Intestinal failure in kaposiform hemangioendothelioma successfully resolved with medical therapy and nearly fat-free oral feeding: A novel case report

Background: Kaposiform hemangioendothelioma and kaposiform lymphangiomatosis (KHE/KLA) are rare, non-malignant lymphovascular anomalies often complicated by Kasabach-Merritt phenomenon (KMP). Morbidity and mortality are high. There are no prior reports of the dietary management in these disorders. Case report: A 2-month-old female presented with hemorrhagic ascites, abdominal compartment syndrome, and severe KMP secondary to extensive retroperitoneal and intestinal KHE/KLA. Following emergent surgical management for abdominal decompression and diverting ileostomy, medical and nutritional therapy with steroids, vincristine, sirolimus, and parenteral nutrition were employed for management. After maintaining a near fat-free enteral diet supplemented with intravenous lipid therapy in the outpatient setting, she underwent successful ileostomy takedown 18 months after initial diagnosis without the need for intestinal resection and maintenance of adequate growth. Conclusion: This case underscores the value of clinical and pathology-guided diagnosis, coordinated medical therapy, and dietary fat restriction for achieving intestinal autonomy and preserving bowel length in intra-abdominal KHE/KLA.