Overview
Capillary hemangiomas, also known as infantile hemangiomas, are benign vascular tumors composed of dilated capillaries. They commonly occur in infants and can present with complications such as Kasabach-Merritt syndrome, characterized by thrombocytopenia and consumptive coagulopathy 3.Diagnosis
Presence of cutaneous or subcutaneous vascular lesions
Clinical features may include rapid growth followed by spontaneous regression 3
Laboratory tests: Thrombocytopenia, prolonged prothrombin time, and low fibrinogen levels in Kasabach-Merritt syndrome 3
Imaging: Ultrasound or MRI to assess lesion extent and complications 3Management
First-line treatments:
- Biopsy: Can lead to prompt elevation of platelet count 3
- Subtotal resection: Immediate increase in platelet count observed in some cases 3
Adjunctive treatments:
- Embolization: Transient increase in platelet counts and fibrinogen levels 3
- Medications to control coagulopathy (e.g., corticosteroids, antifibrinolytics) 3
- Blood product support (platelets, cryoprecipitate) for severe bleeding 3Special Populations
Pediatrics: Infants and young children are most commonly affected; aggressive management required for Kasabach-Merritt syndrome 3
Rare Cases: Primary capillary leak syndrome is extremely rare in children, with fatal outcomes reported in neonates 1Key Recommendations
Biopsy hemangioma in cases of Kasabach-Merritt syndrome to rapidly elevate platelet counts (Evidence: Moderate 3)
Consider subtotal resection for significant thrombocytopenia unresponsive to medical therapy (Evidence: Moderate 3)
Monitor and manage coagulopathy with appropriate blood products and medications (Evidence: Moderate 3)
Aggressive supportive care including inotropes may be necessary in severe cases of capillary leak syndrome (Evidence: Weak 1)References
1 Kulihova K, Prochazkova M, Semberova J, Janota J. Fatal Primary Capillary Leak Syndrome in a Late Preterm Newborn. Indian journal of pediatrics 2016. link
2 Brunetti-Pierri N, Seidel GF, Levy ML, Reid Sutton V. Parkes Weber syndrome occurring in a family with capillary malformations. Clinical dysmorphology 2007. link
3 Larsen EC, Zinkham WH, Eggleston JC, Zitelli BJ. Kasabach-Merritt syndrome: therapeutic considerations. Pediatrics 1987. link