Background and Objectives: Optimal surgical management of the neonate with imperforate anus depends on accurate determination of the level of the rectal pouch. The use of ultrasound to determine the pouch level has been previously described, but has not become widely accepted. The goal of this study is to determine the validity of this measurement according to final surgical findings in patients with anorectal malformation. Materials and Methods: In a cross sectional study,23 children with imperfo-rated anus were evaluated by transperineal ultrasound with a 7.5 MHz sector transducer in the supine and lithotomic positions to determine the pouch level and pouch to perineum (P-P) distance. In all cases, the level of the distal pouch was confirmed by definitive surgery or distal colostogram. The agreement between sonography and surgery or colostogram as gold standard was obtained by weighted kappa test. Results: In five children, the pouch to perineum (P-P) distance was less than 10mm. All of these low lesions were safely treated by a simple perineal anoplasty (minimal posterior sagittal anorectoplasty; i.e. minimal PSARP) and were confirmed as low type. Seven children had P-P distance of 10-15 mm. In the follow up definitive surgery, 5 cases were intermediate and two cases were high. Eleven children had a P-P distance of more than 15 mm, of which ten cases were high lesions and had colostomy at birth. During follow up, 8 cases underwent definitive surgery of PSARP and two of these high cases were confirmed by distal colostogram and one case was categorized as intermediate by definitive PSARP. Measure of agreement (Kappa) was calculated to be 0.791 (P = 0.001) Therefore, Ultrasound correctly predicted the level of the distal pouch in 20 of 23 patients.Conclusion: Transperineal ultrasound can be confidently used prior to surgical decision in children with imperforate anus in addition to physical exam.

Aone-day-old neonatal boy was admitted to the hospital to evaluate his imperforate anus. He was born prematurely (at 36 weeks of gestation) via normal vaginal delivery. He was the second child of non-consanguineous parents. The other sibling was healthy. The neonatal period had been uneventful...

Objectives: The present study aimed to investigate the efficacy of the ultrasound-guided wire localization of the anal tract and sphincter muscle complex in the patients with imperforate anus.Methods: This study was conducted on 20 patients (4 females and 16 males) with imperforate anus referring to the Doctor Sheikh and Akbar Pediatric Hospitals, Mashhad, Iran, between2016 and 2017. The trans-perineal ultrasonography was performed under general anesthesia to identify the location and direction of the anal tract, which was checked by a muscle stimulator. Then, the localization needle was inserted in the center of the anal pit and anal sphincter muscle complex until it reached the rectal pouch.Eventually, by using the wire as a guide, the rectum was brought to the middle of the sphincter complex through the minimally invasive pull-through procedure.Results: The anal sphincter muscle complex (concentric hypoechoic muscle) could be seen in all patients with the thickness of 1.8 -3.6mmand mean of 2.6mm. The anal pit (the multi-layer view) was visualized in all patients, except in two cases with clinically lowtype imperforate anus. Muscle stimulator findings showed that the wire was located in the middle of the sphincter muscle complex in all patients. Mean localization time was 38 min.Conclusions: The ultra sound-guided wire localization of the anal tract facilitated the implementation of a less invasive and anatomically corrected rectal pull-through operation in patients with imperforate anus.

In the last couple of years, with the advancement of imaging methods, the diagnosis of gray matter heterotopia has been more promising. Gray matter heterotopia is a rare disorder in the general population, but recent attention to its psychiatric aspects encouraged us to introduce a patient with a form of gray matter heterotopia, who suffers from the anatomical abnormalities with a variety of psychiatric disorders. Our patient is a 25-year-old woman, who presented a variety of organogenesis disorders such as imperforate anus and rectovaginal fistula with the presence of gray matter heterotopia and history of refractory mood and psychotic disorders during 9 past years. Because of the various clinical manifestations of the disease, syndromic attention to this disease seems to be helpful in diagnosing and treating its various aspects.

Congenital pouch colon (CPC) is an unusual condition in imperforate anus (IA), usually of high variety. This malformation is mostly reported from India, where it comprises about 7% of all anorectal malformations. In this anomaly, the normal colon is very short or absent and is replaced by a pouch like dilatation that usually communicates through a fistula with genitourinary tract or persistent cloaca. We are reporting on three cases of newborns (2 females, and 1 male) with this anomaly diagnosed during September 2001 to September 2004. These three cases comprise less than 3% of all cases of IA during this time period. The two girls had anorectal agenesia, short segment of colon and a pouch with vaginal fistula and persistent cloaca (Type II). Colon in the third case was replaced by a dilated pouch that communicated with a wide fistula to the bladder (Type I). This is very important for pediatric surgeons to be aware of the features of this condition to allow for proper diagnosis and surgical management.

Background: VACTERL association is a congenital abnormality involving several organs. The percentage of involvement of different organs in this illness varies and treatment success depends on the intensity of the accompanied anomalies. This study aimed to investigate the prevalence of VACTERL association in neonates Hospitalized in Be'sat Hospital in Hamadan, Iran. Materials and Methods: This retrospective study was conducted using the descriptive-analytic method and all the neonates who were hospitalized with esophageal atresia and imperforate anus abnormalities in Be'sat Hospital, Hamadan, Iran, from April 2009 to April 2018 were included in the study. Information on the neonates were extracted from the medical records and after being recorded in the checklist, they were analyzed using SPSS v. 16 at a 95% confidence level. Results: 127 neonates were included in this study. 42 neonates (33. 1%) had esophageal atresia, 78 (61. 4%) had imperforate anus, and 7 neonates (5. 5%) had both anomalies. 87 of these neonates (68. 5%) suffered from congenital heart disease. Atrial septal defect (31%), simultaneous presence of atrial septal defect and patent ductus arteriosus (24%), and patent ductus arteriosus (23%) were the most common congenital heart diseases, respectively. 32 neonates (25. 2%) had VACTERL association. Heart, genitourinary, and spinal anomalies were present in 93. 2%, 84. 3%, and 9. 3% of the neonates, respectively. VACTERL association was significantly observed in neonates with imperforate anus (P = 0. 001) Conclusion: VACTERL association is common in neonates who suffer from esophageal atresia or imperforate anus. Considering the high prevalence of anomaly of other organs, in addition to complete physical examination, echocardiography, abdominal ultrasound, and radiography of the spine are recommended.

The standard approach to management of high imperforate anus is colostomy in the newborn period followed by posterior saggital anorectoplasty (PSARP) at 6 to 12 months of age. The purpose of this study was to determine whether a one-stage repair by primary PSARP in the newborn period could be performed without clear determent to the patient’s functional result. Totally 30 newborns with high imperforate anus who underwent primary PSARP without colostomy were studied retrospectively. All cases were studied by sonography, echocardiography, lower vertebra X-ray and finally routine blood and urine laboratory tests. Incontinence was defined as fecal soiling at least twice a day, and patients requiring more than office dilatation were described to have stricture formation. All patients recovered well and were followed for periods ranging from 1.5 to 10 years. There were 3 cases of postoperative wound infection, but no anastomotic dehiscence, stricture formation and fistula recurrence were seen. In cases of constipation and fecal incontinence there were associated anomalies such as sacral dysplasia, kidney dysplasia, ventricular septal defect and vesicoureteral reflux. There were no complications in other cases. The most important factor is patient selection, and one-stage PSARP spares the patient the morbidity of additional surgeries in the standard multistage approach for high imperforate anus. These preliminary results suggest that one-stage PSARP is a safe and viable approach to the management of high imperforate anus without clear determent to future bowel function.

Background and Objective: Imperforate anus is a common anorectal malformation, which is associated with other anomalies. This study was done to determine the associated malformations in newborns with imperforate anus in northern Iran.Methods: This descriptive study was carried out on 40 hospitalized newborns (24 boys and 16 girls) with imperforate anus in Taleghani teaching hospital in Gorgan, northern Iran during 2006-10. Results: From 40 newborns with imperforate anus, 23 newborns (57.5%) had associated anomalies. The commonest associated anomalies was genitourinary (65.2%) followed by heart (47.8%), gastrointestinal (13%) and musculoskeletal anomalies (8.7%).Conclusion: Associated anomalies in newborns with imperforate anus in northern Iran have a high prevalence in comparison with other reports.

Dear editor, We have completed the study entitled “ Ultrasound Guided Wire Localization of Anal Tract in Imperforate Anus” by Alamdaran SA, Joudi M, et al. published in the Iran J Pediatr 2018; 28(4): e65338 (1). We would like to add some commentary to it....

Background: Tailgut cyst (TGC) is a rare congenital lesion that originates from remnants of the embryonic post-anal gut. It presents as a multilocular presacral mass mainly in young women. Microscopically, the cyst lining is composed of different types of epithelium such as stratified squamous, transitional, or glandular.Case Presentation: We present a term female newborn referred to our hospital for evaluation and management of imperforate anus. During dissection of the presacral space to release the rectum, a multicystic mass adherent to the distal part of rectum was detected and completely excised. Histopathology confirmed the TGC diagnosis.Conclusion: TGC is a very rare lesion, but it should be considered in differential diagnosis of any presacral mass, even in infancy. Complete excision is the preferred treatment and can be done more easily neonatally or in infancy.