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Boston, MA, United States

Schook C.C.,Harvard University | Mulliken J.B.,Harvard University | Fishman S.J.,Harvard University | Grant F.D.,Harvard University | And 3 more authors.
Plastic and Reconstructive Surgery

Background: Lymphedema results from maldevelopment of the lymphatic system (primary) or injury to lymphatic vasculature (secondary). Primary lymphedema is far less common than the secondary condition. The purpose of this study was to determine the clinical features of primary lymphedema in the pediatric age group. Methods: The authors' Vascular Anomalies Center database was reviewed for patients evaluated between 1999 and 2010 with onset of lymphedema before 21 years of age. Cause, sex, age of onset, location, and familial/syndromic association were determined. Morbidity, progression, and treatment were analyzed. Results: Lymphedema was confirmed in 142 children: 138 cases (97.2 percent) were primary and four (2.8 percent) were secondary. Analysis of the primary cohort showed that 58.7 percent of the patients were female. Age of onset was infancy, 49.2 percent; childhood, 9.5 percent; or adolescence, 41.3 percent. Boys most commonly presented in infancy (68.0 percent), whereas girls usually developed swelling in adolescence (55.3 percent). Lymphedema involved an extremity (81.9 percent), genitalia (4.3 percent), or both (13.8 percent). The lower limb was most commonly affected (91.7 percent), and 52.9 percent had bilateral lower extremity disease. Eleven percent of patients had familial or syndromic lymphedema. Cellulitis occurred in 18.8 percent of children; 13.0 percent required hospitalization. The majority of patients (57.9 percent) had progression of their disease. Treatment was compression garments alone (75.4 percent) or in combination with pneumatic compression (19.6 percent); 13.0 percent had operative intervention. Conclusions: Pediatric primary lymphedema usually involves the lower extremities. Boys typically are affected at birth, and girls most often present during adolescence. Most patients do not have major morbidity, are successfully managed by compression, and do not require surgical treatment. Copyright © 2011 by the American Society of Plastic Surgeons. Source

Hsi Dickie B.,Hemangioma and Vascular Malformation Center Cincinnati Childrens Hospital Medical Center | Fishman S.J.,Boston Childrens Hospital | Fishman S.J.,Vascular Anomalies Center | Azizkhan R.G.,Hemangioma and Vascular Malformation Center Cincinnati Childrens Hospital Medical Center
Seminars in Pediatric Surgery

The most common hepatic vascular tumor in the pediatric population is the infantile hepatic hemangioma. Although these lesions have a spectrum of presentations, there are three main subtypes that have been described-focal, multifocal, and diffuse. An algorithm on the workup, treatment, and follow-up of these lesions can be based on this categorization. Recent shifts in the management of hemangiomas with beta-blockers (propranolol) have also influenced the treatment of hepatic hemangiomas. This article reviews the current understanding of hepatic hemangiomas and protocols in the management of these patients. © 2014 Elsevier Inc. Source

McCormick A.,Vascular Anomalies Center | Rosenberg S.,Childrens Hospital of Pittsburgh of UPMC | Tier K.,Childrens Hospital of Pittsburgh of UPMC | Balest A.,Childrens Hospital of Pittsburgh of UPMC

The study of vascular anomalies is a rapidly progressing field in medicine. The development of new knowledge in the pathology and management of these disease processes are exemplified in the treatment of hemangiomas with propranolol and generalized lymphatic malformations with sirolimus. Central conducting lymphatic anomalies have traditionally been refractory to medical and surgical interventions. We report a case of a central conducting lymphatic anomaly that was responsive to sirolimus. A 14-year-old boy presented with chylothorax and chyluria with a lymphangiogram demonstrating abnormal lymphatic flow and ref lux along the entire course of the central channels. Traditionally, medical management has been limited to somatostatin and low-fat diet with poor response and surgical interventions that are palliative. Sirolimus allows a new medical option that could improve management of this unresponsive population. © 2016 by the American Academy of Pediatrics. Source

Chute C.,Hemangioma and Vascular Malformations Center | Stein B.,Cincinnati Childrens Medical Center | Sylvia M.B.,Vascular Anomalies Center | Spera E.,Vascular Anomalies Center
Seminars in Pediatric Surgery

Patients with vascular anomalies present specific and unique challenges to providers of their postoperative care. Vascular anomalies can range from localized solitary lesions to diffuse lesions with vessel malformations and associated soft tissue, muscle, organ, and bone involvement. Perioperative issues for these patients can be complicated and include coagulopathies requiring anticoagulation, the need for postoperative surgical drains and specialized wound care, and the use of compression garments to maintain the desired postoperative result. This article will address these specific concerns for patients with vascular anomalies in order to create a framework for consistent and appropriate perioperative care. © 2014. Source

Greene A.K.,Vascular Anomalies Center
The Journal of craniofacial surgery

The field of vascular anomalies is confusing because numerous types of lesions exist, different anomalies often look similar, and imprecise terminology commonly is used. Pharmacotherapy is effective for certain vascular tumors; sclerotherapy generally is the primary treatment of problematic lymphatic and venous malformations. Arteriovenous malformations remain difficult to manage because of their high progression and recurrence rates. Propranolol has gained popularity recently for the treatment of problematic infantile hemangioma, but its efficacy and safety compared with corticosteroid therapy have not been studied. Continuing education is needed to increase the use of accepted biologic terms to describe vascular anomalies; this will improve patient care and facilitate research. As vascular anomalies centers continue to develop, children will have easier access to interdisciplinary expertise. Patients are most likely to be diagnosed and treated properly when managed by a specialist or team focused on these conditions. Recent insight into the etiopathogenesis of infantile hemangioma and vascular malformations may lead to novel therapies for these lesions in the near future. Source

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