Misty mesentery, ascites, and bowel wall thickening in a child: Diagnostic clue for intestinal lymphangiectasia

Primary intestinal lymphangiectasia (PIL) is a rare congenital disorder characterized by lymphatic system obstruction, resulting in the leakage of lymph into the bowel lumen. We present the case of a 6-year-old boy with recurrent diarrhea and weight loss. On examination, bilateral pitting edema in the lower limbs was observed. Laboratory investigations revealed hypoalbuminemia and lymphopenia. Contrast-enhanced CT of the abdomen showed thickening of the jejunum, echogenic fat islands, and enlarged lymph nodes in the mesentery. The diagnosis was confirmed by endoscopic biopsy. The patient was managed with a high-protein diet and replacement of the long-chain triglycerides with medium-chain triglycerides. Gradual improvement in symptoms was observed with regular follow-up. PIL is a protein-losing enteropathy that causes hypoproteinemia, hypolymphopenia, and hypoglobulinemia. PIL usually presents with peripheral edema, weight loss, abdominal pain, and chronic diarrhea. Diagnosis is based on characteristic endoscopic and histopathologic findings. Management involves a multidisciplinary approach, including nutritional modifications, medical therapy, and, in rare cases, surgical resection. PIL remains a challenging diagnosis due to its nonspecific clinical presentation. Clinicians should maintain awareness of this disorder for prompt identification and management.