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Congenital Segmental Dilatation of the Rectosigmoid Colon: A Forgotten Cause of Constipation

Ahmed H. Al-Salem

Department of Peiatric Surgery, Maternity and Children Hopsital, Dammam, Saudi Arabia

 

Correspondnce

AHMED H. AL-SALEM
P.O. BOX 61015; QATIF 31911; SAUDI ARABIA;
FAX: 966 3 8630009;
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.


Abstract

Purpose: To review our experience and discuss the diagnosis and pathogenesis of congenital segmental dilatation (CSD) of the rectosigmoid colon.

Patients and Methods: We studied retrospectively the patients with the diagnosis of CSD of the rectosigmoid colon who were treated in our hospital from 2002 to 2006.

Results: Four patients, all males were found. Their age ranged from 1.3 years to 7 years (mean 3.9 years). All presented with constipation and abdominal distension. The duration of symptoms was variable ranging from as early as immediately after birth to several years prior to presentation. Before surgery, all received several modalities of conservative treatment including dietary manipulation, laxatives, suppositories and enemas with only partial and temporary benefit. All had barium enema which was diagnostic and a preoperative rectal biopsy which showed ganglion cells. One had a temporary colostomy followed by Duhamel’s pull through while the remaining 3 had one stage Duhamel’s pull through after thorough bowel preparation. Two had in addition an anterior reduction of the remaining segment of the rectum. Histology of the resected segment showed normal colon with chronic inflammation and fibrotic changes in some of them as well as normal ganglion cells. Postoperatively, all 4 are doing well, passing once to three times stools per day with no medication and no more abdominal distension.

Conclusions: CSD of the recto sigmoid colon, although a very rare condition, it should be considered in the differential diagnosis of infants and children with constipation and abdominal distension. Barium enema should form part of their investigation and in severe cases, surgical excision is curative.

Key words: segmental dilation of intestines, constipation

 

Congenital segmental dilatation (CSD) of the intestines is a very rare condition of unknown etiology [1]. It is characterized by a localized dilatation that is limited to a bowel segment of the small or large intestines and an abrupt transition between the normal and dilated intestines. The neuronal enteric plexus is normal in the affected segment, proximally and distally. Most cases are asymptomatic discovered during exploration for other conditions namely omphalocele, bowel atresia and anorectal malformations, but there are reports of CSD causing intestinal obstruction, perforation or bleeding [2, 3, 4, 5, 6].

CSD can affect any part of the gastrointestinal tract from the duodenum to the rectum, with the ileum being the most commonly affected site. Within the colon, the recto sigmoid region is the most commonly affected site and the presentation is usually chronic constipation with abdominal distension that is commonly treated as functional constipation or confused with Hirschprung’s disease [2, 7].

This report describes our experience with four patients with CSD of the recto sigmoid colon outlining aspects of diagnosis and management.

PATIENTS AND METHODS

The medical records of all infants and children treated at our hospital with the diagnosis of CSD of the recto sigmoid colon were retrospectively reviewed and the following information was obtained: age at diagnosis, sex, duration of symptoms, investigations, treatment, surgical management and outcome. All had barium enema as part of their investigation and where indicated a rectal biopsy was also obtained.

RESULTS

Over a period of 5 years from January 2002 to December 2006, four children with the diagnosis of CSD of the recto sigmoid colon were treated at our hospital. All were males. Their age at presentation ranged from 1.3 year to 7 year (mean 3.9 years). All presented with chronic constipation which started since birth or early infancy. The constipation was associated with abdominal distension and poor appetite. The constipation was severe that some of them were not passing a normal bowel motion for 2-4 weeks. All received several modalities of treatment including dietary manipulation, laxatives, suppositories and enemas with only partial and temporary benefit. Clinically, all were well with no other associated anomalies.

One of them was slightly subnormal mentally. Three of them had abdominal distension at the time of evaluation. Rectal examination was normal with palpable fecal matter. Plain abdominal x-rays showed markedly loaded recto sigmoid colon with gaseous distension proximally in some of them. One of them presented immediately after birth and his abdominal x-ray showed dilated bowel loops (Fig. 1A) and his subsequent abdominal x-ray showed dilated recto sigmoid colon (Fig. 1 B&C). He was diagnosed to have Hirschprung’s disease, treated conservatively and improved. He was discharged home but continued to have abdominal distension and constipation. He presented to our hospital when he was 4 years old. All had barium enema as part of their investigation and some of them had more than one barium enema prior to their referral to our hospital. This showed markedly dilated recto sigmoid colon with an abrupt transition of the normal colon proximally (Fig. 2&3).

This was also evident in the post evacuation film (Fig. 4). All had rectal biopsy prior to surgery which showed normal ganglion cells. This was done at two levels, 2 and 4 cm above the dentate line.

All four were operated on, three of them underwent one stage Duhamel’s pull through after thorough bowel preparation, while the fourth had a defunctioning colostomy followed by pull through. The majority of the dilated segment was excised and two of them also had anterior reduction of the recto sigmoid colon. Intraoperatively, the affected segment was markedly dilated with a clear transition to a proximally normal colon (Fig. 5). There were also prominent, dilated serosal blood vessels.

Post-operatively, all did well with no mortality or morbidity. All 4 are passing at least one to two motions per day with no medications or enemas. One of them was on oral laxatives for two months postoperatively, but currently, he is off medications and passing stools daily.

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Figure 1. Plain abdominal x-ray on the third day of life showing dilated bowel loops (a) and follow-up x-ray showing dilated rectosigmoid colon (b&c).

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Figure 2. Barium enema showing dilated rectosigmoid colon with transition to a normal proximal colon. Figure 3. Barium enema showing dilated rectosigmoid colon and a normal proximal colon.
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Figure 4. Post-evacuation barium enema showing localized dilatation of the rectosigmoid colon and  a normal proximal colon. Figure 5. Intraoperative photograph showing localized dilatation of the rectosigmoid with normal colon proximally. Note also the dilated prominent serosal blood vessels.

DISCUSSION

Congenital segmental dilatation (CSD) of the intestines is a very rare condition of unknown etiology. It was first described in 1959 by Swenson and Rathauser [1]. They described three cases of CSD of the colon. Several theories have been proposed to explain its pathogenesis. Irving and Lister proposed an extrinsic intrauterine intestinal compression such as an umbilical ring, vitelline vessels or omphalomesenteric band [8]. Whereas this theory may explain the occurrence of CSD in association with omphalocele, it does not however explain its occurrence in the other parts of intestines specially the recto sigmoid colon. Mathe’s et al on the other hand proposed a primitive neuromuscular dysfunction of the bowel, but this does not explain the selective occurrence of CSD [9]. Heller’s et al suggested a disturbance during splitting of the notochord from the endoderm as an etiology for CSD [10]. A notable feature we noticed in our patients is the presence of abundant, dilated and tortuous serosal blood vessels which may suggest a vascular role in its etiology.

CSD can affect any part of the intestines from the duodenum to the rectum with the ileum being the commonest affected site in the small intestines and the rectosigmoid in the colon [2, 7]. De Lorimer et al described a case of CSD affecting the cecum, ascending, transverse and descending colon [11]. CSD of the rectosigmoid colon should be included in the differential diagnosis of chronic constipation in infants and children. Whereas radiological investigations may not be helpful in the diagnosis of segmental dilatation affecting the small intestines, we found barium enema a useful investigation which should form part of the diagnostic evaluation of these children.

Clinically, CSD of the rectosigmoid colon can resemble Hirschprung’s disease and radiologically it may be confused with ultra short segment Hirschprung’s disease. Normal distensibility of the distal bowel on radiological examination and relaxation of the internal sn the colon [2, 7]. De Lorimer et al described a case of CSD affecting the cecum, ascending, transverse and descending colon [11]. CSD of the rectosigmoid colon should be included in the differential diagnosis of chronic constipation in infants and childrethe dilated segment as well as anterior reduction by excising part of the anterior wall of the remaining part of the dilated segment in an elliptical fashion. With this technique, the majority of the dilated segment will be excised. An alternative technique is a low anterior resection with an end to end anastomosis. This however requires an extensive pelvic dissection with the danger of injuring surrounding structures.

In conclusion, CSD dilatation of the recto sigmoid colon, although rare it should be considered in the differential diagnosis of constipation and abdominal distension in infants and children. Barium enema is diagnostic and differentiates this from functional constipation and Hirschprung’s disease by demonstrating a localized dilation of the recto sigmoid colon with an abrupt transition to a normal colon proximally. Rectal biopsy in doubtful cases should differentiate this from Hirschprung’s disease.

 

 

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