Visceral heterotaxia with polysplenia syndrome and haemorrhagic splenic infarction as a rare cause of the acute paediatric abdomen

摘要

A 9-year-old girl with right upper quadrant abdominal pain, unrelated to traum,underwent MRI. Visceral heterotaxy syndrome with a midline liver, right-sided stomach and multiple spleens was diagnosed (Fig. la). The multiple spleens were on the same side as the stomach, predominantly along the greater curve. There was a left-sided aorta and a right-sided, but uninterrupted IVC. There was no malrotation of the bowel. One of the multiple spleens showed hypointense signal on the Tl-weighted images(Fig. lb), heterogeneous hyper- and hypointense signal on the T2-weighted images (Fig. lc) and no enhancement after contrast material administration (Fig. Id); the other spleens demonstrated normal signal and contrast medium enhancement. Torsion of one of the multiple spleens with haemorrhagic infarction was diagnosed. The patient underwent urgent laparoscopy, which confirmed an infarcted spleen with a pedicle under torsion. Uncomplicated resection of the infarcted spleen was performed.