Type Of Anorectal Malformation Research Articles

Clinical application of middle descending colon-double lumen ostomy with distal stoma narrowing in the treatment of anorectal malformation

BackgroundAnorectal malformations (ARMs) are the most common congenital anomaly of the digestive tract. And colostomy should be performed as the first-stage procedure in neonates diagnosed with intermediate- or high-type ARMs. However, the most classic Pe˜na’s colostomy still has some disadvantages such as complicated operation procedure, susceptibility to infection, a greater possibility of postoperative incision dehiscence, difficulty of nursing and large surgical trauma and incision scarring when closing the stoma. We aimed to explore the effectiveness of middle descending colon-double lumen ostomy (MDCDLO) in the treatment of high and intermediate types of anorectal malformations.MethodsWe retrospectively reviewed the data of patients who underwent MDCDLO for high or intermediate types of ARMs between June 2016 and December 2021 in our hospital. The basic characteristics were recorded. All patients were followed up monthly to determine if any complication happen.ResultsThere were 17 boys and 6 girls diagnosed with high or intermediate types of ARMs in our hospital between June 2016 and December 2021. All 23 patients were cured without complications such as abdominal incision infection, stoma stenosis, incisional hernia, and urinary tract infection in the postoperative follow-up time of 6 months to 6 years except one case of proximal intestinal prolapse was restored under anesthesia.ConclusionMDCDLO offers the advantages of simplicity, efficiency, safety, mild trauma, and small scarring in the treatment of high and intermediate types of anorectal malformations.

The treatment of congenital recto-vestibular fistula and recto-perineal fistula and effect of the megarectum on defecation.

This study aims to discuss the treatment of congenital recto-vestibular fistula and recto-perineal fistula, and the effect of the megarectum on defecation. Congenital recto-vestibular fistula or recto-perineal fistula is the most common type of anorectal malformation, and surgical methods include posterior sagittal anorectoplasty, anterior sagittal anorectoplasty, and mid-sagittal anorectoplasty, which can be performed at stage one or stage two after the ostomy. In the later stages of a recto-vestibular fistula, constipation is a common complication. Rectal dilatation is frequently associated with constipation, and the effect of rectal dilatation on defecation should be discussed for patients with congenital recto-vestibular or recto-perineal fistula who had rectal dilatation prior to surgery. Rectal dilatation may be one of the causes of constipation for congenital recto-vestibular fistula and recto-perineal fistula. The patients in this study were 67 children with congenital recto-vestibular fistula or recto-perineal fistula treated in our hospital from March 2013 to February 2017. All patients underwent an MRI of the spine and a barium enema. Six patients with myelodysplasia and sacral agenesis were excluded from this study. There were 18 patients with rectal dilatation (ages: 4-month-old to 1 year old, male: 3, female: 15). Seven of them had anterior sagittal anorectoplasty (group A), and 11 had anorectoplasty with dilated rectum resection (group B). Forty-three patients (ages: 3- to 10 months old, male: 6; female: 37) without a dilated rectum underwent anterior sagittal anorectoplasty (group C). All patients were followed up for 1 year to 5 years. Among the 50 patients who had undergone an anoplasty, 5 out of 7 patients with rectal dilatation developed post-operative constipation, and 3 of them had normal defecation after the second resection of the dilated rectum. Only two out of 43 patients without rectal dilatation developed post-operative constipation. One out of 11 patients with rectal dilatation who underwent anoplasty and resection of the dilated rectum developed post-operative constipation. Patients with congenital recto-vestibular fistula or recto-perineal fistula complicated by rectal dilatation are more susceptible to post-operative constipation. Resection of the dilated rectum at the same time can reduce the incidence rate of constipation. A barium enema should be performed pre-operatively for patients with congenital recto-vestibular fistula or recto-perineal fistula. If the dilated rectum is found, it can be resected at the same time.

ANORECTAL MALFORMATION WITH VAGINAL AGENESIS: A CASE REPORT

Vaginal agenesis is characterised by primary amenorrhea in adolescent women and other abnormalities, such as the absence of the uterus. The incidence of vaginal agenesis or distal vaginal atresia associated with anorectal malformations is difficult to estimate. A 5-year-old patient, at birth, immediately cried, and faeces came out through an orifice in the skin under the vagina. Previously, the patient had planned for a posterior sagittal anorectoplasty (PSARP), but it was known that the patient did not have a vaginal opening at the time of surgery. The patient was diagnosed with a low-type anorectal malformation, vaginal agenesis, and a sigmoidostomy status. PSARP and vaginoplasty were performed while the patient was 7 years old. Clinical and radiographic examination in the first 3 days of age plays a role in determining the type of anorectal malformation and whether or not a colostomy is necessary. In children, MRI radiology plays a role in the preoperative examination to evaluate the size, morphology, and stage of development of the muscular sphincter. Keywords: anorectal malformations, vaginal agenesis.

Three dimension high definition manometry evaluated postoperative anal canal functions in children with congenital anorectal malformations.

We aimed to evaluate the function of the reconstructed anal canal in postoperative anorectal malformations (ARMs) patients through three dimension (3D) high-definition anorectal manometry. From January 2015 to December 2019, 3D manometry was performed as a postoperative functional assessment of patients with ARMs divided into age subgroups based on the time of manometry. Manometric parameters, such as the length of the anorectal high-pressure zone (HPZ-length), the mean resting and squeeze pressure of HPZ (HPZ-rest and HPZ-sqze), recto-anal inhibitory reflex (RAIR), and strength distribution of the anal canal, were collected and compared with age-matched controls. Their functional outcomes were analyzed with SPSS 23.0 software for statistical analysis. 171 manometric measurements were performed on 142 postoperative patients (3 months∼15 years). The HPZ-rest in all patients was significantly lower than in age-matched controls (p < 0.05). HPZ-sqze was notably decreased in patients older than 4 years, whereas other age groups were comparable to controls (p < 0.05). The proportions of asymmetric strength distribution and negative RAIR were higher in ARMs patients. The type of anorectal malformations and lower HPZ-rest were the impact factors affecting postoperative functional outcomes. The majority of the ARMs patients had acceptable functional outcomes. 3D manometry can objectively assess the reconstructed anal canal function. The patients with fecal incontinence had a high proportion of extremely low HPZ-rest and HPZ-sqze, negative RAIR, and asymmetric strength distribution. The manometric details will help the clinicians explore the causes of defecation complications and guide further management.

Indocyanine green fluorescence imaging localization: A helpful addition to laparoscopic dissection and division of rectourethral fistulae

BackgroundRectourethral fistulae (RUF) are the most prevalent type of anorectal malformations in boys, with various surgical treatment methods investigated in recent years. Currently, research is focused on preventing urethral damage or urethral diverticulum formation caused by imprecise dissection during the laparoscopically assisted anorectal pull-through (LAARP) technique. This study aimed to determine the efficacy of indocyanine green (ICG) fluorescence imaging to improve the localization and separation of the RUF during laparoscopic surgery. MethodsICG was intrarectally injected through a pre-inserted gastric tube at the distal enterostomy port to locate the fistula. This retrospective analysis included children with RUF who were treated surgically with ICG fluorescence imaging localization-assisted LAARP between January and June 2022. We investigated the patient demographics, perioperative conditions, and subsequent follow-up results. ResultsFour patients underwent ICG-assisted LAARP. Their median age was 80 days (range, 63–120) and the median duration of each procedure was 145 min (range, 120–165). Postoperatively, the duration of catheter retention and hospital stay was eight days. The children's prognosis was based on the follow-up outcomes of gastrointestinal, urinary tract function, and imaging examination. None of the included patients was diagnosed with urinary diverticulum, urinary tract injury, anal stricture, or rectal prolapse. ConclusionsInjection of ICG at the end of the rectum during LAARP surgery is a feasible adjunct for locating the RUF, providing a greater degree of accuracy for laparoscopic separation and resection of fistulae, thereby decreasing the risk of urological complications.

Delayed vs. early enteral feeding after repair of congenital recto-vestibular fistula: The effect on perineal wound healing.

congenital Recto vestibular fistula represents the commonest type of anorectal malformation in females. The treatment of this anomaly is mainly approached either through anterior or posterior sagittal ano-rectoplasty approach. Several perioperative factors may affect the outcome. One of major postoperative complications is the occurrence of wound infection. We aimed to study the effect of delayed vs. early enteral feeding on the occurrence of perineal wound infection (PWI) after repair of congenital recto vestibular fistula. Fifty-five infants with recto-vestibular fistula were included. They were managed by single stage anterior sagittal anorectoplasty (ASARP) at an age ≥3 months. Groups A and B included infants who started oral intake on the 6th and 2nd postoperative days respectively. Group A infants were kept on peripheral parenteral nutrition (PPN) during the fasting period. Superficial wound infection occurred in three cases in group A while it developed in seven cases in group B. Deep perineal infection occurred in two and five cases in group A and group B respectively. The mean hospital stay was 8 days in group A vs. 13 days in group B when PWI developed. Delayed enteral feeding with PPN keeps the perineal wound less contaminated with stool. This promoted proper and fast healing with lower incidence of PWI. Also, PPN compensates the catabolic effects of both surgical trauma and fasting during the postoperative period and ensures maintenance of normal levels of essential nutrients that allow for proper healing.

Congenital H-type ano-vestibular fistula with normal anus

H-type ano-vestibular fistula with a normal anus is a rare type of anorectal malformations in female children. Although most cases are considered to be congenital, some authors suggested that this was an acquired condition. There is a high incidence of associated anomalies reaching up to 20–60% of the cases. The most common anomalies are anal stenosis, ectopic anus, cardiac and urological malformations. Many surgical techniques have been developed since the first description of this condition by Bryndorf in 1960. However, there is no consensus regarding the optimal surgical procedure due to the low number of cases reported in the literature. After reviewing these surgical techniques and their outcome, we can conclude that the main principles for a good repair are good preoperative bowel preparation, complete excision of the fistulous tract, good closure of the intervening tissue between the rectum and vagina in layers, avoidance of apposing suture lines and good post-operative nursing to keep the wound clean and dry as much as possible.

Surgical Complications and Functional Outcomes in Children with Anorectal Malformations with Vestibular Fistula

Background: Vestibular fistula is one of the most common types of anorectal malformations in female infants. Colostomy prior to definitive anorectoplasty is still controversial. Objective: To compare the complication rates and functional outcomes of the surgical treatments of vestibular anus between colostomy prior to anorectoplasty and treatment without colostomy. Materials and Methods: The medical records of 40 patients with vestibular fistula that underwent definitive surgical treatments at Siriraj Hospital between January 2006 and December 2018 were reviewed. Demographic data, operative records, functional outcomes, and complications were analyzed. Results: In the 40 vestibular fistula patients, colostomy (C) prior to vestibular fistula repair was performed in 18 patients, while vestibular fistula was repaired without colostomy (NC) in the other 22 patients. The C group had more associated anomalies than the NC group, including GI tract anomalies at 23.5% versus 9.1%, spinal anomalies at 47.1% versus 9.1%, and KUB anomalies at 52.9% versus 9.1%. The definitive anorectoplasty for vestibular fistula were anal transposition (31, 77.5%), PSARP (5, 12.5%), and other operations (7, 10%). Anal transpositions were performed in both the NC (90.9%) and C (61.1%) groups, respectively. NC had higher complications than C at 36.4% versus 22.2%. The recurrence of fistula occurred in 9.1% of NC cases versus 0% in C. Anal stricture was found in 18.2% of NC cases versus 5.6% in C. The wound infection rates of these two groups were the same. The complication rate with colostomy was 22.2% with prolapsed colostomy in 11.1% of cases and wound infection in 5.6%. The NC group had higher complication rates of anorectoplasty than the C group, but the overall complications, including colostomy complications, of these two groups were comparable. The Krickenbeck scoring system was used to evaluate the functional outcomes. In the median follow-up period of 1,336 days and 23 patients were evaluated. Even though the NC group had higher complication rates of anorectoplasty than the C group, the functional outcomes of these two groups were the same. Conclusion: Vestibular fistula repair with prior colostomy had lower complication rates of anorectoplasty than primary vestibular fistula repair, but the functional outcomes of these two groups were the same. Keywords: Vestibular; Rectovestibular; Anovestibular

Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study

BackgroundOutcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome. MethodsA multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4–7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups ‘poor’ ≤ 11, and ‘fair’ 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome. ResultsThe study included 111 RVF-patients. Median BFS was 16 (range 6–20). The ‘below normal’ group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management. ConclusionsAlthough median BFS at 4–7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management. Level of EvidenceLevel III.

Comparison of clinical outcome and anal manometry following laparoscopic-assisted anorectoplasty and posterior sagittal anorectoplasty in patients with high and intermediate anorectal malformation: A randomised controlled trial.

Introduction:High and intermediate types of anorectal malformations (ARMs) may be managed by either open posterior sagittal anorectoplasty (PSARP) or by laparoscopic-assisted anorectoplasty (LAARP). Most of the literature favours one approach over the other based on retrospective analysis. We performed this study with the aim to compare the short-term outcomes of both procedures.Materials and Methods:All paediatric patients with high and intermediate ARM were enrolled and randomised into two groups: open PSARP group and LAARP group. Outcome parameters such as faecal continence using Kelly's scoring system, anal manometric parameters and post-operative complications were compared between the groups.Results:A total of 16 patients were included with equal distribution in the open PSARP and LAARP group. Patient's variables were comparable in both the groups. Five patients developed immediate post-operative complications, three in the LAARP and two in the open group. The mean Kelly's score was 3.63 ± 1.6 versus 2.57 ± 1.9 (P = 0.132) for LAARP and PSARP group, respectively. The mean resting pressure was 34.71 ± 6.26 cm of H2O and 35 ± 6.16 cm of H2O (P = 0.384) in LAARP and open group, respectively. Rectoanal inhibitory reflex was demonstrated in 6/7 patients in LAARP group and 5/7 patients in open group.Conclusion:Faecal continence in patients undergoing either of the procedure is comparable. However, wound-related complications are lesser in LAARP procedure.

Reconsidering Diagnosis, Treatment, and Postoperative Care in Children with Cloacal Malformations.

Cloacal malformations are among the most complex types of anorectal malformation and are characterized by the urological, genital, and intestinal tracts opening through a single common channel in the perineum. Long-term outcome is affected by multiple factors, which include anatomical variants of the malformation itself, associated anomalies, and the surgical approach. Reconsidering these variables and their influence on "patient important" function might lead to strategies that are more outcome-driven than focused on the creation of normal anatomy. Key outcomes reflect function in each of the involved tracts and the follow-up needed should therefore not only include the classical fields of colorectal surgery and urology but also focus on items such as gynecology, sexuality, family-building, and quality of life as well as other psychological aspects. Involving patients and families in determining optimal treatment strategies and outcome measures could lead to improved outcomes for the individual patient. A strategy to support delivery of personalized care for patients with cloacal malformations by aiming to define the best functional outcomes achievable for any individual, then select the treatment pathway most likely deliver that, with the minimum morbidity and cost, would be attractive. Combining the current therapies with ongoing technological advances such as tissue expansion might be a way to achieve this.

Sacral Curvature in Addition to Sacral Ratio to Assess Sacral Development and the Association With the Type of Anorectal Malformations.

Introduction: Sacral ratio (SR) is currently the only measurement to quantitatively evaluate sacral development in patients with anorectal malformations (ARM). This study proposes sacral curvature (SC) as a new indicator to qualitatively assess the sacrum and hypothesizes that sacral development, both quantitatively and qualitatively, can be an indicator to predict the type of ARM. The study aims to investigate the difference of SR and SC between ARM types and the association with the type of ARM.Methods and Materials: This study was retrospectively conducted between August 2008 and April 2019. Male patients with ARMs were enrolled and divided into three groups based on the types of ARM: (1) rectoperineal fistulae, (2) rectourethral-bulbar fistulae, and (3) rectourethral-prostatic or rectobladder-neck fistulae. SC was measured in the sagittal views of an MRI or a lateral radiograph of the sacrum.Results: Included in the study were 316 male patients with ARMs. SRs were 0.73 ± 0.12, 0.65 ± 0.12, and 0.57 ± 0.12 in perineal, bulbar, and prostatic/bladderneck fistula, respectively (p < 0.01). The SCs in perineal fistulae and bulbar fistulae were significantly higher than that in prostatic/bladderneck fistulae (0.25 ± 0.04, 0.22 ± 0.14, and 0.14 ± 0.18, p < 0.01). When SR ≥ 0.779, there was an 89.9% of possibility that the child has a perineal fistula. When SR ≤ 0.490 and SC ≤ 0, the possibilities of the child having prostatic/bladderneck fistulae were 91.6 and 89.5%, respectively. SC < 0 was also noted in 27 (27.8%), 19 (10.5%), and no (0%) patients of prostatic/bladderneck, bulbar, and perineal fistulae (p < 0.01), respectively. Sacral defect was noted in 63% of patients with SC ≤ 0, compared to none with SC > 0 (p < 0.01).Conclusions: The higher the rectal level is in an ARM, the lower are the objective measurements of the sacrum. SC ≤ 0 is associated with sacral defects and implies a high likelihood of prostatic/bladderneck fistulae.

To Study the Outcome of Posterior Sagittal Anorectoplasty in Anorectal Malformations

Aim: To assess the effectiveness of posterior sagittal anorectplasty for the repair of anorectal malformations in terms of postoperative complications and functional outcomes. Study design: Descriptive prospective study. Place and duration of study: Sahiwal Teaching Hospital, Sahiwal. From 1st January 2016 to 31st December 2019. Methodology: Forty-two children with high/intermediate anorectal malformations, who underwent posterior sagittal anorectoplasty, were included in the study. Patients with low variety anorectal malformations, recto vestibular/ano vestibular fistula, persistent cloaca and patients with sacral dysraphism, spinal dysraphism, myelomeningocele spina bifida occulta and menifesta were excluded. Demographic information included age, gender, weight, type of anorectal malformation, type of surgery performed, operation time and hospital stay. Immediate post-operative complications were also noted. Bowel habits, constipation and faecal incontinence were evaluated in all patients during the follow up period. Results: Thirty-one (73.80%) were males and 11(26.19%) were females and mean age was 1.72 years. The mean weight at the time of operation was 11.00 kg. High variety anorectal malformations were 14(33.33%) and intermediate were 28(66.66%). Rectourethral fistula 25(59.52%) was most common variety seen in males. Recto bulber urethral fistula in 17(40.47%) and recto prostatic urethral fistula in 8(19.04%) patients were seen. Anal agenesis without fistula 7(16.66%) was most common anomaly in females. Overall complication rate was 15(35.71%). Constipation 11(26.19%) was most common post-operative complication. True fecal incontinence was seen in 3(7.14%) patients. Strong and effective squeeze of anal sphincter was seen in 39 (92.85%) patients and satisfactory bowel habits were present in 37 (88.08%) patients. Conclusion: Posterior sagittal anorectplasty is a useful procedure in the management of high/intermediate anorectal malformations, precise identification and reconstruction of muscle complex result in good anatomical and functional outcome. Immediate post-operative complication rate is also low. Keywords: Anorectal malformations, Posterior Sagittal Anorectoplasty, Postoperative complications, Functional outcomes

Functional fecal and urinary outcomes after sacrococcygeal mass resection in pediatric patients

BackgroundSacrococcygeal masses (SCM) are uncommon in children. The purpose of this study is to review the functional fecal and urinary outcomes following resection of SCM and to determine the impact of a multidisciplinary clinic (MDC) on these outcomes. MethodsA retrospective review was performed of patients who underwent SCM resection between 1979 and 2019. Baylor Social Continence Scale (BCS), Vancouver Symptom Score (VSS) and Cleveland constipation score (CSS) surveys were used to assess fecal and urinary continence at time of most recent follow up. Age, tumor characteristics, histopathology, and type of anorectal malformations (ARM), if present, were also recorded. Results75 patients were included. 51 (69%) patients were females and 23 (31%) had an associated ARM. The median age at resection was 8.5 months (IQR 0–26.8). 41 (56%) patients were followed in the MDC. 27 (82%) of patients seen in the MDC were clean for stool and 26 (87%) were dry for urine, while only 17 (59%) of patients not seen in the MDC were clean for stool and dry for urine (p<0.05). There was improvement in Baylor, Vancouver and Cleveland scores. ConclusionsA multidisciplinary approach to the care of patients following SCM resection may improve bowel and bladder outcomes.

Anorectal malformation with long perineal fistula: one of a special type

The anorectal malformation with long perineal fistula is a rare anomaly in the spectrum of anorectal malformations. Aim of the study is to describe the series of patients with anorectal malformation with long perineal fistula and compare the outcome with patient with standard perineal fistula. From March 2012 to January 2019, 7 patients who suffered from anorectal malformation with long perineal fistula were retrospectively reviewed. Three were operated on primarily by our department, and 4 cases were re-operated after a perineal anoplasty repair performed elsewhere. Four were operated by laparoscopy assisted anorectoplasty, and 3 cases were repaired by posterior sagittal anorectoplasty. The follow-up outcomes were compared with 71 cases of normal perineal fistula (NPF) in the same period. 7 cases have been followed up for 0.5–4 years (M = 2.57 ± 1.26) after definitive surgery. Their bowel function score was lower than normal perineal fistula (SPF = 12, range: 5–18; NPF = 18.5, range: 18–20). Four cases underwent anorectomanometry. The incidence of rectoanal inhibitory reflex was lower in the special type group. (p = 0.14). Three cases of contrast enema using barium: 2 cases of colorectal dilatation and thickening changes, 1 case showed no obvious abnormalities. Anorectal perineal fistula should be examined by distal colostogram at preoperation. This should be altered in: When suspecting a case of anorectal malformation type long perineal fistula a preoperative contrast enema could give insight of the anatomy befor performing a anoplasty.

Morphological criteria for surgical treatment of anorectal malformations in children

To improve treatment outcomes in patients with anorectal malformations via research of morphological criteria and differentiated approach to surgical correction. There were 37 children with various types of anorectal malformations for the period 2000-2019. We analyzed morphological features of atretic rectum wall, fistula, anastomosis with adjacent organs and skin of the perineum. Morphological research of anorectal malformations made it possible to differentiate treatment strategy and explain the causes of unsatisfactory results after perineal and abdominal-perineal proctoplasty. Incidence and severity of complications, as well as early disability were reduced that significantly improved postoperative quality of life. According to the morphological criteria, deeper mobilization of atretic rectum within at least 2.5-3 cm of the rectal «cone» with intact muscular wall is necessary. This approach was valuable to ensure adequate closure function of the rectum, prevent anal incontinence and restore normal appearance of the perineum. These achievements contributed to decrease in the incidence of admissions, redo surgeries and improvement of social adaptation in children.

POST OPERATIVE FUNCTIONAL OUTCOME FOR ANORECTAL MALFORMATIONS IN INFANT AND CHILDREN IN ERBIL

Background/aims: Outcomes of anorectal malformations have greatly improved but many children still experience some form of urinary or fecal incontinence despite optimal management. The aim is to evaluate the functional outcome in infants with anorectal malformation for different surgical procedure including traditional and recent operations and outcome for different type of anorectal malformation. Subjects and Methods : An analytical study include 28 cases of anorectal malformation (16 males and 12 females ) operated by different technique in Erbil city during 2010 - 2016. Cases of cloaca and early deaths were excluded from the study. Results : In male Rectoperineal fistula (25% ) and in female Rectovestibular fistula (25% ) were the most common anomalies. the most common procedure were primary anorectoplasty for perineal fistula in both male and female infants in 10 cases ( 35.71% ) followed by transfistula anorectoplasty for rectovaginal fistula in 7 cases (25%) and PSARP for rectobladder neck and urethral fistula for male infants in 6 cases. The most common functional complication was constipation in 2 (7.15%) cases and only one case (3.57%) developed fecal incontinence . 89.29 percent of the patients had voluntary bowel movements and were totally continent. Conclusion : Despite significant developments in the understanding of the pathophysiology and surgical techniques, the results of surgery of ARM remain far from perfect. An optimal operation restores normal anatomy and preserves all potential sphincter structures. Functional complications, especially treatable ones, such as constipation, should be detected and treated early to achieve an optimal outcome.

Frequency of congenital cardiac anomalies in patients with anorectal malformations.

Objectives: To determine the frequency of congenital cardiac anomalies in patients with anorectal malformations. Study Design: Retrospective study. Setting: Department of Pediatric Surgery, Khyber Teaching Hospital, Peshawar. Period: Jan 2018 to June 2018. Material &amp; Methods: All patients from 0 to 30 days of life both males and females (Inclusion criteria) with diagnosed anorectal malformation on clinical and radiological assessment, underwent cardiovascular work up to identify any cardiovascular disease. Patients with intersex disorders and those operated elsewhere were excluded. Mean and standard deviation were calculated for age. Frequency and percentage were calculated for gender, type of anorectal malformation, genital ambiguity, previous surgical intervention and congenital cardiac anomalies. Data was stratified for age, gender, lesion level, genital ambiguity and previous surgery to see the effect modification. Results: Out of 90 patients with anorectal malformation, there were 49 (54%) males and 41 (46%) females (M: F;1:1.19). with mean age of 7.65 ± 2.43 days. There were 39 (43.34%) patients with low anorectal malformation and 51 (56.67%) patients with high anorectal malformation. Genital ambiguity and previous surgical intervention was present in 8 (8.89%) and 11 (12.23%) patients, respectively. Congenital Cardiac Anomalies were diagnosed in 16 (17.78%) patients with anorectal malformation. Congenital Cardiac Anomalies were mostly seen in age group 1-10 days (21.95%), male children (22.45%) and high anorectal malformation (23. 52%). Conclusion: The frequency of congenital cardiac anomalies in patients with anorectal malformations was found lower than local figures. The majority of congenital cardiac anomalies associated with high anorectal malformation and male gender.

Dyssynergic patterns of defecation in constipated adolescents and young adults with anorectal malformations

We aimed to evaluate the etiologies of constipation in patients with anorectal malformations having a good prognosis for bowel control but a high risk of constipation. We included twenty-five patients from the Odense university hospital in Denmark. Patients were subjected to colon transit time examination and high resolution anorectal manometry (HRAM). The median age was 18 (14–24) and 48% (12/25) were females. Fifty-two % (13/25) of patients were diagnosed with constipation. Types of anorectal malformation were perineal fistula (9/25), rectovestibular fistula (8/25), rectourethral bulbar fistula (5/25) and no fistula (3/25). No difference in neither total colon transit time nor segmental colon transit times were found based on the presence of constipation. Only four of the constipated patients fulfilled criteria for dyssynergic defecation with a dyssynergic pattern at HRAM and prolonged colon transit time. A Type I dyssynergic pattern was dominant in constipated patients (7/13). A Dyssynergic defecation pattern was due to isolated contraction of puborectalis muscle in 9 out of 13constipated patients. We found a dyssynergic pattern during attempted defecation in patients with anorectal malformations disregarded the presence of constipation. In the majority of constipated patients an isolated contraction of the puborectalis muscle was visualized with HRAM.

Anorectal Malformations at a University Teaching Hospital in Southern Nigeria

Ano-rectal malformations comprise a wide spectrum of diseases, which can affect males and females, and involve the distal anus and rectum as well as the urinary and genital tracts. The incidence, type or variety of malformations varies from one region or geographical location to another. Ano-rectal anomalies tend to be associated with other congenital anomalies. The aim of this study is to determine the incidence, presentation and types of ano-rectal malformations and their treatment outcome. It is a prospective observational study from March 2012 to February 2019. Information obtained from cases of ano-rectal anomalies who presented to our health institution during the study period was entered into a proforma. Relevant clinical information was obtained and documented in a structured proforma. A total of 60 patients were seen during the study period. Males were 35 (58.3%) while females were 25 (41.7%). Age at presentation ranged from 2 hours -15 years. High anomalies were seen in 34 (56.7%), low anomalies 18 (30%) and intermediate 8 (13.3%). Associated congenital anomalies were seen in 12 (20%) patients. 28 patients had the traditional three-stage procedure of initial colostomy, PSARP and colostomy closure while 13 patients had double staged procedure of initial colostomy and definitive surgery with colostomy closure at the same time. Fifteen (15) patients had single staged procedure (PSARP) without an initial colostomy. Three (3) patients are awaiting colostomy closure after definitive surgery. Post-Operative complications were seen in 16 patients. A mortality of 10 (16.7%) was observed.