Abstract
Modern approach to the surgical treatment of Hirschsprung's disease (HD) consists in the earliest possible repair and reduction of the number of surgical interventions. Primary one-stage transanal endorectal pull-through (TEPT) technique requires preoperative determination of the length of aganglionic segment. The efficacy of the standard method - contrast enema - is questionable in patients with a poorly defined transitional zone. To present the proposed laparoscopic method for the management pathway in patients with HD, in whom the determination of the length of aganglionic segment with contrast enema was not possible. A retrospective analysis of the diagnostic and therapeutic management employed in 14 patients, from 2 weeks to 55 months of age, with diagnosed HD, treated between January 2013 and May 2020. Laparoscopic histological mapping was performed with the use of 3 laparoscopic ports of 3-5 mm diameter. In all patients, laparoscopic mapping allowed for the determination of the length of aganglionic segment and the mode of surgical treatment. Four children with determined short-segment disease underwent TEPT, while 2 underwent temporary colostomy formation using the Duhamel-Martin-Ikeda method. Five patients with long-segment HD underwent laparoscopic-assisted TEPT. One patient with long-segment disease was treated with a temporary double-barrel colostomy and definitive surgery was performed 3 months later using the Duhamel-Martin-Ikeda method. In 2 patients with an initial diagnosis of HD established using current diagnostic pathway, HD pathology was later excluded based on the results of laparoscopic mapping and repeat rectal suction biopsy. No complications related to the laparoscopic procedure were identified. The method of laparoscopic mapping is effective in the determination of the length of aganglionic segment in children with diagnosed HD. In doubtful cases, it can be the preferred option in establishing the final mode of surgical treatment.
Highlights
Modern approach to the surgical treatment of Hirschsprung’s disease (HD) consists in the earliest possible repair and reduction of the number of surgical interventions
Laparoscopic mapping allowed for the determination of the length of aganglionic segment and the mode of surgical treatment
In 2 patients with an initial diagnosis of HD established using current diagnostic pathway, HD pathology was later excluded based on the results of laparoscopic mapping and repeat rectal suction biopsy
Summary
Modern approach to the surgical treatment of Hirschsprung’s disease (HD) consists in the earliest possible repair and reduction of the number of surgical interventions. The efficacy of the standard method – contrast enema – is questionable in patients with a poorly defined transitional zone. The modern approach to the definite surgical treatment of Hirschsprung’s disease (HD) focuses on the earliest possible repair, and reducing the number of surgical interventions. The efficacy and sensitivity of the standard method used for this purpose – contrast enema – is questionable, especially in the group of patients in whom, because of an early presentation of the disease, bowel distension above the transitional zone is not well demarcated. The lack of visible transitional zone in older children with confirmed HD does not necessarily represent long-segment disease.[6,7] The lack of precise determination of the length of aganglionic segment before operation using the TEPT technique can result in an unwanted intraoperative change of therapeutic strategy
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