Rectovestibular Fistula Research Articles

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.

Congenital pouch colon in girls: Genitourinary abnormalities and their management.

Aims:To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years.Materials and Methods:Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management.Results:Initial examination of the external genitalia showed a “clover-leaf” appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a “high” position (n = 14) or at a relatively normal or “low” position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls.Conclusions:GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.

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.

Chronic Constipation and Megarectum in a 14-Year-Old Patient Operated of Anorectal Malformation—Case Report

The aim of this paper is to present the case of a female patient, 14 years of age, who developed a megarectum and overflow of fecal incontinence as a consequence of chronic and severe constipation, after the surgical repair of her vestibular fistula at the age one. The huge rectum was resected than dissected down to the pelvic floor and a new anus was reconstructed via a posterior sagittal anorectoplasty with excellent results.

Intracannal pain remission in child with amelogenesis imperfecta. Case report

BACKGROUND AND OBJECTIVES: Amelogenesis imperfecta is characterized by enamel structural defects, which may severely affect dental structure in both dentitions. When accompanied by severe decay, it may impair the pulp complex requiring endodontic treatment and in case of incomplete root formation treatment becomes more complex due to pulp volume and anatomic conditions. This study aimed at reporting a clinical case of patient with amelogenesis imperfecta and with multiple incomplete root formation as a consequence. CASE REPORT: Female patient, 12 years old, leucodermic, with painful symptoms, who has looked for dental assistance. At intraoral clinical evaluation teeth presented with shape and size changes, yellowish color, covered by a thin enamel layer with roughened surface and absent in some areas, with anterior vestibular sulcus fistula and without edema. At radiographic evaluation, both dentitions were affected by the abnormality with delayed chronology of permanent teeth eruption. At the end of all evaluations, patient was diagnosed with amelogenesis imperfecta, and periodontal treatment was started, followed by endodontic treatment. CONCLUSION: Amelogenesis treatment is complex, especially when in more advanced stages of dental structure destruction. However, it is possible to reestablish patient’s functionality and esthetics with good planning and multidisciplinary approach.

About the surgical treatment of vestibular window abnormalities

The objective of the present study was to develop the sparing strategy for the surgical treatment of the patients presenting with malformations of the vestibular window based on the results of the evaluation of synopty of the main clues of the internal and middle ears. This article contains the analysis of the effectiveness of the surgical treatment of 17 patients with malformations of the vestibular window operated during the period from 2008 till 2013. It presents the results of investigations into the topographic relationships between the principal structures of the internal and middle ears of special importance for the assessment of the possibilities for the surgical treatment of such patients with the minimal risk of development of postoperative complications. The results of the surgical treatment were estimated based on the data of audiological studies in the early and late postoperative periods. It was shown that none of the patients developed the symptoms of dizziness, unstable gait or sensorineural impairment of hearing in the immediate postoperative period. Good functional results in the form of the reduced bone-air interval (15.2±1.0 dB) were achieved in 13 patients. Nevertheless, the considerable decrease in the sound conductivity in the late postoperative period observed in 64.7% of the patients related to the closure of the vestibular fistula dictates the necessity of developing the novel techniques for its prevention.

Using an operating microscope to re-treat an inferior premolar with two canals

This report aims to present a clinical case of endodontic re-treatment performed on a left inferior premolar with two canals of difficult localization. The importance of anatomical knowledge and the use of an operating microscope, especially in complex reintervention are discussed. An endodontic re-treatment was performed on element 34, which had a prosthetic restoration and vestibular fistula. In clinical examination, it was observed a negative result to the sensitization test and a positive result to vertical percussion test. After all the obturating material was removed, an operating microscope was requested to assess a possible fracture or the presence of another canal. It was noticed the presence of another canal that was treated and obturated as the existing canal. After 12 months, a new radiographic exam was performed, whose result was found asymptomatic and radiographically normal. In complex peculiar anatomy cases, the operating microscope is vital for the localization of extra canal to guarantee treatment success.

PATTERN OF ANORECTAL MALFORMATIONS AND EARLY OUTCOMES OF MANAGEMENT AT MOI TEACHING AND REFERRAL HOSPITAL ELDORET-KENYA.

Objectives: To describe the anatomical sub-types of Anorectal malformations, their management and the early outcome at Moi Teaching and Referral Hospital (MTRH) over a 16 month period. Design: A prospective study. MTRH, in the neonatal Unit and paediatric surgical wards for theinitial capture of patients and initial follow up. The Paediatric Surgical out-patient clinic was used for the subsequent follow ups. All infants diagnosed with ARM (Anorectal malformations) at MTRH from November 2011 to April 2013. Sub-types of the Anorectal malformations, coexisting abnormalities morbidity and mortality rates. There were 42 participants including 24 (57%) males and 18 (43%) females. Neonates presented at an average age of 4 ± 3, three days and older children presented on average age of 152 ± 118, three days. There were 30 (71%) neonates and 12 (29%) older infants. In males, the predominant sub-type was imperforate anus without a fistula found in ten participants (42% of males). In females, the predominant sub-type was recto-vestibular fistula found in 14 participants (78% of females). Mortality occurred in 13 (31%) participants among them ten (24%) had coexisting abnormalities. The main causes of morbidity were: colostomy complications in four (9.5%); wound,infections in one (5%); and wound dehiscence in one (5%). Patients with Anorectal malformations presented late at MTRH. The diagnosis at birth was missed in babies born at home as well as those delivered in health institutions.

Variants of Currarino Syndrome: Embryological Association and Review of Pertinent Literature

Currarino syndrome is a triad of sacral defect, anorectal malformation and a presacral mass. The diagnosis is usually made late in childhood and about 50% of cases are familial with autosomal dominant inheritance. We present two neonates (one with vestibular fistula, and another with cloacal malformation) with the features consistent with Currarino syndrome, but with Altman’s type II sacrococcygeal teratoma, that is, presacral mass having an external sacrococcygeal component also. We believe that this triad should be considered a variant of Currarino syndrome. In first case, excision of the mass along with coccyx, followed by primary Posterior Sagittal AnoRectoPlasty was performed in the same setting. The patient succumbed to death due to septicemia as a result of wound sepsis. Learning from the previous experience, we decided to do a diverting sigmoid loop colostomy followed by posterior sagittal excision of the mass along with coccyx, in same sitting in the second case. There was no recurrence. Though HLXB9 has been identified as the major causative gene in Currarino syndrome, exact pathogenesis is still unclear. We herein highlight the significance of this variant of Currarino syndrome and propose a theory on the basis of an embryological association between the malformation complex.

Hypermethylation of SHH in the pathogenesis of congenital anorectal malformations

ObjectiveThis study sought to examine promoter methylation and expression of the identified sonic hedgehog (SHH) gene in terminal rectal tissues of children with congenital anorectal malformations (ARMs). MethodsTissue samples from the terminal rectum of pediatric patients with ARMs (five cases each of high and intermediate malformation — two cases of rectovesical fistula, two cases of rectourethral prostatic fistula, one case of cloaca with >3cm common channel, four cases of rectourethral bulbar fistula and one case of imperforate anus without fistula, respectively, and ten cases of low malformation — five cases of perineal fistula and five cases of vestibular fistula, respectively), and patients with non-gastrointestinal tract malformation (six cases, anal fistula) were collected and divided into three groups: high-intermediate ARM (ARMhi-int), low ARM (ARMlo), and control (Cont.). Real-time RT-PCR was used to detect mRNA expression levels of the verified differentially methylated gene SHH, and bisulfite genomic sequencing was performed to evaluate DNA methylation in the SHH promoter region. ResultsThe average methylation levels of the SHH promoter were significantly higher in ARMhi-int (0.850±0.030, P=0.0036) and ARMlo (0.540±0.053, P=0.0087) groups than in Cont. group (0.280±0.032). SHH mRNA expression levels were lower in ARMhi-int (0.340±0.015, P=0.0065) and ARMlo (0.530±0.042, P=0.0156) groups than in Cont. group (0.870±0.046). The average methylation levels of the SHH promoter were higher in ARMhi-int group than in ARMlo group (0.850±0.030 vs. 0.540±0.053, P=0.0095), while SHH expression was significantly reduced in ARMhi-int group compared to ARMlo group (0.340±0.15 vs. 0.530±0.042, P=0.0252). The methylation levels of the SHH promoter in ARMhi-int group were negatively correlated with SHH gene expression (r=−0.89, P<0.01). ConclusionsThe SHH gene, which plays a major role in the development of the anorectum and enteric nervous system, is hypermethylated at its promoter, and this is correlated with low levels of SHH gene expression. This epigenetic modification may therefore be responsible for the observed changes in SHH expression, which could in turn underlie the pathogenesis of congenital ARMs.

Single Stage Correction of Rectovestibular Fistula

Introduction: The aim of this study was to determine the feasibility, safety, cost effectiveness and functional outcome between single stage and multiple stage operation of anorectal malformation (ARM) with rectovestibular fistula (RVF).Materials &amp; Methods: This interventional study was conducted on 40 female children born with ARM with RVF from July 2007 to June 2009 in the Department of Pediatric Surgery, Dhaka Medical College Hospital. Their age range was from 3 months to 7 years. Anterior sagittal anorectoplasty (ASARP) was done as definitive treatment of RVF. 20 out of 40 patients underwent single stage procedure and the rest underwent multiple stage procedure. Good preoperative bowel preparation and postoperative wound care were ensured for optimum outcome.Results: Mean operating time was 70 minutes in single stage and 80 minutes in multi stage definitive procedure. Average hospital stay was 10 days in single stage procedure where as 22-24 days in multi stage procedure. Partial perineal wound dehiscence occurred in two patients with single stage procedure and one patient with multi stage procedure. Wound infection was seen in two patients of single stage and also two patients of multi stage procedures. Fecal continence was good in all patients older than 3 years. Average number of bowel movement was 2-3 times daily in older patients and 2-4 in younger patients. Constipation developed in three patients with multiple stage procedure and in two patients with single stage procedure. Both groups were managed conservatively for all complications. In Dhaka Medical College Hospital, treatment cost is mostly free, so we could not compare this cost but the incidental expenses in single stage treatment was Tk. 10,000.00 and Tk. 30,000.00 in multistage treatment in three settings.Conclusion: Adequate preoperative bowel preparation, good surgical skill, proper analgesia and postoperative wound care remain the key factors to produce a cost effective excellent outcome of single stage correction of RVF.DOI: http://dx.doi.org/10.3329/jpsb.v1i2.19523

Comparison Between One Stage Correction of Vestibular Fistula by Transfistula Anorectoplasty (TFARP) and Anterior Sagittal Anorectoplasty (ASARP)

Background: Various innovative approach have been tried for the surgical management of vestibular fistula, most of them have post operative complication like wound infection, wound dehiscence, more hospital stay which compromise the functional out come, financial burden and aesthetic appearance of the perineum. This article details a new technique, Transfistula Anorectoplasty (TFARP), which includes minimal dissection without interruption of perineal body and perineal skin.Materials and methods: This cross sectional comparative study on 43 patients with vestibular fistula conducted in Dhaka Shishu (Children) Hospital from September 2008 to April 2010, about 20 months. Patients were operated according to parent choice after obtaining informed consent and standard bowel preparation. Data on demographics, operation time and ostoperative complications were analyzed systematically by SPSS program. Patients were followed up for a period of 2½ months postoperatively.Results: There is no statistical difference in patient population regarding age, geographical distribution and clinical presentation. Mean operation time was 76.5 min for TFARP and 84.34 min for ASARP. Two cases had wound infection after TFARP operation and 11 patients after ASARP operation. One patient developed partial wound dehiscence after TFARP and was healed after conservative treatment within 7 days. On the other hand 5 patients developed partial wound dehiscence and 4 patients developed complete wound disruption after ASARP which were also treated conservatively need more than 02 week on an average. Mean hospital stay were 6.45 days after TFARP operation and mean hospital stay were 7.87 days after ASARP peration. Twenty neonates and infant who were treated by TFARP operation have good bowel movement without laxative and symmetrical anal contraction after stimulation.Conclusion: TFARP is an operation which produces less morbidity and is more effective and superior procedure than that of ASARP operation and gives better aesthetic appearance of the perineum.DOI: http://dx.doi.org/10.3329/jpsb.v1i2.19525

Spinal ultrasound in patients with anorectal malformations: is this the end of an era?

Even if lumbar magnetic resonance imaging (MRI) is considered the gold standard in the diagnosis of occult spinal dysraphism (SD) in patients with anorectal malformations (ARMs), spinal ultrasound (US) performed up to 5 months of life have been largely used as a screening test. The aim of the present study was to evaluate the accuracy in terms of sensibility and specificity of neonatal US to detect occult SD in patients with ARMs. Retrospective analysis of all patients treated for ARMs between 1999 and 2013 at our institution who underwent both spinal US (up to 5 months of life) and MRI. Sensibility and specificity have been calculated for US based on MRI results. Of 244 patients treated for ARMs at our institution, 82 (34 females, 48 males) underwent both the imaging studies and have been included in this study. ARMs types were: anal stenosis (7), recto-vestibular fistula (19), recto-perineal fistula (3) and cloaca (5) in female and imperforate anus (7) recto-perineal fistula (14), recto-urethral fistula (22), recto-vesical fistula (5) in males. Forty-seven patients (57, 3 % of total, 18 females, 29 males) had some occult SD (tethered spinal cord, spinal lipoma, syringomyelia) at MRI. Only 7 (14, 8 %) patients of those with spinal anomalies at MRI had pathological US studies. In our population, sensibility and specificity of US for diagnosis of occult SD were, respectively, 14, 8 and 100 %. Since it is well known that a screening test must have a high sensibility, our data suggest that spinal ultrasound is not suitable as a screening test for occult spinal dysraphism in patients with ARMs. Furthermore, we strongly advise against the use of US as a screening test for spinal dysraphism to prevent a false sense of security in physician and patients' families.

The influence of anorectal malformations on fertility: a systematic review.

This systematic review aims to give an overview of available evidence concerning the influence of anorectal malformations (ARM) on fertility. We conducted a search in PubMed, EMBASE and Cochrane Library conformed to the PRISMA standards. All studies reporting on fertility and ARM were included. 2,905 studies were identified. Based on title, abstract and full text, nine articles on 429 patients remained to answer the research question. Childbirth rate was the only reported outcome parameter to describe fertility. An overall childbirth rate of 27 % (range 0-57 %) was found. Mean age at time of study ranged from 23 to 35 years. There was no statistical significant difference in childbirth rate between female and male patients, based on seven studies (p = 0.45). Patients with a more complex type of ARM (imperforated anus without fistula, rectourethral bulbar and prostatic fistulas, rectobladderneck fistulas and cloacal malformations) had a lower childbirth rate compared to healthy controls, whereas in patients with a less complex ARM (rectoperineal or rectovestibular fistula) the childbirth rate was similar to healthy controls. Patients with a more complex type of ARM had a significant lower childbirth rate than patients with a less complex type of ARM (18 vs 47 %, respectively) (p = 0.0001). When further dividing these patients by gender, this difference was only seen in female patients (p = 0.04). In patients with a more complex type of ARM a lower childbirth rate was found compared to healthy controls and patients with a less complex type of ARM. The latter was only seen in female patients. However, conclusions concerning fertility in ARM patients have to be taken with caution due to limited quality of the studies. Further investigation is recommended.

Whole-exome resequencing reveals recessive mutations in TRAP1 in individuals with CAKUT and VACTERL association

Congenital abnormalities of the kidney and urinary tract (CAKUT) account for approximately half of children with chronic kidney disease and they are the most frequent cause of end-stage renal disease in children in the US. However, its genetic etiology remains mostly elusive. VACTERL association is a rare disorder that involves congenital abnormalities in multiple organs including the kidney and urinary tract in up to 60% of the cases. By homozygosity mapping and whole exome resequencing combined with high-throughput mutation analysis by array-based multiplex PCR and next-generation sequencing, we identified recessive mutations in the gene TNF receptor-associated protein 1 (TRAP1) in two families with isolated CAKUT and three families with VACTERL association. TRAP1 is a heat shock protein 90-related mitochondrial chaperone possibly involved in antiapoptotic and endoplasmic reticulum-stress signaling. Trap1 is expressed in renal epithelia of developing mouse kidney E13.5 and in the kidney of adult rats, most prominently in proximal tubules and in thick medullary ascending limbs of Henle’s loop. Thus, we identified mutations in TRAP1 as highly likely causing CAKUT or CAKUT in VACTERL association.

Rectovestibular fistula with vaginal atresia: our experience and a proposed course of management.

Rectovestibular fistula with coexisting vaginal atresia poses a surgical dilemma with regard to the timing and type of reconstruction. We present our experience and suggest an appropriate course of management. Seven patients with rectovestibular fistula and coexisting vestibular atresia were operated in our hospital during January 2004 through December 2013. The details of their bowel, menstrual and sexual functions were recorded. Five of the seven patients who underwent anoplasty in childhood presented to us in their teens with primary amenorrhea and cyclical abdominal pain. All five had sigmoid colon neovaginoplasty. Four of these had the uterus or its remnants anastomosed to the neovagina. All four have regular menstrual cycles. One patient is sexually active and has satisfactory sexual function. The bowel function in all the five patients is good. The remaining two patients presented in their infancy and had the anorectovestibular fistula left as the neovagina. The recto-sigmoid was pulled down to form the neoanus. Both these patients have bowel incontinence. We recommend the rectovestibular fistula be used as the neoanus and not as the neovagina. Delayed bowel vaginal replacement has excellent results and allows for optimal assessment of functioning uterine body or remnants.

A study of Craniospinal Imaging in Patients with Anorectal Malformation

Introduction: Anorectal malformations are commonly associated with craniospinal abnormalities like hydrocephalus, tethered cord syndrome, occult spinal dysraphism, syringomyelia, meningomyelocele and Arnold Chiari malformation. Aim of our study was to identify the incidence of craniospinal anomalies in patients with anorectal malformation and to analyse utility of Ultrasound and MRI as a screening tool in these patients to detect such anomalies. Material and Method: 52 infants male and female of anorectal malformation were included. All underwent ultrasonography of brain and spinal cord and 27 patients underwent MRI to detect craniospinal anomalies. Result: Out of 52 cases 38(73.07 %) were males and 14(26.93 %) were females. High type ARM (34.61 %) and Vestibular fistulas(19.23 %) were most common type in male & female child respectively. On ultrasonography in this series no craniospinal abnormality was found. On ultrasonography subarachnoid space was normal in all cases and central canal diameter was found <1cm in all cases. No abnormalities were detected in posterior fossa like cistermagna dilatation, dilated ventricles, Chiari Malformation. MRI spine was done 27 patients with high and low type malformation, but no abnormality was detected. Conclusion: Craniospinal anomaly and tethered cord are not frequently found in patients with anorectal malformation, in Northern Indian Population. As no association was found between craniospinal anomalies and anorectal malformation in the present study, the use of Ultrasonography and MRI as a screening tool is not recommended unless clinically indicated.

Unusual presentation of a rectovestibular fistula as gastrointestinal hemorrhage in a postmenopausal woman.

Background. Anorectal malformations (ARMs) are extremely rare and are usually identified neonatally. It is unusual for these cases to present in the postmenopausal period. This case report describes a postmenopausal patient with ARM and rectovaginal hemorrhage. Case. An 86-year-old, gravida 11, para 9, presented to the emergency department complaining of profuse postmenopausal vaginal bleeding. Her gynecologic history was significant only for an unclear history of an anal abnormality that was noted at birth. Speculum examination revealed profuse rectal bleeding from a rectovestibular fistula exterior to her hymenal ring. Colonoscopic examination revealed severe diverticular disease. Conclusion. This patient was born with an imperforate anus which resolved as rectovestibular fistula and ectopic anus. This case presents a rare clinical circumstance which integrates the fields of obstetrics, gynecology, gastroenterology, and embryology alike.

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

Bowel symptoms in children with anorectal malformation — a follow-up with a gender and age perspective

BackgroundGender specific outcome for children with anorectal malformations (ARM) is rarely reported although it is important for medical care and in parent counseling. PurposeTo assess bowel function according to the Krickenbeck system in relation to ARM-subtype, gender and age. MethodAll children born with ARM in 1998–2008 and referred to two centers in two different countries were followed up. The bowel function in 50 girls and 71 boys, median age 8years, was analyzed. ResultsAmong those with a perineal fistula, incontinence occurred in 42% of the females and in 10% of the males (p=0.005) whereas constipation occurred in 62% of the females and 35% of the males (p<0.001). No bowel symptoms differed between the females with perineal and vestibular fistulas (p>0.3 for every symptom). Sacral malformations were associated with incontinence only in males with rectourethral fistulas. Constipation among the males differed between the age groups: 58% versus 26% (p=0.013). Bowel symptoms did not change with age among the females. ConclusionGender differences in outcome for children with ARM must be considered. Males with perineal fistulas had less incontinence and constipation than the females with perineal fistulas. The females with perineal and vestibular fistulas had similar outcomes.