Authors: Greg Klazura, Joshua Eberhardt.
Background: Although a Duhamel Spur is a well documented phenomena. Literature on its frequency and management is limited. Furthermore, the existence of concomitant Hirschsprung’s Disease (HD) and anorectal malformations (ARM) is exceedingly rare.
Case Summary: A 29 year old male with a history of ARM, HD, and urinary stoma, presented with fatigue and body aches. CT revealed a blind-ending large pouch in the left lower quadrant. According to records from Pakistan, in the OR, the appendix, cecum, ascending colon, and transverse colon were missing as an infant. A small segment of gut ended blindly behind the bladder over the middle of the sacrum. Histology of the terminal portion of the colon revealed the absence of ganglion cells, suggesting HD. When the patient returned to the OR at one year old he likely received both a Posterior Sagittal Anorectoplasty for his ARM and a Duhamel procedure for his HD. During the operation his urethra was injured, repaired and a foley catheter was left in place for an unknown amount of time. At the age of 12 the patient was unable to evacuate his bladder and he was given a suprapubic vesicostomy.
Management: We performed a preoperative colonoscopy. Upon entering the anus there was a smaller lumen and a larger lumen. In the OR the patient was placed in a lithotomy position for cystoscopy and stent placement. Next, we performed a midline laparotomy. The mass was under such tension it virtually sprang forth from the abdomen when the fascia was opened. We then fired a stapler multiple times across the neck of the mass where it arose off of the rectum with the sigmoidoscope in place.
Outcome: Patient was able to tolerate a diet on POD 5. Urology planned for outpatient internalization of nephrostomy tubes. The patient was discharged in stable condition.
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