An otherwise healthy 4-week-old term female of Japanese heritage presented with a 1-week history of asymptomatic progressive, generalized skin lesions. The lesion morphology, distribution, and dermatopathology result was consistent with Sweet syndrome. The patient was found to have a congenital type H rectovestibular fistula. This case highlights the rare association of rectovestibular fistula in neonatal Sweet syndrome which has only been described in neonates of Japanese heritage.

BACKGROUND: Female anorectal malformation is a correctable congenital defect. Delayed manifestations in patients with anal deformities are uncommon, especially after adolescence.
METHODS: The clinical data of a 19-year-old adult female patient with congenital anal atresia accompanied by rectovestibular fistula as the main manifestation was retrospectively analyzed. Diagnosis was made based on the patient\'s clinical symptoms, signs, imaging showing the fistula, X-ray and magnetic resonance imaging results. The preoperative examination was improved. Anorectoplasty was performed. The patient exhibited an improvement in quality of life and presented no evidence of fecal incontinence during the 6-mo follow-up.
CONCLUSIONS: Transfistula anorectoplasty is a reasonable and reliable surgical method for the treatment of adult congenital anal atresia and rectovestibular fistula.

UNASSIGNED: To assess the effect of megarectum on postoperative defecation of female patients with congenital rectovestibular fistula or rectoperineal fistula.
UNASSIGNED: From March 2013 to February 2021, 74 female patients with congenital rectovestibular fistula or rectoperineal fistula were treated. The age of patients ranged from 3 months to 1 year. Barium enema and spinal cord MRI were performed in all children. 4 patients were removed from the study because of spinal cord and sacral agenesis. Finally, 70 patients underwent one-stage anterior sagittal anorectoplasty (ASARP). Anal endoscopy and anorectal pressure measurement were performed 1 year after surgery. All patients were divided into two groups depending on the presence of megarectum (+) and (-) and observed for constipation and anal sphincter function.
UNASSIGNED: 16 patients (4 months to 1 year) were complicated with megarectum, and 5 patients (3 months to 9 months) were without megarectum. The incision infection was seen in 3 patients. All patients were followed up for 1 year to 5 years. Fecal soiling was seen in 2 patients and constipation in 14 patients. Among 16 patients with megarectum, soiling was seen in 1 patient and the constipation in 12 patients. Among 54 patients without megarectum, soiling was seen in 1 patient and constipation in 2 patients. There was a significant difference in the incidence of postoperative constipation between the two groups (megarectum (+) 75% vs. megarectum (-) 3.7% (P < 0.05)). However, there was no significant difference in the score of anal sphincters between the two groups (P < 0.05). And there was no significant difference in anal resting pressure (P = 0.49) and length of anal high pressure area (P = 0.76). 7 patients with constipation and megarectum acquired normal anal function after the dilated rectum was resected.
UNASSIGNED: Megarectum increases the possibility of difficult postoperative defecation in the patients with congenital rectovestibular fistula or rectoperineal fistula. However, constipation was not associated with ASARP postoperative effects on sphincter function. Resection of megarectum is helpful to the improvement of constipation.

UNASSIGNED: congenital Recto vestibular fistula represents the commonest type of anorectal malformation in females. The treatment of this anomaly is mainly approached either through anterior or posterior sagittal ano-rectoplasty approach. Several perioperative factors may affect the outcome. One of major postoperative complications is the occurrence of wound infection. We aimed to study the effect of delayed vs. early enteral feeding on the occurrence of perineal wound infection (PWI) after repair of congenital recto vestibular fistula.
UNASSIGNED: Fifty-five infants with recto-vestibular fistula were included. They were managed by single stage anterior sagittal anorectoplasty (ASARP) at an age ≥3 months. Groups A and B included infants who started oral intake on the 6th and 2nd postoperative days respectively. Group A infants were kept on peripheral parenteral nutrition (PPN) during the fasting period.
UNASSIGNED: Superficial wound infection occurred in three cases in group A while it developed in seven cases in group B. Deep perineal infection occurred in two and five cases in group A and group B respectively. The mean hospital stay was 8 days in group A vs. 13 days in group B when PWI developed.
UNASSIGNED: Delayed enteral feeding with PPN keeps the perineal wound less contaminated with stool. This promoted proper and fast healing with lower incidence of PWI. Also, PPN compensates the catabolic effects of both surgical trauma and fasting during the postoperative period and ensures maintenance of normal levels of essential nutrients that allow for proper healing.

BACKGROUND: Wound dehiscence of the anocutaneous anastomosis and/or reconstructed perineal body is a feared perioperative complication after posterior sagittal anorectoplasty (PSARP). Dehiscence of the perineal body can have long-term negative implications for fecal continence, sexual intimacy, obstetrical outcomes, and the need for reoperative surgery. We describe a modification to the traditional PSARP for repair of an imperforate anus with a rectovestibular, perineal, or absent fistula by sparing the perineal body, eliminating postoperative perineal body dehiscence and potentially improving long-term functional outcomes.
METHODS: A retrospective review of female patients with a rectovestibular, perineal, or absent fistula who underwent PSARP with sparing of the perineal body was performed.
RESULTS: Six patients underwent PSARP with sparing of the perineal body between 2019 and 2022. There were no perioperative complications. At follow-up, all patients are having regular bowel function without difficulty and have an excellent appearance of their perineal body.
CONCLUSIONS: PSARP for a rectovestibular, perineal, or absent fistula can be safely performed with a more limited incision to avoid disruption of the perineal body. This eliminates the need for perineal body reconstruction during the procedure, thereby preventing wound dehiscence. Given the significant long-term adverse sequelae of wound dehiscence and resultant inadequate perineal body, we believe this modification to the PSARP warrants strong consideration.

BACKGROUND: VACTERL association is defined by the presence of 3 or more anomalies in any of the following systems: vertebral, anorectal, cardiac, trachea-esophageal, renal, or limb. This study hypothesized that the presence of VACTERL association would correlate with an increased risk of gynecologic anomalies in patients with anorectal malformation (ARM).
METHODS: This study is a cross-sectional, retrospective analysis from the prospectively collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The 834 female patients with ARM who were enrolled in the registry by January 1, 2020 were included in this study. The relationship of VACTERL association with presence of a gynecologic anomaly was evaluated with Fisher\'s exact test. The relationship of each VACTERL system with presence of a gynecologic anomaly was assessed in patients with cloaca, rectovestibular fistulas and rectoperineal fistulas. P-values reported were based on a 2-sided alternative and considered significant when less than 0.05.
RESULTS: 834 patients with ARM underwent VACTERL screening and gynecologic evaluation with the three most common subtypes being cloaca (n = 215, 25.8%), rectovestibular fistula (n = 191, 22.9%) and rectoperineal fistula (n = 194, 23.3%). A total of 223 (26.7%) patients with ARM had gynecologic anomalies. VACTERL association was seen in 380 (45.6%) of patients with ARM. Gynecologic anomalies were present in 149 (39.1%) vs. 74 (16.3%) of subjects with vs. without VACTERL association (p < 0.001). VACTERL association did not significantly increase the risk of gynecologic anomaly in patients with cloaca and VACTERL (n = 88, 61.5%) vs. cloaca without VACTERL (n = 39, 54.2% p = 0.308). VACTERL association increased the risk of gynecologic anomalies in patients with rectoperineal fistulas (n = 7, 14.9% vs n = 9, 6.1% p = 0.014) and rectovestibular fistulas (n = 19, 31.1% vs. n = 13, 10.0% p<0.001). In patients with ARM who had a VACTERL association, when one of the associated anomalies was renal, there was an even higher risk of having an associated gynecologic anomaly (n = 138, 44.2% vs. n = 85, 16.3% p<0.001).
CONCLUSIONS: VACTERL association in patients with rectoperineal and rectovestibular fistulas correlates with an increased risk of gynecologic anomalies. The presence of VACTERL associated findings, especially renal, should prompt a thorough evaluation of the gynecologic system.
METHODS: III. Retrospective comparative study.

BACKGROUND: Outcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome.
METHODS: A multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4-7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups \'poor\' ≤ 11, and \'fair\' 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome.
RESULTS: The study included 111 RVF-patients. Median BFS was 16 (range 6-20). The \'below normal\' group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management.
CONCLUSIONS: Although median BFS at 4-7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management.
METHODS: Level III.

OBJECTIVE: The purpose of this study was to compare the postoperative outcomes of neonatal versus delayed repair of rectoperineal and rectovestibular fistulae using a multi-center pediatric colorectal specific database. We hypothesized that the incidence of 30-day postoperative complications is not significantly different between these two surgical treatment strategies.
METHODS: We performed a retrospective, observational study of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) database. We included any patient from the database that underwent primary surgical repair of a rectoperineal or rectovestibular fistula. Neonatal repair was defined as occurring within 14 days of birth, and delayed repair as occurring after that period. The primary outcome was the occurrence of postoperative complications within 30 days.
RESULTS: 164 patients were included in the study (123 rectoperineal, 41 rectovestibular); the majority (81%) were repaired in a delayed fashion. Patients that underwent delayed repair had lower birth weights and were more likely to be female than those that underwent neonatal repair. Wound breakdown/dehiscence was the most common complication in both groups (Delayed 5.3% v. Neonatal, 6.5%). We found no significant difference in the incidence of any postoperative complication between groups (Delayed 6.0 v. Neonatal 6.5%, p = 1.0).
CONCLUSIONS: We concluded there was no significant difference in the incidence of 30-day postoperative complications for neonatal versus delayed repair of rectoperineal and rectovestibular fistulae, suggesting that both strategies are safe and may have excellent short-term outcomes in appropriately selected patients.

OBJECTIVE: To evaluate the long-term results of surgical correction of H-type fistula in girls with a normal anus.
METHODS: There were 7 patients with rectovestibular fistula and 3 patients with rectovaginal fistula with a normal anus were observed from 2014 to 2019 in the Surgical Department No. 1 of the Russian Children\'s Clinical Hospital. Upon admission, all patients underwent genital examination, vaginoscopy, rectal examination and probing the fistulous canal, irrigography, abdominal and retroperitoneal ultrasound. They were also examined by a gynecologist and genital smears were obtained. Surgical treatment was determined depending on the height and diameter of the fistula for each child. One patient underwent perineal fistulectomy, three patients - anterior anorectoplasty. Invaginated fistula extirpation, abdominoperineal proctoplasty and perineal fistulectomy using a pad flap between the defects were used in two cases, respectively. Patients were followed-up for the period from 6 months to 1 year after the last recurrence. Follow-up examination, irrigography and functional examination of sphincter were performed.
RESULTS: Two (20%) patients did not require redo surgery. In 6 (60%) cases, recurrences didn\'t occur within a year after the second surgery, in 2 (20%) cases - after 3 operations. Recurrent H-type fistula appeared after 3 of 4 perineal fistulectomy procedures, 3 of 9 anterior anorectoplasty, 2 of 2 abdominoperineal proctoplasty and 2 of 3 invaginated fistula extirpation. Hypotension of internal anal sphincter and neo-rectal ampulla, recurrent vulvovaginitis were diagnosed in 2 patients in 6 months after anterior anorectoplasty.
CONCLUSIONS: We recommend anterior anorectoplasty and perineal fistulectomy using a pad flap between the defects for the treatment of H-type fistula to minimize the risk of recurrence.
UNASSIGNED: Оценка отдаленных результатов хирургической коррекции H-фистул у девочек при нормально сформированном анусе.
UNASSIGNED: Семь пациенток с диагнозом «ректовестибулярный свищ» и 3 пациентки с диагнозом «ректовагинальный свищ» при нормально сформированном анусе находились под наблюдением и прооперированы в хирургическом отделении №1 РДКБ. При поступлении в отделение произведены осмотр наружных половых органов, вагиноскопия, ректальное исследование и зондирование свищевого хода, ирригография, УЗИ органов брюшной полости и органов забрюшинного пространства, проведены консультация гинеколога и исследование отделяемого мочеполовых органов. Для каждой пациентки подобрана тактика оперативной коррекции состояния в зависимости от высоты расположения и диаметра свища: иссечение свища промежностным доступом — у 1 пациентки, брюшно-промежностная проктопластика, иссечение свища промежностным доступом с использованием прокладки между дефектами и инвагинационная экстирпация свища — выполнены каждая у 2 пациенток, передняя аноректопластика — у 3 пациенток. Наблюдение за пациентками проводилось от 6 до 12 мес после последнего рецидива: проведены контрольный осмотр, ирригография и функциональное исследование сфинктерного аппарата прямой кишки.
UNASSIGNED: У 2 (20%) пациенток мы смогли скорректировать состояние, проведя одну операцию по устранению свища, у 6 (60%) пациенток рецидивы не отмечены в течение года после второго оперативного вмешательства, у 2 (20%) пациенток — после 3 операций. После 3 из 4 операций иссечения свища промежностным доступом, 3 из 9 передних аноректопластик, 2 из 2 брюшно-промежностных проктопластик и 2 из 3 инвагинационных экстирпаций свища отмечен рецидив H-фистулы. У 2 пациенток через полгода после передней аноректопластики диагностированы гипотония внутреннего сфинктера и неоампулы прямой кишки, рецидивирующий вульвовагинит.
UNASSIGNED: Для минимизации риска рецидива H-фистулы рекомендуем проведение передней аноректопластики или иссечение свища промежностным доступом с использованием прокладки между дефектами.

Long term survival of animals with major congenital anomalies is very rare. This report documents the 18-month survival of a dog with multiple anomalies including atresia ani. An 18-month-old black Cocker Spaniel bitch was presented for evaluation of prolapsed glands of the third eyelid involving both the eyes. Clinical examination revealed a single perineal opening, fecal matter in the vestibule, distended abdomen, hypoplastic vulva, and the absence of a tail without any neurological deficits. Abdominal contrast radiography revealed a distended colon with fecal stasis, rectovestibular fistula, termination of the rectum as a blind pouch, lumbar scoliosis due to block vertebrae, and the presence of only two hypoplastic coccygeal vertebrae. The case was diagnosed as atresia ani type II with rectovestibular fistula, hypoplastic vulva, lumbar scoliosis, and anury, in the global context of a caudal regression syndrome. The wide aperture fistula, connected to the vestibule, undamaged spinal cord and sacrum without any neurological deficits were the favorable prognostic factors that maintained continence and allowed the dog to survive to adult life with these anomalies. Thus, an appropriate bowel management program and specialty care can improve the quality of life and longevity of this animal.