Management Of Anorectal Malformations Research Articles

Anorectal malformations in low and middle-income countries- spectrum, burden and management

The clinical presentation, diagnosis and management of anorectal malformation has been well described in the literature, however the experience with these conditions in low-and middle-income countries is often shaped in unique ways due to the social, cultural and economic factors at work in these regions. This leads to adaptation of modifications in management options for these babies that usually present as delayed cases with added poor prognostic factors like sepsis leading to need for emergency resuscitation and overall increased morbidity and mortality. This article explores the anomaly from a global surgery lens and outlines the spectrum of the anomaly, burden faced in the resource constrained environment and the management options adopted for successful management under the available circumstances.

The Effect of Post War Limited Resources in Management of Anorectal Malformations in Mosul City

The Effect of Post War Limited Resources in Management of Anorectal Malformations in Mosul City

A review of the trend in the management of anorectal malformation in a major pediatric surgical center in India

A review of the trend in the management of anorectal malformation in a major pediatric surgical center in India

Experience with the management of anorectal malformations in Ibadan, Nigeria.

Introductionanorectal malformation is a multi-systemic birth defect of the distal gastrointestinal tract, the management of which is challenging to the surgeons, the patients and the parents. The presence of associated congenital malformations may worsen the outcome with consequent psychosocial effects on the patients and the parents. The characteristics of anorectal malformations with the challenges associated with their management and the outcomes are therefore presented here.Methodsall patients managed for anorectal malformations from January 2003 to December 2017 were studied. Patients´ demography, clinical presentations, types of malformations, associated anomalies, procedures performed, post-operative complications and management outcome were obtained and analysed.Resultseighty-eight children with anorectal malformations comprising 61 (69.3%) boys and 27 (30.7%) girls were studied with 76 (86.3%) patients presenting within the first year of life. Low anorectal malformation was observed in 14 (15.9%) patients, 71 (80.7%) patients had intermediate or high malformations and cloacal malformation was present in 3 (3.4%) patients. Associated congenital malformations were observed in 18 (20.5%) patients with 10 (55.6%) patients associated with intermediate or high malformations and urogenital system was the most common system whose anomalies were associated with anorectal malformations in 12 (13.6%) patients. Anoplasty was performed on 14 (15.9%) patients, posterior sagittal anorectoplasty was performed on 67 (76.1%) patients, abdominosacroperineal pull through on 4 (4.6%) patients and posterior sagittal anorectovaginourethroplasty on 3 (3.4%) patients. Six (6.8%) neonates died.Conclusionimmediate post-operative outcome was good; however, good functional outcome can only be assessed in an atmosphere of good follow-up which is still a problem in our environment.

Two-stage repair for delayed presentation of anorectal malformation with vestibular and perineal fistulae in females

Background/aim In spite of advances in knowledge and techniques in the management of anorectal malformation (ARM), delayed presentation of female ARM with vestibular and perineal fistulae is quite common especially in developing countries. In this literature, there is no standardized surgical algorithm for late presentation of these female ARM. This study aimed to present the experience of two pediatric surgery tertiary centers regarding delayed presentation of vestibular and perineal fistulae using two-stage procedures. Patients and methods This prospective study was conducted on 28 female patients with late presentation of vestibular and perineal fistulae from November 2014 to April 2019. Lower contrast study was done for all patients. Laxative, frequent rectal wash enemas, and Hegar’s dilators were used for 2–3 weeks preoperatively. All patients were repaired by anterior sagittal anorectoplasty (ASARP) with covering high sigmoid loop colostomy, and colostomy closure was performed after 2–3 months. Krickenbeck continence score was used for assessment of anal continence. Results Of 28 patients included, 25 patients completed the study. There were 14 patients with perineal and 11 with vestibular fistulae. The age of patients ranged from 8 months to 18 years. The main reasons for delay were socioeconomic, unawareness, and overlapping causes. Sphincter-saving ASARP was used in 15 patients and the classic ASARP was used in 10 patients. Tapering proctoplasty was needed in one patient. Minor wound infection developed in three patients. Constipation occurred in 11 (44%) patients. Follow-up period ranged from 6 to 50 months (median 30 months). Good continence was obtained. Conclusion Although delayed presentation of vestibular and perineal fistulae in female patients is accompanied by comorbidities, with good surgical management, successful results can be obtained by using two-stage procedure.

Divided and Separated Sigmoid Colostomy in Anorectal Malformation : Six Years Experience at Dhaka Shishu Hospital

Background : There are various surgical options for management of anorectal malformations (ARM). Colostomy is a common part of the management of high anorectal malformation in the pediatric population.Objective : The aim of this study was to find out the most common complications after formation of divided sigmoid colostomy.Methods : This prospective observational study was done in the Division of Pediatric Surgery, Bangladesh Institute of Child Health and Dhaka Shishu (Children) Hospital, Dhaka from January 2012 to December 2017. Divided and separated sigmoid colostomy was doneon 116 patients admitted with ARM during this period. Follow up on 2nd, 4th and 8th week after operation was done and in each follow up patients were assessed clinicallyfor wound infection, skin excoriation, prolapse of colostomy, retraction of colostomy and parastomal hernia. If colostomy complications were found then managed as per standard method.Results : The mean age of patients was 2.43±1.29 days and majority of the patients 66 (56.9%) were male.About half of the patients 50(43.1%) had fistula and 21(18.1%) patients had associated anomaly. Among 116 patients 51 (43.97%) developed complications after colostomy. The most common complication was skin excoriation found in 35 (30.2%) patients followed by wound infection in 8(6.9%). Prolapse and retraction of colostomy was found only in 6 (5.17%)and 2 (1.7%) cases respectively. No patient developed parastomal hernia and there was no mortality.Conclusion : Divided and separated sigmoid colostomy provided satisfactory outcome in the management of high anorectal malformation. Skin excoriation was the most common complication and few developed wound infection and prolapse of colostomy.Northern International Medical College Journal Vol.9(2) Jan 2018: 311-314

Management of Anorectal Malformation: Experience from Ethiopia

Background: Anorectal malformations are among the most common congenital malformation seen in children. There is paucity of data regarding the incidence and treatment outcome of this disease from Ethiopia. Methods: This is a retrospective review of patients treated for ARM at the TASH from March 2010-February 2014. Relevant statistical analysis was done and the results presented in tables and graphs. Results: Six patients (6%) presented at early neonatal age (before the age of 4 days) and 14 (14%) presented between 4 days and one month. A total of 57 associated congenital malformations were diagnosed in 44/99(44%) patients. The two most common type of ARM diagnosed among the female patients were recto- vestibular fistula which occurred in 39/56 (70%) andperineal fistula accounting for 6/56 (11%). ARM with urethral fistula was the commonest diagnosis among the male patients, making up 10/43 (23%). Post-operative outcome showed that 64 (64%) had a very good functional outcome. Conclusions: The diagnosis of anorectal malformation is late. Nearly half of the patients were found to have associated malformationsKey words: Anorectal malformation, Associated malformations, Postero-sagittal anorectoplasty

Revision Surgery in the Management of Anorectal Malformations: Experience from a Tertiary Center of India.

Aim and Objectives:Despite the significant advancements in the management of anorectal malformations (ARMs), there are various surgical and functional complications reported. Complications are closely related with the surgical techniques adopted and the types of malformations. In this article, we present our experiences with ARM patients who required reoperation after unsuccessful previous surgeries or who had developed complications related to the previous surgical techniques.Materials and Methods:We retrospectively reviewed clinical and electronic records of all the patients with ARM who were operated for ARMs in our institute from June 2010 to May 2016. All ARM patients who needed reoperation were included in the study. These patients were previously operated outside our institute and referred to us with ongoing problems of constipation, stool impaction with overflow incontinence, perineal soiling, and difficult urination.Results:There were 31 patients (M:F = 2.1:1) of ARM, reoperated for 38 indications during the above-mentioned period. Five patients had more than one problem. Presentation included neoanal stenosis (11), complete obliteration of neoanus (2), malpositioned neoanus (2), persistent/recurrent rectourethral fistula (3), iatrogenic rectovaginal fistula (4), rectal prolapse (5), large widened neoanus with soiling (2), and urethral stricture (2), which required revision interventions. Six patients had megarectum. All patients showed improvement in their symptoms after revision surgery, but 10 (41.7%) patients required further regular bowel management program (BMP) to avoid the soiling and constipation. Fourteen (58.3%) patients stayed clean without regular BMP.Conclusion:All these complications had clear explanations and are well described in the literature. Revision surgery in such patients had fair outcome, but some sort of BMP was required. Both posterior sagittal anorectoplasty and anterior sagittal anorectoplasty are excellent techniques for revision surgery with few simple modifications.

Current concepts in the management of anorectal malformations

Anorectal malformations (ARMs) occur in approximately 1 of every 5000 newborns and management still differs widely among practitioners. In this review, we adress some of the current accepted concepts in management. Missmanagement can have devastating consequences such as faecal incontinence, urinary incontinence and sexual disfunction. We briefly review common pecularities of the most common cases the and initial management oft he newborn. In this article I intend to present a brief overview without going into details but hopefully will motivate interest and further reading. Further the recommendation is made that case referal to a centre with a high volume load provides the highest benefits fort he patient in the form of better prognosis and long term management.

Current management of anorectal malformation in Egypt

Background/aimAnorectal malformation (ARM) represents a wide spectrum of anomalies. Its management includes various strategies. This survey aims at detecting the current preferences of Egyptian pediatric surgeons regarding the management of ARM.Materials and methodsA survey was circulated individual

Colostomy for anorectal malformation in a tertiary center in Nigeria

Background: Anorectal malformations constitute a wide spectrum of congenital anorectal abnormalities ranging from absent anus to anal membranes and fistulae. It is one major indication for the creation of colostomy in early neonatal life. This study was aimed at o describe and highlight the place of colostomy in the management of anorectal malformations. This article also reviews the issues relating to the choice of colostomy in anorectal malformation and common complications.Methods: The study was a prospective study carried put at a tertiary medical centre in Nigeria. It involved patients with a anaorectal malformation requiring the creation of colostomy.Results: A total of 19 neonates with anorectal malformation was seen over the period. The male to female ratio was approximately 1:2 with a female preponderance. Rectovestibular fistula was the commonest indication in females while recto-urethral fistula was commonest indication in males. Elective colostomy accounted for 78.9%.Conclusion: Anorectal malformation is the commonest indication for the creation of colostomy; this is closely followed by hircshsprungs disease. It is usually a part of the staged repair of anorectal malformation.The use of colostomy is acceptable as important in the initial management of patients with anorectal malformation. This affords the neonates the ability to feed and evacuate from the stoma. Complications are common and not life-threatening.Keywords: Colostomy, congenital, anorectal

Survey on the management of anorectal malformations (ARM) in European pediatric surgical centers of excellence.

Various management strategies for anorectal malformations (ARM) have been proposed. The aim of this study was to assess the current management in centers of excellence in Europe. An online survey on the pre- and post-operative concepts, surgical techniques, and the management of complications was sent to the representative experts of 28 selected European centers of pediatric surgery with special expertise in the treatment of ARM. The survey was completed by 25 experts from 14 countries. To assess the level of the rectal pouch in newborns 60% of participants perform a prone cross-table X-ray and 52% ultrasound. If an ostomy is required, 84% create divided Peña stomas. Primary repair in the newborn period is performed in females with rectoperineal (92%), rectovestibular (60%), and no fistula (32%), and in males with rectoperineal (92%), rectourinary (17%) and no fistula (38%). For 68 % of surgeons, the PSARP is the preferred surgical approach for "low" malformations. Laparoscopically assisted pull-throughs are routinely performed by 48% of experts for ARM with bladderneck and 28% for rectoprostatic fistula. 88% perform postoperative dilations. The management of ARM in Europe is very heterogeneous. High-quality clinical studies are needed to provide scientific evidence for the optimal treatment strategies.

Laparoscopic approach in the management of anorectal malformations.

Seventeen years have passed since the first description of the laparoscopic approach for anorectal malformation and approximately 68 articles have been published on the subject. In this review article, we aim to describe the advantages as well as the indications and contraindications of this approach when dealing with each specific type of anorectal malformation, according to what has been described in the literature and to our own experience. The ideal and undisputable indication for laparoscopy remains for cases in which the abdomen needs to be entered to repair the malformation. Only 10% of male patients with anorectal malformation are born with a recto-bladder neck fistula that requires an abdominal approach, this represents an ideal indication for laparoscopy. In females, only the complex cloacae with a common channel length greater than 3 cm are the ones that require a laparotomy; they represent about 30% of the cloacae. However, the repair of this type of cloacae also requires sophisticated and technically demanding maneuvers that have never been done laparoscopically. In cases of recto-urethral prostatic fistulas the malformation can be repaired either way: laparoscopically or posterior sagitally. In all other malformations: recto-perineal fistula, recto-urethral bulbar fistula, anorectal malformation without fistula, rectal atresia, recto-vestibular fistula; no justification for laparoscopy could be found; and in some cases, laparoscopy is contraindicated. In the published reports, there is no evidence supporting the idea that laparoscopic repair results in better functional results when compared with non-laparoscopic operation; there is a tendency to omit information relevant to bowel control such as the characteristics of the sacrum and the presence or absence of tethered cord; and most authors do not compare results between comparable malformations.

Invited comment on van der Steeg et al.: European consensus meeting of ARM-Net members concerning diagnosis and early management of newborns with anorectal malformations.

A conglomerate of European pediatric surgeons [1], united in the anorectal malformations network (ARMs-Net), refined the ‘Krickenbeck classification’ [2] that has served as a gold standard for the clinical management of anorectal malformations (ARM) over the past decade. With expanding experience when dealing with ARM and continuing improvement in diagnostic and therapeutic means over the years, this is a welcome addition that deserves general support. Regarding the differentiation between “experienced” surgeons and “not,” it is clear that ARMs are an infrequently occurring congenital anomalies and that extensive experience is not easy to acquire, if not in an ARM center. When to do a primary repair or when to perform a colostomy is clarified. However, this is also a signal that maybe in the future, patients with these pathologies should not be managed by everyone, but should be referred to specific centers that have a large experience. The addition of temporary dilatation of the fistula in female patients with a fistula > H5 is refreshing. Particularly these days, when possible negative effects of anesthesia and/or surgery in neonates is in consideration, delay until the infant is somewhat older, yet before the onset of psychological sequelae, is a positive development. Therefore, temporary dilatation should not only be limited to premature infants or those with severe associated congenital anomalies, but may be appropriate for all infants. Some restraint is warranted with some individual, yet unpublished, experience with puncturing the skin over the anus to determine the distance to the rectum. This is a highly subjective means of determining a distance that can be determined more objectively and repetitively by means of X-ray and/or ultrasound.

Anorectal Malformation: Paediatric Problem Presenting in Adult.

This is a case report of 22-year-old girl admitted with abdominal distension, vomiting, and chronic constipation since birth. Abdomen was distended, and perineal examination revealed imperforate anus with vestibular fistula (ARM). So far worldwide very few cases have been reported about anorectal malformation presenting in adulthood, and thus extremely little data is available in the literature about an ideal management of anorectal malformation in adults. In our case in the treatment instead of conventional procedure of posterior sagittal anorectoplasty (PSARP) anal transposition was done and till two years after the definitive treatment during follow-up patient has been doing well with Kelly's score of six. Our experience suggests that anal transposition provides satisfactory outcome in adults presenting late with anorectal malformation.

Management of anorectal malformations in the era of laparoscopy

AimDespite the advances of laparoscopic surgery in paediatric surgery, its role in anorectoplasty remains controversial. This review article aims at discussing current evidences regarding this new operative approach.MethodsLiterature review for laparoscopic anorectoplasty was performed in combination with experience from our centre. Details on operative techniques, surgical complications and postoperative outcomes were highlighted.ResultsLaparoscopic anorectoplasty (LAR) can be performed safely and effectively in early infantile period with modern small‐sized laparoscopic instruments. Postoperative complications have been reported to range from 0.8 to 7.2 per cent. Apart from general surgical complications, fistula and cyst formations at the posterior urethra are two specific complications. Functional outcomes are not inferior to traditional posterior sagittal anorectoplasty (PSARP) with less surgical trauma and better cosmetic result especially in high‐type malformations. Similarly, outcomes of LAR are comparable with PSARP under manometric and radiological assessments.ConclusionsBased on current evidences, LAR appears to be at least as good as PSARP. In high‐type malformations, we recommend that LAR be considered as the operation of choice. However, good laparoscopic skills and proper laparoscopic instruments are essential when performing this operation.

Management of Ano-Rectal Malformations in a Tertiary Level Children’s Hospital of Nepal

Introduction: Ano-rectal malformation (ARM) comprises a wide spectrum of diseases that involves the distal anus and rectum as well as the urinary and genital tracts. The factors that need to be considered during treatment are the type of anomaly, associated anomalies and patients general condition. The aim of this study is to analyze the types of anorectal malformations and their management in a tertiary level children’s hospital. Materials and Methods: The medical records of patients with the diagnosis of anorectal malformation managed in the hospital during a period of January 2009 to January 2014 were reviewed. Results: There were 187 cases of ARM 126 (67.37%) males and 61 (32.62%) females. High/Intermediate type was 129 (68.98%). Low ARM was 58 (31.01%). In male high type ARM with recto-urethral fistula was seen in 60 (47.61%). ARM with Rectovestibular fistula(RVF) was seen in 40 (65.5%) female. High and intermediate ARMs were managed initially with colostomy and low ARM with primary anoplasty. ARM with rectovestibular fistula in females were managed with Anterior Sagittal Anorectoplasty without colostomy. Common cloaca in female was managed initially with colostomy followed by posterior sagittal ano-recto-vagino-urethroplasty. Colostomy closure was done after adequate size of anus was achieved with anal dilation. Conclusion: The type of ARM in newborn is diagnosed by perineal examination and X-rays. Low type ARM was managed by primary surgery without colostomy. High anomaly and complex defect was treated with stage surgeries. DOI: http://dx.doi.org/10.3126/jnps.v33i3.8190 J. Nepal Paediatr. Soc. 2013;33(3):196-200

Update on the management of anorectal malformations

Thirty-three years ago, on 10 August 1980, in Mexico City, the first patient with an anorectal malformation was operated on using the posterior sagittal approach. At that time it was not obvious that we were actually opening a "Pandora's box" that continues to give many positive surprises, a few disappointments, and the constant hope that each day we can learn more about how to improve the quality of life of children born with all different types of anorectal malformations. In November 2012, patient number 3000 in our database was operated in the city of Cochabamba, Bolivia; during one of our International Courses of Anorectal Malformations and Colorectal Problems in Children. The goal of this article is to give a brief update on the current management of patients with anorectal malformation, based on the multiple lessons learned during this period.

Management of anorectal malformation: Changing trend over two decades in Zaria, Nigeria

Anorectal malformation is a common congenital defect and its management has evolved over the years. This is a review of the trend in the management of this condition in a major paediatric surgical centre in Nigeria over two decades. A retrospective analysis of 295 patients with anorectal malformations managed from January 1988 to December 2007 was carried out. There were 188 boys and 107 girls aged 1 day-9 years (median 8 years) at presentation. There were 73 (54.5%) and 106 (65.8%) emergency operations in groups A and B, respectively. There were 61 (45.5%) and 55 (34.2%) elective operations in groups A and B, respectively. Regarding treatment, in group A, patients requiring colostomy had transverse loop colostomy, while in group B, sigmoid (usually divided) colostomy was preferred. The definitive surgery done during the two periods were: group A: cutback anoplasty 29 (47.5%), anal transplant 5 (8.2%), sacroabdominoperineal pull through (Stephen's operation) 6 (9.5%) and others 21 (34.4%). In group B, posterior sagittal anorectoplasty (PSARP) 46 (83.7%), anal transplant 1 (1.8%), posterior sagittal anorectovaginourethroplasty (PSARVUP) 2 (3.6%) and anal dilatation 6 (10.9%) were done. Early colostomy-related complication rates were similar in the two groups (P > 0.05). The overall late complication rate was 65.5% in group A and 16.4% in group B (P < 0.05). The mortality was 25 (18.6%) in group A compared to 17 (10.6%) in group B (P < 0.05). There have been significant changes in the management of anorectal malformations in this centre in the last two decades, resulting in improved outcomes.

Congenital Anorectal Malformations: Anything New?

Management of anorectal malformations, especially that of proximal and more severe forms have developed significantly in the last 25 years. Echography and magnetic resonance imaging have improved the diagnostic accuracy, especially in terms of associated anomalies that are the main cause of fatalities among patients with anorectal malformations. Animal models, novel molecular biological techniques and knock-out gene models have widened our knowledge on aetiology and pathogenesis of these anomalies. From early 1980s, the posterior sagittal anorectoplasty originally designed by deVries and Pena has systematized the surgical management of anorectal anomalies leading to improved functional outcomes. Surgical mini-invasive techniques are increasingly applied to the management of these anomalies, although their role is as yet not clearly established. Long-term functional follow data are also available today indicating a reasonable favourable faecal and urinary control in the majority of patients with anorectal malformations. For the patients with less favourable functional outcome there are bowel and bladder management options that provide sufficient social continence.