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ORIGINAL ARTICLE
Year : 2012  |  Volume : 1  |  Issue : 3  |  Page : 122-125

A collective review of cases with imperforate anus managed in a teaching hospital


Department of Surgery, Division of Pediatric Surgery, King Abdul Aziz University Hospital, Jeddah 21589, Saudi Arabia

Date of Web Publication15-Jan-2013

Correspondence Address:
Jamal S Kamal
Department of Surgery, King Abdul Aziz University Hospital, PO Box 80215, Jeddah 21589
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0521.106080

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  Abstract 

Objectives: The current study aimed to review the cases of imperforate anus, their demographic distribution, associated anomalies, results of management, and defining cases that require further management. Materials and Methods: A total of 123 cases of different types of imperforate anus were managed over a period of 20 years. Results: The patient group comprised 70 males and 53 females. Recto-vestibular fistula was the commonest type seen (27%), followed by ano-perineal fistula (20%). Only two cases of cloaca (1.6%) and one iatrogenic recto-vaginal fistula were seen. The commonest associated anomaly was hypospadius (79.6%) in males, followed by hydronephrosis and abnormal sacrum in both sexes. Most cases were managed by posterior sagittal anorectoplasty (72%). Voluntary bowel control and fecal continence were found in 100% of cases with ano-perineal fistula, in 93.3% of cases with recto-vestibular fistula, and in 63% of those with recto-urethral fistula. Fecal soiling was found in 12 patients; three of them had sacral anomalies and they were offered Malone appendicostomy. Eleven patients had mucosal prolapse; in two of them the prolapse was complete. Five patients possessed mislocated anus but kept clean by daily enemas and diet control. Conclusions: Although the number of cases with imperforate anus treated in our hospital was relatively small, the outcome of management was favorable and matching with reports from specialized centers. It is advisable to keep those patients under regular follow-up and updating their contact information frequently for further assistance.

Keywords: Congenital, imperforate anus, pediatric, management


How to cite this article:
Kamal JS, Rayes OM, Kurdi MO, Abdulhaleem AR, Farsi M. A collective review of cases with imperforate anus managed in a teaching hospital. Saudi J Health Sci 2012;1:122-5

How to cite this URL:
Kamal JS, Rayes OM, Kurdi MO, Abdulhaleem AR, Farsi M. A collective review of cases with imperforate anus managed in a teaching hospital. Saudi J Health Sci [serial online] 2012 [cited 2020 Dec 3];1:122-5. Available from: https://www.saudijhealthsci.org/text.asp?2012/1/3/122/106080


  Introduction Top


Imperforate anus is a congenital anomaly that is frequently observed in pediatric surgical units with an incidence of 1 per 4000-5000 live births reported globally. [1],[2] This abnormality used to be classified into three types: low, intermediate, or high categories, depending on the site of the proximal blind end in relation to the levator and puborectalis muscles.

This classification has no impact on prognosis or determining the way of management, so it is replaced with those affecting males and females, with or without fistula, and the site of the fistula. In females, a fistula inside the vagina is considered as cloaca with its two types: long and short common channel. [3],[4] Fistulas are found in 85-90% of the cases between the lower end of the rectum and the perineum, female genitalia, or urinary tract. Associated anomalies may also occur. [5],[6] Prognostic features in terms of fecal continence can be anticipated by the degree of sacral development seen in pelvic X-ray (sacral ratio). [7],[8],[9] Cases of imperforate anus are managed surgically by reconstruction of a new anal opening within the external sphincter. Several techniques were described. In 1982, the posterior sagittal approach was introduced: since then it became widely accepted as the standard approach for all types of imperforate anus. [10] Following this procedure; voluntary bowel control and fecal continence greatly improved, and it reached up to 100% in the covered anus and those associated with lower perineal fistulas. However, the percentage is decreasing in cloaca and those associated with higher fistula and high blind rectum. [10],[11] The common postoperative complications are chronic constipation with pseudo-incontinence or true fecal incontinence. These complications although not treatable are manageable by additional procedures like Malone appendicostomy for antegrade enemas and the bowel management popular program. [12],[13]


  Materials and Methods Top


The medical records of patients admitted, managed, and followed up at KAUH between Jan 1989 and Dec 2008 with the diagnosis of imperforate anus or the procedure were received and reviewed. A total of 123 cases of all types of imperforate anus were identified. A data collection sheet was designed to record the demographic and clinical data for each patient including the contact information. Patients older than 3 years of age, particularly those who missed follow-up for a long time, and their contact information, were called for reevaluation. All data were analyzed using SPSS program.


  Results Top


Of the 123 imperforate anus cases reviewed in this study, the most common type found was recto-vestibular fistula (27%) followed by ano-perineal fistula (20%) [Table 1] and [Table 2]. Associated anomalies were seen in 98 of these patients (79.6%), the most common being hypospadius. A breakdown of these anomalies according to sex and type of management were summarized [Table 3] and [Table 4]. Posterior sagittal anorectoplasty (PSARP) was the most common procedure among the current cohort of patients (59%). We had a total of six deaths, unrelated to their problem. More than half of the patients missed follow-up, a few months from their last procedure since the majority of them had no contact information. The group that did not present at follow-up and above 3 years of age (51 patients) was reevaluated, their mean age 11.3 years (range 4-27 years) for their voluntary control of defecation, bowel habits, and for any urinary problems. They also received a local examination for soiling, and site of the anal opening, the presence of external sphincter contraction, and anal squeeze pressure were recorded [Table 5]. Fecal soiling was seen in 12 patients; three of them had sacral anomaly and they were offered Malone appendicostomy for regular antegrade colonic wash. In one female patient, her fecal soiling was noticed to be due to associated limb anomaly (short left forearm), thus preventing adequate cleaning. Mucosal prolapse was seen in 11 patients; in two of them the prolapse was complete that required mucosal trimming. The rest were partial and had no effect. Mislocated anus was noticed in five patients; however, all of them were cleaned by regular enemas and dietary precautions. The overall voluntary bowel control without soiling was 72.5% in all reviewed patients.
Table 1: Different types of imperforate anuses seen in males


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Table 2: Different types of imperforate anuses seen females


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Table 3: The associated anomalies seen in our imperforate anus patients


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Table 4: The procedures followed in the current study


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Table 5: Summary of the clinical findings in the evaluated patients


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Three of the patients (one male and two females) were married, and had normal children, and that constituted the longest follow up-period. In the patient cohort, there were also two brothers, both mentally retarded, with a recto-vesical and a recto-urethral fistula who were the only siblings with the same anomaly.


  Discussion Top


Imperforate anus is one of the frequently seen congenital anomaly in pediatric surgical units. It occurs as a result of a failure in the descent of the urorectal septum and the persistence of the cloacal membrane during the development of the tail end of the embryo during the sixth week of gestation. [14],[15],[16] No genetic or environmental factors are found to be associated with this anomaly; however, it was reported in siblings and it may occur with an increasing incidence in those born to mothers who have taken lorazepam during pregnancy. [17]

Imperforate anus used to be classified into three main types depending on whether the blind end is above, within, or below the sphincter muscle complex (wingspread classification). This classification has now been shifted to those in males or females and to the site of the associated fistula. In females, imperforate anus may be associated with fistulae to the perineum or vaginal vestibule and rarely to the vagina. In males, this condition may be associated with a fistula in the perineum or between the blind lower end of the rectum and urethra (bulbar or prostatic) or the bladder neck. Hence, the fistula cannot be observed by clinical examination. In both sexes, isolated imperforate anus can also occur and the blind lower end may be very low and covered by a thin membrane or high where the blind lower end is high, within or above the levator ani muscles. Cloaca is a rare variant seen in females where the rectum, vagina, and urethra open together in a common channel. [18],[19] Fecal continence and voluntary fecal control with an anal opening at the normal site is the goal of surgical approaches to the imperforate anus. This procedure involved passing the rectum within the levator and external sphincter muscles. The lower anomalies are managed by excision of the membrane and anoplasty, typically with an excellent outcome.

Cases with a perineal fistula and/or recto-vestibular fistulae are managed by anoplasty and anal transposition (anterior or posterior sagittal), with or without colostomy. [20],[21],[22] Other types were managed via an abdominoperineal approach (variations of the Stephens technique involving the passing of the rectum within the puborectalis sling with a temporary colostomy). In this approach, the voluntary bowel control and fecal continence was not fully appreciated. [23],[24],[25] The posterior sagittal anorectoplasty (PSARP), described by Pena and deVries in 1982, greatly improved the outcome. [1],[26],[27],[28],[29] It allows full visualization of the sphincter complex and clearly shows the relationship between the rectum, the urologic system, and the surrounding structures, [30],[31] and it is widely accepted as the treatment of choice for these anomalies. This procedure may also be facilitated by laparoscopy. [32],[33],[34] Imperforated anus has also been reported in dogs for which similar operative procedures have been described. [35]

The current study of human cases aimed to evaluate the results of management over this long period and to identify those in need of further help in terms of fecal continence. All types of imperforate anus were been seen and different approaches were applied in their management.

Nine of the studied cases were treated using the Stephens technique and during follow-up, five of these individuals were found to have fecal incontinence with a patulous anus. These cases underwent a revision anorectoplasty using the posterior sagittal approach and subsequently obtained better control. A total of 72 of our patients were managed by PSARP. For those operated upon more than 10 years, some of them could not be contacted due either to change in their addresses or other reasons; however, a great response and willing for review from parents and the patients themselves were found. This indicated their need for assurance or further help. Some significant complications like soiling or pseudo-incontinence (12) and mucosal prolapse (11) were observed. Soiling was found to be as a result of constipation or true incontinence, and it was frequently reported after treatment of imperforate anus. The patients were guided for further management by regular enemas through the neoanus or constructing a Malone appendicostomy, diet regulations, and the use of laxatives. In this regard, we drew the attention that in one of the patients who suffered from vestibular fistula, her soiling was due to a congenital short left forearm. She was unable to clean herself by her left hand and she was not constipated. Mucosal prolapse might cause irritation due to mucus secretions and may need trimming, but this problem might disappear as the external sphincter contraction improves. [34]

The overall fecal continence rate in the cases was 72.5%, which is consistent with those reported by others. [35],[36],[37] Persistent fecal incontinence was expected in three patients due to the associated sacral anomalies. It is apt to mention that no long-term reports were found regarding the incidence, types, and distribution of this anomaly in Saudi Arabia, but there were sporadic reports about this anomaly as part of gastrointestinal tract malformations in general, and this could be the first one with such extensive review from Saudi Arabia. [38],[39],[40]

In conclusion, the distribution and types of imperforate anus seen at our hospital was matched with that reported in the literature, and the results we obtained in our long-term review were encouraging. [41]

It is very important to keep patients treated for imperforate anus under continuous and unlimited follow-up, and advise them to report any change of contact information.

 
  References Top

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    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]


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