Splenic cystic lymphangiomatosis in association with omental varices and portal hypertension

Abstract

Rational:Lymphangiomatosis is rare and benign, and slowly proliferating lymphatic vessels of unknown etiology and visceral lymphangiomatosis involving the spleen is rare. Since lymphangiomatosis may be asymptomatic or present as a sense of fullness, splenic cystic lymphangiomatosis is a disease of little concern.Patient concerns:A 34-year-old woman suffering from progressive epigastric fullness after oral intake for two weeks.Diagnoses:Physical examination showed a palpable mass which was more than 10 cm in size over the left hypochondrium. An abdominal computed tomography disclosed marked splenomegaly with multiple cystic lesions in the spleen, causing external compression with right-sided deviation of the adjacent organs and varices in the upper abdomen. Esophagogastroduodenoscopy revealed portal hypertensive gastropathy.Interventions:Conventional total splenectomy was performed in this patient because of an enlarged spleen and unknown etiology, preoperatively. Upon surgery, splenomegaly with polycystic content and varicose vessels over the omentum were noted. Autologous spleen transplantation was not performed because of limited orthotopic and vascularized spleen.Outcomes:The patient is doing well 18 months after splenectomy.Lessons:This was a rare case presenting with splenic cystic lymphangiomatosis in association with omental varices and portal hypertension. Splenic cystic lymphangiomatosis should be considered in the differential diagnosis of patients with a palpable painless mass over the left hypochondrium.

Visceral lymphangiomatosis of the spleen and adrenal gland: a rare presentation

“…Portal venous hypertension and omental varices are some known associations with systemic lymphangiomatosis. 5…”

“…Portal venous hypertension and omental varices are some known associations with systemic lymphangiomatosis. 5 Management options for visceral lymphangiomatosis involving the spleen include therapeutic embolisation for focal involvement. While laparoscopic splenectomy is considered as the procedure of choice for diffuse involvement with normal or moderately enlarged spleens, open splenectomy is preferred in cases with gross splenomegaly.…”