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Ostertag-Hill CA, Fevurly RD, Kulungowski AM, Christison-Lagay ER, McGuire AM, Rialon KL, Duggan EM, Murillo R, Zurakowski D, Staffa SJ, Alomari AI, Kozakewich HPW, Al-Ibraheemi A, Fishman SJ, Dickie BH. The Natural History of Congenital Hepatic Hemangiomas. J Pediatr 2025; 281:114523. [PMID: 40023218 DOI: 10.1016/j.jpeds.2025.114523] [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] [Received: 10/25/2024] [Revised: 01/20/2025] [Accepted: 02/22/2025] [Indexed: 03/04/2025]
Abstract
OBJECTIVE To characterize the clinical presentation, radiologic and histologic findings, and management options for congenital hepatic hemangioma (HH) with a focus on describing its natural history and rate of involution. STUDY DESIGN Retrospective review of patients in our HH registry with a congenital HH born between 2004 and 2022 was performed. HH volumes were calculated using the formula for an ellipsoid. Multiple analyses of HH volume change were performed using nonlinear regression with generalized estimating equations, Kaplan-Meier curves, and logrank test. RESULTS We identified 96 infants with a congenital HH. Of these, 32% were diagnosed prenatally, 23% developed heart failure, and 23% developed respiratory failure. On average, patients demonstrated 43% residual HH volume at 12 months (95% confidence interval: 27%-60%) and 16% residual volume at 24 months (95% confidence interval: 0%-35%). No difference in time to 50% HH volume reduction between patients with and without medical therapy was observed (P = .894). CONCLUSION Congenital HH represents the hepatic counterpart of the cutaneous congenital hemangioma and is clinically, histologically, and radiographically distinct from infantile HH. Most lesions reach >80% involution by 24 months of age with no differences in time to involution observed with medical therapy. A clear understanding of the clinical behavior and evolution of congenital HH is critical to early recognition of associated morbidity and prompt review of an incorrect diagnosis.
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Affiliation(s)
- Claire A Ostertag-Hill
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - R Dawn Fevurly
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - Ann M Kulungowski
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | | | - Anna M McGuire
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - Kristy L Rialon
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - Eileen M Duggan
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - Rudy Murillo
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - David Zurakowski
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - Steven J Staffa
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - Ahmad I Alomari
- Department of Radiology, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | | | | | - Steven J Fishman
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA
| | - Belinda H Dickie
- Department of Surgery, Vascular Anomalies Center, Boston Children's Hospital, Boston, MA.
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Grimaldi C, de Ville de Goyet J, Bici K, Cianci MC, Callea F, Morabito A. The role of liver transplantation in the care of primary hepatic vascular tumours in children. Front Oncol 2022; 12:1026232. [PMID: 36505841 PMCID: PMC9730342 DOI: 10.3389/fonc.2022.1026232] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2022] [Accepted: 10/31/2022] [Indexed: 11/25/2022] Open
Abstract
Liver transplantation (LT) is the standard of care for many liver conditions, such as end-stage liver diseases, inherited metabolic disorders, and primary liver malignancies. In the latter group, indications of LT for hepatoblastoma and hepatocellular carcinoma evolved and are currently available for many non-resectable cases. However, selection criteria apply, as the absence of active metastases. Evidence of good long-term outcomes has validated the LT approach for managing these malignancies in the context of specialist and multidisciplinary approach. Nevertheless, LT's role in treating primary vascular tumours of the liver in children, both benign and malignant, remains somewhat controversial. The rarity of the different diseases and the heterogeneity of pathological definitions contribute to the controversy and make evaluating the benefit/risk ratio and outcomes quite difficult. In this narrative review, we give an overview of primary vascular tumours of the liver in children, the possible indications and the outcomes of LT.
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Affiliation(s)
- Chiara Grimaldi
- Department of Pediatric Surgery, Meyer Children’s Hospital, University of Florence, Florence, Italy,*Correspondence: Chiara Grimaldi,
| | - Jean de Ville de Goyet
- Department of Pediatrics, IRCCS-Istituto Mediterraneo per i Trapianti e Terapie ad altra specializzazione (ISMETT) (Institute for Scientific-Based Care and Research-Mediterranean Institute for Transplantation and Advanced Specialized Therapies), Palermo, Italy
| | - Kejd Bici
- Department of Pediatric Surgery, Meyer Children’s Hospital, University of Florence, Florence, Italy
| | - Maria Chiara Cianci
- Department of Neuroscience, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy
| | - Francesco Callea
- Department of Histopathology, Bugando Medical Centre, Catholic University of Healthy Allied Sciences, Mwanza, Tanzania
| | - Antonino Morabito
- Department of Neuroscience, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy
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Gong X, Li Y, Yang K, Chen S, Ji Y. Infantile hepatic hemangiomas: looking backwards and forwards. PRECISION CLINICAL MEDICINE 2022; 5:pbac006. [PMID: 35692445 PMCID: PMC8982613 DOI: 10.1093/pcmedi/pbac006] [Citation(s) in RCA: 17] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/09/2022] [Revised: 01/28/2022] [Accepted: 02/06/2022] [Indexed: 02/05/2023] Open
Abstract
Infantile hepatic hemangiomas (IHHs) are common benign tumors seen in the liver of infants. IHHs are true infantile hemangiomas (IHs) and have phases of proliferation and involution parallel to those of cutaneous IHs. The definition and classification of IHH are still confusing in the literature. The mechanisms during the pathogenesis of IHH have yet to be discovered. The clinical manifestations of IHH are heterogeneous. Although most IHH lesions are asymptomatic, some lesions can lead to severe complications, such as hypothyroidism, consumptive coagulopathy, and high-output congestive cardiac failure. Consequently, some patients can possibly encounter a fatal clinical condition. The heterogeneity of the lesions and the occurrence of disease-related comorbidities can make the treatment of IHH challenging. Oral propranolol is emerging as an effective systemic approach to IHH with obvious responses in tumor remission and symptom regression. However, the precise clinical characteristics and treatment strategies for patients with severe IHH have not yet been well established. Here, we summarize the epidemiology, pathogenic mechanism, clinical manifestations, diagnosis, and treatment of IHH. Recent updates and future perspectives for IHH will also be elaborated.
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Affiliation(s)
- Xue Gong
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
| | - Yanan Li
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
| | - Kaiying Yang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
| | - Siyuan Chen
- Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu 610041, China
| | - Yi Ji
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu 610041, China
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Rela M, Rammohan A, Reddy MS. Liver Tumors in Children. TEXTBOOK OF PEDIATRIC GASTROENTEROLOGY, HEPATOLOGY AND NUTRITION 2022:983-994. [DOI: 10.1007/978-3-030-80068-0_72] [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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Ji Y, Chen S, Yang K, Xiang B, Jiang X, Xu X, Li L, Qiu T, Zhou J, Dai S, Zhang X, Lu G, Kong F, Yang G, Qiu Q. Screening for infantile hepatic hemangioma in patients with cutaneous infantile hemangioma: A multicenter prospective study. J Am Acad Dermatol 2021; 84:1378-1384. [PMID: 33285272 DOI: 10.1016/j.jaad.2020.11.062] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2020] [Revised: 10/12/2020] [Accepted: 11/19/2020] [Indexed: 02/08/2023]
Abstract
BACKGROUND Abdominal ultrasonography has been proposed to screen for infantile hepatic hemangioma (IHH) in patients with multiple cutaneous infantile hemangiomas (IHs). OBJECTIVES The aim of this study was to establish the optimal cutoff point for the number of cutaneous IHs needed to screen for IHH. METHODS We performed a prospective, multicenter study to screen for IHH in patients younger than 9 months who had multiple cutaneous IHs (n ≥ 3) on ultrasonography. For comparison, a group of patients with 1 or 2 focal cutaneous IHs was also recruited. RESULTS In total, 676 patients with at least 3 cutaneous IHs and 980 patients with 1 or 2 focal cutaneous IHs were enrolled. Thirty-one patients were found to have IHH. A higher number of cutaneous IHs was associated with an increased risk of IHH (R = 0.973; P < .001). Receiver operating characteristic curve analysis showed that 5 cutaneous IHs was the optimal cutoff point to screen for IHH, with an area under the curve of 0.872 (P < .001; 95% confidence interval, 0.789-0.955). LIMITATIONS This was an uncontrolled study. CONCLUSIONS Screening for IHH is recommended in patients younger than 9 months who present with 5 or more cutaneous IHs.
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Affiliation(s)
- Yi Ji
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China.
| | - Siyuan Chen
- Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, China.
| | - Kaiying Yang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Bo Xiang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Xian Jiang
- Department of Dermatology, West China Hospital of Sichuan University, Chengdu, China
| | - Xuewen Xu
- Department of Burns and Plastic Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Lizhi Li
- Department of Pediatric Surgery, Fujian Provincial Hospital, Fuzhou, China
| | - Tong Qiu
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Jiangyuan Zhou
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Shiyi Dai
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Xuepeng Zhang
- Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, China
| | - Guoyan Lu
- Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, China
| | - Feiteng Kong
- Department of Pediatric Surgery, Sichuan Women and Children's Hospital, Chengdu, China
| | - Gang Yang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China; Department of Pediatric Surgery, Chengdu Shangjin Nanfu Hospital, Chengdu, China
| | - Qingxia Qiu
- Department of Pediatric Surgery, Chengdu Shangjin Nanfu Hospital, Chengdu, China
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6
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Zavras N, Dimopoulou A, Machairas N, Paspala A, Vaos G. Infantile hepatic hemangioma: current state of the art, controversies, and perspectives. Eur J Pediatr 2020; 179:1-8. [PMID: 31758313 DOI: 10.1007/s00431-019-03504-7] [Citation(s) in RCA: 34] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/30/2019] [Revised: 08/11/2019] [Accepted: 10/09/2019] [Indexed: 12/13/2022]
Abstract
Infantile hepatic hemangioma (IHH) is a common vascular tumor, distinctive for its perinatal presentation, rapid growth during the first year of life, and subsequent involution. Although they generally follow a benign course, some tumors have been reported to undergo malignant transformation. The diagnosis of IHH is based on patient's medical history, physical examination, and imaging. Moreover, the management of this vascular tumor is based on clinical presentation and includes observational, medical, surgical, and radiological interventional treatment options. The present review presents the currently available data in the literature on the diverse aspects of the terminology, epidemiology, clinical presentation, pathogenesis, diagnosis, indications for surgery, malignant potential, and long-term outcomes of these tumors.Conclusion: No formal guidelines have yet been established for the treatment of these hepatic lesions, and the therapeutic strategies implemented vary widely from simple observation to medical, radiological, and surgical interventions in the prism of multidisciplinary teams.What is Known:• Infantile hepatic hemangioma is the most common benign tumor of the liver in infancy, but despite its benign nature, it can present with life-threatening complications.• The treatment strategies range from simple observation to a series of medical, surgical, and radiological interventions.What is New:• This review gives an overview of the developments and current status about the management of IHH.• The aim of this study is to clear up the confusion and controversy that exists about terminology, diagnosis, and treatment of IHH.
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Affiliation(s)
- Nikolaos Zavras
- Department of Paediatric Surgery, "ATTIKON" General University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Anastasia Dimopoulou
- Department of Paediatric Surgery, "ATTIKON" General University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
| | - Nikolaos Machairas
- Third Department of Surgery, "ATTIKON" General University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Anna Paspala
- Third Department of Surgery, "ATTIKON" General University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - George Vaos
- Department of Paediatric Surgery, "ATTIKON" General University Hospital, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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Lekwuttikarn R, Josephs S, Teng JM. Successful Medical Management of Life-threatening Hepatic Hemangioma in Neonates. Pediatrics 2019; 144:peds.2019-1339. [PMID: 31511312 DOI: 10.1542/peds.2019-1339] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/28/2019] [Indexed: 11/24/2022] Open
Abstract
Hepatic hemangioma (HH) is a common asymptomatic, self-limiting benign vascular tumor of the liver in neonates. Although complicated HHs are rare, they have significant risks of morbidity and mortality, especially during the perinatal period. Because of the high risks of complications from surgical interventions, there is an unmet need for effective medical therapy. We report 2 neonates with life-threatening HH who were evaluated for a liver transplant before being treated successfully with combined medical therapy, which included sirolimus, corticosteroids, and propranolol.
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Affiliation(s)
- Ramrada Lekwuttikarn
- Division of Pediatric Dermatology, Department of Dermatology and.,Section of Dermatology, Division of Ambulatory and Adolescent Medicine, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
| | - Shellie Josephs
- Division of Pediatric Radiology, Department of Radiology, School of Medicine, Stanford University, Stanford, California; and
| | - Joyce M Teng
- Division of Pediatric Dermatology, Department of Dermatology and
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Osada A, Araki E, Yamashita Y, Ishii T. Combination therapy of propranolol, levothyroxine, and liothyronine was effective in a case of severe consumptive hypothyroidism associated with infantile hepatic hemangioma. Clin Pediatr Endocrinol 2019; 28:9-14. [PMID: 30745728 PMCID: PMC6356094 DOI: 10.1297/cpe.28.9] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2018] [Accepted: 11/01/2018] [Indexed: 11/12/2022] Open
Abstract
Infantile hepatic hemangioma (IHH) can be accompanied by consumptive hypothyroidism. We
report the case of a 4-mo-old boy who showed massive hepatomegaly, peripheral coldness,
lethargy, and failure to thrive. An enhanced computed tomography scans demonstrated
multiple hemangiomas in both lobes of the liver, and a thyroid function tests showed
severe hypothyroidism: TSH 561.5 µIU/mL, free triiodothyronine (fT3) 1.0 pg/mL,
and free thyroxine (fT4) < 0.7 ng/dL. IHH gradually regressed following
propranolol treatment and fT4 increased to a low normal level (1.0 ng/dL) by
high dose replacement of levothyroxine, while fT3 remained very low (< 1.0
pg/mL), even following high doses of levothyroxine; fT3 eventually normalized
following the administration of liothyronine. We suggest that treatment strategies should
be individualized based on thyroid function, and that the combination therapy of
propranolol for anti-tumor treatment and levothyroxine and liothyronine for respective
thyroid hormone replacement is effective, particularly in cases of severe consumptive
hypothyroidism due to multiple IHHs.
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Affiliation(s)
- Asami Osada
- Department of Pediatrics, Yokohama Municipal Citizen's Hospital, Yokohama, Japan.,Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
| | - Eriko Araki
- Department of Pediatrics, Yokohama Municipal Citizen's Hospital, Yokohama, Japan
| | - Yukio Yamashita
- Department of Pediatrics, Yokohama Municipal Citizen's Hospital, Yokohama, Japan
| | - Tomohiro Ishii
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
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9
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Shen WC, Liang PC, Wu JF, Hsu CT, Chen HS, Chen HL. Combined Transarterial Embolization and Propranolol as Effective Treatment for Hepatic Hemangioma with Kasabach-Merritt Syndrome and Heart Failure in a Neonate. Pediatr Neonatol 2017; 58:191-193. [PMID: 27373825 DOI: 10.1016/j.pedneo.2016.01.008] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2015] [Revised: 12/14/2015] [Accepted: 01/08/2016] [Indexed: 11/26/2022] Open
Affiliation(s)
- Wan-Chen Shen
- Department of Pediatrics, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan
| | - Po-Chin Liang
- Department of Medical Imaging, National Taiwan University Hospital, Taipei, Taiwan
| | - Jia-Feng Wu
- Department of Pediatrics, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan
| | - Chien-Ting Hsu
- Department of Pediatrics, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan
| | - Ho-Sheng Chen
- Department of Pediatrics, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan
| | - Huey-Ling Chen
- Department of Pediatrics, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan; Department of Medical Education and Bioethics, National Taiwan University College of Medicine, Taipei, Taiwan.
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Li K, Wang Z, Liu Y, Yao W, Gong Y, Xiao X. Fine clinical differences between patients with multifocal and diffuse hepatic hemangiomas. J Pediatr Surg 2016; 51:2086-2090. [PMID: 27686480 DOI: 10.1016/j.jpedsurg.2016.09.045] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/20/2016] [Accepted: 09/12/2016] [Indexed: 10/21/2022]
Abstract
BACKGROUND It has been reported that multifocal and diffuse hepatic hemangiomas are true infantile hemangiomas for which a continuum probably exists. We determined the similarities and fine differences between the two types of hemangioma and identified the multifocal subgroup of type, which needs timely treatment. MATERIAL AND METHODS Twenty-four patients (4 males and 20 females; age 114±142days) with multifocal or diffuse hepatic hemangiomas who were treated between January 2000 and June 2015 were studied. For the multifocal type, patients were divided into countable (MC) and uncountable (MU) subgroups. The medical data were analyzed retrospectively. RESULTS The clinical presentations included hepatomegaly (n=11), dyspnea (n=7), heart failure (n=9), hypothyroidism (n=6), and anemia (n=1). There were 19 and 5 patients with multifocal and diffuse types, and 6 and 13 patients in the MC and MU groups, respectively. There were significant differences between the multifocal and diffuse types with respect to hepatomegaly, heart failure, dyspnea, and hypothyroidism. However, there was no difference between the MU group and the diffuse type except for hypothyroidism. Observation was commonly recommended for patients with the multifocal type, and their survival rate was clearly higher than for patients with the diffuse type. Both the MU and diffuse-type groups needed treatment, unlike MC patients. The complete remission rate was higher in the MC group than in the MU and diffuse-type groups. Patients with the diffuse type were more likely to die. CONCLUSIONS Patients with diffuse-type hepatic hemangioma are at high risk and need active treatment. The MU group for the multifocal type is unique and has high similarities to the diffuse type with respect to clinical presentation and treatment, which suggests that a continuum of the disease phenotypes exists. LEVEL OF EVIDENCE Case-control study, level III.
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Affiliation(s)
- Kai Li
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 201102, Shanghai, China.
| | - Zuopeng Wang
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 201102, Shanghai, China
| | - Yinhua Liu
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 201102, Shanghai, China
| | - Wei Yao
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 201102, Shanghai, China
| | - Yin Gong
- Department of Radiology, Children's Hospital of Fudan University, 201102, Shanghai, China
| | - Xianmin Xiao
- Department of Pediatric Surgery, Children's Hospital of Fudan University, 201102, Shanghai, China
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11
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Ji Y, Chen S, Xiang B, Xu Z, Jiang X, Liu X, Wang Q, Lu G, Yang L. Clinical features and management of multifocal hepatic hemangiomas in children: a retrospective study. Sci Rep 2016; 6:31744. [PMID: 27530723 PMCID: PMC4987646 DOI: 10.1038/srep31744] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2016] [Accepted: 07/26/2016] [Indexed: 02/05/2023] Open
Abstract
Multifocal hepatic hemangioma (MHH) is a benign hepatic tumor that is commonly diagnosed in children with multiple cutaneous infantile hemangiomas (IHs). We present a review of all children with MHH at our institutions. Of the 42 patients, the median age at presentation of MHH was 2.5 months. Thirty-six (85.7%) patients had cutaneous IHs. Twelve (28.6%) patients were symptomatic at presentation. There was no significant association between the number of hepatic hemangiomas and the number of cutaneous IHs. Fourteen (33.3%) patients received some form of treatment for hepatic hemangiomas. The most common type of treatment was oral prednisone in 8 patients, followed by oral propranolol in 6 patients. Two patients were totally resistant to prednisone treatment. They died from congestive heart failure or respiratory distress and coagulopathy. Two patients with problematic facial IH were treated with intralesional triamcinolone injection. The remaining 26 patients were managed with imaging surveillance. On follow-up, all of the survivors had a favorable outcome. Our study suggests that the clinical features of MHH are variable. Our data emphasize the treatment strategy that aggressive treatment is indicated in symptomatic or progressive MHHs, whereas observation management of asymptomatic patients with a few small lesions is safe and appropriate.
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Affiliation(s)
- Yi Ji
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China
| | - Siyuan Chen
- Pediatric Intensive Care Unit, West China Hospital of Sichuan University, Chengdu, 610041, China
| | - Bo Xiang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China
| | - Zhicheng Xu
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China
| | - Xiaoping Jiang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China
| | - Xingtao Liu
- Department of Vascular & Interventional Radiology, Chengdu Women and Children’s Central Hospital, Chengdu, 610091, China
| | - Qi Wang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China
| | - Guoyan Lu
- Pediatric Intensive Care Unit, West China Second University Hospital, Sichuan University, Chengdu, 610041, China
| | - Li Yang
- Department of Radiology, West China Hospital of Sichuan University, Chengdu, 610041, China
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12
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Wu L, Zhao L, Lu Y, He L, Hu X. Interventional embolization of congenital intrahepatic shunts in children. Pediatr Radiol 2016; 46:541-7. [PMID: 26637318 DOI: 10.1007/s00247-015-3497-3] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/20/2015] [Revised: 08/28/2015] [Accepted: 10/28/2015] [Indexed: 12/30/2022]
Abstract
BACKGROUND Congenital intrahepatic shunts in children are rare and precise treatment strategies have not been established. OBJECTIVE The purpose of this study was to present our experience with transcatheter embolization in a descriptive case series of congenital intrahepatic shunts in children. MATERIALS AND METHODS We retrospectively studied 10 children with symptomatic congenital intrahepatic shunts who were treated with embolization at our institute between January 2008 and December 2014. RESULTS Seven children had arteriovenous shunts in association with hepatic hemangiomas, two had arterioportal shunts and one had portosystemic shunts. The major presentations were congestive heart failure and severe anemia in the groups with arteriovenous and arterioportal shunts, respectively. Following embolization, two children died in the arteriovenous group, one from progressive liver dysfunction and the other from abdominal compartment syndrome. One child in the arterioportal group required liver transplantation after initial embolization. With mean post-procedure follow-up of 15 months (range 4-54 months), all the remaining children were well, with resolution of the symptoms. CONCLUSION Interventional embolization provides an alternative to surgical ligation or hepatic resection in children with clinically significant intrahepatic shunts. For lesions with complex morphology, embolization may be inadequate and liver transplantation might be the only therapeutic option.
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Affiliation(s)
- Lin Wu
- Cathererization Laboratory, Cardiovascular Center, Children's Hospital of Fudan University, Shanghai, China
| | - Lu Zhao
- Cathererization Laboratory, Cardiovascular Center, Children's Hospital of Fudan University, Shanghai, China
| | - Ying Lu
- Cathererization Laboratory, Cardiovascular Center, Children's Hospital of Fudan University, Shanghai, China
| | - Lan He
- Cathererization Laboratory, Cardiovascular Center, Children's Hospital of Fudan University, Shanghai, China
| | - Xihong Hu
- Department of Radiology, Children's Hospital of Fudan University, 399 Wan Yuan Road, Shanghai, 201102, People's Republic of China.
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13
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Moyakine AV, Vleuten CJMVD. Propranolol for infantile hemangioma: Current state of affairs. World J Dermatol 2016; 5:4-16. [DOI: 10.5314/wjd.v5.i1.4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/27/2015] [Revised: 10/19/2015] [Accepted: 12/14/2015] [Indexed: 02/06/2023] Open
Abstract
Infantile hemangioma (IH) is the most common benign tumor seen in infancy. This review provides up-to-date information on the pathophysiology, variations in clinical presentation, and natural history of IH, elaborating on associated anomalies, such as PHACE(S) syndrome and LUMBAR syndrome. Because of the benign and self-limiting characteristics seen in more than 90% of cases of IH, a conservative approach is usually chosen. However, some circumstances, such as ulceration, vision loss, breathing difficulties, or potential disfigurement, will require treatment during the proliferative phase. For decades, treatment of IH has primarily consisted of corticosteroids or surgery. Since 2008, propranolol has become the treatment of first choice. In this article, we bring to light the crucial changes in the treatment of IH over the past years. To date, there is still a lack of data on the possible long-term effects of propranolol treatment in young infants. A theoretical probability of the central nervous system being affected (that is, impairment of short- and long-term memory, psychomotor function, sleep quality, and mood) has recently been suggested. This review highlights research topics concerning these long-term adverse effects. Finally, information is provided on the potential instruments to measure IH severity and activity in clinical trials and/or in clinical practice and the recently developed and first-validated IH-specific quality-of-life questionnaire.
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Scuderi V, Tomassini F, Troisi RI. Transplantation for Benign Liver Lesions. BENIGN TUMORS OF THE LIVER 2015:233-251. [DOI: 10.1007/978-3-319-12985-3_18] [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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