Introduction:
Kaposiform hemangioendothelioma is a rare tumor, of vascular origin, which predominates in childhood. Although it is frequently a vascular cutaneous anomaly, it is known that some cases can involve bones, mediastinum and retroperitoneum, being even rarer when it occurs in the small intestine, representing only 0.05% of all intestinal neoplasms.
Presentation of case:
Female patient, 21 days old, born at 38 weeks, is admitted to the hospital due to bilious vomiting, dehydration and major abdominal distension. An X-ray of the acute abdomen suggests type I jejunal atresia. An exploratory laparotomy was performed, with the presence of a stenotic area in the proximal jejunum, which was chosen for resection of the stenotic area and end-to-end terminal anastomosis, with post-pyloric jejunostomy. The presence of kaposiform hemangioma was confirmed by immunohistochemistry, and the patient had no further complications.
Discussion:
The presence of hemangiomas in childhood is frequent, is but it very rare when it appears in the gastrointestinal location, as reported in the present case. A common presentation is intestinal bleeding, being manifested as anemia or, sometimes, acute and potentially fatal anemia. Other forms of rare presentation include intussusception, perforation and obstruction, the latter being out patient's last manifestation. The diagnosis of this alteration can be difficult, and often performed only in the intraoperative period of an exploratory laparotomy.
Conclusion:
The presence of bilateral vomiting and intestinal obstruction in newborns opens the possibility of a wide range of possible diagnoses, such as the presence of intestinal atresia. Kaposiform intestinal hemangioma is an uncommon cause in these clinical manifestations, but it should be included in the differential diagnosis.