Early surgical intervention in a patient with Kasabach-Merritt phenomenon

Area of Science:

• Pediatric Surgery
• Hematology
• Oncology

Background:

• Kasabach-Merritt phenomenon (KMP) is a rare condition characterized by a vascular tumor that sequesters platelets and coagulation factors.
• KMP can lead to severe coagulopathy, including thrombocytopenia, hypofibrinogenemia, and elevated fibrin split products.
• Diagnosis requires a high index of suspicion in infants presenting with a vascular mass and bleeding diathesis.

Observation:

• A 3-day-old male infant presented with a firm, 3-cm subcutaneous mass.
• Laboratory findings revealed thrombocytopenia, decreased fibrinogen, and increased fibrin split products, consistent with KMP.
• The vascular lesion was surgically resected.

Findings:

• Complete resection of the vascular lesion was achieved without complications.
• Post-operative recovery was uneventful, with resolution of the coagulopathy.

Implications:

• Early surgical intervention is a viable and effective therapeutic strategy for Kasabach-Merritt phenomenon.
• Prompt diagnosis and management can prevent life-threatening complications associated with KMP.
• This case highlights the importance of considering surgical resection as a primary treatment option for KMP.