The anal canal is absent in most types of anorectal malformations, therefore one cannot expect “normal” bowel functions in most cases of anorectal malformations. The introduction of posterior sagittal anorectoplasty by Devries and Pena~ in the early 80s has revolutionized the management of high anorectal malformations and gained wide acceptance worldwide in short period and now has become standard procedure1. Many patients achieve very satisfactory bowel and urinary control, whereas others either remain fecally incontinent or suffer from functional disorders. Fecal incontinence, defined as the inability to control the emission of flatus and/or feces, can become an obstacle in the occupational, social, emotional, sportive, and sexual spheres of a person’s life, leading to psychiatric disorders and even loss of independence.2 Quality of life (QOL), defined by the World Health Organization as “the individual’s perception of their position in life in the context of culture and the system of values in which they live and in relation to their goals, expectations, standards, and concerns”.3 Thus, QOL has become in the last decade an important indicator of medical outcomes. The relevance of quality of life assessment in children with ARM was confirmed in an early study by Ditesheim and Templeton4, who used a questionnaire scoring system that included items such as school attendance, social relationships, and physical capacities. Bai eta l5 used the Achenbach’s child behavior checklist to assess the QOL in babies with ARM. We are sharing our experience to assess the QOL in children with ARM in this part of the world by using various scoring systems.

It was a randomized controlled prospective study conducted between September 2013 to January 2016. the department of paediatric surgery, Kurnool Medical College, Kurnool, Andhra Pradesh. The study material included all children above 3 years age with various congenital anorectal anomalies and had completed all stages of surgeries. The mean follow up period was 3 years (ranging from 3-6years). A standard questionnaire based on various scoring systems(Kelly’s score 6,7 Penās score 8, Bai etal 5 score) were given to individuals/parents to assess fecal continence by inquiring about (1).stool pattern, (2).frequency of stools, (3)degree of soiling/smearing,(4)ability to perceive the urge to defecate, (5)episodes of fecal incontinence, (6)dietary restrictions, (7)use of laxatives or enemas. Social life was assessed based on (8)peer rejection and (9)school absenteeism in school going children. Based on these scores the QOL was assessed. The evaluation of continence was graded using Kelly score and Penā score of continence. The quality of life was assessed using criteria given by Bai et al. Patients were categorized according to their score as follows; a score of 9-13 is clinically good, 5-8 as fair and 0-4 as poor.

A total of 77 children participated in this study. (Males 45, females 32) Mean age was 4.5 years, ranging from 3-7 years. Twenty two children were operated for high anorectal malformations (18 were males and 4 were females), 23 for intermediate (17 males,6 females) and 32 for low anorectal malformations(LARM). Twenty two of 32 children with LARM were females and rest were males (Table 1,2,). Table 1: Male anomalies Type of Anomaly No. of Cases Perineal fistulas 08 Rectobulbar urethral fistula 11 Rectoprostatic urethral fistula 10 Rectobladder neck / Rectovesical fistula 09 Pouch colon 03 No fistula 04 Total 45 Table 2: Female anomalies Anomaly No. of cases Anovestibular fistula 21 Rectovestibular fistula 06 Rectovaginal 03 Pouch colon 01 Vestibular anus 01 Total 32 A total of Twenty three children with high or intermediate ARM underwent posterior sagittal anorectoplasty (PSARP) and 22 had abdomino perineal pull through (AP Pull through). Among children with low ARM, all 10 male babies had anoplasty where as 22 female babies with LARM had either anal transposition(16)or limited PASRP(6).A total of 51 complication(Table-3) both major and minor were noticed during follow up and among them 14 children required interventions. Babies with Low ARM had good prognosis. Nine of the 10 male babies had normal stool pattern, only one baby suffered with constipation, where as twelve of 22 female babies who underwent anal transposition or limited PSARP suffered with constipation. Of the 23 children with PSARP five had normal stool pattern, twelve suffered with constipation and rest 6 children had increased frequency of stools with minor soiling. Twenty one of the 22children who had AP pull through suffered with increased frequency of stools and major soiling. Poor QOL scores were seen for those who had under gone AP Pull through procedure(QOL 2-5) where as those who had PSARP had fair QOL scores( 6-9), and children with anoplasty and anal transposition had good QOL scores(8-13). Male babies with high ARM had more number of school absenteeism as compared to females probably because females had lesser degrees of soiling and had more number of lower anomalies as compared to males. We used Bai etal,5 Kelly’s 6,7and Pena 8 scoring systems in this study to assess QOL.(Table 4). Table-4: Bai et al, QOL score according to type of surgery Type of surgery QOL score Anoplasty/anal transposition 8-13 PSARP 6-9 Abdomino Perineal Pullthrough 2-5 QOL was assessed in 77 children aged 3 years and above using the scoring system developed by Bai et al5. Table 5: School absenteeism and grade of soiling. Grade of soiling Males Females Total Grade 0 - - Grade I 04 06 10 Grade II 13 06 19 Grade III 16 02 18 Total 33 14 47 School absenteeism rate (Table-5) was higher in males as compared to females in this study. This was because girls had more low anorectal anomalies as compared to males. Those with grade2 soiling 19/77(25%) and grade3 soiling18/77(23.3%) had higher school absenteeism as compared to those with grade1 10/77(13%) or no soiling 30/77(39%). Table 6: School absenteeism according to type of ARA Type of ARA Males Females Total High 19 03 22 Intermediate 13 05 18 Low 01 06 07 Total 33 14 47 High type of ARA were associated with higher school absenteeism in male children in this study (Table 6) A total of 77 children participated in this study. (Males 45, females 32) Mean age was 4.5 years, ranging from 3-7 years. Twenty two children were operated for high anorectal malformations (18 were males and 4 were females), 23 for intermediate (17 males,6 females) and 32 for low anorectal malformations(LARM). Twenty two of 32 children with LARM were females and rest were males (Table 1,2,).

Anorectal malformations (ARMs) are the most common pediatric alimentary tract anomalies, with an incidence in live births of 1:1,500- 5,000 9. ARMs are classified using Krickenbeck and Wingspread classification into high-, intermediate and low-type; and are also grouped depending upon cloacal and rare malformations.10 PSARP, advocated by Pena in 1982, is a significant evolution in the management of ARMs 11. The principle of PSARP is to dissect the muscles of continence in a midline plane through a posterior approach without damaging the nerve supply. Different scoring systems have come up to assess the functional outcomes after the surgical correction of ARMs 12. The average QOL score in this study was 7.4. In a study by Viju John etal13 the average QOL score was 9.1.The high QOL score by Viju John etal is due to the fact that they have included only intermediate ARM as compared to our study which include all low, intermediate and high anomalies. Soiling in this study was graded based on Pena scoring system 8. Of the 28 (36%) children in good group, 18(23.3%) had no soiling, and 10(13%) had grade 1 soiling. Out of 27(35%) children in fair group, 17(22%) had major soiling and 10(13%) had minor soiling. Out of 22(29%) in poor group, 21(27%) had major soiling and 1(3%) had minor soiling. In study by Viju John et al 11.9% had no soiling, 28.57% had grade 1 soiling, 16.6% had grade2 and 42.85% had grade 3 soiling. The variation seen between the two studies was again because Viju John et al used only cases with intermediate anomalies for their studies. Wilfried Kroihe ARM group, there were nosoiling in 22 (52.38%) patients, grade 1 soiling in 13 (30.95%), grade 2soiling in 3 (7.14%), and grade 3 soiling in 4 (18.18%) patients QOL scores were better in children with low anorectal anomalies (9-13), as compared to intermediate (6-9) and high (2-5) anomalies because, children with low anomalies had lower grade of soiling and were more continent as compared to those with intermediate and high anomalies. Similar observations were reported by Zhihua Hong etal,14 who have done QOL assessment in 138 children with ARM using kelly’s and JSGA scores.QOL was good in 48,fair in 89,and poor in 1.They also reported that functional outcome of patients with highARM were worse than those with intermediate and low type. All our 32 children with LARM have good QOL (9-13). This is in comparison with Helena Wigander etal 15 who compared 44 children that were operated for LARM with normal controls and found no difference regarding QOL. QOL score was low in children who had associated VACTERL anomalies (21/77) and those who had associated syndromes .Associated genitourinary anomalies were most common, followed by cardiac anomalies. There were two cases of Down syndrome in this study. Patients with sacral agenesis and hemi vertebrae had poor QOL compared to those with normal sacrum. We agree with Zhihua Hong etal14, who reported that associated malformation and High ARM directly lead to negative outcomes. QoL scores also varied according to the type of surgery, those who had anoplasty / anal transposition had better scores (9-13), than those who had undergone PSARP (6-9) and those who had Abdomino-perineal pull through procedure had (2-5). Most of those who had undergone anoplasty or anal transposition had constipation with minor grade soiling. Those who had undergone PSARP had higher degree of constipation. 52% needed some kind of dietary modification, 80% needed laxatives and 40% needed enemas to manage constipation. All those who had under gone AP Pull through procedure had fecal incontinence and grade 3 soiling, except one female patient who had constipation with grade 1 soiling. This may be due to bowel adaptation and patient adjusting the diet as needed, and because of early bowel management programme. QoL score was lower in those children who had major postoperative complications (51/77) as compared to those with no complications in same study group. Most commonly seen complications were anal stenosis, mucosal prolapse, and wound infection. QoL score was lower in those who had to undergo redo surgery (14/77) probably because of presence of fibrosis and loss of sphincter muscle complex during redo surgery, there were five cases of recurrent fistulas in this study QoL score was better in those, who had regular follow up and, who followed regular anal dilatation programme as compared to those who were irregular in follow up and anal dilatation programme. This is in contrast to Kumar B etal 15 who performed redo surgeries in 31 patients and reported better results in 21 patients and only 10 patients required bowel management programme(BMP) and 58.3% stay clean without need for BMP. QoL scores also varied according to the stooling pattern, QoL was better in those who had normal stool pattern ( 25%), as compared to those who had constipation (30/77) 39%. It was worse in those who had increased frequency, due to fecal incontinence (28/77) 36.3%.Constipation was most common sequelae in children in this study. Low anomalies 13/32 (40.6%) had more tendency to constipate than high anomalies 2/30 (6.6%). In female anomalies 12/22 (54.5%) with anovestibular fistula, had constipation. In male anomalies 07/45(16%) with recto urethral bulbar fistula and 03/45 with recto urethral prostatic fistula had constipation. Fecal incontinence was seen in 28/77(36.3%), who had high anomalies like recto bladder neck/ recto vesical fistula, rectovaginal fistulas and high anomalies with no fistulous communication. All of them had increased frequency of stools with grade 3 soiling. Anju Goyal etal 16 evaluated 80 children with various anorectal malformations and found that functional outcome scoring decreased significantly with increasing severity of ARM. However they found no difference in the QOL between patients with ARM and controls. These findings were also comparable to the findings in other studies mentioned in the discussion.14 School absenteeism in preschool children (3-4y) was 48% in this study which was52.3% in study by Viju John et al13. And was equated to restricted socializing by parents in the society due to their child’s soiling habit and the attendant embarrassment. Parents were reluctant to send their kids to school at early age because of fear of soiling and hence most of these children were studying in lower class than their peers. Most children with major soiling faced peer rejection and had few friends and were reluctant to socialize with kids of their own age. Zheng H et al 17 reported that Children with low-type ARM can achieve good bowel control and QOL. However, although ARMs are benign, several children with this condition suffer from anal function problems that affect QOL. Similar observations seen in our female children who suffered with constipation which can be managed with dietary modifications and laxatives. We agree with Zheng H etal that Redo operations, mislocated anus, and incorrect constipation treatment are the iatrogenic causes of fecal incontinence. Julia Leitner etal18 review the literature to assess the quality of life in children and adolescent who were operated for anorectal malformations, by searching relevant databases PubMed". " CINAHL, PsycInfo" and “Cochrane” and concluded that children and young people with anorectal malformations have a worse quality of life than their healthy peers. We completely agree with Leitner etal that these children requires early support (bowel management program, psychologist, group therapy), as delay may cause multiple problems in the social, physical and psychological areas in children and adolescents with anorectal malformations. It is also important to the parents that they learn to accept the child's illness and try to help the children to cope their illness better. With the above support in childhood it will be possible to accept the disease better by parent as well as child and to integrate it into their self-image and self confidence. C Grano etal19 compared the QOL of109 Italian children and adolescents with ARM to the QOL of a large control sample of 336 healthy peers. They observed that children with ARM showed lower emotional, social and school functioning compared to healthy peers. Similar findings were observed in our study. Though our follow up is limited We completely agree with the author that this difference does not seen in the adolescent group as the children with ARM grown as adolescence they may have learned to overcome these difficulties.

Quality of life was better in male children with low anorectal anomalies. Female children with low anomalies suffered with constipation after corrective surgery however it can be managed with dietary modifications alone or with help of laxatives and QOL was good with less peer rejection and low school absenteeism. Quality of life was fair in those who had under gone PSARP. QOL was poor in those who had under gone AP Pull through for HARM due to major soiling and School absenteeism was also more prevalent in this group due to peer rejection.

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