Treatment of a congenital diaphragmatic hernia with associated wandering spleen: Case report of a 17-year-old girl

INTRODUCTION: A congenital diaphragmatic hernia (CDH) is rarely diagnosed in adults and can allow passage of abdominal viscera into the chest cavity. A particularly rare association is a wandering spleen due to absence of its diaphragmatic and retroperitoneal attachment which predisposes to elongation of the vascular pedicle with risk of torsion, infarction and rupture. PRESENTATION OF CASE: A 17-year-old girl presented with a two-day history of increasing abdominal pain. Examination identified an abdominal mass. Computer tomography (CT) chest, abdomen and pelvis revealed a significantly enlarged wandering spleen with signs of torsion and an associated large left CDH with viscera in the chest cavity. The patient proceeded to an open splenectomy and repair of CDH. Post-operatively the patient developed ileus and required a temporary chest tube for pneumothorax, but otherwise progressed well. DISCUSSION: Untreated CDH with a symptomatic wandering spleen is an extremely rare diagnosis with only one similar previous case report. Clinical detection is unlikely, making CT scanning the diagnostic test of choice. Surgery is recommended given the high morbidity and mortality of associated complications of both conditions. Splenic preserving options are favoured, however the majority of identified cases require splenectomy because of associated torsion or splenomegaly. Reduction of the CDH should be performed with primary closure of the defect and mesh reinforcement where possible. CONCLUSION: CDH with associated wandering spleen in adults presents an extremely rare but clinically important diagnosis. Prompt surgical management as reported in this case should be performed to minimise immediate and future complications.