Reply to Dr. Chung and colleagues

We thank Dr. Chung and colleagues for their comments on our study [1]. The following are our responses to the queries raised.

We agree that infancy is the best opportunity to treat Hirschsprung’s disease (HSCR); however, in most third world countries, HSCR symptoms are frequently missed during infancy, although delayed diagnosis does not translate to poorer functional outcomes [2]. The patients in our study likely had ignored or misdiagnosed symptoms previously. Despite the delay, our results demonstrate excellent functional outcomes. The median age of patients in our study was 4 years and not 7.5 years as stated by the authors (Table  1 s) [1].

The diagnosis of HSCR is based on three cardinal features: history, barium enema, and rectal biopsies confirming the absence of ganglia by immunohistochemistry [2] [3]. All three parameters, and anorectal manometry in addition, were used in our study. The relevant clinical findings were also detailed, though briefly owing to editorial constraints on the number of written characters. HSCR was independently confirmed clinically and histopathologically by respective experts (V.B., a pediatric gastroenterologist; S.B., a gastrointestinal pathologist).

Treatment of ultrashort-segment HSCR with surgical myotomy/myectomy has demonstrated good long-term outcomes and can be the procedure of choice for older children [4] [5]. Per-rectal endoscopic myotomy (PREM) eliminates the limitation of surgical myotomy length. Myotomy relieves functional obstruction, and the proximal ganglionated colon can initiate good peristalsis (except where dilated and flaccid, when surgical outcomes are also likely to be suboptimal).

Our study was retrospective; therefore, outcomes reporting was limited by the availability of data. However, the reported outcomes (bowel movement frequency, laxative requirement, and presence of soiling) form important components of standardized functional bowel scores. The absence of incontinence and enterocolitis highlights excellent restoration of bowel function post-PREM and may be a distinct advantage over surgical pull-through. Although the follow-up is limited, most adverse events or recurrences are likely to manifest within the time period of our study.

In conclusion, we urge the authors to consider PREM as a significantly less morbid alternative, with optimal functional outcomes compared with surgical pull-through. We agree that further research is required on this subject.

Publikationsverlauf

Artikel online veröffentlicht:
21. April 2022

• References

• 1 Bapaye A, Dashatwar P, Biradar V. et al. Initial experience with per-rectal endoscopic myotomy for Hirschsprung's disease: medium and long term outcomes of the first case series of a novel third-space endoscopy procedure. Endoscopy 2021; 53: 1256-1260
• 2 Sharma S, Gupta DK. Hirschsprung's disease presenting beyond infancy: surgical options and postoperative outcome. Pediatr Surg Int 2012; 28: 5-8
• 3 Knowles CH, De Giorgio R, Kapur RP. et al. The London Classification of gastrointestinal neuromuscular pathology: report on behalf of the Gastro 2009 International Working Group. Gut 2010; 59: 882-887
• 4 Shehata SM, El-Banna IA, Gaber AA. et al. Long-term evaluation of modified lateral anorectal myomectomy for low-segment Hirschsprung disease. Arch Surg 1998; 133: 269-271
• 5 Sawin R, Hatch E, Schaller R. et al. Limited surgery for lower-segment Hirschsprung's disease. Arch Surg 1994; 129: 920-924 discussion 924-925