Case Report: Life-threatening Kasabach–Merritt phenomenon in a 2-month-old child

TL;DR

A 2-month-old infant with severe symptoms was diagnosed with a rare vascular condition called Kasabach–Merritt Phenomenon and successfully treated with a multidisciplinary approach.

Contribution

This is the first reported case of Kasabach–Merritt Phenomenon in a critically ill infant with this specific clinical presentation.

Findings

The patient exhibited life-threatening symptoms including hypertension, coagulopathy, and ascites.

Diagnosis of Kasabach–Merritt Phenomenon was confirmed after extensive diagnostic procedures.

Treatment with methylprednisolone and sirolimus led to successful recovery and discharge.

Abstract

We present a rare case of a 2-month-old girl, born at term, who was admitted to the pediatric intensive care unit for fussiness, increasing abdominal distension, and intermittent diarrhea for one week. She was found to be hypertensive, coagulopathic, and suffering from unrelenting ascites. Over the course of the following month, her symptoms became life-threatening, and she was intubated, sedated, and paralyzed. She underwent multiple diagnostic and therapeutic procedures, including exploratory laparotomy, MRI, colonoscopy, and multiple peritoneal drains. After several weeks, the medical teams reached a consensus diagnosis of a rare and complex vascular anomaly characterized by a life-threatening Kasabach–Merritt Phenomenon (KMP). KMP is known to occur in association with several anomalies, including kaposiform hemangioendothelioma and kaposiform lymphangiomatosis. Life-saving therapies were initiated, including methylprednisolone and sirolimus. The patient was ultimately discharged and sent home with her parents. To our knowledge, this is the only reported case describing this constellation of symptoms in a critically ill infant. Multidisciplinary cooperation was key in saving the patient's life. Critical care providers should consider vascular anomalies in patients with similar presentations.

Figures