to birth, generally by antenatal ultrasound. After delivery, most infants do well and remain asymptomatic for a long time. However, complications may occur beyond infancy, including in adolescence and adulthood. Prenatal diagnosis is sometimes missed and detection may occur later, either by chance or because of unexplained recurrent or persistent respiratory symptoms or signs, with difficult implications for family counseling and substantial delay in surgical planning. Although landmark studies have been published, postnatal management of asymptomatic children is still controversial and needs a resolution. Our aim is to provide a focused overview on a number of unresolved issues arising from the lack of an evidence-based consensus on the management of patients with CTM. We summarized findings from current literature, with a particular emphasis on the vigorous controversies on the type and timing of diagnostic procedures, treatments and the still obscure relationship between CTM and malignancies, a matter of great concern for both families and physicians. We also present an algorithm for the assessment and follow-up of CTM detected either in the antenatal or postnatal period. A standardized approach across Europe, based on a multidisciplinary team, is urgently needed for achieving an evidence-based management protocol for CTM.
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Affiliation(s)
- Federica Annunziata
- Division of Paediatrics, Department of Translational Medical Sciences, Federico II University, Naples, Italy
| | - Andrew Bush
- Department of Paediatrics and Paediatric Respiratory Medicine, Imperial College and Royal Brompton Hospital, London, United Kingdom
| | - Francesco Borgia
- Divisions of Cardiology and Cardiothoracic Surgery, Department of Advanced Biomedical Sciences, Federico II University, Naples, Italy
| | - Francesco Raimondi
- Division of Paediatrics, Department of Translational Medical Sciences, Federico II University, Naples, Italy
| | - Silvia Montella
- Division of Paediatrics, Department of Translational Medical Sciences, Federico II University, Naples, Italy
| | - Marco Poeta
- Division of Paediatrics, Department of Translational Medical Sciences, Federico II University, Naples, Italy
| | - Melissa Borrelli
- Division of Paediatrics, Department of Translational Medical Sciences, Federico II University, Naples, Italy
| | - Francesca Santamaria
- Division of Paediatrics, Department of Translational Medical Sciences, Federico II University, Naples, Italy
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