DOI: 10.36347/sjmcr.2024.v12i04.008

Pages: 412-416

Introduction: Waldmann's disease, or primary intestinal lymphangiectasia (PIL), is an uncommon condition characterized by digestive lymphatic dilatations, predominantly observed in the pediatric population and rarely in adults. First described in 1961, its etiology remains unknown, and it presents with diverse manifestations, from chronic diarrhea to ascites. Diagnosis involves intestinal biopsies and radiological assessments, with treatment primarily focused on dietary modifications and, in exceptional cases, surgery. Observation: This report details a unique case of Waldmann's disease diagnosed in a 52-year-old man with a history of type 2 diabetes. Presenting with a complex clinical picture including generalized edematous syndrome, chronic diarrhea, and ascitic manifestations, the patient underwent a comprehensive diagnostic evaluation ruling out renal, hepatic, and cardiac causes. Endoscopic examinations revealed multiple duodenal lymphangiectasias, supporting the diagnosis of exudative gastroenteropathy. The patient responded positively to dietary interventions with medium-chain triglycerides, showing a significant regression of symptoms. Discussion: Waldmann's disease, a rare pathology with unknown prevalence, typically manifests in early childhood. However, the case presented here highlights the atypical onset in adulthood, emphasizing the importance of considering this diagnosis in cases of exudative enteropathy with an early onset. Clinical signs, including lower limb edema, chronic diarrhea, and visceral effusions, may vary, making a definitive diagnosis challenging. Diagnostic modalities such as imaging, biopsies, and specialized tests like Alpha-1 antitrypsin clearance play a crucial role in confirming the condition. Complications, including the risk of neoplasia and immunological abnormalities, require vigilant follow-up. Treatment primarily involves medical and dietary approaches, with encouraging results. Surgical resection may be considered .........