A Rare Case of Ectopic Splenic Transplantation in a 16 Year Old with Hereditary Spherocytosis

Background: Hereditary Spherocytosis is a well known cause of hemolytic anemia. Splenectomy is indicated in patients who are on chronic transfusions to decrease the ongoing hemolysis. Transplantation of ectopic splenic tissue after splenectomy, although rare, is possible. There has been 1 such documented case report in the adult population, but none in the pediatric population. Clinical symptoms are uncommon, however, can include vague abdominal pain, nausea, vomiting, and organomegaly. Given its proximity to surrounding structures and organs, extra splenic tissue is often mistaken as gallstones, pancreatic masses, lymphomas, neuroendocrine tumors, and even hepatocellular carcinoma. It is understood that in the presence of trauma, ectopic splenic tissue is transplanted into the surrounding parenchyma, thereby allowing its regeneration. However, the etiology of ectopic splenic tissue regeneration in the absence of trauma, such as in this case, has never been reported.

Objective: We report a 16 year old female with a history of hereditary spherocytosis s/p splenectomy who presented with 2 years of intermittent, dull, non radiating RUQ abdominal and midsternal pain. RUQ ultrasound revealed multiple progressively increasing masses in the hepatic and biliary regions. Further characterization on MRI of the abdomen and pelvis revealed 4 large echogenic hepatic lesions and multiple small echogenic lesions in the gallbladder concerning for hepatocellular carcinoma. Core needle biopsy of the lesion was indicated.

Design/Methods: Chart review was performed through the electronic medical record to obtain information about the patient and case.

Results: Core needle biopsy of the hepatic lesion was consistent with spleen tissue; no hepatocytes present; no evidence of carcinoma. The findings are consistent with splenosis or overgrowth of ectopic spleen tissue. A scintigraphy with sensitive heat-denatured technetium-99m-labeled red blood cells is recommended to evaluate other hepatic masses.

Conclusion(s): Transplantation of ectopic splenic tissue in a patient who has undergone total splenectomy is an extremely rare finding in pediatric patients.

No relevant conflicts of interest to declare.