Splenic torsion of a wandering spleen and polysplenia syndrome in a 10-year-old boy with cyanotic congenital heart disease: A case report
Year: 2026
Type: article
Study design: Case Report
Abstract: Introduction Splenic torsion is an unusual cause of acute abdomen in children, usually occurring in a wandering spleen. Its association with polysplenia has rarely been reported. Case Presentation We report a 10-year-old boy with a complex cyanotic congenital heart disease who presented with one week of left upper quadrant abdominal pain and non-bilious vomiting. He had previously undergone open heart surgery (a Glenn procedure) at two years of age for a hypoplastic left ventricle with double outlet right ventricle. Examination at the time of presentation, revealed central and peripheral cyanosis, digital clubbing, and left hypochondrial tenderness. Ultrasound demonstrated a normal spleen with a separate accessory splenic tissue (splenunculus) at its hilum and a bulky pancreas. Contrast-enhanced computed tomography of the abdomen showed tortuous splenic vessels, a seperate splenunculus at splenic hilum, and a previously undiscovered double inferior vena cava, raising suspicion for underlying heterotaxy syndrome. At laparotomy, a wandering spleen (with the splenunculus at its hilum) was found to be in torsion on its own axis through two complete rotations. Ligamentous attachments were absent and the spleen failed to regain perfusion after detorsion; splenectomy was therefore performed. Incidental intestinal malrotation with a narrow mesentery and mobile caecum was identified and treated with a Ladd’s procedure and appendectomy. The patient remained ventilated in ICU for 2 days and was discharged uneventfully on postoperative day 6. He received post-splenectomy vaccination 2 weeks later. Conclusion Splenic torsion due to wandering spleen should be included in the differential diagnosis of children with congenital heart disease who develop acute abdominal pain.
Institution: Pakistan Army
Language: English
Subfield: Cardiology and Cardiovascular Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $1,130
OpenAlex ID: W7204654647