Unusual occurrence of acquired hypoganglionosis following surgery for Hirschsprung's disease

Abstract

Purpose: Acquired hypoganglionosis or aganglionosis after surgery for Hirschsprung's disease is a littler described situation. To investigate and describe the mechanism of acquired aganglionosis or hypoganglionosis following sugery in the pulled-through bowel which had been previously confirmed as ganglionated. Case report: A full-term boy initially presented with polymalformatif syndrome and dwarfism. The symptoms started at birth with abdominal distension and failure to pass meconium in the first 48 hours of life which was relieved by the use of a rectal tube. At the age of 4 months he developed abdominal distention and acute bowel obstruction. He was operated on and during surgery a narrow distal recto-sigmoid transition zone was found. A diverting right transverse colostomy was done. A barium enema was done and showed a recto-sigmoid transition zone. A rectal biopsy suction was performed and showed aganglionic segment. At the age of 8 months, a Soave pull-through procedure was performed. Ganglion cells were documented in the pull-through segment. The recurrent symptom patterns of abdominal distention constipation, and failure to thrive developed. Another contrast enema was performed two years later and shows massive colonic dilation with a rectosigmoid zone of transition. A redo Soave pull-through was done. Histology concluded to a hypoganglionosis. The CD56 staining was positive confirming aganglionosis. After surgery and during follow-up the boy was in a good condition, without abdominal distention. Conclusion: The cause of acquired aganglionosis or hypoganglionosis could be secondary to destruction of neuroganglionic cells due to secondary hypoxemia. That's why this diagnosis should be considered when recurrent obstructive symptoms appear several months following pull-through for HD.

Keywords

• Hirschsprung disease
• Constipation
• Acquired
• Hypoganglionosis