European Journal of Cardiovascular Medicine

Constipation is a common problem seen in the follow-up of patients with anorectal malformation (ARM) after surgery. It is most common in patients with rectosigmoid preservation after pull-down surgery for ARM. [1,2] This constipation occurs after surgery for mechanical or functional reasons. Inadequate treatment can result in mega rectum/mega rectosigmoid and lead to faecal impaction and overflow incontinence.[3] It is a well-established fact that long-term constipation can lead to urinary problems. [2,3,4,5] In practice, this common problem is usually unrecognized/ignored by parents and sometimes until it becomes a major problem. Treated by a physician. It negatively affects the physical, behavioural and mental development of the child. Frequent urination problems associated with constipation can lead to growth failure in children. [2,3,4,5,6,7,8]

Hence this retrospective observational study was done to study the patients with constipation after surgery for ARM in a tertiary hospital in India.

This was a retrospective observational study done in the department of General Surgery in a tertiary medical college from 2018 to 2022 on patients of anorectal malformations. We reviewed the follow-up records of all patients presenting with complaints of constipation in the outpatient department, either at our institution or outside the ARM. Data analysed included age at presentation, gender, clinical presentation, diagnostic evaluation performed, need for revision surgery, management, postoperative complications, and follow-up results. In this case, the Crackenback protocol is used for evaluation. [9]

Additional dimensions

All patients who were older than 3 years (toilet trained) and complained of diarrhoea more than 3 months after surgery for ARMs were included in this study.

Exclusion criteria

Patients with missing records, age <3 years (not toilet trained), or patients who could not be followed for more than 3 months were excluded from the study.

At the time of presentation, the clinical manifestations are constipation, difficulty in defecation and urination, or tabular effect with impaired fecal and urinary flow. After the initial consultation, all patients were clinically evaluated. Patients with suspected mechanical abnormalities are investigated radiologically, including X-rays, ultrasonography (USG), and water-soluble contrast enemas to determine anatomy. Patients with mechanical obstruction underwent surgery again after informed consent from their parents/guardians and followed up according to the method established by Crackenback in 2005 to assess the results. [9]

The bowel management program (BMP) involves complete bowel obstruction and bowel emptying using an enema and rectal lavage as the first step. Sometimes the use of a nasogastric solution of polyethylene glycol (PEGLEC) is necessary. Regular counselling and reassurance of parents/guardians and patients is required. Toilet training and dietary changes are the main focus of management. A diet high in Fiber and protein is usually recommended. We euthanized the patients and taught them to use laxatives carefully and gradually reduce the bowel/bowel lavage in the follow-up. We used the BMP model during the 1st month after neoanus reconstruction and during the variable period of anal dilatation. We continue to follow-up the chart of each patient by asking leading questions and recorded complaints / satisfaction from parents and patients of variable psychiatric patients.

A total of 572 patients were operated on in our surgery unit in which 130 (22.73%) patients were found to be constipated. During follow-up. 36 patients with complaints of constipation were operated outside/elsewhere and subsequently presented to our outpatient department. These 166 ARM patients with preserved rectosigmoid (130 managed by us and 36 managed elsewhere) presented with a primary complaint of constipation. 12 of the 166 patients were less than 3 years of age and were not toilet trained, so were excluded from the study. A total of 154 patients were included in the study. Their ages ranged from 38 months to 168 months (median - 60 months) and 60 were male. Primary diagnoses included rectourethral fistula (52; 33.77%; 16rectoprostatic and 36 bulbourethral), vestibular fistula (50; 32.47%), perineal fistula/ectopic anus (36; 23.38%), rectovaginal fistula (41%) and imperforate anus (41%). %) were without fistula (8; 5.19%). Among these 154 patients, 58.44% (90) patients were operated through posterior sagittal route and remaining 41.56% (64) patients were operated through anterior sagittal route. Presenting complaints were excessive straining/crying during defecation and passing hard stools usually once every 2-3 days. 32 (20.78%) patients had complaints of stool impaction and dirtiness, while 20 (12.99%) patients also had complaints of difficult urination. Almost all the patients were using some type of drugs/laxatives/enemas on an irregular basis.

A detailed history was taken of each patient with emphasis on dietary habits, defecation and urine. Each patient was thoroughly examined. 56 (36.36%) patients were underweight (<5th percentile), while 6 (3.89%) patients were underweighting below the 3rd percentile. Anal stenosis was present in 40 (25.97%) patients (Male - 16; Female - 24), while 10 (6.49%) patients (Male - 6; Female - 4) had significant posterior aspect with adequate anal opening. A displaced anus was observed in 8 (5.19%) patients (male - 4; female - 6) [Figure 1]. No anatomical problem in the remaining patients [96; 62.34%].

In all patients (114 patients) except those with anal stricture (40 patients), BMPs (reassurance, fecal stone dissection, rectal irrigation/enema/PEGLEC, dietary changes, toilet training, etc.) were tested for 6 to 8 weeks. 86 (75.44%) of 114 patients responded well to BMP and did not require further surgery, while 28 (24.56%) patients (M-16, F-12) remained symptomatic after attempts at conservative management. they stayed. These patients underwent further radiological evaluation (abdominal USG with X-ray of the abdomen and spine). Only two patients presented with partial hypoplasia of the sacrum. Water-soluble contrast studies showed rectal/recto colonic dilatation in 26 patients [Figure 2].

In this cohort, 37.66% (58) patients had anatomical strictures/strata at the neoanus site (anal stenosis - 40, posterior ledge - 10 and displaced anus - 8) which was easily diagnosed only after detailed careful examination. About 75.44% (86 of 114) patients responded well after BMP and had good bowel control at follow-up (twenty patients with anal stenosis were excluded from the BMP trial). About 24.56% (28 out of 114) patients remained symptomatic (partial response) after the BMP trial, with 22 patients having known anatomical problems (posterior ledge - 10 and displaced anus - 8). Only 10 patients without any mechanical problem did not respond to the BMP trial.

Surgery was planned in 68 patients (for anal stenosis - 40, posterior ledge - 10, displaced anus - 8 and with dilated rectum/rectosigmoid - 10) but parents/guardians of two patients refused/did not consent to revision surgery. Finally, only 64 patients underwent surgery. Anoplasty (26), minimal PSARP (22), ASARP (10) and PSARP with dilated rectum (6) were performed in these patients. There were no deaths in this cohort. Superficial wound infections were observed in six patients. All patients were kept on rectal dilation and BMP for variable periods after redo surgery at our institution. 36 patients were lost to follow-up (only 6 patients from the operated group). The remaining 86 of 118 (72.88%) patients are doing well (continence, clean without regular BMP) but 32 of 118 (27.19%) patients still require some form of laxative and bowel wash/enema (grade I /II Constipation). The incidence of constipation was higher in patients operated by the anterior sagittal route (27.58%) than the posterior sagittal route (23.94%), but it was not statistically significant (P = 0.5). 8 patients required anticholinergic medication (oxybutynin) for their urinary problems, while 24 patients were still underweight (<5th percentile). Follow-up ranged from 3 months to 5 years.

Stool control is determined by three main factors: sensation in the rectum, good motility of the colon, and good control of the sphincter muscles. [2], [3], [4], ARM patients have abnormal voluntary muscles and the degree of muscle complex development varies. Most people with ARM are born without an anal canal, except for those with rectal atresia. Therefore, emotions do not exist. It seems that the patient can only perceive the protrusion of the rectum (proprioception). The most important clinical effect is that liquid stool or soft stool material may not be felt by the patient because the rectum is not dilated. [2, 3, 4, 5, 6, 7, 8]

The exact cause of constipation problems is unknown. Approximately 95% of all cases of constipation are functional, and only 5% are due to organic causes. [10] Constipation is considered a hypomotility disorder secondary to chronic bowel dilatation due to rectal stricture or anal canal angle/stenosis due to anterior displacement and consequent proximal bowel dilatation. The vicious cycle of reduced exercise capacity leads to bloating and constipation. In addition, this problem of constipation has been seen to be worse in lower defects. [2,3,4,5,6,7,8] Perirectal incision is also believed to cause some degree of denervation and lead to constipation. [2, 3, 4]

Another major factor affecting the degree of constipation appears to be the location of the colostomy. In patients with a transverse loop colostomy, distal colonography usually shows gross dilatation of the rectosigmoid colon, especially if closure of the colostomy is delayed for a long time. These patients experience more severe constipation after ARM repair and colostomy closure. In fact, there is a direct relationship between the extent of rectosigmoid expansion and the duration of colostomy closure. [2, 3, 4, 5, 6, 7, 8, 11].

The act of stretching in the dilated intestine and creating a neoanus can cause a blockage or a bulge in the distal part of the posterior wall of the stretched intestine (near the neoanus). [11] It causes faecal stasis and constipation, which leads to chronic dilatation, diastasis of the rectum, and decreased bowel motility.

The greater the dilatation of the rectum, the greater the aggravation of constipation and later, constipation becomes a self-perpetuating and self-aggravating condition. In this cohort, five patients did not have any anatomical/mechanical problem but still did not respond to BMP trial due to a dilated rectum/rectosigmoid. It is an important finding because it denotes hypomotility/ectasia of the dilated proximal bowel due to long-standing constipation. Non-compliance or poor compliance is a subjective finding related with satisfaction of patients/parents/guardians to BMP and the need for frequency/amount of laxative and bowel washing/enema required. Constipation is a very serious problem because eventually it provokes faecal incontinence, even in patients who were born with a potential for a bowel control (overflow pseudo incontinence).[4,5,6,7,8,11,12]

Long-term constipation may lead to urinary problems. The bladder and the distal bowel, both have almost the same nerve supply and the functional abnormality of any organ affects others (bowel and bladder cross talk).[12] Loaded colon can compress the bladder neck and cause incomplete bladder emptying. Perineal soiling and the incomplete bladder emptying are leading cause of recurrent urinary tract infections and cystitis. Long-term infection and the outlet obstruction may lead to trabeculated bladder and detrusor-sphincter dyssynergia. Any other associated anomaly in urinary system exaggerates the problems. [4,5,6,7,8,11,12]

BMP can eliminate the constipation and the associated urinary problems.[12] We also observed it in 20 patients who presented with urinary problems including frequency, burning micturition and fever. Incomplete emptying of bladder, dribbling of urine and burning sensation during urination were usual complaints. USG showed evidence of cystitis and thick-walled bladder in 12 patients, in which 8 patients needed anticholinergic support. The main problems encountered in BMP for poor compliance are ignorance, illiteracy, variable intelligence of parents/guardians and frequent change of guardians during follow-up. [2,3,4,5,6,7,8,13,14] Another issue in the management of constipation in post-surgery ARM is its association with Hirschsprung's disease. Although this is very rare, it may coexist and aggravate the problem of constipation.[15] Constipation after ARMs surgery needs early attention, but in routine practice, diagnosis is usually delayed. It should be evaluated properly, and the anatomical causes should be treated early. Minor early interventions/surgery or even medication and proper counselling can eliminate this problem. [13,14] Rarely, does a massively dilated rectosigmoid needs excision because of ectasia/hypomotility, but it should be avoided to prevent faecal incontinence. Recurrent constipation and persistent perineal soiling have adverse effect on the physical mental and psychosocial growth of patients. [7,8,13]

This study has various limitations because of its retrospective nature and probable selection bias. There were limitations in follow-up too. Results were based on the subjective opinion of parents and patients of variable intelligence and on their satisfaction. In conclusion, constipation is a common problem after ARMs surgery, especially in patients having preserved rectosigmoid after pull-through surgery. It is advisable to create relatively larger neoanus after pull-through procedures because the relative growth of neoanus is less due to local fibrosis which leads to narrowing of neoanus, especially in redo cases. [13,14] Neoanus dilatation should be instituted early in the post-operative period for variable lengths of time according to the need.[14] The key in these patients is to manage constipation proactively. Mechanical obstruction should remove early. The patients must be followed regularly. Repeated counselling, laxatives and dietary manipulations should begin at the first sign of constipation.

Constipation is a common problem after ARMs surgery, especially in patients having preserved rectosigmoid after pull-down surgery. It is advisable to create relatively larger neoanus after pull through with early practice of neoanus dilatation, especially in redo cases. Repeated counselling, laxatives and dietary manipulations should begin at the first sign of constipation.

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