Giant intra-abdominal lymphatic malformation arising from the cisterna chyli-thoracic duct region in a five-year-old boy: a case report

Abstract

Lymphatic malformations are rare congenital vascular anomalies, and intra-abdominal involvement is uncommon. We report a 5-year-old boy with progressive abdominal distension for more than 6 months and intermittent abdominal pain. Ultrasonography and magnetic resonance imaging demonstrated a giant multiloculated cystic lesion measuring approximately 21 × 13 × 23 cm, occupying most of the abdominal cavity and markedly displacing the abdominal viscera. Because of the lesion size and severely restricted intra-abdominal working space, open surgical excision was performed. Intraoperatively, part of the malformation arose from the cisterna chyli-thoracic duct region and contained inspissated chylous material. Complete excision was achieved without bowel resection. Histopathology and immunohistochemistry confirmed cystic lymphangioma, while a separately submitted lymphatic structure was compatible with the thoracic duct. Recovery was uneventful, with no recurrence during 3 years of follow-up.