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Polke M, Polke N, Piel S, Brunnemer E, Wälscher J, Buschulte K, Warth A, Heussel CP, Eichinger M, Frankenstein L, Eichhorn M, Miliauskas S, Herth FJF, Kreuter M. Pulmonary lymphangiomatosis: insights into an ultra-rare disease. Respir Res 2024; 25:416. [PMID: 39593123 PMCID: PMC11600747 DOI: 10.1186/s12931-024-03040-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2024] [Accepted: 11/12/2024] [Indexed: 11/28/2024] Open
Abstract
BACKGROUND Pulmonary lymphangiomatosis (PL) is an ultrarare disease characterized by diffuse infiltration of the lung, pleura and/or mediastinum by abnormal lymphatic proliferation. Consented diagnostic or treatment approaches are not established. We therefore aimed to collect data on diagnostics and treatments in a cohort of patients with PL from a tertiary center for rare lung diseases. METHODS Clinical, radiological and outcome data from PL patients were collected retrospectively. RESULTS 12 patients were diagnosed between 1996 and 2022 in our center. PL was diagnosed more commonly in female (58%), never smokers (75%) and younger patients (mean age 42 years). Main clinical symptoms comprised haem- and chyloptysis (58%) and dyspnea on exertion (83%). Pulmonary function was mostly restrictive (mean VC 59%) with impaired DLCO (mean 65%). Radiological assessment mainly showed mediastinal involvement (83%), and pleural effusion (67%), pleural thickening (67%) and bronchial wall thickening (67%) while interstitial changes were rare. Diagnosis was confirmed by surgical or transbronchial cryobiopsy. 8 patients were treated with sirolimus, 3 of these combined with a surgical intervention and in one case surgical intervention was necessary 9 months after initiation of sirolimus. Clinical and radiological improvement was demonstrated for all patients treated with sirolimus. 1 patient received a lung transplant due disease progression. Survival rates were 90% after a mean follow up of at least 3 months. CONCLUSION This case series illustrates the variability of the clinical presentation of PL. Among our patients, those treated with sirolimus showed significant clinical, functional and radiological improvement. However, further investigation is needed to understand the pathogenesis of lymphangiomatosis in order to establish therapeutic approaches.
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Affiliation(s)
- M Polke
- Center for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
- Translational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany.
- Member of ERN-LUNG, Heidelberg, Germany.
| | - N Polke
- Center for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
| | - S Piel
- Center for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
| | - E Brunnemer
- Center for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
| | - J Wälscher
- Center for Interstitial and Rare Lung Diseases, Pneumology Department, Ruhrlandklinik, University Hospital, University of Essen, Essen, Germany
| | - K Buschulte
- Center for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
- Translational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany
| | - A Warth
- Translational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany
- Institute of Pathology, University of Heidelberg, Heidelberg, Germany
| | - C P Heussel
- Translational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany
- Dagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
| | - M Eichinger
- Translational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany
- Dagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
| | - L Frankenstein
- Department of Cardiology, University of Heidelberg, Heidelberg, Germany
| | - M Eichhorn
- Department of Thoracic Surgery, University of Heidelberg, Heidelberg, Germany
| | - S Miliauskas
- Department of Pulmonology, Lithuanian University of Health Sciences, Kaunas, Lithuania
| | - F J F Herth
- Center for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany
- Translational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany
- Member of ERN-LUNG, Heidelberg, Germany
| | - M Kreuter
- Center for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center, Mainz, Germany
- Center for Pulmonary Medicine, Department of Pulmonary, Critical Care & Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany
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Yanagisawa A, Tamiya A, Takimoto T, Sumikawa H. Generalized lymphatic anomaly involving the pleura and bone in an older male: A case report. Respir Med Case Rep 2023; 46:101961. [PMID: 38187118 PMCID: PMC10770584 DOI: 10.1016/j.rmcr.2023.101961] [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: 09/11/2023] [Accepted: 12/05/2023] [Indexed: 01/09/2024] Open
Abstract
Generalized lymphatic anomaly (GLA) is a congenital malformation of the lymphatic vessels that is often diagnosed in early childhood. Owing to the rarity and heterogeneity of its clinical course, GLA is frequently misdiagnosed, especially in adults. A 67-year-old man was incidentally found to have bone and pleural lesions. Multiple bone lesions detected on magnetic resonance images were mistaken for malignancy, and pathological evaluation led to the diagnosis of GLA. GLA should be considered in the differential diagnosis of multiple bone lesions, and a proactive biopsy to confirm the diagnosis may help avoid unnecessary invasive procedures.
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Affiliation(s)
- Atsushi Yanagisawa
- Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Kitaku Nagasone-cho 1180, Sakai City, Osaka, 591-8555, Japan
| | - Akihiro Tamiya
- Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Kitaku Nagasone-cho 1180, Sakai City, Osaka, 591-8555, Japan
| | - Takayuki Takimoto
- Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Kitaku Nagasone-cho 1180, Sakai City, Osaka, 591-8555, Japan
| | - Hiromitsu Sumikawa
- Department of Radiology, National Hospital Organization Kinki-Chuo Chest Medical Center, Kitaku Nagasone-cho 1180, Sakai City, Osaka, 591-8555, Japan
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Alhasan AS, Daqqaq TS. Extensive abdominal lymphangiomatosis involving the small bowel mesentery: A case report. World J Clin Cases 2021; 9:9990-9996. [PMID: 34877341 PMCID: PMC8610896 DOI: 10.12998/wjcc.v9.i32.9990] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/26/2021] [Revised: 07/16/2021] [Accepted: 07/29/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Abdominal lymphangiomatosis is a rare benign condition accounting for less than 1% of all the cases of lymphangiomatosis. Management usually involves radical surgical excision; however, depending upon the extent of involvement, patient condition, and absence of complications, conservative management can be also considered.
CASE SUMMARY We present the case of a 32-year-old male who presented with short onset abdominal pain and melena. Physical examination findings were within normal limits, except for left lower abdominal tenderness. Upper gastrointestinal endoscopy was within normal limits. Abdominal and pelvic ultrasound and computed tomography (CT) scan revealed numerous, variably-sized cystic lesions within the abdominal cavity, exclusively and extensively affecting the small bowel mesentery with sparing of the retroperitoneum. The diagnosis was confirmed by CT and cytological examination. Radical surgical excision was technically impossible in this patient because of the extensive involvement of the mesentery; therefore, the patient was managed conservatively.
CONCLUSION Extensive and exclusive small bowel mesentery involvement in abdominal lymphangiomatosis is rare. Imaging modalities play an important role in establishing the diagnosis and conservative management can be considered when surgery is technically impossible.
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Affiliation(s)
- Ayman S Alhasan
- Department of Radiology and Medical Imaging, College of Medicine Taibah University, Madinah 42353, Saudi Arabia
| | - Tareef S Daqqaq
- Department of Radiology and Medical Imaging, College of Medicine Taibah University, Madinah 42353, Saudi Arabia
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Kourouni I, Abramovich CM, Tamarkin SW, Tomashefski JF, Sivak ED. A perplexing airspace: peace of mind now or later. Breathe (Sheff) 2021; 17:210017. [PMID: 34295413 PMCID: PMC8291947 DOI: 10.1183/20734735.0017-2021] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/21/2021] [Accepted: 03/05/2021] [Indexed: 11/10/2022] Open
Abstract
A 36-year-old, nonsmoking woman with a history of asthma presented for a second opinion about a “cavitating” right middle lobe (RML) lesion that was found incidentally 6 months prior, during preoperative evaluation for cholecystectomy. The lesion in question was pleural based, measuring 5.4×4×4.5 cm with thin and thick inner septations along with low-density right hilar and mediastinal adenopathy (figure 1a–c). A perplexing right middle lobe lesion in a young woman. Peace of mind now or later?https://bit.ly/3veB5wE
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Affiliation(s)
- Ismini Kourouni
- Division of Pulmonary, Critical Care and Sleep Medicine, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
| | - Caroline M Abramovich
- Dept of Pathology, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
| | - Stephen W Tamarkin
- Dept of Radiology, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
| | - Joseph F Tomashefski
- Dept of Pathology, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
| | - Edward D Sivak
- Division of Pulmonary, Critical Care and Sleep Medicine, Case Western Reserve University (MetroHealth), Cleveland, OH, USA
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Late diagnosis of generalized lymphangiomatosis in a woman presenting with respiratory distress. Radiol Case Rep 2020; 15:1189-1193. [PMID: 32550956 PMCID: PMC7292890 DOI: 10.1016/j.radcr.2020.05.021] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/20/2020] [Revised: 05/11/2020] [Accepted: 05/11/2020] [Indexed: 01/15/2023] Open
Abstract
Generalized lymphangiomatosis (GLA) is a rare lymphatic abnormality, mostly affects children and young individuals and can be a diagnostic challenge because of wide spectrum of clinical manifestations. A 26-year-old woman presented to the emergency department of our institution with respiratory distress and hypoxia. The patient reported similar episodes for the past 10 years without a definite diagnosis. The imaging study demonstrated findings suggestive of GLA with pulmonary, retroperitoneal and osseous involvements which was confirmed on pathological studies from a lung biopsy. A concise review of the clinical, imaging and pathological findings of GLA is provided in this study. A comprehensive history and physical examination, laboratory and pathological work up and imaging is required to make the diagnosis of GLA. The characteristic imaging findings play an essential role to rule out other possible diagnoses and raise the possibility of GLA.
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Gurskytė V, Zeleckienė I, Maskoliūnaitė V, Mickys U, Šileikienė V. Successful treatment of diffuse pulmonary lymphangiomatosis with sirolimus. Respir Med Case Rep 2020; 29:101014. [PMID: 32071853 PMCID: PMC7013175 DOI: 10.1016/j.rmcr.2020.101014] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2020] [Revised: 01/28/2020] [Accepted: 01/31/2020] [Indexed: 10/27/2022] Open
Abstract
Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease characterized by uncontrolled proliferation of anastomosing lymphatic channels in the lungs, pleura and mediastinum. Several palliative treatment options have been suggested for this condition, such as surgical interventions, radiotherapy and systemic medications. However, the existing treatment modalities yield inconsistent results, and their use is often limited by toxic side effects. The aim of this case report is to demonstrate the diagnostic challenges of a rare disease and improvement in the condition of a DPL patient treated with sirolimus. A 27-year-old man presented to the pulmonologist with exertional dyspnea, chronic cough and intermittent hemoptysis. Upon medical investigation, a chest computed tomography (CT) scan revealed soft tissue masses infiltrating the mediastinum and bilateral interlobular septal thickening. A surgical biopsy was performed, and pathological tissue analysis showed findings consistent with the diagnosis of DPL. Treatment with sirolimus was initiated, maintaining trough concentrations between 10 and 15 ng/ml. At 21 months of treatment, the patient reported reduced symptoms of cough and dyspnea. A CT scan showed decreased interstitial thickening and reduced infiltrations in the mediastinum. Moreover, pulmonary function tests revealed a significant increase in FEV1 and FVC. The authors believe this is the first article reporting pulmonary function improvement in an adult DPL patient treated with sirolimus. Therefore, sirolimus therapy should be considered for DPL patients as it may be effective in improving their condition and preventing disease progression.
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Affiliation(s)
- Viktorija Gurskytė
- Faculty of Medicine, Vilnius University, M. K. Čiurlionio Str. 21/27, LT-03101, Vilnius, Lithuania
| | - Ingrida Zeleckienė
- Centre of Radiology and Nuclear Medicine, Vilnius University Hospital Santaros Klinikos, Santariškių Str. 2, LT-08661, Vilnius, Lithuania
| | - Vygantė Maskoliūnaitė
- National Centre of Pathology, Affiliate of Vilnius University Hospital Santaros Klinikos, P. Baublio Str. 5, LT-08406, Vilnius, Lithuania
- Department of Pathology, Forensic Medicine and Pharmacology, Institute of Biomedical Sciences, Faculty of Medicine, Vilnius University, M. K. Čiurlionio Str. 21/27, LT-03101, Vilnius, Lithuania
| | - Ugnius Mickys
- National Centre of Pathology, Affiliate of Vilnius University Hospital Santaros Klinikos, P. Baublio Str. 5, LT-08406, Vilnius, Lithuania
- Department of Pathology, Forensic Medicine and Pharmacology, Institute of Biomedical Sciences, Faculty of Medicine, Vilnius University, M. K. Čiurlionio Str. 21/27, LT-03101, Vilnius, Lithuania
| | - Virginija Šileikienė
- Clinic of Chest Diseases, Immunology and Allergology, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Čiurlionio Str. 21/27, LT-03101, Vilnius, Lithuania
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A rare case of retroperitoneal and mesenteric lymphangiomatosis. Radiol Case Rep 2019; 15:11-14. [PMID: 31762861 PMCID: PMC6849486 DOI: 10.1016/j.radcr.2019.09.039] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2019] [Revised: 09/27/2019] [Accepted: 09/30/2019] [Indexed: 12/15/2022] Open
Abstract
Lymphagiomatosis are rare benign malformations of the lymphatic system. They are more commonly seen during childhood and are frequently asymptomatic and incidentally found in the adult patient. We report a case of a 31-year-old male who presented initially with melena. Computer tomography scan revealed multiple confluent, fluid-density lesions encasing the retroperitoneum and mesentery. A laparotomy and incisional biopsy of the mesenteric lesion was performed. Histologic examination demonstrated fibrofatty tissue with prominent, thick-walled endothelial-lined vessels. The histologic and computer tomography findings were consistent with a diagnosis of retroperitoneal and mesenteric lymphangiomatosis. The patient was subsequently discharged home well.
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8
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Nicolas G, Shebli A, Minari AM, Sleimen AEH, Jaoude FA, Terro JJ, Zeidan M, Khairallah M, Hashem M. Propranolol Alleviating the Challenging Clinical Course of Birth Onset Generalized Lymphangiomatosis. A Case Report. Surg Case Rep 2019. [DOI: 10.31487/j.scr.2019.05.04] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
Background: Cystic hygromas, interchangeably named Lymphangiomas, are rare, congenital, benign lesions due to an abnormal lymphatic system development that tend to occur mostly in the head, neck, and oral cavity.
Case Presentation: This is a case of 18 year old male patient Syrian, smoker, with a history of multiple cystic hygromas since birth along with a generous surgical history of multiple laparotomies for intrabadominal cystic excisions, splenectomy herniorrhaphies (bilateral inguinal hernias and 2 incisional hernias due to laparotomies) left orchieopexy then testiculectomy, scrotal skin graft , multiple abdominal radioguided cystic drainage and sclerotherapy; presented to our care center for fever, dry cough, pleuretic chest pain, and mild abdominal pain with watery diarrhea.
Conclusion: Cystic Hygroma is still being considered as a rare entity and can virtually occur in the whole body sites mostly at head and neck regions as explained above. Challenges in this disease is by finding cases where the presence of such cystic lesions is in extremely rare and unusual sites as well as different treatment modalities and prognostics depending on the site and size of the detected cystic formations.
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Abstract
RATIONALE Diffuse pulmonary lymphangiomatos (DPL) is a rare aggressive lymphatic disorder characterized by proliferation of anastomozing lymphatic vessels and extremely rare in adult patients. PATIENT CONCERNS We report a case of diffuse pulmonary lymphangiomatosis in 59-year-old man presented with cough and sputum for 2 months. DIAGNOSES Combining clinical manifestations with results of radiological, bronchoscopy, and surgical lung biopsy, it was consistent with the diagnosis of DPL. INTERVENTIONS After bronchoalveolar lavage and biopsy, symptom of cough got worse suddenly accompanied by excessive chyloptysis. The patient received an emergency surgical intervention and low fat medium chain fat treatment. OUTCOMES The patient was discharged with a much better health condition. LESSONS This case report is the oldest patient reported in the English literature, to the best of our knowledge. Serious complications of bronchoscopy should be considered, especially in DPL patients with severely enlarged mediastinum or with thin-walled translucent vesicles under endoscopy.
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Affiliation(s)
| | - Liyun Mi
- Department of Respiratory Medicine
| | | | | | - Yunqing Chen
- Department of Pathology, Affiliated Hospital of Qingdao University, Qingdao City, Shandong, China
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Yu X, Jia N, Ye S, Zhou M, Liu D. Primary chylopericardium: A case report and literature review. Exp Ther Med 2017; 15:419-425. [PMID: 29375697 DOI: 10.3892/etm.2017.5383] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2017] [Accepted: 06/29/2017] [Indexed: 12/26/2022] Open
Abstract
Primary chylopericardium (CP) is a rare clinical condition in which chylous fluid containing high concentrations of triglyceride accumulates in the pericardial cavity. The present study reports a case of CP that was successfully treated by reconstruction surgery of thoracic duct. To improve the ability to diagnosis and treat this rare disease, the current study also systematically extracted 104 reported cases of primary or idiopathic CP from the past 60 years (January 1950 to December of 2015), and reviewed the clinical manifestation, etiology, diagnosis and treatment of these cases. The age at diagnosis varied between 6 weeks and 79 years with a mean age of 27.95±16.50 years. Asymptomatic patients accounted for 39.42% of cases, while the most common initial symptoms were dyspnea (44.23%) and coughing (10.58%). Jugular venous distention and distant heart sound was identified in 23 (22.12%) and 34 cases (32.69%), respectively. Cardiomegaly in X-ray scans was detected in the majority of patients (93.27%). In addition, lymphoscintigraphy and lymphangiography were helpful in identifying the source of chyle. Regarding the etiology, idiopathic cases accounted for 35.56% of the included cases in the present study. The most important cause of primary CP was abnormal connection or accumulation of lymph fluid in the pericardium (37.50%). Conservative therapy included low-fat or medium-chain triglyceride diet, as well as total parenteral nutrition. The majority of patients (71.2%) required surgery for definitive therapy, and thoracic duct ligation was the most preferred surgical procedure, performed in 44.23% of cases. Follow-up was reported in 64 cases, and all patients survived during the mean follow-up period of 12 months. Therefore, it is suggested that surgical management is the most successful treatment method and is associated with a favorable prognosis.
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Affiliation(s)
- Xue Yu
- Department of Cardiology, Beijing Hospital, National Center of Gerontology, Beijing 100730, P.R. China
| | - Na Jia
- Department of Cardiology, Beijing Hospital, National Center of Gerontology, Beijing 100730, P.R. China
| | - Sanxia Ye
- Department of Internal Medicine, Beijing Shijitan Hospital, Beijing 100038, P.R. China
| | - Min Zhou
- Department of Cardiology, The First People's Hospital of Changzhou, Changzhou, Jiangsu 213003, P.R. China
| | - Deping Liu
- Department of Cardiology, Beijing Hospital, National Center of Gerontology, Beijing 100730, P.R. China
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Luisi F, Torre O, Harari S. Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis). Eur Respir Rev 2017; 25:170-7. [PMID: 27246594 PMCID: PMC9487238 DOI: 10.1183/16000617.0018-2016] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2016] [Accepted: 04/21/2016] [Indexed: 01/05/2023] Open
Abstract
Generalised lymphatic anomaly (GLA), also known as lymphangiomatosis, is a rare disease caused by congenital abnormalities of lymphatic development. It usually presents in childhood but can also be diagnosed in adults. GLA encompasses a wide spectrum of clinical manifestations ranging from single-organ involvement to generalised disease. Given the rarity of the disease, most of the information regarding it comes from case reports. To date, no clinical trials concerning treatment are available. This review focuses on thoracic GLA and summarises possible diagnostic and therapeutic approaches. Possible diagnostic and therapeutic approaches to generalised lymphatic anomaly (lymphangiomatosis)http://ow.ly/4n4pgU
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Affiliation(s)
- Francesca Luisi
- Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Multimedica IRCCS, Milan, Italy
| | - Olga Torre
- Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Multimedica IRCCS, Milan, Italy
| | - Sergio Harari
- Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Multimedica IRCCS, Milan, Italy
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Alsmady MM, Aladaileh MA, Al-Zaben K, Saleem MM, Alimoglu O. Chylopericardium presenting as cardiac tamponade secondary to mediastinal lymphangioma. Ann R Coll Surg Engl 2016; 98:e154-e156. [PMID: 27388545 DOI: 10.1308/rcsann.2016.0203] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
Mediastinal lymphangioma is a rare entity and chylopericardium is a rare form of pericardial effusion. We report a case of acute chylous cardiac tamponade due to a cervicomediastinal lymphangioma in a one-year-old boy. A chest x-ray revealed marked cardiac enlargement and echocardiography showed massive pericardial effusion. Emergency surgery was performed whereby a pericardial window was created, followed by excision of the lymphangioma.
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Wang Z, Li K, Yao W, Dong K, Xiao X, Zheng S. Successful treatment of kaposiform lymphangiomatosis with sirolimus. Pediatr Blood Cancer 2015; 62:1291-3. [PMID: 25598153 DOI: 10.1002/pbc.25422] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/25/2014] [Accepted: 12/11/2014] [Indexed: 11/12/2022]
Abstract
Generalized lymphatic anomaly (GLA) is a rare and often fatal congenital lymphatic disorder that also commonly affects bone. Kaposiform lymphangiomatosis (KLA) is a novel subtype of GLA with poor prognosis and no proper treatment guidelines. A 9-year-old male with recurrent pleural effusion was clinically diagnosed as KLA. Following sirolimus therapy at a dose of 0.8 mg/m(2) twice daily, pleural effusion was significantly decreased and the general status of the patient markedly improved. The clinical course indicates that sirolimus may present an effective therapeutic option in KLA. Moreover, KLA should be considered in differential diagnosis for cases of GLA with coagulopathy.
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Affiliation(s)
- Zuopeng Wang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Kai Li
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Wei Yao
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Kuiran Dong
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Xianmin Xiao
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Shan Zheng
- Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, People's Republic of China
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Simple lymphangioma to generalized lymphatic anomaly: role of imaging in disclosure of a rare and morbid disease. Case Rep Radiol 2015; 2015:603859. [PMID: 25954563 PMCID: PMC4410542 DOI: 10.1155/2015/603859] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2015] [Accepted: 03/30/2015] [Indexed: 11/20/2022] Open
Abstract
Generalized lymphatic anomaly is a rare multisystem congenital disorder in which multiple organs are involved. Imaging features often overlap with other complex lymphatic anomalies and diagnosis is difficult. Treatment options are limited, not remedial and prognosis is poor. We report a 12-year-old male who presented with axillary and chest wall lymphangioma but was subsequently diagnosed as having diffuse lymphangiomatosis affecting lungs, liver, spleen, and bones on computerized tomography scan. We suggest complete radiological evaluation of susceptible adolescent children with lymphangioma to avoid diagnostic delay in this morbid condition. We also discuss radiological features of other similar complex lymphatic anomalies and crucial role of imaging in diagnosis.
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15
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Lymphangiomatose diffuse : une cause rare d’anémie. Arch Pediatr 2015; 22:380-2. [DOI: 10.1016/j.arcped.2015.01.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/18/2013] [Revised: 11/04/2013] [Accepted: 01/06/2015] [Indexed: 11/16/2022]
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Lin RY, Zou H, Chen TZ, Wu W, Wang JH, Chen XL, Han QX. Abdominal lymphangiomatosis in a 38-year-old female: Case report and literature review. World J Gastroenterol 2014; 20:8320-8324. [PMID: 25009412 PMCID: PMC4081712 DOI: 10.3748/wjg.v20.i25.8320] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/20/2013] [Revised: 02/09/2014] [Accepted: 04/09/2014] [Indexed: 02/06/2023] Open
Abstract
Lymphangioma is an uncommon benign tumor that develops in the lymphatic system. Abdominal lymphangiomatosis is extremely rare in adult patients, and the clinical symptoms of this condition are complicated and atypical. We report a case of abdominal lymphangiomatosis in a 38-year-old female who presented with intestinal bleeding and protein-losing enteropathy, as well as lesions in the lung and bones. A computed tomography scan revealed multiple small cystic lesions without enhancement. Histological examination revealed microscopic cysts were submucosal, with walls composed of thin fibrous tissue, and D2-40 stained highlight the lining of the lymphatic channels by immunohistochemical method. We make a comparison with the cases reported before, and also discuss the diagnose of diffuse pulmonary lymphangiomatosis and Gorham’s disease.
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Abstract
Chylothorax, the accumulation of chyle in the pleural space, is a relatively rare cause of pleural effusion in children. It can cause significant respiratory morbidity, as well as lead to malnutrition and immunodeficiency. Thus, a chylothorax requires timely diagnosis and treatment. This review will first discuss the anatomy and physiology of the lymphatic system and discuss various causes that can lead to development of a chylothorax in infants and children. Then, methods of diagnosis and treatment will be reviewed. Finally, complications of chylothorax will be reviewed.
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Affiliation(s)
- James D Tutor
- Program in Pediatric Pulmonary Medicine, University of Tennessee Health Science Center; Le Bonheur Children's Hospital; and St. Jude Children's Research Hospital, Memphis, Tennessee
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18
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Maeda S, Fujino Y, Tamamoto C, Suzuki S, Fujita A, Takahashi M, Ohno K, Uchida K, Tsujimoto H. Lymphangiomatosis of the systemic skin in an old dog. J Vet Med Sci 2012; 75:187-90. [PMID: 22986273 DOI: 10.1292/jvms.12-0321] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
A 13-year-old, neutered male miniature dachshund was presented with a one-month history of bilateral symmetrical swelling in the pinnae and carpal, cubital and tarsal joints, and swelling in the tail. The lesions were histopathologically characterized by multiple dilated lymphatic vessels lined by a single attenuated layer of endothelial cells. The subcutis was predominantly involved. A number of spindle-shaped cells lining the irregular vessels were observed. Morphological atypia was not evident in these cells. Immunohistochemical analyses revealed that the proliferating endothelial cells were positive for factor VIII-related antigen and CD31. Based on the clinical presentation and histopathological features, the dog was diagnosed with lymphangiomatosis. Treatment with anti-inflammatory prednisolone improved the symptoms.
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Affiliation(s)
- Shingo Maeda
- Department of Veterinary Internal Medicine, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Tokyo, Japan
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Walker CM, Takasugi JE, Chung JH, Reddy GP, Done SL, Pipavath SN, Schmidt RA, Godwin JD. Tumorlike Conditions of the Pleura. Radiographics 2012; 32:971-85. [DOI: 10.1148/rg.324115184] [Citation(s) in RCA: 41] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
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20
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Lesser DJ, Young LR, Hagood JS. Rare Childhood Lung Disorders. KENDIG & CHERNICKÂS DISORDERS OF THE RESPIRATORY TRACT IN CHILDREN 2012:877-885. [DOI: 10.1016/b978-1-4377-1984-0.00060-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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21
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Caballero Y, Pérez D, Cano JR. Diffuse pulmonary lymphangiomatosis with mediastinal affectation. Arch Bronconeumol 2011; 47:474-5. [PMID: 21821337 DOI: 10.1016/j.arbres.2011.06.001] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2011] [Revised: 05/24/2011] [Accepted: 06/04/2011] [Indexed: 11/16/2022]
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22
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Shah V, Shah S, Barnacle A, Sebire NJ, Brock P, Harper JI, McHugh K. Mediastinal involvement in lymphangiomatosis: a previously unreported MRI sign. Pediatr Radiol 2011; 41:985-92. [PMID: 21660638 DOI: 10.1007/s00247-011-2124-1] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/16/2010] [Revised: 01/23/2011] [Accepted: 01/29/2011] [Indexed: 11/28/2022]
Abstract
BACKGROUND Multifocal lymphangiomatosis is a rare systemic disorder affecting children. Due to its rarity and wide spectrum of clinical, histological and imaging features, establishing the diagnosis of multifocal lymphangiomatosis can be challenging. OBJECTIVES The purpose of this study was to describe a new imaging sign in this disorder: paraspinal soft tissue and signal abnormality at MRI. MATERIALS AND METHODS We retrospectively reviewed the imaging, clinical and histopathological findings in a cohort of eight children with thoracic involvement from this condition. RESULTS Evidence of paraspinal chest disease was identified at MRI and CT in all eight of these children. The changes comprise heterogeneous intermediate-to-high signal parallel to the thoracic vertebrae on T2-weighted sequences at MRI, with abnormal paraspinal soft tissue at CT and plain radiography. CONCLUSION Multifocal lymphangiomatosis is a rare disorder with a broad range of clinicopathological and imaging features. MRI allows complete evaluation of disease extent without the use of ionising radiation and has allowed us to describe a previously unreported imaging sign in this disorder, namely, heterogeneous hyperintense signal in abnormal paraspinal tissue on T2-weighted images.
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Affiliation(s)
- Vikas Shah
- Department of Radiology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK.
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23
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Thoracic lymphangiomatosis with massive chylothorax after a tumor biopsy and with disseminated intravenous coagulation--lymphoscintigraphy, an alternative minimally invasive imaging technique: report of a case. Surg Today 2011; 41:978-82. [PMID: 21748615 DOI: 10.1007/s00595-010-4383-0] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2010] [Accepted: 07/16/2010] [Indexed: 10/18/2022]
Abstract
Thoracic lymphangiomatosis in childhood is a rare disorder that frequently has poor response to medical therapy as well as a poor prognosis. This disease is often misdiagnosed, and a definitive diagnosis is usually delayed because of the rarity and slow course of the disease. No previous reports have so far described the usefulness of lymphoscintigraphy in the diagnosis of lymphangiomatosis, although some authors have reported the efficacy of lymphoscintigraphy for evaluating chylothorax. A 6-year-old boy presented with a diffuse mediastinal mass and received an open mediastinal biopsy for a definitive diagnosis, which led to the occurrence of massive chylothorax postoperatively. A diagnosis of lymphangiomatosis was finally made based on the lymphoscintigraphic findings demonstrating an obstruction of the thoracic duct. This report describes a rare case of thoracic lymphangiomatosis diagnosed at autopsy, and suggests that the minimally invasive technique of lymphoscintigraphy should be employed to obtain a definitive diagnosis at an early stage.
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24
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El-Essawy MT, Hassan HH. Cystic hygroma of the neck with multiple bone lymphangiomas. THE EGYPTIAN JOURNAL OF RADIOLOGY AND NUCLEAR MEDICINE 2011. [DOI: 10.1016/j.ejrnm.2011.05.009] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022] Open
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Jang EK, Kim CR, Choi CM, Oh YM, Shim TS, Lee SD, Kim WS, Kim DS, Song JW. A Case of Pulmonary Lymphangiomatosis. Tuberc Respir Dis (Seoul) 2011. [DOI: 10.4046/trd.2011.70.5.428] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
Affiliation(s)
- Eun Kyung Jang
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Chang Rae Kim
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Chang Min Choi
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Yeon Mok Oh
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Tae Sun Shim
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Sang Do Lee
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Woo Sung Kim
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Dong Soon Kim
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Jin Woo Song
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Abstract
Lymphangiomatosis is a rare condition characterized by multiple abnormalities of the lymphatic system. Diagnosis is often difficult, as chronic, intermittent, or acute pain; edema; and other symptoms may affect the respiratory, gastrointestinal, renal, hepatic, skeletal, and other organ systems. We report the case of a patient who first presented with lymphedema in childhood and was treated intermittently for related symptoms before diagnosis was achieved 36 years later. Plain film radiography, bone scanning, computed tomography, magnetic resonance imaging, and lymphoscintigraphy were used to arrive at a diagnosis. Information derived from all scan types was combined to derive a diagnosis of lymphangiomatosis. Lymphoscintigraphy provided direct evidence of the abnormal lymphatic flows associated with lymphangiomatosis. Lymphangiomatosis presents a diagnostic challenge; information from several scan types, including lymphoscintigraphy, is useful in deriving this diagnosis.
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27
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Harnisch E, Sukhai R, Oudesluys-Murphy AM. Serious complications of pulmonary biopsy in a boy with chylopericardium and suspected pulmonary lymphangiomatosis. BMJ Case Rep 2010; 2010:2010/may06_1/bcr0820092206. [PMID: 22736725 DOI: 10.1136/bcr.08.2009.2206] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Lymphangiomatosis is a rare disorder. It is commonly localised in the skeletal system and called Gorham's disease, and in thoracic or abdominal organs. Involvement of the pericardium is rare and has been described in less than 20 patients worldwide. The case of a 14-year-old boy presenting with asymptomatic chylopericardium and interstitial lung disease is presented. After lung biopsy, performed to confirm the diagnosis of pulmonary lymphangiomatosis, he developed chylothorax and massive loss of chyle via chest drains. Thoracic duct ligation did not result in clinical improvement. Treatment with interferon α2b was given and because of clinical deterioration radiotherapy was added to the treatment. This resulted in a rapid decrease of chyle production in the patient.
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28
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Abstract
OBJECTIVE This review will focus on radiographic description of lymphangiomas, lymphangiohemangiomas, pulmonary lymphangiomatosis, lymphangiectasis, lymphangioleiomyomatosis, lymphatic dysplasia, and traumatic lymphatic injury. CONCLUSION Diseases of the thoracic lymphatic system have a wide variety of unique radiographic manifestations, all of which can be explained by the underlying pathophysiology and relationship to the normal distribution of lymphatics in the chest.
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29
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Abstract
Chylothorax is the accumulation of chyle in the pleural space, as a result of damage to the thoracic duct. Chyle is milky fluid enriched with fat secreted from the intestinal cells and lymphatic fluid. Chylothorax in children, is most commonly seen as a complication of cardiothoracic surgery but may occur in newborns or conditions associated with abnormal lymphatics. The diagnosis is based on biochemical analysis of the pleural fluid, which contains chylomicrons, high levels of triglycerides and lymphocytes. Investigations to outline the lymphatic channels can prove helpful in some cases. Initial treatment consists of drainage, dietary modifications, total parenteral nutrition and time for the thoracic duct to heal. Somatostatin and its analogue octreotide may be useful in some cases. Surgery should be considered for patients who fail these initial steps, or in whom complications such as electrolyte and fluid imbalance, malnutrition or immunodeficiency persist. Surgical intervention may be attempted thoracoscopically with repair or ligation of the thoracic duct.
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Affiliation(s)
- Manuel Soto-Martinez
- Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Parkville, Melbourne, Victoria 3052, Australia.
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30
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Steinacher I, Lamprecht B, Lobendanz M, Zoller H, Dartevelle P, Fadel E, Studnicka M. Successful surgical treatment of thoracic multiorgan lymphangiomatosis. Wien Klin Wochenschr 2009; 121:644-7. [DOI: 10.1007/s00508-009-1249-9] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2008] [Accepted: 08/24/2009] [Indexed: 12/01/2022]
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Kinnier CV, Eu JPC, Davis RD, Howell DN, Sheets J, Palmer SM. Successful bilateral lung transplantation for lymphangiomatosis. Am J Transplant 2008; 8:1946-50. [PMID: 18671675 PMCID: PMC3732029 DOI: 10.1111/j.1600-6143.2008.02340.x] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Lymphangiomatosis is a rare disease of lymphatic proliferation for which no adequate treatment is known. We report the first successful case of bilateral lung transplantation for the treatment of end-stage pulmonary lymphangiomatosis. A successful outcome was achieved with continued survival beyond 4 years posttransplant and stable lung function. The primary obstacles to significant gains in pulmonary function were thoracic, skeletal and abdominal lymphangiomatosis, which led to pulmonary restriction. Our report demonstrates that pulmonary lymphangiomatosis should be included among those diseases for which lung transplantation is considered potentially beneficial treatment but also emphasizes the importance of screening patients carefully for chest wall and abdominal lymphangiomas that may impede recovery.
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Affiliation(s)
- CV Kinnier
- Division of Pulmonary Medicine, Duke University Medical Center, Durham, NC
| | - JPC Eu
- Division of Pulmonary Medicine, Duke University Medical Center, Durham, NC
| | - RD Davis
- Cardiothoracic Surgery, Duke University Medical Center, Durham, NC
| | - DN Howell
- Pathology, Duke University Medical Center, Durham, NC
| | - J Sheets
- Division of Pulmonary Medicine, Duke University Medical Center, Durham, NC
| | - SM Palmer
- Division of Pulmonary Medicine, Duke University Medical Center, Durham, NC
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34
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Stoss S, Kalbermatten DF, Robertson A, Bruder E, Rasmus M, Gambazzi F, Pierer G. Large cystic tumour at the chest wall mimicking an echinococcosis: a case report. J Plast Reconstr Aesthet Surg 2007; 61:e13-6. [PMID: 18060851 DOI: 10.1016/j.bjps.2007.10.040] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2006] [Revised: 04/23/2007] [Accepted: 10/18/2007] [Indexed: 10/22/2022]
Abstract
The authors report an atypical late onset of a big axillary lymphatic malformation in a 41-year-old male. Considering the patient's history and clinical findings at first presentation, the swelling was highly suspicious for malignancy or cystic echinococcosis. A consequent CT showed non infiltrative growth with inhomogeneous density but remained non conclusive regarding diagnosis. Subsequently incision biopsy revealed lymphatic tissue and raised suspicion for lymphatic malformation. The tumour was excised completely and showed no recurrence in a 1-year follow up. Late onset lymphatic malformations can mimic malignant tumours or other rare conditions such as echinococcosis which has to be taken into consideration as differential diagnosis especially in known areas of hydatid diseases.
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Affiliation(s)
- S Stoss
- Department of Plastic and Reconstructive Surgery, University of Basel, Switzerland.
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35
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Ono K, Kikuchi A, Miyashita S, Iwasawa Y, Miyachi K, Sunagawa S, Takagi K, Nakamura T, Sago H. Fetus with prenatally diagnosed posterior mediastinal lymphangioma: characteristic ultrasound and magnetic resonance imaging findings. Congenit Anom (Kyoto) 2007; 47:158-60. [PMID: 17988258 DOI: 10.1111/j.1741-4520.2007.00164.x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
Prenatal diagnosis of lymphangiomas located in the posterior mediastinum has been reported to be extremely rare. We present a fetus with a prenatally diagnosed posterior mediastinal lymphangioma found at 28 weeks' gestation. Fetal ultrasound and magnetic resonance imaging (MRI) revealed a 46 x 26 x 30 mm multicystic mass extending above the diaphragm, located in the posterior mediastinum. The mass was symmetrically shaped and surrounded the thoracic aorta. No remarkable change was noted in the size, shape and texture of the cyst by serial ultrasound and the fetus did not develop cardiac failure or hydrops in the antenatal period. Postnatal chest X-ray and MRI confirmed the prenatal findings. The infant was asymptomatic, so he was placed on close follow-up without any medical or surgical treatment.
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Affiliation(s)
- Kyoko Ono
- Department of Obstetrics, Center for Perinatal Medicine, Nagano Children's Hospital, Nagano, Japan
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36
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Zisis C, Spiliotopoulos K, Patronis M, Filippakis G, Stratakos G, Tzelepis G, Bellenis I. Diffuse lymphangiomatosis: are there any clinical or therapeutic standards? J Thorac Cardiovasc Surg 2007; 133:1664-5. [PMID: 17532983 DOI: 10.1016/j.jtcvs.2007.02.005] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/24/2006] [Accepted: 02/07/2007] [Indexed: 11/22/2022]
Affiliation(s)
- Charalambos Zisis
- Department of Thoracic and Vascular Surgery, Evangelismos Hospital, Athens, Greece.
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Abstract
Generalized lymphangiomatosis is a rare disease that is characterized by widespread bony and soft tissue involvement of lymphangioma. Radiological evaluation is crucial because the site and extent of the lymphangioma are important prognostic factors. We reported here on three cases of generalized lymphangiomatosis and all three cases showed similar radiologic findings, but a different clinical course. The CT, US and MR images showed sharply defined, non-enhanced cystic lesions involving the mediastinum, bones, spleen, lung and lower neck. The whole body MR imaging with the short tau inversion recovery (STIR) sequence showed good capability for evaluating the extent of disease.
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Affiliation(s)
- Dong Hyun Yang
- Department of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul 138-736, Korea
| | - Hyun Woo Goo
- Department of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul 138-736, Korea
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38
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Chen YL, Lee CC, Yeh ML, Lee JS, Sung TC. Generalized Lymphangiomatosis Presenting as Cardiomegaly. J Formos Med Assoc 2007; 106:S10-4. [PMID: 17493902 DOI: 10.1016/s0929-6646(09)60359-4] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Lymphangioma refers to the local proliferation of well-differentiated lymphatic tissue. Generalized lymphangiomatosis is rare. We report a previously healthy 8-month-old infant who suffered from tachypnea with mild fever for 2 weeks. Imaging studies revealed a well-defined, large mass occupying the mediastinum, which presented as cardiomegaly. The disseminated mass extended to the thymus, lung, and spleen. Lymphangiomatosis was diagnosed by biopsy. Drainage of the pericardial fluid and total parenteral nutrition did not result in improvement of chylopericardium. Secondary hypogammaglobulinemia and septic shock developed sequentially. Surgical removal of the mediastinal mass and spleen were performed. Daily subcutaneous injection of interferon (IFN) alpha-2b was then given for 3 months. No recurrence was noted during 2 years of follow-up. IFN alpha-2b may be considered as an alternative for the treatment of generalized lymphangiomatosis.
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Affiliation(s)
- Yi-Ling Chen
- Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan
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39
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Timke C, Krause MF, Oppermann HC, Leuschner I, Claviez A. Interferon alpha 2b treatment in an eleven-year-old boy with disseminated lymphangiomatosis. Pediatr Blood Cancer 2007; 48:108-11. [PMID: 16007599 DOI: 10.1002/pbc.20461] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
Disseminated lymphangiomatosis is a rare disease mostly observed in children and young adults. If no surgical removal can be achieved, the prognosis is poor, especially for patients with thoracic localization and pleural effusions. Next to pleural drainage, therapeutic options include radiotherapy, local, and systemic pharmacotherapy. An 11-year-old boy presented with disseminated lymphangiomatosis involving thorax with massive pleural effusions, retroperitoneum, and bones. In immunohistochemical analysis, the tissue biopsy stained positive for vascular endothelial growth factor-receptor 3 (VEGFR-3). The patient has been treated with interferon alpha 2b for 2 years, and achieved a good clinical and radiological response.
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Affiliation(s)
- Christian Timke
- Departments of Pediatrics, University of Kiel, Schwanenweg 20, Kiel, Germany.
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40
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Graziuso S, Ormitti F, Cerasti D, Menozzi R, Piazza P, Cerasoli G, Crisi G, Zompatori M. Percutaneous sclerotherapy for lymphangioma and soft-tissue venous malformation located in the maxillofacial region in children and young adults. Neuroradiol J 2006; 19:385-93. [PMID: 24351228 DOI: 10.1177/197140090601900320] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/03/2006] [Accepted: 05/11/2006] [Indexed: 11/16/2022] Open
Abstract
To demonstrate that percutaneous sclerotherapy for lymphangioma using a new fibrosing agent (OK-432) and for soft-tissue venous malformation with alcoholization can improve management of these congenital vascular abnormalities. Between February 2003 and November 2005 five patients with lymphangioma, ranging in age from 23 months to 18 years (mean age = 9 years) and four patients with soft-tissue venous malformation, raging in age from 25 months to 18 years (mean age = 11 years) underwent percutaneous sclerotherapy. Ultrasound Computed tomography and/or Magnetic Resonance imaging were performed beforehand to evaluate the anatomic boundaries of the malformation. General anesthesia is mandatory for children under three years. Direct puncture of the mass with a 23-gauge venous infusion set (butterfly) was performed by means of palpation alone or with ultrasonographic guidance using OK-432 PICIBANIL (0.1-0.2 mg dilute in 10 ml normal saline) for lymphangioma and alcohol in venous malformation. The volume of sclerosing solution varied from 0.2 to 15 ml per injection course. Processing time was less than 20 minutes. Swelling of lesion, pain, local inflammatory reactions and mild fever (37.5°-39°) in lymphangioma, were constant findings after sclerotherapy. Satisfactory results (when the regression was estimated to be more than 50% of the initial volume; negative in inspection, but positive in palpation and imaging study) were obtained in four patients with head and neck lymphangioma. One patient was completely cured with an excellent (when there was a complete regression of malformations; negative in inspection, palpation and imaging study) result. All patients with soft-tissue venous malformation were satisfied with the results. In conclusion, in consideration of its low cost, rare complications and good results, we strongly recommend percutaneous sclerotherapy in the treatment of head and neck lymphangioma and soft-tissue venous malformation in children.
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Affiliation(s)
- S Graziuso
- Dipartimento di Scienze Cliniche, Sezione Diagnostica per Immagini, Università degli Studi di Parma. -
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41
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Tamay Z, Saribeyoglu E, Ones U, Anak S, Güler N, Bilgic B, Yilmazbayhan D, Gun F. Diffuse thoracic lymphangiomatosis with disseminated intravascular coagulation in a child. J Pediatr Hematol Oncol 2005; 27:685-7. [PMID: 16344679 DOI: 10.1097/01.mph.0000193476.14493.06] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
An 8.5-year-old girl who presented with chronic cough and hemoptysis underwent a CT scan of the chest showing diffuse mediastinal and parenchymal infiltration and pleural effusion, and laboratory findings showed disseminated intravascular coagulation. Disseminated lymphangiomatosis was diagnosed after an open-lung biopsy. She was treated by systemic steroids, interferon, tamoxifen, chemotherapy, and radiation but died of respiratory failure and disseminated intravascular coagulation 2 years after the diagnosis. This patient represents a rare presentation of diffuse pulmonary lymphangiomatosis together with disseminated intravascular coagulation, involving both the mediastinum and pulmonary parenchyma, in a child.
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Affiliation(s)
- Zeynep Tamay
- Division of Pediatric Allergy and Chest Diseases, Department of Pediatrics, Istanbul University, Istanbul Medical School, Turkey.
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42
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Abstract
Tumors of the mediastinum represent a wide diversity of disease states. The location and composition of a mass is critical to narrowing the differential diagnosis. The most common causes of an anterior mediastinal mass include the following: thymoma; teratoma; thyroid disease; and lymphoma. Masses of the middle mediastinum are typically congenital cysts, including foregut and pericardial cysts, while those that arise in the posterior mediastinum are often neurogenic tumors. The clinical sequelae of mediastinal masses can range from being asymptomatic to producing symptoms of cough, chest pain, and dyspnea. This article will review the anatomy of the mediastinum as well as the different clinical, radiographic, and prognostic features, and therapeutic options of the most commonly encountered masses.
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Affiliation(s)
- Beau V Duwe
- Department of Internal Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA
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Schultz K, Rosenberg AE, Ebb DH, Mankin HJ. Lower-extremity lymphangiomatosis. A case report with a seventeen-year follow-up. J Bone Joint Surg Am 2005; 87:162-7. [PMID: 15634828 DOI: 10.2106/jbjs.d.01892] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Affiliation(s)
- Karl Schultz
- Department of Orthopaedic Surgery, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA
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45
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Affiliation(s)
- Edith M Marom
- Division of Diagnostic Imaging, Box 57, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX 77030, USA.
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Papagiannopoulos K, Van Raemdonck DEM, De Boeck K, Lerut T. Pediatric thoracic lymphangiomatosis: is chest wall resection too radical? Ann Thorac Surg 2004; 77:695-7. [PMID: 14759463 DOI: 10.1016/s0003-4975(03)01146-9] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 05/12/2003] [Indexed: 11/26/2022]
Abstract
Lymphangiomas account for 5% to 6% of all pediatric neoplasms. Complex pathologic processes often present a diagnostic and treatment challenge. Radical and major surgery might have a role in complex cases when medical treatment fails.
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Abstract
An 8-year-old boy who presented with a mediastinal mass, pulmonary infiltrates, and disseminated intravascular coagulation was diagnosed with lymphangiomatosis. Despite medical management, he developed multiple organ failure and died. The authors discuss the diagnostic findings, medical management, and pathology and review 52 additional cases of thoracic lymphangiomatosis from the literature. Patients presented with chylothorax (49%), a mass (47%), pulmonary infiltrates (45%), bone lesions (39%), splenic lesions (19%), cervical involvement (15%), disseminated intravascular coagulation (9%), and skin involvement (7%). Children (<16 years) had a worse prognosis than older patients (39% vs. 0% mortality). All patients who died had either parenchymal lung involvement or pleural effusion. Thoracic lymphangiomatosis should be included in the differential diagnosis of a mediastinal mass with pulmonary findings.
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Affiliation(s)
- Ofelia A Alvarez
- Department of Pediatrics, University of Miami, Miami, Florida 33101,USA.
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Giguère CM, Bauman NM, Smith RJH. New treatment options for lymphangioma in infants and children. Ann Otol Rhinol Laryngol 2002; 111:1066-75. [PMID: 12498366 DOI: 10.1177/000348940211101202] [Citation(s) in RCA: 89] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
Lymphangiomas are congenital malformations of the lymphatic system. These lesions occur most often in the head and neck area, and their treatment continues to be a challenge. Fortunately, a number of advances have occurred in the diagnosis and management of lymphatic malformations in the past decade. The purpose of this article is to clarify the embryology, pathogenesis, histopathology, and classification of these lesions, as well as to describe their various forms of clinical presentation. We provide a complete review of the diagnostic measures available and thoroughly discuss new therapeutic interventions proposed to treat lymphangiomas.
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Affiliation(s)
- Chantal M Giguère
- Department of Otolaryngology-Head and Neck Surgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242, USA
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50
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Islam K. Thoracic lymphangioma. Indian J Pediatr 2001; 68:359-60. [PMID: 11370448 DOI: 10.1007/bf02721850] [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] [Indexed: 10/22/2022]
Abstract
Thoracic lymphangioma is a benign congenital condition that may involve any organ within the chest. Here, a case of intra thoracic isolated extramediastinal lymphangioma is reported. The child had respiratory distress from neonatal period. Surgery completely cured the patient. However, it is only the 3rd reported case in English literature.
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Affiliation(s)
- K Islam
- Department of Pediatric Surgery, Sylhet MAG Osmani Medical College, Sylhet 3100, Bangladesh.
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