How to manage a late diagnosed Hirschsprung′s disease

Abstract

Background:How to manage a late diagnosed Hirschsprung's disease (HD) and how to avoid calibre discrepancy?Subjects and Methods:A retrospective study of all patients diagnosed with HD over 2 years in our hospital from January 2009 to December 2012. Data were analysed for clinical presentations, investigations, surgical procedures and post-operative outcome.Results:Fifteen patients, operated by one single surgeon, were included in this study. The mean age was 6 years (2-16 years). Patients had an ultra-short segment type in 4 cases, rectosigmoid type in 9 cases and descending colonic aganglionosis in 2 cases. Rectal wash out was effective in 12 patients. A blowhole transverse colostomy was performed in 2 patients. Twelve patients underwent one single stage endorectal pull-through. Anastomosis incongruence was avoided by a plication procedure never described before. The assessment of post-operative outcomes by the paediatric incontinence and constipation scoring system revealed a normal continence function in all our patients, but 3 patients suffered from soiling secondary to constipation.Conclusion:One single stage pull-through can be safe and effective in children with late diagnosed HD. Routine rectal washout is a good way to prepare the colon. In some cases, blowhole colostomy can be an option. Anastomosis incongruence is a challenge; we describe a plication procedure to avoid it.

Transanal-only Swenson-like pull-through for late diagnosed Hirschsprung disease

“…Challenges with the transanal-only approach occur when there is a large amount of proximal colonic dilation, and therefore, in this instance, we recommend a laparoscopic approach for intraoperative mobilization of the descending and proximal colonic mobilization off the left retroperitoneum [9]. If at the time of anastomosis of the dilated ganglionated bowel, anal caliber size discrepancy is encountered a trick can be employed by placing four quadrant sutures and then bisecting each quadrant again and again with interrupted sutures [10].…”

Late Diagnosis of Hirschsprung's Disease: Definition and Implication on Core Outcomes

“…Diagnostic delay is not uncommon and can be multifactorial, both in the developing and developed countries. 5,6 The incidence of delayed diagnosis is between 17 and 37%. 6 It can also happen in both short and long segment HD.…”

Urinary Incontinence Secondary to Fecal Impaction in a Patient With Likely Undiagnosed Hirschsprung’s Disease

“…Patients with delayed onset of Hirschsprung’s disease have had bowels that were able to compensate, though up to a certain point. Additionally, there is usually a history of delayed passage of meconium in patients with Hirschsprung’s diagnosed after infancy [ 3 , 7 ].…”

“…About 80% of patient's Hirschsprung's disease are diagnosed in the first few months of life, with up to 90% failing to pass meconium before 24 hours of life [4,5]. Those who present later in life often have a prolonged history of constipation that is managed by the pediatrician with laxatives and stool softeners [1,[5][6][7][8]. We present the case of an eight-year-old male with chronic constipation since birth who presented to the emergency department with signs concerning spinal cord compression.…”