Abdominal kaposiform hemangioendothelioma presenting as volvulus and recurring intestinal obstruction in a neonate: a...

Kaposiform hemangioendothelioma is a rare vascular tumor primarily occurring in infants and children. The most common sites for kaposiform hemangioendothelioma are extremities, with very few cases of abdominal kaposiform hemangioendothelioma reported in neonates. Making a diagnosis of Kaposiform hemangioendothelioma can be challenging when the patient presents with generalized symptoms such as bilious vomiting and constipation that can be attributed to other more common causes of intestinal obstruction.

A 28-day-old Pakistani baby girl presented with bilious vomiting and constipation. The abdomen was distended, and scans showed generalized dilation of bowel loops. A preliminary diagnosis of intestinal obstruction due to volvulus was made, and the patient underwent an exploratory laparotomy. Postoperatively, the patient remained unwell with profuse aspirates in nasogastric and no improvement in ileus. Repeat scans were again suggestive of midgut volvulus, which was initially managed conservatively, but later redo surgery was done. Ischemic bowel was resected with histopathology showing kaposiform hemangioendothelioma. In the meantime, the baby developed an abdominal collection, infection, and short gut syndrome due to resection of the bowel. The family was counseled regarding the prognosis, and the patient was transferred from hospital to home upon request. The baby passed away 2 days later.

While extremely rare, abdominal kaposiform hemangioendothelioma can be an important and potentially lifesaving differential diagnosis to consider in neonates and infants with recurring and nonresolving intestinal obstruction. The management is complex, and prognosis can be poor in diffuse, congested lesions involving a large part of the gut.