Successful Splenectomy for Massive Isolated Splenic Lymphangiomatosis in a Resource-Limited Centre: A Case Report.

Abstract

Background:

Isolated splenic lymphangiomatosis is a rare benign condition in which malformed lymphatic channels proliferate within the spleen without involvement of other organs. Giant forms that produce massive splenomegaly requiring complex open surgery are uncommon.

Case Presentation:

A 26-year-old woman presented with a two-year history of progressively increasing abdominal distension, early satiety, and constipation. Abdominal examination revealed a firm, lobulated mass occupying almost the entire abdominal cavity. Contrast-enhanced CT showed massive splenomegaly with multiple non-enhancing hypodense splenic cysts extending from the left upper quadrant to the pelvis, with no radiological evidence of extra-splenic involvement. After preoperative vaccination against encapsulated organisms, open splenectomy was performed via midline laparotomy using sequential caudal vascular control. The resected spleen measured 42 × 24 × 19 cm and weighed 5.4 kg. Intraoperative blood loss was approximately 100 mL and operative time was two hours. Histopathology confirmed splenic lymphangiomatosis with Gamna-Gandy bodies. The patient was discharged on postoperative day 4 and was asymptomatic at follow-up with complete resolution of abdominal distension.

Conclusion:

Open splenectomy with sequential caudal vascular control allowed safe complete resection in this patient with massive isolated splenic lymphangiomatosis. Favourable outcomes are attainable with careful preoperative planning and systematic operative technique.