Management of children afflicted with hirschsprung’s disease: a narrative review

Abstract

Introduction: Hirschsprung's disease is a genetic anomaly with an incidence of 1 in 5000 births, predominantly affecting females at a ratio of 1:4. It is characterized by the absence of ganglion cells in the enteric nervous system in the distal segment of the gastrointestinal tract. Approximately 90% of individuals with Hirschsprung's disease present with symptoms in the neonatal period. Classic symptoms include abdominal distension (>90%), vomiting (>85%), which can be bilious, and the absence of meconium passage in the first 24 hours of life (>60%). Diagnosis is established through biopsies (gold standard), contrast enema, and anorectal manometry. While awaiting the results of rectal biopsy, saline rectal irrigations should be initiated one to three times daily to achieve functional bowel obstruction relief and enable enteral feeding until the pull-through operation. There are many surgical approaches for Hirschsprung's disease, including transabdominal approach (TAB) and transanal pull-through (TERPT). TAB mainly includes Swenson, Duhamel, Duhamel and Soave techniques. Swenson involves the removal of the affected area entirely with a termino-terminal anastomosis to the anal canal. In Duhamel, the normal colon is usually brought through the posterior rectal space and anastomosed with the contralateral rectum. In Soave, saline is injected into the rectum after circularly cutting the rectal muscular layer, leaving the mucosa intact at the level of the dentate line. Later, De La Torre-Mondragon proposed a new treatment called single-stage TERPT, a minimally invasive surgery with an anal approach that eliminates abdominal adhesions and pelvic nerve injury. The most commonly performed operation is the Duhamel procedure. The benefits of Duhamel include decreased stool frequency and increased consistency, but they must be balanced with the risk of pouch dilation and stool stasis. TERPT is superior to TAB in terms of hospital stay and constipation. However, there are still a large number of randomized studies to be verified, and more trials are expected to be conducted in the future to determine the best surgery. Among the postoperative complications are enterocolitis, dermatitis, fecal incontinence, and obstruction, with enterocolitis having the highest morbidity and mortality. Alternative treatments, although lacking evidence to prove their efficacy, include botulinum toxin, probiotics, and laser therapy. Objective: To synthesize the main and most up-to-date knowledge on the management of children with Hirschsprung's disease, from diagnosis to definitive treatment. Methods: This study is a narrative review. To carry out this work, systematic reviews, meta-analyses, and cohort studies published in the last five years (2018 to 2022) were searched in the PUBMED scientific database. The keywords used were "Hirschsprung disease" and "Surgical treatment." Sixteen articles were found in the database.