Journal of Pediatric Surgery 49 (2014) 564–569

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Megarectosigmoid in children with anorectal malformations: Long term outcome after surgical or conservative treatment Helena Borg a,⁎, Marc Bachelard b, Ulla Sillén a a b

Department of Pediatric Surgery, The Queen Silvia, Children's Hospital, The Sahlgrenska Academy at University of Gothenburg, Sweden Department of Pediatric Radiology, The Queen Silvia, Children's Hospital, The Sahlgrenska Academy at University of Gothenburg, Sweden

a r t i c l e

i n f o

Article history: Received 12 June 2013 Received in revised form 1 August 2013 Accepted 3 August 2013 Key words: Anorectal malformation Megarectosigmoid Excision of megarectum Bowel management Contrast enema Bowel functional outcome

a b s t r a c t Purpose: Megarectosigmoid (MRS) is commonly seen in children with anorectal malformations (ARM) and contributes to the high incidence of constipation. Surgical resection has been advocated by some, whereas others propose intense bowel management as the treatment of choice. The aim of this study was to evaluate outcome of both bowel function and configuration after surgical or conservative treatment of MRS in ARM patients. Materials and methods: The study included 79 patients with ARM, excluding perineal fistula, (48 boys, 31 girls) from 1986 to 2007. MRS was diagnosed at colostomy formation or contrast enema performed in the neonatal period. Early in the period, the majority of the patients were treated surgically, whereas in the late 1990s, a conservative approach with intensified bowel treatment was implemented. Contrast enema and bowel function investigations were performed repeatedly during follow-up. Results: MRS, according to radiological criteria, was diagnosed in 26/79 (33%) of the ARM children. Bowel functional outcome was similar regardless of surgical or conservative treatment and comparable to function in ARM children with non-MRS. The radiological signs of rectal dilatation and elongation disappeared after surgical intervention, but normalisation of the rectosigmoidal configuration was also seen with age in the conservative group. Conclusions: Bowel functional outcome in ARM children with MRS was similar after either surgical or conservative treatment during follow-up. The radiological signs of rectal dilatation and elongation disappeared also in the conservatively treated patients over time. © 2014 Elsevier Inc. All rights reserved.

Prognosis for bowel function in children with anorectal malformation (ARM) can be predicted early from factors such as type of fistula, spinal cord malformation and sacral anomalies. Bowel functional outcome in children with ARM has also been shown to improve with age as regards continence, soiling and constipation, but as a group, these children do not achieve the level of healthy children [1]. In addition to the above-mentioned factors, psychosocial pathology and the presence of megarectosigmoid (MRS) influence the extent of improvement. MRS contributes to chronic constipation and overflow faecal incontinence [2–6]. Wide variation is reported in the prevalence of MRS (10–50%) in ARM children, and the mechanism behind the dilated distal bowel is not fully understood [2,4,6]. The dilatation is often recognized as early as in the neonatal period, which has been related to congenital dysmotility of the distal bowel or stenosis of the ARM fistula [6–8]. Overflow from a loop or transverse colostomy early in life has also been proposed as a cause. In addition, MRS can be acquired as a result of inadequate treatment of constipation in childhood [8,9]. The treatment of MRS is intensified bowel management, whereas surgical

⁎ Corresponding author. Department of Pediatric Surgery, The Queen Silvia Children's Hospital, 416 85 Gothenburg, Sweden. E-mail address: [email protected] (H. Borg). 0022-3468/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2013.08.003

treatment with excision of the dilated bowel segment has been advocated in selected cases [4,8,10]. Before 1997, surgical treatment for MRS was in favour at our department, however, in the later era, an early- implemented bowel management strategy has dominated. The aims of this study were to report the long- term bowel functional outcome in ARM patients with surgically or conservatively treated MRS and to report on the development of the dilated bowel in the conservatively treated children vs. the children where the segment was surgically removed. In order to describe the change in radiological imaging of MRS over time certain landmarks were defined and tested. Benefits and drawbacks of the different treatment methods are also addressed.

1. Patients This material was based on a partially retrospective study of 79 children with ARM, excluding perineal fistulas, born between 1986 and 2007. All patients were treated with a diverting colostomy within 48 h of birth and the rectosigmoid configuration was frequently also documented during this operation. Anorectal reconstruction (posterior sagittal anorectoplasty, PSARP) was performed at a median age 8 months (range 3-120). For distribution of the type of fistulas see Table 1.

H. Borg et al. / Journal of Pediatric Surgery 49 (2014) 564–569 Table 1 Classification of the ARM patients included in the study according to MRS and non-MRS. Type of fistula

N

MRS surgery

MRS conservative

Non-MRS

Total Non-fistula (NF) Bulbar fistula (BF) Prostatic fistula (PRF) Bladder neck fistula (BNF) Vestibular fistula (VF) Cloaca Rectourethral fistulaUnspecified

79 8 10 25 2 27 3 4

12 1 2 4

14 1 3 4

5

6

53 6 5 17 2 16 3 4

In 67 of the 79 patients the rectum/colon was investigated with a contrast enema in the neonatal period. This was done either via an existing fistula, by a transperineal injection (guided by plain x-ray or ultrasound) or as a distal colostogram. Twenty-six out of 79 (33%) patients with ARM were diagnosed with MRS. In the early part of the period, a majority of the patients with severe constipation and MRS were offered a resection of the rectosigmoid (12 patients). In the late part of the period, on the other hand, a conservative attitude with intensified bowel treatment was implemented (14 patients). There was an overlap in treatment strategy between the early and late era in the middle of the 1990s. In the 12 surgically treated patients a rectosigmoid resection was performed at a median age of 4.7 years (1.4–6.5). One-third of the patients in each MRS group had sacral dysplasia and one patient in each group had sacral agenesis. Spinal cord malformation, tethered cord, was diagnosed in 2 patients from the late era, and in the early group one boy had syringomyelia without tethering. No sign of neurogenic bladder dysfunction (NBD) was seen during follow-up. 2. Methods 2.1. Strategy for follow-up of MRS patients, early vs. late period All patients have been followed at the clinic on a regular basis. In the early period when MRS was found, the patients were put on a contemporary bowel management therapy. If they did not improve clinically a resection of the dilated bowel segment was advocated. Radiological follow up was performed in all surgically treated cases. In the later period, contrast enema was performed routinely 6 months post stoma closure. The information from this investigation was helpful when designing an individual bowel management strategy for MRS patients [1,11]. Constipation treatment with stool softeners (osmotic drugs, or in children from 9–12 months PEG3350) were introduced in combination with assisted emptying. If microclysma was not sufficient, saline enemas (volumes 150–500 ml, 3–7 times per week) were introduced and were always accompanied by potty training with the aim to avoid emptying in a diaper. Thorough information was given to the parents, underlining the importance of adherence to the instructions. These conservatively treated patients were investigated with a follow up contrast enema at a median age 6.4 y. Two patients (1VF, 1 PRF) with previously diagnosed MRS who needed intensive bowel treatment owing to severe constipation, declined late follow up contrast enema. 2.2. Surgical method The dilated rectosigmoid surgery was performed via a low transverse incision. The peritoneum was incised and the dilated bowel mobilized below the peritoneal reflection. Special care was taken not to divide the superior haemorrhoidal branches to the distal rectum. The resection

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was performed leaving 6–9 cm of the distal rectum, which was anastomosed end-to-end with normal calibre sigmoid colon. The anastomosis was hand sewn in two layers. There were no surgical complications. Routine pathology was performed in each patient.

2.3. Radiological method All contrast enemas performed in ARM children included in the study were reviewed retrospectively by an experienced paediatric radiologist (MB) together with the paediatric surgeon (HB). In order to assess a change in configuration of the megarectum in relation to the different treatment strategies, conservative or surgical, measures of the rectum in relation to pelvic anatomical landmarks were performed according to a standardized protocol in each individual. Radiologically the normal rectum is characterized by an oval lemon shape, where the widest part is located within the pelvis. It is significantly wider than the sigmoid which, unlike the rectum, is haustrated and the rectosigmoid transition is located below the promontory/iliac crest (S2-3) and deviates from the midline. Megarectosigmoid is characterized by a cylindrical or saccular shape with widening and midline elongation above the promontory/iliac crest. The transition to normal calibre sigmoid colon is often sharp. In the present study elongation (ELO) of the rectum above S2-S3 was registered in relation to level of vertebrae. For calculation of changes in elongation, each vertebra from S5 to Th11 was given a numerical value from 1 to 12. Since the radiological examinations have been performed in individuals of different ages and sizes, it was impossible to use a fixed width at the pelvic brim as a discriminator between normal and abnormal bowel [12]. Therefore, the width of the bowel in a frontal projection was measured at different levels: above the promontory at the level of S1 (SS 1) and at the widest part of the rectum (RR 1) within the pelvic ring (Fig. 1). In MRS, SS 1 is often wider or equal to RR 1. To illustrate this relationship the sigmoid/rectal ratio (SR) was calculated (SS 1/RR 1). Bowel emptying of contrast was estimated from 1-5, where 1 was poor and 5 complete.



S R1 R Fig. 1. Radiological image of megarectosigmoid (MRS) in an ARM patient. SS1 line illustrates bowel width above promontory and RR1 line rectal width within pelvic ring, measures used for calculation of sigmoid-rectal (SR) ratio.

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Eleven children with ARM (5 girls, 6 boys) without radiological MRS, were used as controls (control group 1). The contrast enemas in the control patients were measured as described above and thus used for the evaluation of the diagnostic criteria used for the radiological diagnose of MRS.

promontory in relation to rectal width within the pelvic ring with a median 0.60 (range 0.44–0.71) in non-MRS and median 0.93 (range 0.62–1.5) in the MRS group (p b 0.0001), (Table, http://hdl.handle. net/2077/33592). Cut-off level for SR ratio differentiating megarectum from non-MRS could be identified. According to the ROC curve it was 0.64 (p = 0.0411).

2.4. Evaluation of bowel function 3.2. Surgically versus conservatively treated MRS Evaluation of bowel function was performed prospectively at 5, 10 and 15 years, in the group of patients between 1995–2007. Bowel function was evaluated using a structured questionnaire and three weeks´ registrations of bowel movements, episodes of faecal leakage and soiling [1]. The type of bowel treatment was also recorded. Bowel functional outcome was evaluated with a scoring instrument described by Rintala and Lindahl, with slight modifications [1,13,14]. The maximum functional bowel score was 14. Constipation was reported as grades 0–3 according to the Krickenbeck method [15]. The patients in the early period (1986–1994) were evaluated retrospectively from medical records. Since the patients were followed by only two surgeons (HH and HB) and one stoma/bowel therapist, the recording of grade of constipation and soiling and bowel treatment was consistent and reliable. Fourteen ARM children without MRS, evaluated concerning bowel function as mentioned above, were used as controls (control group 2) [1]. 2.5. Statistical method Continuous variables were described with median and range and categorical variables with number and percentage. For comparison between two groups Mantel-Haenszel chi-square exact test was used for ordered categorical variables and Mann–Whitney U test for continuous variables. Changes over time within group were analysed with Wilcoxon signed rank test for continuous variables and Sign test for ordered categorical variables. All significance tests were two-tailed and conducted at the 0.05 significance level. 3. Results 3.1. Radiological findings in MRS versus non-MRS ARM patients MRS was diagnosed radiologically in 26 patients. In a majority of the patients the MRS was observed as early as in the neonatal period. Elongation of the rectum was below or at the level of the S2 vertebrae in the control ARM patients without MRS, whereas the elongation was above S1 in all MRS children, with a variation between Th11-S1 (p b 0.0001), (Table, http://hdl.handle.net/2077/33592). The rectal width below S3 was similar in MRS and non-MRS patients, whereas the bowel at the promontory level was wider in MRS patients. This was shown in calculations of SR ratio, the width of the bowel at the

3.2.1. Radiological findings The diagnostic contrast enema was performed at an earlier age in the conservative group than in the surgical group (median age 1.5 y and 2.9 y) (Table 2). Elongation of the rectum in both groups was similar at the baseline examination (p = 0.6736), and reached above the promontory (S1) in all but one patient from the surgical group (Table 2, Fig. 2). Elongation at follow-up in the surgical group after median 3.2 y had normalized in all children and did not exceed the level of S2-S3. However, in the conservative group, after median 6.4 y, a decrease to normal level was found in only two thirds of the patients, and in 4 children the rectum still reached above the promontory. Difference in elongation between the baseline and the follow-up examination was significant in both groups (surgical p = 0.0005 and conservative 0.0010) (Table 2). Width of the bowel above the promontory was similar in both groups at baseline examination (p = 0.2796). Decrease in width of the bowel above the promontory post resection in the surgical group was evident and illustrated by the change in sigmoid/rectal ratio between baseline and follow-up examination from median 0.97 to 0.5 (p = 0.001). A significant but less pronounced decrease in this ratio was also seen in the conservative group from 0.92 to 0.56 (p = 0.0059). Bowel emptying improved (conservative 7/10, surgical 9/12) from baseline to follow-up and there was no significant difference between the two groups (p = 0.524). 3.2.2. Bowel function 3.2.2.1. Bowel score. There was no significant difference in bowel scoring at ages 5, 10 and 15 years between the surgically and conservatively treated MRS patients (p = 0.5535, p = 0.5900, p = 0.4562). This was also true when comparing bowel score at the same ages with the control group 2 of ARM patients without MRS (p = 0.7613, p = 0.1379, p = 0.5687) (Fig. 3). When analyzing improvement in bowel score over time in the surgically and conservatively treated MRS patients, a significant change was found from 5–15 y only in the surgical group (p = 0.0078). However, since the conservative group had a shorter follow up, we analyzed the bowel score for each patient at the last visit, although this could not be classified as a 10 or 15-year follow-up. Improvement over time in bowel score could then also be found in a larger number of patients in the conservative group between age 5

Table 2 Radiological findings in contrast enema in ARM patients with MRS, conservative vs. surgical treatment. Variable Age baseline (years) Age follow-up (years) Elongation baseline Elongation follow-up Diff elongation (Follow-up – baseline) SR ratio baseline SR ratio follow-up Diff SR ratio (Follow-up – baseline)

Conservative (n = 14) 1.5 (0.1; 8.3) n = 14 10.0 (2.8; 16.0) n = 12 7.0 (6.0; 12.0) n = 14 4.5 (3.0; 7.0) n = 12 -2.0 (-5.0; 0.0) n = 12 0.92 (0.64; 1.05) n = 14 0.56 (0.30; 1.00) n = 11 -0.20 (-0.46; 0.09) n = 11

Variables are presented as median (min; max).

p-Value within group

Surgery (n = 12)

0.0010

2.9 (1.3; 6.0) n = 12 6.7 (4.0; 12.0) n = 12 6.8 (5.0; 11.5) n = 12 3.5 (3.0; 4.0) n = 12 -3.3 (-8.5; -1.5) n = 12

0.0059

0.97 (0.62; 1.50) n = 12 0.50 (0.40; 0.64) n = 12 -0.48 (-1.00; 0.01) n = 12

p-Value within group

p-Value between groups

0.0005

0.0473 0.3120 0.6736 0.0013 0.0376

0.0010

0.2796 0.0485 0.0138

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Fig. 2. Individual values of rectal elongation from baseline to follow-up in ARM patients with MRS treated conservatively or surgically (blue dots). Only baseline value for controls (red dots).

and last follow-up at median age 10 (range 2.8–16) (p = 0.001) (Table 3, Fig. 4).

3.2.2.2. Constipation grade. At age 5, constipation grade 3 was overrepresented in both the surgical and the conservative MRS group (8/ 11 (73%) and 9/14 (64%), respectively). At age ten the number of children with constipation grade 3 was reduced in both groups (6/11 (55%) and 4/9 (44%)). Further reduction was seen at age 15 (2/11 (18%) and 1/4 (25%) (Fig. 5). Constipation grade was lower in the control group at 5 and 15 years.

3.3. Histological findings Histopathological findings in the excised rectal specimens were normal in 8/12 (67%) patients. Fibrosis was seen in 2 patients,

Fig. 3. Change in bowel score by treatment and age group in MRS and non MRS ARM patients.

muscular hypertrophy in one and in one boy there was a suspicion of intestinal neuronal dysplasia (IND). 4. Discussion In this study we have shown that ARM children with megarectosigmoid have the same bowel function outcome as they grow up, irrespective of surgical or conservative treatment of the dilated bowel. In the surgical group the radiological appearance was clearly normalised earlier, i.e. directly after resection, without evidence of late recurrence in dilatation. However, normalisation of the rectosigmoid configuration was also successively seen with age in the conservative group. The prevalence of MRS in the present study was 33%, which agrees with other studies [2,4,6]. To make a fair comparison of the results of different MRS treatment strategies in ARM patients it is important to ensure that the groups are equally well defined before treatment. One drawback of this study was that the patient groups were from different time periods, and other that part of the study was retrospective. Since criteria for making a megarectal diagnosis is not very well defined for growing individuals we made an effort to verify the megarectum on previously performed contrast enema investigations objectively, by presenting radiological criteria for the diagnosis of MRS [12]. We found that the most important discriminating variable was the elongation of the rectum above the promontory, according to comparison with contrast enemas in ARM children without MRS. The elongation of the rectum was clearly normalised directly after resection in the surgically treated group and did not re-dilate during follow-up. In the conservatively treated group the decrease in elongation to close to normal was an ongoing process as the child grew up. We also used a sigmoid-rectal ratio to illustrate the dilatation of the megarectum above the promontory, which also seemed to follow normalisation of the bowel configuration. In fact, a cut-off point was identified for the ratio to differentiate between children with and without MRS. Using the above-described criteria, we found that the two groups from different time periods were radiologically similar regarding grade of megarectum. We also established a comparable degree of

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Table 3 Change in bowel score over time with analysis of bowel score at last follow-up (FU) in each patient. Variable

Change bowel score 5–10 y Worsen Unchanged Improved Change bowel score 5 y−last FU Worsen Unchanged Improved Change bowel score 10y − last FU Worsen Unchanged Improved

Conservative (n = 14)

1 (8%) 3 (25%) 8 (67%)

0 (0%) 1 (8%) 11 (92%)

0 (0%) 6 (50%) 6 (50%)

p-Value within group

p-Value between groups

0.0391

2 (20%) 3 (30%) 5 (50%)

0.4531

0.4031

0.0010

0 (0%) 2 (2%) 8 (80%)

0.0078

0.5714

0.0313

1 (8%) 3 (25%) 8 (67%)

0.0391

1.0000

p-Value within group

Surgery (n = 12)

severe constipation in the groups before treatment, which we found to be important, since a connection between megarectum and severity of constipation has previously been reported in ARM patients [6]. The surgically treated patients were mainly from the early part of the study when bowel management was less consistently implemented as compared to the later era. Our study clearly shows that excision of the dilated rectum does not lead to normalisation of bowel function. Even when the grade of constipation decreased, there was still a need for additional bowel management throughout childhood, which was consistent with the situation for non-surgically treated MRS patients. The results of this study were comparable with the outcome after surgical management of idiopathic megarectum in chronic constipated non-ARM children who were resistant to medical treatment, except for the fact that these patients recovered normal bowel function within a shorter time period [16].

Fig. 4. Change in bowel score during follow-up in ARM patients with MRS, conservative vs. surgical treatment between 5 y and last follow-up. Dotted line represents median and solid line mean for the respective group.

Fig. 5. Number of MRS patients and controls with each constipation grade at 5, 10 and 15 years.

The conservatively treated patients were from the later era, when a firm, consistent bowel management strategy was implemented at our department. This might be one of the explanations for the similar bowel function results seen in the conservatively treated patients as compared with the surgically treated group. The improved strategy included close contact between the bowel therapist/paediatric surgeon and the child/ parent, and teaching the parents the importance of enema treatment to avoid faecal impaction. The radiological imaging of the MRS was used as a pedagogical tool to make them understand the risks associated with retention of stool and overflow soiling. In most ARM children with megarectum, dilatation of the rectosigmoid is recognized as early as the neonatal period, which was also the case in the present study [6,7]. The aetiology is unknown for this congenital form. The abnormality has been suggested to lie within the muscle wall itself, which is strengthened from a recent histological analysis of colon in patients with impaired large bowel motility [17]. Miyahara et al. indicated that colonic dysmotility in ARM may be associated with impaired maturation of enteric neurons, since improvement in faecal continence is delayed, as compared with healthy children [17]. Since MRS is characterised by pronounced hypomotility in the dilated rectosigmoid it could be hypothesized that there are more immature enteric neurons in the bowel segment. To our knowledge the immaturity question has not been addressed in the aetiology of MRS. It could be valuable to re-evaluate the resected specimens in the present study concerning grade of maturity in enteric neurons. The immaturity theory might also support the fact that megarectum may develop later in life, but this was not confirmed in our study. The type of original colostomy and postoperative complications such as anal or stoma stricture formation and inadequate bowel management may be contributory factors [4,10]. An immature bowel may be more vulnerable to distension and incomplete emptying, with permanent dilatation as a result. The early implemented bowel management therapy in the conservative group apparently prevented late rectal dilatation, as was verified by the radiological follow-up. An interesting finding from an earlier study was that none of the patients with severe spinal cord malformation causing poor anal sphincter function had MRS [1,11]. However, two patients with tethered cord but with no signs of neuropathic bowel impairment in

H. Borg et al. / Journal of Pediatric Surgery 49 (2014) 564–569

the present study had MRS. It may therefore be suspected that good anal sphincter function is required in order to develop megarectum, a theory in line with previous reports. Both Levitt et al. and Rintala et al. have shown that patients with a good prognosis for bowel control are at greater risk of having or developing MRS [6,18]. This study makes it clear that MRS is commonly seen in ARM children and is an important contributor to the high degree of constipation found in these patients. Bowel functional outcome seems to be similar after surgical and conservative treatment during followup until adolescence. In addition, long term functional outcome in non-MRS ARM children was not different. This study also confirms that not only the symptoms of MRS, but also the radiological signs of rectal dilatation and elongation disappear over time in conservatively treated patients. Therefore our recommendation is conservative treatment of MRS with consistent and intensive bowel management therapy during growing up. However, in therapy resistant severe cases surgical treatment might be considered. References [1] Borg HC, Holmdahl G, Gustavsson K, et al. Longitudinal study of bowel function in children with anorectal malformations. J Pediatr Surg 2013;48:597–606. [2] Pena A, el Behery M. Megasigmoid: a source of pseudoincontinence in children with repaired anorectal malformations. J Pediatr Surg 1993;28:199–203. [3] Powell RW, Sherman JO, Raffensperger JG. Megarectum: a rare complication of imperforate anus repair and its surgical correction by endorectal pullthrough. J Pediatr Surg 1982;17:786–95.

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[4] Levitt MA, Kant A, Pena A. The morbidity of constipation in patients with anorectal malformations. J Pediatr Surg 2010;45:1228–33. [5] Keshtgar AS, Ward HC, Richards C, et al. Outcome of excision of megarectum in children with anorectal malformation. J Pediatr Surg 2007;42:227–33. [6] Rintala R, Lindahl H, Marttinen E, et al. Constipation is a major functional complication after internal sphincter-saving posterior sagittal anorectoplasty for high and intermediate anorectal malformations. J Pediatr Surg 1993;28:1054–8. [7] Hedlund H, Borg H, Jacobsson B: Megarectosigmoid in imperforate anus. Presented at the BAPS meeting, Istanbul, Turkey, July 23-25,1997 [8] Brent L, Stephens FD. Primary rectal ectasia. A quantitative study of smooth muscle cells in normal and hypertrophied human bowel. Prog Pediatr Surg 1976;9:41–62. [9] Pena A. Anorectal malformations. Semin Pediatr Surg 1995;4:35–47. [10] Levitt MA, Pena A. Anorectal malformations. Orphanet J Rare Dis 2007;2:33. [11] Borg H, Holmdahl G, Olsson I, et al. Impact of spinal cord malformation on bladder function in children with anorectal malformations. J Pediatr Surg 2009;44:1778–85. [12] Preston DM, Lennard-Jones JE, Thomas BM. Towards a radiologic definition of idiopathic megacolon. Gastrointest Radiol 1985;10:167–9. [13] Rintala RJ, Lindahl H. Is normal bowel function possible after repair of intermediate and high anorectal malformations? J Pediatr Surg 1995;30:491–4. [14] Rintala RJ, Lindahl HG, Rasanen M. Do children with repaired low anorectal malformations have normal bowel function? J Pediatr Surg 1997;32:823–6. [15] Holschneider A, Hutson J, Pena A, et al. Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg 2005;40:1521–6. [16] Lee SL, DuBois JJ, Montes-Garces RG, et al. Surgical management of chronic unremitting constipation and fecal incontinence associated with megarectum: a preliminary report. J Pediatr Surg 2002;37:76–9. [17] Miyahara K, Kato Y, Seki T, et al. Neuronal immaturity in normoganglionic colon from cases of Hirschsprung disease, anorectal malformation, and idiopathic constipation. J Pediatr Surg 2009;44:2364–8. [18] Levitt MA, Pena A. Outcomes from the correction of anorectal malformations. Curr Opin Pediatr 2005;1:394–401.

Megarectosigmoid in children with anorectal malformations: long term outcome after surgical or conservative treatment.

Megarectosigmoid (MRS) is commonly seen in children with anorectal malformations (ARM) and contributes to the high incidence of constipation. Surgical...
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