Jejunal lymphangioma causing intussusception in an adult: An unusual case with review of the literature.

Introduction: Adult intussusception is rare, and 90% are due to a lead point secondary to a pathologic condition. Lymphangioma is an uncommon tumor of the lymphatic system and is rarely found within the small bowel. Small bowel lymphangioma causing intussuception in an adult is a rare occurrence, with three very distinct rare pathologies occurring simultaneously.

Case description: A 70-year-old male patient with multiple pre-existing pathologies such as advanced ADPKD, multiple persistent tubulovillous colon polyps and colon cancer in situ, was hospitalized due to rapid weight loss of 20 lbs, hematemesis, and abdominal pain. He was subsequently found to have jejunal intussusception caused by two lymphangiomas of the small bowel. The portion of intussuscepted jejunum was resected and final diagnosis on pathology was two jejunal lymphangiomas.

Discussion: Lymphangiomas of the small bowel are rare, but increasing in incidence due to the accessibility of endoscopic evaluations. A hypothetical connection between lymphangioma and ADPKD is unknown, but both diseases are built on a foundation of cystogenesis. There is little known about the effect ADPKD on cystogenesis and tumor formation extra-renally, and there is a possible correlation between genetic mutations in polycystin and cystic tumors such as lymphangiomas.

Conclusion: Lymphangioma, although rare in the small bowel, is a possible cause of intussusception and should be considered on the differential of abdominal pain in adults. The pathogenesis of polycystic kidney disease has implications that could predispose to cystic development beyond the kidney, and more research into the genetic mechanism behind the disease is necessary to support or deny this claim.