Duodenal obstruction - A rare complication of Langerhans Cell Histiocytosis – a case report

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Langerhans cell histiocytosis (LCH) has a highly variable presentation, of which gastrointestinal involvement is extremely rare. Gastrointestinal involvement portends a poor prognosis in LCH. Herein, we describe the presentation, progression, management, and outcome of an 11-month-old female infant with LCH. The patient presented with severe acute malnutrition, hepatosplenomegaly, and gastrointestinal involvement in the form of persistent vomiting. She was managed with chemotherapy initially and subsequently developed complete duodenal narrowing. She underwent Kimura's duodenojejunostomy and was improving after that. Hence, chemotherapy was restarted. Two months later, she developed features of intestinal obstruction with intraoperative findings of ileal gangrene requiring adhesiolysis, ileal resection, and ileostomy. Subsequently, she succumbed to sepsis. This case report aims to highlight the atypical gastrointestinal manifestations of LCH and the challenges faced in managing such cases.