Wandering Spleen in a Patient With Significant Medical History

Abstract

The clinical presentation of a wandering spleen is characterized mainly by unspecific acute symptoms, ranging from diffuse abdominal pain to left upper/lower quadrant and referred shoulder pain to asymptomatic. This has challenged accelerated medical care and impeded the acquisition of confirmatory diagnosis; therefore, increasing morbidity and mortality risks. Splenectomy is an established operative procedure for a wandering spleen. However, there has not been enough literature emphasizing the clinical history of congenital malformations and surgical corrections as inferential tools for facilitating a decisive and informed procedure. The case presented is of a 22-year-old female who reported to the emergency department with a five-day persistent left upper quadrant and left lower quadrant (LLQ) abdominal pain, associated with nausea. According to the medical history, the patient had a significant history of vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL) associated with congenital anomalies. By the age of eight years, the patient had undergone multiple surgical interventions, including tetralogy of Fallot repair, an imperforate anal repair with rectal pull-through, Malone antegrade continence enema (MACE), and bowel vaginoplasty. Computed tomography imaging of the abdomen revealed evidence of a wandering spleen in the LLQ with associated torsion of the splenic vasculature (whirl sign). Intra-operatively, appendicostomy was identified extending from the cecum in a near mid-line position, to the umbilicus, and carefully incised distally, preventing injury to the appendicostomy. The spleen was identified in the pelvis, and the individual vessels were clamped, divided, and ligated. Blood loss was minimal with no post-operative complications. This rare case report adds valuable teaching points about the treatment of wandering spleen in individuals with VACTERL anomalies.

Keywords: appendicostomy; malone antegrade continence enema (mace); post-splenectomy vaccines; vacterl; vacterl association; wandering spleen; whirl sign.