Unusual Vascular Anomalies in Pediatric Hand and Plastic Surgery

INTRODUCTION: Rare pediatric vascular tumors and malformations can be diagnostically and therapeutically challenging. They may present atypically, mimic more common conditions, or be life-threatening. Understanding of the various conditions, early recognition, and multidisciplinary management are essential for successful treatment. METHODS: A retrospective review was performed of selected pediatric vascular anomaly cases managed at a tertiary referral center between March 2023 and August 2025. Cases were chosen based on diagnostic complexity, rarity, and the requirement for multidisciplinary and/or targeted systemic therapy. CASE PRESENTATION SUMMARY: Five cases are presented: kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon, congenital hemangioma, generalized lymphatic anomaly, and PIK3CA-related overgrowth spectrum disorder presenting with multifocal osteolytic lesions and fractures. Diagnoses were established through radiologic, histopathologic, and targeted genetic and molecular testing. Management combined wound care and reconstruction with systemic therapies. Propranolol was used as first-line therapy in infantile hemangioma, corticosteroids and sirolimus were used in vascular tumors with hematological complications, and alpelisib was utilized in PIK3CA-related disease. Outcomes were variable but demonstrated significant symptomatic and functional improvement with good overall tolerance of systemic therapy. CONCLUSION: These cases highlight the heterogeneity of pediatric vascular anomalies and their potential to mimic nonvascular pathology. Hand and plastic surgeons play a key role in recognition, biopsy, wound care and reconstruction, and rehabilitation within multidisciplinary teams. Novel targeted systemic therapies, such as sirolimus and alpelisib, alongside more traditional agents, including propranolol and corticosteroids, are central where surgery alone is inadequate. Awareness of these rare vascular anomalies is essential for timely diagnosis and treatment.

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Publication Details

Journal
Annals of Plastic Surgery
Published
2026-04-22
DOI
https://doi.org/10.1097/sap.0000000000004755
Primary Topic
Vascular Malformations and Hemangiomas
Type
article
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article

Unusual Vascular Anomalies in Pediatric Hand and Plastic Surgery

Ramesh Vidyadharan, Andrea Jester, Sanjeev Sharma
Annals of Plastic Surgery
Vascular Malformations and Hemangiomas
article

Unusual Vascular Anomalies in Pediatric Hand and Plastic Surgery

Ramesh Vidyadharan, Andrea Jester, Sanjeev Sharma
article en

Abstract

INTRODUCTION: Rare pediatric vascular tumors and malformations can be diagnostically and therapeutically challenging. They may present atypically, mimic more common conditions, or be life-threatening. Understanding of the various conditions, early recognition, and multidisciplinary management are essential for successful treatment. METHODS: A retrospective review was performed of selected pediatric vascular anomaly cases managed at a tertiary referral center between March 2023 and August 2025. Cases were chosen based on diagnostic complexity, rarity, and the requirement for multidisciplinary and/or targeted systemic therapy. CASE PRESENTATION SUMMARY: Five cases are presented: kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon, congenital hemangioma, generalized lymphatic anomaly, and PIK3CA-related overgrowth spectrum disorder presenting with multifocal osteolytic lesions and fractures. Diagnoses were established through radiologic, histopathologic, and targeted genetic and molecular testing. Management combined wound care and reconstruction with systemic therapies. Propranolol was used as first-line therapy in infantile hemangioma, corticosteroids and sirolimus were used in vascular tumors with hematological complications, and alpelisib was utilized in PIK3CA-related disease. Outcomes were variable but demonstrated significant symptomatic and functional improvement with good overall tolerance of systemic therapy. CONCLUSION: These cases highlight the heterogeneity of pediatric vascular anomalies and their potential to mimic nonvascular pathology. Hand and plastic surgeons play a key role in recognition, biopsy, wound care and reconstruction, and rehabilitation within multidisciplinary teams. Novel targeted systemic therapies, such as sirolimus and alpelisib, alongside more traditional agents, including propranolol and corticosteroids, are central where surgery alone is inadequate. Awareness of these rare vascular anomalies is essential for timely diagnosis and treatment.

Annals of Plastic Surgery
ING Direct (US), National Centre of Scientific Research "Demokritos" (GR), Center For Policy Research (US), Birmingham Children's Hospital (GB)
Good health and well-being
Openalex Percentile: Top 18%
Vascular Malformations and Hemangiomas
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