Hirschsprung’s disease

Abstract

A 28-year-old, primagravida woman, gave birth to a boy at 40 gestational weeks. On delivery, the baby was found to have blood stained liquor. This was followed by non-bilious vomiting after feeds and a distended abdomen. He was transferred to NPICU at 36 hours of age due to persistent vomiting and failure to open his bowels. An initial diagnosis of septic shock necessitated the following management plan: nurse in an incubator, establish intravenous access and perform a septic screen. Nevertheless, at 50 hours of age, persistent bilious vomiting and abdominal distension persisted. An upper GI contrast study excluded a malrotation. Hirschsprung’s Disease was then suspected and confirmed on punch rectal biopsies. A surgical operation was carried out, which included a mapping laparotomy and a colostomy. Three days after the operation was performed, he developed wound dehiscence and required re-repair in theatre. The small and large intestine were cleaned and replaced in the abdomen and the stoma site secured. Swabs taken from the wound grew Enterococcus faecalis and Pseudomonas aueriginosa.