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OBJECTIVE: Patients with anorectal malformations (ARMs) may have concurrent gynecologic abnormalities. As patients grow, they typically transition from pediatric subspeciality care and seek adult OB/GYN related services. We aimed to assess adult OB/GYN physicians' knowledge, competency, and comfort meeting the sexual and reproductive health care needs of patients with ARM. METHODS: We performed a cross-sectional observational survey-based study of graduates from a single academic OB/GYN residency program from 2013-2022. Physicians were surveyed on experience, comfort, and challenges caring for patients with ARMs and given a knowledge assessment. Descriptive and comparative statistics between those who did and did not complete a pediatric and adolescent gynecology (PAG) rotation were generated. RESULTS: There were 59 respondents (53.6%). Fewer than half (39.0%) report caring for a patient with ARM, an appendicovesicostomy (12.3%) or an appendicostomy (5.4%). Most felt uncomfortable (80.4%) or felt they lacked competence caring for these patients (81.8%). The majority (64.3%) felt ARMs should be discussed in residency. Only one physician (1.7%) answered all questions in the knowledge assessment correctly; 33.9% did not answer any question correctly. On subgroup analysis, more physicians completing a PAG rotation recalled learning about ARMs (83.3 vs 51.9%, p=0.03); however, there were no differences in experience, comfort, competence, or willingness to learn. CONCLUSION: OB/GYN providers report lack of knowledge and comfort in caring for patients with ARMs. Development of a standardized OB/GYN residency curriculum and education for practicing OB/GYN physicians is necessary to allow access to knowledgeable sexual and reproductive health for this patient population.

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PURPOSE: Congenital anorectal stenosis is managed by dilations or operative repair. Recent studies now propose use of dilations as the primary treatment modality to potentially defer or eliminate the need for surgical repair. We aim to characterize the management and outcomes of these patients via a multi-institutional review using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. METHODS: A retrospective database review was performed using the PCPLC registry. The patients were evaluated for demographics, co-morbidities, diagnostic work-up, surgical intervention, current bowel management, and complications. RESULTS: 64 patients with anal or rectal stenosis were identified (57 anal, 7 rectal) from a total of 14 hospital centers. 59.6% (anal) and 42.9% (rectal) were male. The median age was 3.2 (anal) and 1.9 years (rectal). 11 patients with anal stenosis also had Currarino Syndrome with 10 of the 11 patients diagnosed with a presacral mass compared to only one rectal stenosis with Currarino Syndrome and a presacral mass. 13 patients (22.8%, anal) and one (14.3%, rectal) underwent surgical correction. Nine patients (8 anal, 1 rectal) underwent PSARP. Other procedures performed were cutback anoplasty and anterior anorectoplasty. The median age at repair was 8.4 months (anal) and 10 days old (rectal). One patient had a wound complication in the anal stenosis group. Bowel management at last visit showed little differences between groups or treatment approach. CONCLUSION: The PCPLC registry demonstrated that these patients can often be managed successfully with dilations alone. PSARP is the most common surgical repair chosen for those who undergo surgical repair. LEVEL OF EVIDENCE: III.

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INTRODUCTION AND IMPORTANCE: Anal stenosis following hemorrhoids surgery is rare, and very distressing to the patients. It is graded according to its severity namely mild, moderate or severe. Majority of severe cases require surgery. The treatment is complex shown by the multiplicity of flaps and techniques described in the literature. This case report then reminds surgeons about the complications of hemorrhoidectomy and the complexity of their management. In addition, it challenges the surgeons about their practice on hemorrhoids. CASE PRESENTATION: A 30-year-old male who presented with difficulty in passing stool with intermittent low abdominal pain for 4 weeks following a surgery for hemorrhoids. A House Advancement Flap was designed to match the transverse incisions and hence the width of the mucosal defect to be replaced. Any intra- or postoperative complications were noted and the patient was discharged on the second postoperative day from the hospital. Patient had no complaints in the post-discharge period. CLINICAL DISCUSSION: Overzealous hemorrhoidectomy is one of the primary causes of stenosis of anal canal. Other causes that have been reported include anorectal diseases, other anorectal surgeries and radiotherapy. Physical examination majorly leads to the diagnosis of anal stenosis. Its treatment is conservative in mild cases while in severe anal stenosis a House Advancement Flap is one of the possible surgical management options. CONCLUSION: Anal stenosis is a rare condition which requires good evaluation for its better management. Given its simplicity and successful results, a House Advancement Flap anoplasty is a reliable treatment of severe anal stenosis.

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Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive disorder characterized by a rash that progresses to poikiloderma. Other common features include sparse hair, eyelashes and eyebrows, short stature, variable skeletal abnormalities, dental defects, cataracts, hypogonadism, and an increased risk for cancer, especially osteosarcoma and skin cancer. RTS is caused by biallelic pathogenic variants in ANAPC1 (Type 1 RTS) or RECQL4 (Type 2 RTS). We present an African girl with Type 2 RTS caused by a nonsense variant and an intronic variant in RECQL4. The patient presented precocious puberty, which has not been previously reported in RTS and that was treated with a GnRH analog, and anal stenosis, which has only been reported once. This case highlights the need to consider deep intronic variants in patients with RTS when pathogenic variants in the coding regions and exon/intron boundaries are not identified and expands the phenotypic spectrum of this disorder.

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PURPOSE: Anorectoplasty and pull-through procedure can be performed with extensive mobilization or tension anastomosis, which can compromise bowel blood perfusion. We aimed to analyze the hypoxia biomarker values and histopathological findings in both conditions to correlate the occurrence of anal stenosis and defecation disorders in experimental models. METHODS: We created anorectal reconstruction models with impaired vascularization of the anorectum (group I) and tension anastomosis (group II) in rats. A third group of animals underwent sham operation (group III) and another as controls (group IV). Hypoxia biomarker values were assessed in all groups. The histopathological changes on the postoperative days 3 and 35, anal stenosis and defecation disorders on day 35 were compared. RESULTS: Hypoxia biomarker values confirmed postoperative ischemia in groups I-III compared to control. Group I and II rats had a similarly pronounced ischemia with histopathologic changes in the anorectum on the postoperative day 3 and accompanied by severe fibrosis on day 35. Compared to the sham operation, both groups showed defecation disorders with significant anal stenoses. CONCLUSION: Extensive rectal mobilization to about the same extent as tension anastomosis has a major impact on postoperative rectal ischemia, resulting in severe fibrotic changes in the anorectum and defecation disorders in the long term.

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AIM: The optimal surgical treatment for anatomical anal stenosis (AS) remains to be determined. The aim of this study was to determine the rates of complications and recurrence after anoplasty for anatomical AS and, wherever feasible, compare the outcomes for the various techniques. METHOD: A PROSPERO-registered systematic review was reported following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Medline, PubMed, Embase, Cochrane Library of Systematic Review, Scopus and Web of Science were searched for articles published up to May 2021. Studies that assessed the outcomes of anoplasty in adult patients with anatomical AS were selected. The primary outcomes were complications and recurrence. The methodological quality of studies was appraised using the Joanna Briggs Institute critical appraisal tools. RESULTS: From the total of 2705 unique screened records, 151 were assessed for eligibility. Only 29 studies (two prospective) met the inclusion criteria, reporting data on 556 patients [mean age 53 (18-83) years, 46% female]. Previous history of surgery for haemorrhoidal disease accounted for three quarters of cases. A total of 14 types of anoplasty were found, with the Y-V flap being the most performed technique [27% of cases (n = 149)]. Complications frequently occurred, with a pooled prevalence of 10.2% (95% CI 3.9%-24.1%) after Y-V flap and 11.5% (5.3%-23.0%) after rhomboid/diamond flap. Patients undergoing house flap achieved better results in terms of clinical improvement, satisfaction and quality of life compared with Y-V flap and rhomboid/diamond flap. When considering only studies with at least 12 months of follow-up, the pooled prevalence of recurrence was 4.7% (2.2%-9.8%), with significantly higher rates observed in the prospective versus retrospective series [pooled prevalence 18.9% (11.5%-29.5%) vs. 3.6% (1.7-7.8%), respectively; p < 0.001]. CONCLUSION: Both complications and recurrence were significantly lower after house flap compared with rhomboid/diamond and Y-V flap. Better designed multicentre studies with longer follow-up are needed to confirm these findings. PROSPERO REGISTRATION NUMBER: CRD42021239493.

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In the past, an anteriorly located anus was often misdiagnosed and treated as an anorectal malformation (ARM) with a perineal fistula (PF). The paper aims to define the criteria for a normal anus, an anterior anus (AA) as an anatomic variant, and milder types of ARM such as congenital anal stenosis (CAS) and PF. An extensive literature search was performed by a working group of the ARM-Net Consortium concerning the subject "Normal Anus, AA, and mild ARM". A consensus on definitions, clinical characteristics, diagnostic management, and treatment modalities was established, and a diagnostic algorithm was proposed. The algorithm enables pediatricians, midwives, gynecologists, and surgeons to make a timely correct diagnosis of any abnormally looking anus and initiate further management if needed. Thus, the routine physical inspection of a newborn should include the inspection of the anus and define its position, relation to the external sphincter, and caliber. A correct diagnosis and use of the presented terminology will avoid misclassifications and allow the initiation of correct management. This will provide a reliable comparison of different therapeutic management and outcomes of these patient cohorts in the future.

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Anal stenosis, which develops as a result of aggressive excisional hemorrhoidectomy, especially with the stoutly use of advanced technologies (LigaSure®, ultrasonic dissector, laser, etc.), has become common, causing significant deterioration in the patient's quality of life. Although non-surgical treatment is effective for mild anal stenosis, surgical reconstruction is unavoidable for moderate to severe anal stenosis that causes distressing, severe anal pain, and inability to defecate. The problem in anal stenosis, unlike anal fissure, is that the skin does not stretch as a result of chronic fibrosis due to surgery. Therefore, the application of lateral internal sphincterotomy does not provide satisfactory results in the treatment of anal stenosis. Surgical treatment methods are based on the use of flaps of different shapes and sizes to reconstruct the anal caliber and flexibility. This article aims to summarize most-used surgical techniques for anal stenosis regarding functional and surgical outcomes.

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Anorectal strictures are a notoriously difficult to treat phenotype of perianal Crohn's disease. Quality of life is diminished due to ongoing pain, incontinence, difficulty with stool evacuation, and recurrent medical and surgical treatments. Medical therapy is aimed at treating luminal disease and mucosal ulceration to prevent worsening of fibrosis. Clinical examination and endoscopic intervention can be used for serial dilations of strictures. Unfortunately, despite optimal medical therapy and endoscopic intervention with serial anal dilations, surgery with intestinal diversion or proctocolectomy may be required as part of the treatment algorithm in a significant proportion of patients.

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INTRODUCTION: After hemorrhoidectomy, anal stenosis occurs, which is an uncommon but severe consequence. The majority of severe cases require advancement flap anoplasty. PRESENTATION OF CASE: A 50-year-old female patient with a history of hemorrhoidectomy 10 months prior to admission complained of difficulty defecating, pain, and incomplete evacuation sensation, as well as a hole on the right side of the anal canal through which feces unintentionally passed. On the physical examination, we found that the anal lumen was partially obstructed, which did not allow the insertion of a finger. There was an impression of a perineal fistula at 5 and 7 o'clock, which was connected to the anal canal 3 cm from the edge of the anus. The patient was diagnosed with severe anal stenosis with perianal fistula. The patient underwent fistulectomy and advancement flap with perianal skin. In the outpatient follow-up clinic in the first and second weeks, the patient showed no complications, and no recurrence of her complaints was found. DISCUSSION: Several corrective surgical techniques have been applied to restore a healthy lining to the constricted portion of the anal canal. We performed a combination of simple cutaneous advancement flap and fistulectomy to manage the patient with severe anal stenosis following hemorrhoidectomy with concurrent anal fistula. CONCLUSION: A combination of fistulectomy and simple cutaneous advancement flap anoplasty is a simple, safe, and effective surgical option for the management of severe anal stenosis with concomitant anal fistula.

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AIM: Lateral internal sphincterotomy (LIS) remains a standard for chronic anal fissure even though other surgical techniques have shown high efficacy. Faecal incontinence is a well-documented complication of LIS. We devised modified open posterior internal sphincterotomy (m-OPIS) with sliding skin graft (SSG), which is a combined procedure of OPIS and anal advancement flap. The aim of this study is to evaluate m-OPIS+SSG. METHODS: This was a retrospective, observational, single-arm study. m-OPIS+SSG was performed for chronic anal fissure and anal stenosis. m-OPIS involved incision of the internal sphincter muscle at the posterior midline until four fingers could be passed. The incision wound was closed by anastomosis of the anoderm and skin. Then, an arcuate skin incision was created and the skin graft was advanced into the anal canal. Follow-up was conducted by clinical consultation and telephone interview. Faecal continence was assessed by Cleveland Clinic Faecal Incontinence (CCFI) score. RESULTS: m-OPIS+SSG was performed in 143 patients. The mean patient age was 50±16 years. The success and overall recurrence rates after m-OPIS+SSG were 99% and 0.7%, respectively, with a median follow-up period of 16.3 years. One patient developed incontinence with liquid stools once during the 6-month period. None of the other patients suffered permanent faecal incontinence postoperatively. The postoperative CCFI score was 0.5±0.9. CONCLUSIONS: We consider m-OPIS+SSG as one of the efficacious options of procedure for chronic anal fissure and anal stenosis, owing to its high success rate, low recurrence rate and no postoperative complication of serious faecal incontinence.

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INTRODUCTION AND IMPORTANCE: Anal stenosis is narrowing of anal canal that may result from true anatomic stricture or functional stenosis. Anal stenosis from irritant chemical application for hemorrhoid is rare and tends to be more severe. There is scarcity of data in the literature regarding anal stenosis secondary to traditional medicine application for the treatment of hemorrhoid. This case report can create awareness to promote health education and health advancement, especially in areas with wide spread use of traditional medicine. In addition, it can motivate general surgeons to prepare themselves to handle such cases in the absence of colorectal surgeons. PRESENTATION OF THE CASE: A 75 years old male farmer presented with worsening of difficulty of passing feces and flatus and intermittent abdominal distention of 3 months. The patient has history of application of irritant chemical by traditional healer for treatment of hemorrhoid. Physical examination led to diagnosis of severe anal stenosis. Bilateral house advancement flap anoplasty done by a general surgeon with excellent result. DISCUSSION: The commonest cause of anal stenosis is hemorrhoidectomy. Other causes include other anorectal surgeries, anorectal diseases, and radiotherapy. Diagnosis of anal stenosis is by physical examination. Treatment is conservative for mild cases and advancement flap anoplasty for moderate to severe cases. CONCLUSION: Health education and health advancement can create awareness, hence preventing people from having wrong treatments. House advancement flap anoplasty is a good option for the treatment of anal stenosis in resource limited setup, as it is easy to do and has good outcome.

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Background Hemorrhoids are a common condition that presents with bleeding per rectum, pain at rest and defecation, mucosal discharge, and prolapse. Surgical hemorrhoidectomy is the treatment method of choice for Grade 3 and Grade 4 hemorrhoids. Hemorrhoidectomy is associated with postoperative pain and no single surgical technique has been proved to significantly reduce the pain. We analyzed in our study the effect of lateral internal sphincterotomy with hemorrhoidectomy on postoperative pain, anorectal function, and retention of urine after the Milligan and Morgan technique. Methods This randomized, prospective, and comparative study included 200 Grade 3 and Grade 4 hemorrhoids patients who were scheduled for surgical management. The patients were classified randomly into two groups with an equal number of participants: Group A underwent Milligan & Morgan open hemorrhoidectomy and Group B underwent lateral internal sphincterotomy (LIS) in addition to Milligan and Morgan open hemorrhoidectomy. Postoperative pain was recorded using the Visual Analog Scale (VAS) score for up to 48 hours. Postoperative bleeding, urinary retention, and bowel and gas incontinence were noted. Long-term follow-up at six and 24 months for anal stenosis, anal fissure, incontinence, and recurrence was also noted. Results Patients who underwent LIS showed a significant reduction in postoperative pain at 12 hours (p=0.0008*), 24 hours (p=0.000*), and 48 hours (p=0.003*); the time taken to request rescue analgesia was similar between the two groups (p=0.07). Side effects, such as postoperative bleeding and urinary retention, were significantly lower after LIS (p=0.001* and p=0.01*, respectively), and gas incontinence was significantly higher after LIS (p=0.002*). The long-term outcomes of anal fissure were significantly higher without LIS at six months (p=0.02*) and 24 months (p=0.04*) and those of anal stenosis were significantly higher without LIS at six months (p=0.04*). Conclusions From our study, we conclude that postoperative pain, bleeding, and urinary retention were significantly lower after LIS, and gas incontinence was transient. The long-term outcomes, which included anal stenosis and anal fissure, were significantly lower after LIS. However, bowel and gas incontinence and recurrence were not altered. Therefore, we conclude that the addition of LIS to hemorrhoidectomy improves patient outcomes in terms of postoperative pain and anorectal function.

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BACKGROUND: Colorectal anastomosis using the double stapling technique (DST) has become a standard procedure. However, DST is difficult to perform in patients with anal stenosis because a circular stapler cannot be inserted into the rectum through the anus. Thus, an alternative procedure is required for colorectal anastomosis. CASE PRESENTATION: A 78-year-old woman presented with bloody stool. Colonoscopy and computed tomography revealed advanced low rectal cancer without lymph node or distant metastasis. We initially planned to perform low anterior resection using a double stapling technique or transanal hand-sewn anastomosis, but this would have been too difficult due to anal stenosis and fibrosis caused by a Milligan-Morgan hemorrhoidectomy performed 20 years earlier. The patient had never experienced defecation problems and declined a stoma. Therefore, we inserted an anvil into the rectal stump and fixed it robotically with a purse-string suture followed by insertion of the shaft of the circular stapler from the sigmoidal side. In this way, side-to-end anastomosis was accomplished laparoscopically. The distance from the anus to the anastomosis was 5 cm. The patient was discharged with no anastomotic leakage. Robotic assistance proved extremely useful for low anterior resection with side-to-end anastomosis. CONCLUSION: Performing side-to-end anastomosis with robotic assistance was extremely useful in this patient with rectal cancer and anal stenosis.

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BACKGROUND: Hemorrhoidal disease represents one of the most common anorectal disorders in the general population. Energy devices, such as LigaSureTM scalpel, have reshaped the concept of hemorrhoid surgery and in turn, have improved patient outcomes and simplified the work of surgeon. OBJECTIVE: The study aims to evaluate the outcomes of LigaSureTM hemorrhoidectomy (LH) analyzing main post-operative complications rate, length of stay, operating time, and time to return to work. METHODS: In this monocentric descriptive study, from June 2001 to February 2019, 1454 consecutive patients, treated with LH for grade III and IV hemorrhoids, were analyzed. Complications were classified in early, late, and long-term if they occurred within 1 month, between 1 and 2 months or after 2 months, respectively. RESULTS: 90.2% of patients were treated in day surgery regimen and the mean operating time was 14.3 minutes. The post-operative pain decreased from 3.7 mean VAS on the 1st postoperative day to 0.1 mean VAS on 30th post-operative day. Early complications rate was 2.1%: urinary retention accounted for 1.8% of patients. 0.3%-of patients experienced postoperative bleeding and only one required reoperation. Late complications rate was 5.8%: anal stenosis, incomplete healing, and anal fissure were detected in 3.6%, 1.2%, and 1% of patients, respectively. The long term complications rate was 5.3%: anal fistula, soiling, perianal abscess, and recurrence were identified in 0.2%, 0.1%, 0.3%, and 4.8% of patients, respectively. CONCLUSION: LH is a safe and fast procedure with a proven minimal complication rate.

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BACKGROUND: To date, only 23 cases of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with duplex kidney have been reported. We present the first reported case of MRKH syndrome with solitary duplex kidney and anal stenosis. CASE: A 17-year-old Chinese girl presented with primary amenorrhea and fully developed secondary sex characteristics. Ultrasonography of the abdomen and pelvis revealed the absence of the right kidney, a left duplex kidney, and a primordial uterus. Surgery for anal stenosis was performed when she was 1 year of age. The patient had a normal 46, XX karyotype.

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AIM: Anal stenosis (AS) is a rare but disabling disorder that often represents a complication of anorectal surgery. The aim of our study was to assess the safety and functional outcome of a modified rhomboid flap (MRF) in the treatment of moderate and severe AS. METHODS: Between January 2002 and September 2017, 50 consecutive patients with moderate and severe AS who underwent an MRF were retrospectively included. Anal continence (Cleveland Clinic Incontinence Score) and symptoms (Obstructed Defaecation Syndrome Score) were assessed preoperatively and postoperatively at 12 months. Furthermore, anal calibre was measured both preoperatively and postoperatively at 1, 6 and 12 months. RESULTS: The mean follow-up period was 97 ± 48.3 (33-180) months. The main aetiology was a previous excisional haemorrhoidectomy (N = 23; 46%). The mean preoperative anal calibre was 9.96 ± 2.68 (5-15) mm and there was a statistically significant improvement in all three periods (P < 0.0001) of postoperative evaluation (1, 6 and 12 months) with a mean difference, obtained comparing preoperative and 12 months anal calibre, of 14.1 ± 2.72 (P < 0.0001). Statistically significant improvement in both Cleveland Clinic Incontinence Score and Obstructed Defaecation Syndrome Score was observed in all patients at 12 months. The overall success rate was 96% (48/50 patients). CONCLUSION: The use of an MRF is a safe and suitable option for the treatment of moderate and severe AS. The possibility of tailoring the flap, based on the degree as well as the level of AS, is the key.

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Anal stenosis (AS), which is a very rare but serious complication, usually develops after hemorrhoidectomy. It may be mild, moderate or severe depending on the grade of stenosis. While mild and moderate types can be monitored with conservative treatment, most of the severe type require advancement flap anoplasty. There are several commonly used flap types such as Y-V, diamond and house-type flaps, but the best results belong to house-type flaps. In this paper, we aimed to present a case of severe post-hemorrhoidectomy AS, in which we performed house advancement flap anoplasty, with its video demonstrating the procedure step by step.

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BACKGROUND: Congenital pouch colon (CPC) is a rare variant of anorectal malformation. In male patients, CPC communicates distally with the urogenital tract by a large fistula. The CPC cases which do not fulfill the criteria as mentioned in the classical description are reported along with the pertinent literature review. MATERIALS AND METHODS: This was a retrospective study from January 2004 to December 2017 of male children with Type IV CPC. We evaluated clinical presentation, primary management, anatomical relationship, previous surgical intervention, definitive management, result, and outcome in terms of continence status on Templeton score. RESULTS: Fifty-one children were included in the study among whom 36 children (Group 1) had a colovesical fistula and 15 children (Group 2) had no communication of the pouch with the genitourinary tract. In Group 2 children, the clinical presentations and management were varied: 4 underwent primary pull-through procedure, whereas 11 underwent staged procedure. Group 2 included three children in whom a narrow and thin-walled anal canal or anal canal with lower rectum was present, which was incorporated during the pull-through procedure. On continence assessment, only one child in Group 1 had "good" continence score compared to four children (three having anal canal) in Group 2. CONCLUSION: CPC Type IV can present without genitourinary tract communication (fistula), contrary to its emblematic description. Awareness about anatomical variations and adaptation of surgical technique accordingly is vital. The identification of the anal canal with or without the lower part of the rectum (even though apparently narrow and thin walled) and incorporation of this in bowel continuity lead to better outcomes in terms of continence.