Anorectal Malformations In Children Research Articles

Analysis of concepts and strategies in the medical and surgical treatment of anorectal malformations in children (literature review)

The concept of the surgical treatment of anorectal malformations is based on an in-depth knowledge of the anatomy, physiology and morpho-pathology of the colo-recto-anal and perineal area. The given study elucidates how the evolution of medico-biologic knowledge has influenced surgical tactics in the correction of anorectal malformations over the centuries. Despite the successes achieved at the current stage, there are still gaps in the medical-surgical treatment of anorectal malformations, which generate postoperative morbidity in the group of these patients, which indicates the need to further improve the curative management of these congenital anomalies.

Хірургічне лікування природженої аноректальної вади розвитку з ректовезикальною норицею в хлопчиків

Congenital rectovesical fistula (RVF) is the most complex variant of anorectal malformations (ARM) in boys, characterized by a fistula connecting the rectum with the bladder. Purpose - to evaluate the results of surgical correction of RVF in children, using a differentiated approach to the diagnosis of this defect, depending on the anatomical options, the clinical course of associated defects, and, based on this, to develop the main tactical requirements for the treatment of this complex congenital ARM in children. Materials and methods. The study included 9 boys: 7 - RVF without pouch colon and 2 - RVF with congenital pouch colon, which accounted for 5.8% of all treated anorectal anomalies (n=154). Results. In patients with RVF without pouch colon: a double-stem disconnecting colostomy was performed in 3 (33.3%) and a double-stem loop colostomy in 4 (44.4%) boys. In children with RVF and pouch colon: a longitudinal, narrowing, stapler coloplasty of the large intestinal "pouch" was performed, with removal of the terminal stoma (n=1); formation of a double-stem disconnecting stoma without separation of the RVF, and at the II stage - elimination of the RVF, removal of the large intestinal «bag» and preservation of the ileostomy - as a permanent, lifelong stoma (n=1). In 7 (100%) patients with RVF without pouch colon at the II stage, abdomino-perineal posterosagittal anorectoplasty with separation of RVF was performed (n=5). At the III stage, 5 patients with RVF without a pouch colon underwent local access stoma closure. At the stages of primary correction of RVF 55.6% (n=5) of children had coloproctological complications. Conclusions. The use of a differentiated approach to diagnosis and methods of anorectoplasty, as well as the use of a complex of rehabilitation treatment in patients with RVF without a pouch colon made it possible to obtain good results in 50% and satisfactory results in 50% of children. Congenital pouch colon - a significant expansion of part or the entire colon already in the fetus, makes it possible to suspect and diagnose this defect in the prenatal period, and to carry out delivery and surgical correction in a specialized perinatal center. The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the Local Ethics Committee of the institution mentioned in the work. Informed consent of the patients was obtained for the research. No conflict of interests was declared by the author.

Features of the Genitourinary System in Anorectal Malformations in Children

In anorectal anomalies, combined malformations of the urogenital system are often observed [1,5,7]. This can be explained by the close relationship between the embryonic development of these organs and systems. It is no coincidence that a lot of attention in the literature of the last decade is paid to urological problems of anorectal anomalies [ 12,15,20]. However, the true frequency has not yet been established and the structure of pathological conditions of urogenital system in anorectal anomalies has not been fully studied, the consequences of their delayed diagnosis have not been analyzed, treatment and tactical and operative technical aspects have not been developed and the organizational side of the issue needs improvement

Anaesthesia in Correction of Anorectal Defects in Children

Anorectal abnormalities are among the most common congenital malformations in children requiring emergency surgical interventions. The objective is to analyze existing domestic and foreign publications on the features of anesthesia in anorectal abnormalities in children. Subjects and Methods. The search for publications was carried out in the abstract bases of Russian Scientific Citation Index, PubMed and Scopus according to the key words: anorectal abnormalities, anesthesia, regional anesthesia, children. 105 publications were found, 49 studies were selected for analysis. Results. For the purpose of anesthesia, both general and regional anesthesia are used to correct anorectal anomalies. Among regional techniques, spinal and caudal anesthesia is most often used. The most effective anesthesia option for anorectal surgery is saddle anesthesia since it is accompanied by a significant decrease in the tone of the anal sphincter. Combined use of general anesthesia and caudal analgesia contributes to the reduction of intraoperative blood loss and early restoration of bowel functions. Conclusion. Further research is needed to establish rapid pos t-o perative recovery protocols for anorectal malformations in children.

Anorectal malformation in children

The lecture presents modern information about embryogenesis, associated anomalies, diagnosis, and treatment of various forms of anorectal malformations (ARM) in children. During the initial examination of ARM children the authors recommend the following sequence of actions for a neonatologist and a surgeon: catheterization the esophagus in order to exclude combined esophageal atresia; survey radiography of the abdominal organs in direct or lateral projections to exclude concomitant duodenal atresia, dilated intestinal loops and primary assessment of the condition of the spine and sacrum; echocardiography to detect concomitant heart and large vessel defects (occur in 30% of ARM newborns), Ultrasound of the kidneys and bladder. A special X-ray study to determine the location of the intestine al atresia is carried out by a pediatric surgeon no earlier than 18-24 hours after birth in newborns without an anus on the perineum. It is believed that after this period of time, the gas will fill the atresized part of the intestine, which is determined on the side image at the level of the coccyx or sacrum or in the abdominal cavity, depending on the type of pathology. To determine the prognosis of the functional state of the pelvic organs before surgery, it is recommended to determine the sacral index, since a visual assessment of the state of the sacrum and coccyx (counting the number of vertebrae) in most cases does not allow a quantitative assessment of the detected changes. The bowel management program provides training for the patient’s parent or child on how to empty the bowel in order to achieve a “clean” period (without constipation and fecal smearing) during the day. The schedule is developed individually for each patient, usually within one week. The issues of postoperative rehabilitation of patients at the stage of treatment in the clinic are discussed.

Quality of Life and Anorectal Malformations: A Single-Center Experience.

PurposeThe treatment and long term clinical outcomes of anorectal malformations (ARM) in children have always been the focus of pediatric surgeons. This study aimed at reporting our experience as far as long-term follow-up of ARM in children is concern.MethodsWe enrolled patients treated between 1999 and 2019, and established selection criteria to choose appropriate subjects. A validated questionnaire was used to determine long-term quality of life outcomes.ResultsOut of a total of 48 patients treated within the study period, 28 were enrolled in this study. Among the latter, more than 35% had at least one long-time complication, and more than 90% had a good lifestyle. Urinary and fecal continence was achieved in more than 95% of the patients using medical devices.ConclusionThis study aimed to bring up new concepts; taking into consideration all aspects of life in patients with ARM, from school life to sexuality, while evaluating fecal and urinary continence. This is essential for the improvement of the skills of the different specialists involved in the management of these patients, and for the implementation of strategies that can improve postoperative function. Most especially, it will also help improve communication between doctors to ensure an adequate transition of these children into adult life.

OUTCOME OF POSTERIOR SAGITTAL ANORECTOPLASTY IN ANORECTAL MALFORMATIONS WITH AND WITHOUT FISTULA IN TERTIARY CARE CENTRES OF NORTH INDIA

Introduction: Anorectal malformations (ARM) have been a source of concern for centuries. The reported incidence of ARM is 1 in every 2500 to 5000 live births but maybe even more frequent in certain 1,2 developing countries . Although ARM comprise approximately 0.2-0.3% births. They have been reported to comprise up to 3,4,6-24 1.2% of reported birth defects. Approximately 36.4% are isolated lesions and 63.6% are associated with other anomalies . ⁵ Studies demonstrated several mutations of HLXB9 associated with ARM . 33 patients with intermediate 25 Method and Material: ARM were included A thorough perineal examination, complete haemogram, urine analysis, colposcopy and . radiological studies were conducted. Patients underwent PSARP, the comparison of late complications and functional outcome at the end of 1 year follow- up in ARM patients with and without stula was done. Fischer Exact Test was done for getting Two tailed P- value. Observation and Results: Out of 33 patients 16 males were without stula and 17 males were with rectourethral stula. 33 patients underwent PSARP, 8 cases (24.24%) had early complications. Late complications in 12 cases (36.36%). No postoperative complications were recorded in 13 (39.39%) children. At end of follow-up, 31 remaining patients (including patients with supercial wound infection) had normal looking perineal body and anus with good contraction. No recurrence of stula, stenosis of anus or anterior displacement of rectum. PSARP is a useful procedure for the correction Conclusion: of ARM in children in developing countries and quality of life depends on clinical status of patient and age at the time of presentation. No signicant statistical differences in quality of life was seen in patients with stula vs patients without stula post PSARP

Results of Colostomy Use in Children with Anorectal Malformation

Introduction. Anorectal malformations in children are still one of the most challenging problems in pediatric coloproctology. The incidence of anorectal malformations in recent years has no tendency to decrease and, according to various authors, ranges from 1 in 4000-5000 live births. Most pediatric surgeons continue to adhere to the opinion about the advisability of preliminary colostomy and delayed proctoplasty at the age of 6-18 months or when the child reaches a certain body weight (8-10 kg.). They are motivating this tactic with the possibility of creating an optimal condition for performing a complex intervention, reducing anesthetic risk, avoiding technical errors. Objective is to improve treatment outcomes for anorectal malformations in children with prior colostomy. Materials and methods. The work is based on the results of treatment of 154 children with anorectal malformation, with preliminary colostomy in the clinic of the Tashkent Pediatric Medical Institute for the period from 2000 to 2020. Along with routine and general clinical examination methods, all children underwent: X-ray of the abdominal cavity, colostography, fistuloirrigography, excretory urography, cystography, ultrasound of the perineum (small pelvis), neurosonography (NSG) screening tests. Results. 154 (10*0%) children had colostomy as a palliative stage of treatment. Of these 117 (76%) children developed colostomy on the first day of life, with the development of intestinal obstruction. In 37 (24%) children, the formation of a colostomy was performed directly by us. 9 (5.8%) children as the first stage before primary radical correction with a high form of the defect and 10 (6.5%) children previously operated on and requiring re-corrective operations, 5 (3.2%) patients underwent colostomy after the development of complications in the early postoperative period. In 13 (8.5%) cases, colostomies were formed with identified concomitant anomalies and defects that clinically "dominated" over anorectal malformation. In 2 (5.4%), a double sigmastoma was imposed, in 2 (5.4%) a distal single-barreled sigmastoma, in 5 (13.5%) the Hartmann type terminal sigmastoma. Conclusion. The use of colostomy in children with anorectal malformation made it possible to carry out the necessary surgical tactics in a timely and differentiated manner. To reduce the frequency, nature of complications and early disability, to improve the quality of life and social adaptation of patients.

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.

Diagnostics and Treatment of Fistula Forms of Rare Regional Variants of Anorectal Malformations in Children

Diagnostics and Treatment of Fistula Forms of Rare Regional Variants of Anorectal Malformations in Children

Repeated Reconstructive Surgeries for Postoperative Complications of Anorectal Malformations in Children

Repeated Reconstructive Surgeries for Postoperative Complications of Anorectal Malformations in Children

Актуальні проблеми класифікації аноректальних вад розвитку в дітей у сучасних умовах (огляд літератури)

Given the diversity of anatomical and functional manifestations of congenital anorectal malformations, the most relevant remains development of classification of this pathology, for the same understanding by doctors of the manifestations of this defect and developing optimal common criteria for treatment tactics and strategies. The paper presents historical and current information on the classification of anorectal malformations in children. The main contradictory issues of the existing classifications are identified. The relevance of the use of Krickenbeck classification in the treatment of these defects is highlighted. Under the influence of different colorectal schools, historically, different educational and methodological approaches to the treatment of anorectal malformations. The first International Congress of Pediatric Surgeons was held in Melbourne, Australia, in 1970. The Melbourne meeting established an international classification of anorectal malformations. It was based on the development of defects at the stages of embryogenesis and their division by sex. Also, they are divided into: low, intermediate and high anorectal malformations. The second World Meeting of Pediatric Surgeons was held in Wingspread, Wisconsin, USA, in 1984, when F. D. Stephens and E. Smith, with a group of experts from around the world, proposed a detailed PAHR classification. The advantage of the Wingspread classification was that it could be used to predict the most optimal surgical treatment for various variants of anorectal defects. The third international conference on the development of standards for the treatment of congenital anorectal malformations took place in Krickenbeck, Germany, in 2005. This conference radically changed the latter classification, removing the terms «low», «intermediate» and «high» and the differences between female and male anomalies. The assessment depended on the presence and type of fistula. The reason for adopting such a classification was the great difficulty in comparing the results of different operations according to the old Wingspread classification. The peculiarity of this classification is the great contribution of colleagues from the Indian and Asian subcontinents to the surgical treatment of several anomalies, in particular: Pouch colon, which are rare in other countries. Today, pediatric surgeons in Ukraine generally do not use the Krickenbeck classification, despite the fact that it is generally accepted in most countries. This leads to the application of different approaches to the surgical treatment of congenital anorectal malformations, which emphasizes the relevance of further study of this issue. The informed consent of the patient was obtained for conducting the studies. Key words: congenital anorectal malformation, classification, children.

Characteristics of Anorectal Malformations in Children at the United States‐Mexico Border

Anorectal malformations (ARMs) are a group of congenital malformations affecting the lower gastrointestinal, urogenital, and/or gynecological systems. They occur in approximately 1 in 5000 live births, with a slight male predominance. ARMs can appear in isolation or in association with other anomalies. The literature contains little information about the characteristics of ARMs in United States Hispanic populations. An institutional review board-approved chart review was conducted in all patients with ARMs seen at a single institution in El Paso, Texas, from January 2012 to December 2015. Data regarding demographics, types of ARMs, associated syndromes, and long-term complications were recorded using the Krickenbeck classification system. Of the 37 patients included in the study, 20 were boys and 17 were girls. The most common ARMs were rectoperineal fistula (46%), rectourethral fistula, and cloacal malformation. Constipation was the most common long-term complication, especially in patients with recto-perineal fistula. Children in the Borderland community may have a higher percentage of recto-perineal fistula than previously reported. Postoperative complications in Hispanic children with ARM were comparable to those reported in other populations.

STRUCTURE OF THE PERIANAL REGION IN CHILDREN WITH ANORECTAL MALFORMATIONS

Objective: To study the morphological factors in the structure of the abdominal part of the colon and perineum in anorectal malformations in children. Material and methods: In 10 patients (1 day-2, 5 years) with anorectal developmental defects, a complex histological study of myo- and neurostructures of abdominal fragments from the rectum was performed, which were removed during surgery, with proctoplasty for the correction of the congenital pathology of the anorectal region. The study group consisted of 2 patients with unsupported forms of vice, 7 patients with fistula in the urinary system and one child with fistula per perineum. Results of the study. The search for the study was focused on revealing the fibers of the transversely striated muscle tissue of the subcutaneous part of the external anal sphincter, as one of the factors of its structure (presence). It was determined that the structural elements of the structure of the perianal region and pelvic floor in children with anorectal defects have certain morphological features, namely: the skin and subcutaneous fat, at the location of the necessary location of the external anus, in 80% of cases did not contain the muscular elements of the surface part of the external anal sphincter; the wall of the apical part of the rectum was characterized by hypoplasia of the mucosa and in most cases of the muscle layer with the fullness of their vessels and the presence of autonomic intermuscular ganglia. Conclusion. The structural elements of perianal area and pelvic bottom anatomy of children with аnorectal development defestes have morphological peculiarities are certain, namely: in the place of needed location of external anal hole the skin and hypodermic-fatty cellular had not contain the muscular elements of superficial part external anal sphincter at 80% cases; the wall of apical part of rectum characterized by the hypoplasia of mucous membrane and the muscular layer with the plethora of their vessels in most cases and presence of vegetative intermuscular neuroganglions.

Outcome of Laparoscopic Treatment of Anorectal Malformations in Children.

Operative correction of anorectal malformations (ARMs) remains a challenge in pediatric surgery. The study aimed to evaluate the outcomes of laparoscopic treatment of ARM in children. From 2007 to 2014, we performed 104 laparoscopic-assisted anorectal pull-through procedures in children with a mean age of 11.3±0.4months and ARMs. Clinical assessment, surgical durations, complications, and postoperative outcome were investigated. The mean duration of the operation was 126.5±17.2min. Mean intraoperative bleeding was 20±5.7g. Three (2.9%) children required conversion to laparotomy. One (0.9%) child developed a pelvic abscess, requiring an additional intervention. Three to six months after laparoscopic-assisted anorectal pull-through, 72 patients were hospitalized for stoma closure. Good functional results were achieved in 39 (54.2%) patients. On examination of the perineum, 11 (15.3%) patients were found to have mucosal prolapse. The circular symmetric anal reflex to tactile stimulation was confirmed in 53 (73.6%) patients and a tactile weakened anal reflex in 5 (6.9%) children. At the 1-year follow-up, constipation was present in nine (12.5%) patients and soling in two (2.8%) patients. A barium enema study performed after the operation showed good outcomes in 67 (93.1%) patients and fair outcomes in 5 (6.9%) patients. None had a poor outcome. Our experience confirms that laparoscopic-assisted anorectal pull-through enabled complete correction of ARM in the meanwhile avoiding damage to the rectum and anus. Also, the technique of double bipolar myostimulation of muscle complex in laparoscopic ARM might lead to these better results.

Late Diagnosis of Anorectal Malformations in Children

Anorectal malformations (ARM) include a wide spectrum of congenital defects of the anus, anal canal and rectum, which are often associated with congenital anomalies of other organ systems. They appear with different clinical presentations ranging from simple imperforate anal membrane to very complex defects. They are usually detected immediately after birth or early at neonatal age, but some patients with the milder defects are diagnosed at a later age. Objectives: The aim of this study is to analyze the incidence of patients with ARM diagnosed after neonatal period, clinical features and consequences of delayed diagnosis. Materials and Methods: Since 2010 we performed a combined retrospective and prospective analysis of a total of 76 patients with anorectal malformations, diagnosed and treated at our clinic between 2005 and 2014. We analyzed patients’ age at the time of diagnosis, sex, type of defect, associated anomalies and postoperative functional outcome. Results: 18 patients with ARM (23.68%) were diagnosed beyond the neonatal period. Of them 11 were female (61.11%) and 7 male patients (38.89%). The earliest diagnosed patient was a male of three months of age, while the latest diagnosed patient was a female of 8.5 years of age. All patients had low type anomalies, presented with anal stenosis (16), perineal fistula (1) and vestibular fistula (1). Six patients (33.33%) were also diagnosed with associated anomalies. Conclusion: High incidence of delayed diagnosis of ARM highlights the importance of a careful, comprehensive clinical examination of the perineum of newborns. All patients with severe and chronic constipation must be evaluated for eventually missed diagnosis of low type anorectal malformations.

Constipation is a Major Complication after Posterior Sagittal Anorectoplasty for Anorectal Malformations in Children

To assess whether constipation or fecal incontinence is a major late complication after posterior sagittal anorectoplasty in patients with anorectal malformation (ARM). We retrospectively enrolled 188 children, 85 low-type ARM (L-ARM) and 103 high-type ARM (H-ARM), who had complete medical records of bowel habits and medication histories after posterior sagittal anorectoplasty for anorectal malformation in Mackay Memorial Hospital. Stool characteristics as well as physical and medication history were evaluated. The symptom severity (SS) scoring system was used to assess changes in bowel habits. During a mean follow-up period of 4.3 years, constipation was found to be the most common late complication in both groups of patients (64.5% in the L-ARM group and 78.6% in the H-ARM group). Compared to constipation, stool incontinence was much less frequent, with 4.7% in L-ARM and 3.9% in H-ARM. There was no significant difference in mean SS scores between the two groups. Constipation was the most common late sequela in children after correction of ARM in our study.

Laparoscopic repair of anorectal malformations at the Red Cross War Memorial Children's Hospital: taking stock

BackgroundThe current standard repair for anorectal malformations in children is a posterior sagittal anorectoplasty. Recently, laparoscopic-assisted anorectoplasty (LAARP) was performed at the Red Cross Children's Hospital. MethodsA detailed case note review was conducted. Patient outcome was prospectively evaluated by colorectal nurse specialists using the Krickenbeck standardized questionnaire. Comparison among patients undergoing posterior sagittal anorectoplasty was performed. ResultsBetween September 2005 and June 2009, 24 children underwent LAARP. Sixteen had associated anomalies, including 7 children with renal and 4 children with cardiac abnormalities. Median age at surgery was 7.5 months (range, 2.6-15.0 months). Subtypes of anorectal malformation were as follows: vestibular, 2; bulbar, 9; prostatic, 7; vesical, 3; and with no fistula, 3. There was a 16% early complication rate. Redo-anoplasty was required in 9 patients. Eleven children had difficulties with follow-up. Thirteen children had regular follow-up and were analyzed further. Toilet training had been completed in 7 children (median age, 4.3 years; range, 3.5-6 years). Six children developed voluntary bowel motions. Six children are awaiting toilet training or are unable to train because of incontinence. ConclusionsAnal stenosis was the most common complication post-LAARP. Etiology appeared to be multifactorial, but poor compliance with dilatations was a leading cause.

How did the surgeons treat neonates with imperforate anus in the eighteenth century?

Anorectal malformations (ARMS) are one of those challenging topics of pediatric surgery. The developments in assessing and approaching patients with these anomalies have been made in the last decades and the methods described in older textbooks functioned as a guide in planning these attempts (Kiely and Peña in Pediatric surgery, Mosby, Missouri, pp 1425-1449, 1998; Grosfeld in Anorectal malformations in children, Springer-Verlag, Berlin, pp 3-15, 2006). The aim of this study is to present the attitude of a surgeon of eighteenth century to the treatment of anorectal malformations, and the evolution in the history of the anomaly. The part about imperforate anus in a textbook of surgery, found in a second-hand bookstore, was translated. The description and the classification of the anomaly, the methods of approaching these cases together with some case reports were presented and compared with today's practice. The historical background of the anomaly was evaluated not only with regard to the book of Heister specifically but also to the other data obtained in the literature. The anomaly was reported to be "not rarely" observed. The obstetricians were warned to examine a newborn baby completely for early diagnosis. The classification of the anomaly was made according to the properties of the membrane covering the anus but prompt treatment, initiating with its simple excision, was suggested in all types. Better results in cases whose anus was covered with a thin, delicate membrane were reported. The results show that routine neonatal examination for all babies was recommended in this Textbook of Surgery which had been published 260 years ago. The physical deterioration due to delay was well described. A broad classification of imperforate anus was made and successful outcome in low-type anomalies of today was reported with some case samples. It is clear that all the efforts starting from Soranus until today improved the understanding of the anomaly. Combining previous information with today's practice in the meetings focused totally on anorectal malformations, where the leading surgeons shared their experiences and re-evaluated the problems encountered, enlightened the future status of this interesting topic of pediatric surgery.

Mortality and morbidity associated with late diagnosis of anorectal malformations in children

The aim of this study was to highlight the timing of diagnosis of anorectal malformations and the morbidity and mortality associated with any delay. We retrospectively reviewed all patients with anorectal malformations presenting to the Children's Hospital, Leicester between 1998-2005. Patient demographics included age at diagnosis, presenting complaint, type of anorectal malformation and sex. Associated malformations were carefully recorded as well as morbidity and mortality occurring as a consequence of the delay in diagnosis. Out of a total of 52 patients, 28 (53%) had delay in diagnosis of the malformation. This delay was associated with significant morbidity in 19% and sadly included two deaths (4%), both being directly related to the missed diagnosis. Despite the established, routine, systematic pre-discharge examination in all neonates, a significant proportion of anorectal malformations are not diagnosed at this time. This delay is the cause of significant morbidity and mortality. Simple perineal examination will clearly identify the existence of all anorectal malformations and therefore such morbidity and mortality is totally avoidable.