Wandering spleen in a Cornelia De Lange young woman: a case report

this is the first reported case of splenic volvulus (SV) due to Wandering Spleen (WS) in a patient with Cornelia de Lange syndrome (CdLs). The aim of this paper is to share our experience regarding the diagnostic and therapeutic management of this rare condition. an 18-year-old young woman affected by CdLS (NIPBL mutation), previously treated for ileal volvulus due to intestinal malrotation, was referred to our institution with vomiting, diarrhoea and suspected abdominal pain. Urgent abdominal ultrasound and computed tomography (CT) revealed splenic volvulus. She underwent emergency laparotomy which confirmed splenic voluvlus associated with a wandering spleen; the splenic hilum was derotated and splenopexy was performed through the placement of a tailored polypropylene surgical mesh. The postoperative course was regular: analgesics were administered for 5 days and antibiotic therapy for 7 days; she was discharged on postoperative day 7 in good general condition after abdominal ultrasound examination. Follow up was uneventfull: postoperative ultrasounds performed 1 and 6 months after surgery showed a spleen of normal volume and morphology with preserved vascular pattern. splenic volvulus should be always included in the differential diagnosis of abdominal pain in patients with CdLs in whom diagnosis and management may be complicated by neurological impairment, low compliance and non-specific symptoms.