2019 Volume 55 Issue 1 Pages 89-94
The patient was diagnosed as having extensive aganglionosis and was therefore subjected to jejunostomy and managed by home parenteral nutrition (HPN). He admitted owing to the onset of fever associated with a bloodstream infection at 2 years and 6 months. Thereafter, he suddenly became pale and demonstrated both tachycardia and hypotension. Ascites were identified on ultrasound examination and computed tomography, which revealed a splenic rupture. We successfully performed splenic artery embolization. However, since there was an increase in the size of subcapsular hematoma, we performed splenectomy 8 days later. After that, the patient demonstrated a good clinical course and was finally discharged on postoperation day 32. The patient was readmitted 3 months later owing to oliguria and lifelessness. We suspected overwhelming postsplenectomy infection (OPSI) and started to treat the patient immediately, but he unfortunately died in a month. The occurrence of a nontraumatic splenic rupture with extensive aganglionosis is very rare, and therefore, we have herein report the findings of this case.
