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 Table of Contents  
Year : 2017  |  Volume : 50  |  Issue : 1  |  Page : 40-43

Intractable constipation in an adult with megarectosigmoid following repair of low-type anorectal malformation

1 Department of Surgery, Division of Pediatric Surgery, Mackay Memorial Hospital, Taipei, Taiwan
2 Department of Pediatric Surgery, Changhua Christian Hospital, Changhua, New Taipei City, Taiwan
3 Department of Surgery, Division of Pediatric Surgery, Mackay Memorial Hospital, Taipei; Department of Medicine, Mackay Medical College, New Taipei City, Taiwan
4 Department of Surgery, Division of Colorectal Surgery, Mackay Memorial Hospital, Taipei, Taiwan

Date of Web Publication28-Feb-2017

Correspondence Address:
Chin-Hung Wei
No. 92, Section 2, Zhongshan North Road, Zhongshan District, Taipei City 10449
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/fjs.fjs_4_17

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A 55-year-old male patient received an anoplasty at infancy for low-type anorectal malformation, and intractable constipation was observed since. The patient presented with colitis at the emergency department. Computed tomography revealed feces with a megarectosigmoid of diameter 18 cm. Hirschsprung disease was suspected on the basis of the imaging findings. Therefore, a rectal biopsy was performed, which indicated the presence of normal ganglion cells. Bowel management was implemented with one large-volume enema to empty the huge rectal pouch daily. The patient was free of constipation and had an improved nutritional status with appropriate weight gain in 3 months. A laparoscopic Swenson pull-through was performed. The hospital course was uneventful. Enemas, loperamide, and water-soluble fibers were required for frequent soiling because of postoperative hypermotile colon. The treatments were weaned off gradually. At the latest follow-up (2 years postoperatively), the patient had regular voluntary bowel movements with complete bowel control.

Keywords: Anorectal malformations, bowel management, constipation, megarectosigmoid, laparoscopy, Swenson pull-through

How to cite this article:
Hsu YJ, Fu YW, Wei CH, Chen MJ. Intractable constipation in an adult with megarectosigmoid following repair of low-type anorectal malformation. Formos J Surg 2017;50:40-3

How to cite this URL:
Hsu YJ, Fu YW, Wei CH, Chen MJ. Intractable constipation in an adult with megarectosigmoid following repair of low-type anorectal malformation. Formos J Surg [serial online] 2017 [cited 2021 Sep 24];50:40-3. Available from: https://www.e-fjs.org/text.asp?2017/50/1/40/201183

  Introduction Top

Constipation after anorectal malformation (ARM) repairs is common, particularly in low-type ARM with good prognosis for bowel control. Long-term fecal impaction can severely dilate the bowel, leading to megarectosigmoids. A recent study reported that bowel management and surgical resection of the megarectosigmoid have comparable results with regard to the bowel function and colonic size at 15 years of age.[1] However, only a few studies have reported the state of patients with megarectosigmoids during adulthood.

In this case report, we describe an adult patient with low-type ARM who experienced intractable constipation since infanthood and underwent a surgical operation for megarectosigmoid.

  Case Report Top

A 55-year-old male patient received an anoplasty at infancy for low-type ARM, and intractable constipation was observed since. The patient presented with intermittent diffuse abdominal pain at the emergency department. Physical examination revealed distended abdomen with hyperactive bowel sounds and without obvious tenderness or rebounding pain. Laboratory data indicated marked leukocytosis. Computed tomography revealed a megarectosigmoid of diameter 18 cm [Figure 1]. After treating bowel rest, administering empiric antibiotics, and decompressing the rectal tube, abdominal pain improved and fever subsided.
Figure 1: Abdominal computed tomography revealed a megarectosigmoid

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On the basis of the imaging findings, Hirschsprung disease was suspected. Therefore, a rectal biopsy was performed, which indicated the presence of normal ganglion cells. Bowel management was performed with one large-volume enema to empty the huge rectal pouch daily. The patient was then free of constipation and achieved an improved nutritional status with appropriate weight gain. Three months after bowel management, a laparoscopic Swenson pull-through was performed.[2] Total bowel preparation was accomplished with polyethylene glycol 3350.

During the operation, the patient was held in a lithotomy position. Four 5-mm ports were placed in the umbilicus, right lower quadrant, left lower quadrant, and right upper quadrant. Huge rectal pouch occupied most of the peritoneal cavity, particularly in the pelvis. Dilated rectum was adhesive to the retroperitoneum, liver, gall bladder, and surrounding small bowels because of previous episodes of toxic megacolon. Adhesions were lysed. Laparoscopic dissection was continued on a Swenson plane toward the distal rectum as low as possible. Only a few centimeters of transanal dissection were required to pull through the rectum [Figure 2]. The patient was discharged on postoperative day 10 uneventfully. Pathology revealed slightly decreased ganglion cells without increased acetylcholinesterase activity. The cosmetic wound outcome was satisfactory [Figure 3].
Figure 2: Laparoscopic Swenson pull-through. Laparoscopic rectal dissection was made along the Swenson plane distally. Short transanal dissection was required to pull through the rectum

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Figure 3: (a) Pull-throughed megarectosigmoid. (b) Four 5-mm ports: Umbilicus, right-lower quadrant, left-lower quadrant, and right-upper quadrant. Cosmetic outcome was satisfactory

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Frequent watery bowel movements and soiling were observed during the early postoperative period. For reducing the frequency of bowel movements, enemas were provided two times daily. Enemas were stopped 1.5 months postoperatively, and loperamide and water-soluble fibers were prescribed to slow down peristalsis and make feces bulky. Continence was achieved at 2 months postoperatively. Loperamide was weaned off at 5 months postoperatively. In the latest follow-up at 24 months postoperatively, the patient had voluntary bowel movements 2–3 times per day without soiling or fecal accident.

  Discussion Top

The underlying cause of constipation in ARMs remains unclear. Severe constipation may result in megarectosigmoids, subsequently worsening fecal impaction and overflow pseudoincontinence. A study hypothesized that perirectal dissection during ARM repairs causes a degree of denervation, resulting in constipation.[3] However, some children born with ARMs have a congenital dilated rectosigmoid colon. Megarectosigmoid progresses because of an inadequate constipation treatment in early childhood.[4] Studies have suggested that constipation, chronic bowel dilatation, and hypomotility form a vicious cycle.[4],[5] Early and aggressive bowel management is effective in interrupting this cycle.

Because of the disease nature of megarectosigmoid and constipation, connecting it to Hirschsprung disease is easy. The incidence of Hirschsprung disease in patients with ARMs is similar to that in general population.[6] In our case, Hirschsprung disease was suspected when the patient suffered severe constipation and megarectosigmoid. Pathology revealed slightly decreased ganglion cells without increased acetylcholinesterase activity. Histologically, hypoganglionosis, hyaline degeneration, and fibrosis of the smooth muscle rather than typical Hirschsprung disease are frequently observed in the dilated colon. Neuronal cell density may decrease as the bowel diameter increases.[7]

Bowel management is a useful and effective treatment for megarectosigmoids and constipation. The patient was treated with large-volume enemas to empty the colon daily according to the bowel management program in Cincinnati.[8] After bowel management, the patient was free from constipation and achieved an enhanced nutritional status; meanwhile, his quality of life improved. Because of the satisfactory bowel management outcomes, the need for surgery was debatable.

Studies have suggested that surgical excision of the dilated bowel segment is an effective method for severe constipation with megarectosigmoid in ARMs.[7],[9],[10],[11] Postoperative laxative use could be decreased substantially in most cases, whereas laxative use could be totally weaned off in some cases. Nevertheless, soiling is a common complication.[7] Recently, Borg et al. described the long-term follow-up of ARMs with megarectosigmoids,[1] suggesting that early implementation of bowel management was helpful in normalizing the bowel function and megarectosigmoid size up to adolescence. The results of the conservative treatment were comparable with that of the surgical treatment.

Here, we presented the case of a 55-year-old man. Megarectosigmoids have been rarely documented in patients of this age. Furthermore, normalization of rectosigmoid configuration after conservative treatment has not been reported. Moreover, normalization of colon size and function at this age is highly unlikely. Surgical excision can be a definite treatment option after a detailed discussion between patients and physicians. To decrease the volume of enemas and improve the quality of life, we performed surgical excision of the megarectosigmoid and appendicostomy for postoperative antegrade enemas.[12] However, the appendix had been fibrotic because of the several episodes of colitis; therefore, appendicostomy was abandoned.

Pre- and post-operative bowel management are imperative. The patient experienced diarrhea-like soiling after surgery on several occasions because a hypermotile colon had formed. Studies have reported that soiling is a common complication and can be controlled with bowel management because most of the ARM patients with megarectosigmoids have better sphincter function than those with incontinence.[13] In our case, we aimed to slow the colon, make the stool bulky, and provide scheduled toileting. The patient had recovered bowel function with continent, regular bowel movements.

  Conclusion Top

Even in low-type ARM, patients should be carefully managed to avoid constipation. Although surgical excision may be a definitive treatment option for adult patients with megarectosigmoids, both pre- and post-operative bowel management are essential for improving the quality of life.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Borg H, Bachelard M, Sillén U. Megarectosigmoid in children with anorectal malformations: Long term outcome after surgical or conservative treatment. J Pediatr Surg 2014;49:564-9.  Back to cited text no. 1
Wei CH, Fu YW, Levitt MA, Dickie BH. Laparoscopic swenson pullthrough: How low we can reach laparoscopically? J Laparoendosc Adv Surg Tech A Videoscopy 2015;26(2).  Back to cited text no. 2
Levitt MA, Kant A, Peña A. The morbidity of constipation in patients with anorectal malformations. J Pediatr Surg 2010;45:1228-33.  Back to cited text no. 3
Levitt MA, Peña A. Anorectal malformations. Orphanet J Rare Dis 2007;2:33.  Back to cited text no. 4
Burjonrappa S, Youssef S, Lapierre S, Bensoussan A, Bouchard S. Megarectum after surgery for anorectal malformations. J Pediatr Surg 2010;45:762-8.  Back to cited text no. 5
Peña A, Levitt MA. Colonic inertia disorders in pediatrics. Curr Probl Surg 2002;39:666-730.  Back to cited text no. 6
Li L, Yan-Xia W, Xia-Na W, Jin-Zhe Z. Posterior sagittal approach: Megasigmoid resection and anal reconstruction for severe constipation and fecal incontinence after anoplasty. J Pediatr Surg 2000;35:1058-62.  Back to cited text no. 7
Bischoff A, Levitt MA, Bauer C, Jackson L, Holder M, Peña A. Treatment of fecal incontinence with a comprehensive bowel management program. J Pediatr Surg 2009;44:1278-83.  Back to cited text no. 8
Keshtgar AS, Ward HC, Richards C, Clayden GS. Outcome of excision of megarectum in children with anorectal malformation. J Pediatr Surg 2007;42:227-33.  Back to cited text no. 9
Lee SL, DuBois JJ, Montes-Garces RG, Inglis K, Biediger W. Surgical management of chronic unremitting constipation and fecal incontinence associated with megarectum: A preliminary report. J Pediatr Surg 2002;37:76-9.  Back to cited text no. 10
Levitt MA, Martin CA, Falcone RA Jr., Peña A. Transanal rectosigmoid resection for severe intractable idiopathic constipation. J Pediatr Surg 2009;44:1285-90.  Back to cited text no. 11
Levitt MA, Soffer SZ, Peña A. Continent appendicostomy in the bowel management of fecally incontinent children. J Pediatr Surg 1997;32:1630-3.  Back to cited text no. 12
Borg HC, Holmdahl G, Gustavsson K, Doroszkiewicz M, Sillén U. Longitudinal study of bowel function in children with anorectal malformations. J Pediatr Surg 2013;48:597-606.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3]

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