Gastric volvulus and wandering spleen in Pitt-Hopkins syndrome: first paediatric case report.

Background: Acute gastric volvulus is a rare condition in children, and delayed diagnosis may lead to gastric ischemia, perforation, or even death. It is sometimes associated with a wandering spleen, a condition in which the spleen migrates from its normal anatomical position due to the absence of fixation ligaments. We report the first known case of a patient with Pitt-Hopkins syndrome (PTHS) presenting with simultaneous acute gastric volvulus and a wandering spleen. Case report: A 6-year-old male with PTHS was urgently referred for acute abdominal pain and a 24 h history of non-bilious and non-bloody emesis. The x-ray showed a massive gastric dilatation, and an upper gastrointestinal series (UGI) revealed a gastric outlet obstruction. An emergency laparoscopy revealed a gastric mesoaxial volvulus with a hypotonic wall with no sign of ischemia or perforation associated with a wandering spleen. Gastropexy was performed by anchoring the gastric greater curvature to the anterior abdominal wall, covering the spleen in a good position in the left upper abdomen, completely covered by the gastric fundus. The patient made an uneventful recovery and was completely asymptomatic. Conclusion: To our knowledge, this is the first reported case of simultaneous gastric volvulus and wandering spleen in a patient with PTHS. Laparoscopic gastropexy is a straightforward and effective procedure that combines the advantages of previously described surgical techniques.