Long-term functional outcomes of patients with Hirschsprung disease following pull-through

Hirschsprung Disease (HSCR) is one of the most common congenital anomalies, characterized by failure of neural crest migration in the colon causing aganglionosis starting in the distal part of the colon, resulting in functional obstruction [1]. It is estimated that the incidence is 1:5,000 live birth and more commonly in males by 4 to 1 compared to females [1]. A previous study showed a higher incidence in Indonesia, which is 1:3,250 live birth [2].

A definitive treatment for HSCR is pull-through surgery [3]. Several pull-through surgeries are transabdominal endorectal (Soave), Swenson, Duhamel, transanal endorectal pull-through (TEPT), and laparoscopic (Georgeson) approach. Several studies have reported the functional outcomes of HSCR patients following pull-through [4‐8]. However, the studies that compare long-term functional outcomes from these different surgical approaches are very limited [9]. Long-term outcomes are defined by the patient’s ability to control defecation, social function, and quality of life [10]. One of the methods used to measure bowel function as a long term HSCR outcome is the bowel function score (BFS) [11]. Therefore, we aimed to describe the long-term outcomes of patients with HSCR who underwent transabdominal Soave, Duhamel, and TEPT in our institution.

Here, we show that most of our patients have good long-term functional bowel outcomes following transabdominal and transanal pull-through surgeries. Previously, we determined the functional outcomes after several definitive surgeries [5, 8]. However, there are some novelties in this study: 1) we determined the long-term outcomes in three different techniques (vs. two procedures [5] vs. one method [8]); 2) we included the transabdominal and transanal approaches (vs. two transabdominal procedures [5]), and 3) at least three years' follow-up after pull-through (vs. no defined time of follow-up [5, 8]). Our study provided another report of the functional bowel outcomes between three different pull-through techniques from a different population, i.e., Indonesia (vs. a Western country [9]). Another strength of our study is a prospective design and the length of follow-up of at least three years after definitive surgery. Several studies reported the long-term functional outcomes, mostly from Western countries [16‐20]. Our study provided an additional critical report on the long-term bowel function from a South-East Asian country, i.e., Indonesia. A recent study showed that although impairment of functional outcomes is common after surgery, intestinal function was improved following the patient's age [17]. In contrast, other studies reported that the impairment of bowel function does not change with increasing age [7, 18]. They suggested that continuous follow-up and management is essential for these patients’ groups [18].

In our study, patients who underwent transabdominal Duhamel and Soave procedures might have a poorer functional bowel outcome than patients who underwent TEPT (Table 2). There are several advantages of TEPT over transabdominal procedures, including its minimally invasive approach, better cosmetic result, and avoidance of abdominal contamination [21]. Moreover, there is a recognized heterogeneity of operative time and hospital stay, implying variation of skills of the surgeons and postoperative care in associated hospitals [22]. Long-term functional outcomes of HSCR patients who underwent pull-through surgery vary and are deemed due to the operator's experiences. Thus, preferences on the choices of the pull-through technique rely significantly on the operator [23].

Interestingly, our study also reveals that type of definitive procedures might influence the incidence of soiling and accidents (Table 3). There is still conflicting evidence on which procedure is associated with soiling. Soiling in postoperative HSCR patients is due to damaged sensation and sphincter mechanism in primary repair or affected colonic motility after resection of rectosigmoid, the fecal reservoir. These can be seen with damaged/absence of anal canal and/or sphincters due to improper surgical technique in both approaches, either transabdominal or transanal [24]. Fecal incontinence risk could be reduced by creating anastomoses higher than the dentate line [25]. This fecal incontinence could improve over time, but some patients with more severe symptoms require redoing the pull-through [26].

In this study, we used the BFS, not the Krickenbeck classification, to determine the long-term outcomes of patients with HSCR after pull-through. While the Krickenbeck classification is easily applied in clinical practice [2, 5, 8], it is originally designed for patients with anorectal malformation (ARM). It should be noted that there are some differences between HSCR and ARM. Patients with HSCR show normal anal canal and sphincter and usually do not reveal the spinal cord and vertebrae anomalies [5].

Some weaknesses of our study are noted, such as 1) small sample size; 2) an unequal number of patients between three procedures; and 3) we only determined the functional bowel outcomes and the type of pull-through surgery according to overall means without considering other factors that might affect the findings, including sex, degree of aganglionosis, surgeons’ skills, age at HSCR diagnosis, age at definitive surgery, and coexisting dysganglionoses. In addition, a recent study showed that age at definitive surgery, i.e., neonatal vs. delayed primary pull-through, did not affect the functional bowel outcomes [16].

Our study shows that the type of definitive surgery might affect the long-term bowel functional outcome; particularly, the TEPT approach might have some advantages over the transabdominal Soave and Duhamel procedures.