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Submandibular hemangioma as the initial manifestation of Kasabach-Merritt syndrome
1Department of Otolaryngology, Hyogo Prefectural Awaji Hospital, Sumoto, Japan.
Abstract:
Kasabach-Merritt syndrome (thrombocytopenia, consumption coagulopathy and occasional hemolysis) is an infrequent but often fatal complication of rapidly growing hemangiomas in infants. We describe a 1-month-old infant with a huge hemangioma involving the left submandibular region associated with a severe consumptive coagulopathy, who was successfully treated with transfusion of blood products, prednisone and radiation therapy. It is stressed that pediatric otorhinolaryngologists should always be aware of the lethal status of this condition in infants.
Insights
Kasabach-Merritt syndrome, a rare complication of infantile hemangiomas, can be fatal. This case highlights successful treatment using blood products, prednisone, and radiation therapy for a severe presentation.
Area of Science:
- Pediatric Oncology
- Hematology
- Vascular Anomalies
Background:
- Kasabach-Merritt syndrome is a rare, life-threatening complication associated with rapidly growing infantile hemangiomas.
- It presents with thrombocytopenia, consumptive coagulopathy, and potential hemolysis.
Observation:
- A 1-month-old infant presented with a large submandibular hemangioma.
- The infant exhibited severe consumptive coagulopathy.
Findings:
- Successful treatment was achieved with a combination of blood product transfusions, prednisone, and radiation therapy.
- This multimodal approach effectively managed the coagulopathy and hemangioma.
Implications:
- Pediatric otorhinolaryngologists must maintain high awareness of Kasabach-Merritt syndrome's potential lethality in infants.
- Early recognition and prompt, aggressive treatment are crucial for improving outcomes in affected infants.