Degenerated Waldmann's Small Bowel Disease: An Exceptional Entity

Primary intestinal lymphangiectasia, also known as Waldmann's disease, is a rare pathology first described in 1961, typically presenting in childhood or adolescence. It is characterized by exudative enteropathy of lymphatic origin. Diagnosis is based on clinical, biological, and endoscopic criteria after excluding secondary forms. Complications can arise, the most severe being malignant non-Hodgkin's lymphoma. We report the case of a 39-year-old man with a history of hypothyroidism, managed for exudative enteropathy linked to Waldmann's disease with a lowfat diet and vitamin supplementation. He was admitted with acute intestinal obstruction. An abdominal CT scan confirmed acute mechanical small bowel obstruction. Emergency surgery revealed staged small bowel stenoses and a 3 cm tumoral lesion. A carcinological bowel resection was performed, with delayed restoration of digestive continuity due to the patient's critical condition. Pathology identified mucinous adenocarcinoma associated with intestinal lymphangiectasia. Postoperative complications included a digestive fistula, fever, pancytopenia, and neurological symptoms such as seizures. A myelogram revealed hemophagocytosis, and macrophage activation syndrome was suspected. Despite treatment, the patient developed status epilepticus and died three months later.