Outcome of Single Stage Correction of High Variety of Anorectal Malformation.

The standard treatment of high variety anorectal malformation (ARM) is the staged approach. A growing interest in one stage correction of high variety ARM was noted recently. The aim of this study was to examine the feasibility, safety and outcome of single stage correction of High variety ARM. This prospective study was carried out in the Department of Paediatric Surgery, Mymensingh Medical College Hospital (MMCH), Mymensingh, Bangladesh from July 2012 to September 2013. It was conducted among 30 patients, all having high ARM. The type of ARM was diagnosed by clinical examinations and imaging studies like Invertogram and Transperineal USG. Other associated congenital anomalies were excluded. Out of 30 patients 11(36.67%) were male and 19(63.33%) were female. The age incidence ranges from 2 days to 5 months with the mean±SD age 1.70±1.63 months. The type of fistula present along with ARM in male varies from rectobulber urethral fistula, recto prostatic urethral fistula, recto vesical fistula and in one case without any fistula. Type of fistula was rectovestibular, rectovaginal and one cloacal malformation among the female patients. The treatment modalities in male patients vary in transabdominal pull through anorectoplasty and PSARP, in which the former was done more (72.73%). In case of female patients, treatment modalities vary in transabdominal pull through and anorectoplasty and transfistulous anorectoplasty, with the later done more (52.63%). Regarding post operative complications, there was 1(14.29%) wound infection in male and 1(33.33%) in female, partial wound disruption in 1(14.29%) male patient, mucosal prolapse 2(28.57%) in male and 1(33.33%) in female, anal stenosis 1(14.29%) in male and 1(33.33%) in female, perianal soiling in 1(14.29%) male patient. All the complications were treated by adopting various measures. One stage correction of High variety ARM in both male and female patients is technically feasible. The safeties of this approach depend on the adherence to strict inclusion criteria. The early post operative complications are acceptable and can be managed successfully.

One or Two Stages Procedure for Repair of Rectovestibular Fistula: Which is Safer? (A Single Institution Experience).

BACKGROUND: Rectovestibular fistula (RVF) is the most common type of anorectal malformations in females. The need for a diverting colostomy during correction of defect has ignited a heated debate. In this study, we reviewed the girls with RVF that had been treated by either one or two stage procedure in the past 10 years in our institution to define whether one stage or two stage procedures is safer and more beneficial for the patients. MATERIALS AND METHODS: Seventy girls with RVF that had been operated from January 2005 to January 2015 were studied retrospectively. Data were obtained from medical hospital records. The cases were divided into two groups. Group A (46 patients): were operated by two stages technique (simultaneous sigmoid colostomy and anterior sagittal anorectoplasty [ASARP]). Group B (24 patients): were operated by one stage (ASARP without covering colostomy). The short-term outcome as regard wound infection, wound dehiscence, anal stenosis, anal retraction, recurrence of fistula as well as complications of colostomy was reported. The long-term outcome as regard soiling, constipation and voluntary bowel movement was evaluated. RESULTS: The age of patients at the time of surgery ranged from 3 months to 2 years (mean; 9.5 months). In Group A, seven patients (15.2%) developed wound infection, two patients developed wound disruption. One patient developed anterior anal retraction and required redo-operation, anal stenosis was noticed in five (10.9%) patients. Complications from colostomy had occurred in nine patients (19.5%). In Group B, wound infection occurred in ten patients (41.7%). Seven patients (29.2%) developed wound disruption. Anal stenosis occurred in eight patients (33.3%). Five patients required redo-operation because of anal retraction in three patients and recurrence of fistula in the other two patients. Constipation recorded in 15 patients (32.6%) of Group A and in ten patients (41.3%) of Group B. Soiling was reported in six girls (13.04%) of Group A and five girls (20.8%) of Group B. CONCLUSION: The avoidance of colostomy is not outweighed achieving sound operation and continent child. Two stages correction of RVF is safer and more beneficial than one stage procedure, especially in our locality and for our paediatric surgeons during their learning curve.

Cloacal malformation patients report similar quality of life as female patients with less complex anorectal malformations.

PURPOSE: Cloacal malformations are the most complex type of anorectal malformation in females. This study aimed to report quality of life (QoL) of patients with a cloacal malformation for the first time in literature. MATERIALS AND METHODS: Female patients with an anorectal malformation participating in a follow-up program for congenital malformation survivors aged 5 or older were eligible for this study. QoL was assessed with the PedsQL™ 4.0 inventory. Scores of patients with a cloacal malformation (CM) were compared with those of female patients with rectoperineal or rectovestibular fistulas (RP/RV) and with reference data. RESULTS: A total of 59 patients (67% response rate; 13 patients with cloacal malformation) were included, QoL was assessed by patient self-report at median age of 12years (8-13), and by parent proxy-report at median age of 8years (5-12). There were no differences between groups regarding the presence of associated anomalies, with also no differences regarding anomalies in the urinary tract (CM vs. RP/RV=31% vs. 15%, p=0.237). Scores of the cloacal malformations group were similar to those of the comparison group, except the proxy-reported scores on school functioning (60.0 vs. 80.0, p=0.003). Proxy-reported scores of cloacal malformation patients were significantly lower than reference values on total QoL-score, psychosocial health, and emotional and school performance. Patients (irrespective of type of ARM) who suffered from fecal soiling reported significantly lower scores with regard to psychosocial health (71.7 vs. 81.7, p=0.034) and its subscale school performance (65.0 vs. 80.0, p<0.001). QoL-scores reported by cloacal malformation patients did not differ significantly from the reference values of the healthy population. Parents of cloacal malformation patients reported significantly lower total QoL, emotional and school performances, as well as a lower general psychosocial health for their children relative to reference data of healthy children. CONCLUSION: Patients with cloacal malformations and females with less complex anorectal malformations report similar QoL. Parents of cloacal malformation patients report more problems on several psychosocial domains relative to the healthy reference group. To monitor these matters, long-term follow-up protocols should contain multidisciplinary treatment including periodical assessment of psychosocial well-being.

[Congenital recto-vaginal fistula associated with a normal anus (type H fistula) and rectal atresia in a patient. Report of a case and a brief revision of the literature]. / Asociación de fistula rectovaginal congénita con ano normal (Fístulas Tipo H) y atresia rectal en una paciente. Reporte de un caso y breve revisi6n de la literatura.

Congenital recto-urogenital type fistulas with a normal anus and rectal atresia, represent both anorectal malformations that are infrequently seen in clinical practice. We describe the case of a girl with an association of these two anomalies, together with a double vagina who, on her seventh day of life, expelled feces through her genitals. The malformations were corrected by means of a posterior sagittal approach, descending from the rectum to the anus without perineal dissection. The vaginal septum was resected thru the vulva. There is no evidence of recurrence of the recto-vaginal fistula.

Asociación de fistula rectovaginal congénita con ano normal (Fístulas Tipo H) y atresia rectal en una paciente. Reporte de un caso y breve revisión de la literatura / Congenital recto-vaginal fistula associated with a normal anus (type H fistula) and rectal atresia in a patient. Report of a case and a brief revision of the literature.

Las malformaciones ano-rectales del tipo de fístulas recto-urogenitales congénitas con ano normal y atresia rectal son anomalías poco frecuentes. Se discute el caso de una niña con la asociación de estas dos entidades, acompañada además de una vagina doble, quien fue llevada a la consulta a los siete días de vida por la expulsión de heces a través de los genitales. Las malformaciones fueron corregidas a través de un abordaje sagital posterior, con descenso del recto hasta el ano sin disección perineal. El tabique vaginal fue resecado a través de la vulva. En la actualidad no hay evidencia de recurrencia de la fístula recto-vaginal.

Congenital recto-urogenital type fistulas with a normal anus and rectal atresia, represent both anorectal malformations that are infrequently seen in clinical practice. We describe the case of a girl with an association of these two anomalies, together with a double vagina who, on her seventh day of life, expelled feces through her genitals. The malformations were corrected by means of a posterior sagittal approach, descending from the rectum to the anus without perineal dissection. The vaginal septum was resected thru the vulva. There is no evidence of recurrence of the recto-vaginal fistula.

Rectovestibular fistula with normal anus: a treatment alternative.

Congenital rectovestibular fistulas with normal anus are a rare form of anorectal malformations, especially in the Western hemisphere. Due to its rarity, consensus on preoperative management, surgical technique and postoperative care is still in debate. We describe a specific case with its management plan and outcomes while providing an up to date literature review on current management trends.

The diagnostic value of MRI fistulogram and MRI distal colostogram in patients with anorectal malformations.

Contrast fistulogram (FG) and distal pressure colostogram (DPCG) are standard diagnostic methods for the assessment of anorectal malformations. Pelvic magnetic resonance imaging (MRI) earned a place among essential diagnostic methods in preoperative investigations after the Currarino syndrome and a high incidence of associated spinal dysraphism were described. The aim of our study was to evaluate the possibility of substituting FG and DPCG by a modified pelvic MRI, e.g. MRI fistulogram (MRI-FG) and MRI colostogram (MRI-DPCG). The prospective study involved 29 patients with anorectal malformations who underwent a modified pelvic MRI. The length and course of fistulas and rectum, and the presence of sacral anomalies were studied on MRI images and compared with images obtained by radiologic examinations. Modified MRI brought identical results as contrast studies in 25 patients when related to the fistula and rectum length and course. MRI was more accurate for the detection of sacral anomalies. MRI-FG was the only imaging method used in the four most recent patients. The results support the assumption that conventional contrast examinations for the assessment of anorectal malformations can be replaced by MRI, thus reducing the radiation dose.

Anterior sagittal anorectoplasty: An alternative to posterior approach in management of congenital vestibular fistula.

BACKGROUND: Better exposure, possibility of extension if needed and precise placement of the anal canal within the external sphincter complex have made the posterior and anterior sagittal approaches more popular and established for the correction of anovestibular fistula. The mini posterior sagittal anorectoplasty (PSARP) was the procedure of choice for female ARM at our center till date. As an alternative surgical option, we performed anterior sagittal anorectoplasty (ASARP) in 15 cases of anovestibular fistula and compared them with 12 cases of vestibular fistula operated by PSARP technique. PATIENTS AND METHODS: Fifteen female infants with vestibular fistula who had anterior sagittal anorectoplasty (ASARP) procedure were reviewed. The procedure and its outcome were evaluated. RESULTS: The manoeuvering during anesthesia and operative access were quite easier in ASARP compared to PSARP. Delineation of plane in ASARP between rectum and vagina was easier and clearer in comparison to PSARP. Rent occurred in the posterior vaginal wall in three cases of ASARP and two cases of PSARP. There were two cases of wound infection in each group. Three cases of PSARP group developed anal stenosis and constipation while one in the ASARP group developed constipation. CONCLUSION: Anesthesia and access in ASARP makes it an easier alternative option to PSARP in the management of anovestibular fistula in girls.

Abnormalities in "low" anorectal malformations (ARMs) and functional results resecting the distal 3 cm.

PURPOSE: "Low" anorectal malformations (ARMs) are considered minor anomalies of the distal rectum and anal-canal development. Nonetheless, the prognosis of affected patients is far from excellent, as some degree of constipation is a frequent complaint in the long-term follow-up. Constipation in "low" ARM has been reported in 42%-70% of cases. Vestibular fistulas seem to have the highest rate of constipation (not less than 61.4%). The aim of this study was to evaluate all the histological wall abnormalities of ARM with recto-perineal and recto-vestibular fistulas in order to identify features that could explain the bowel dysfunctions. Moreover, the resection of distal perineal and vestibular fistulas (last 3 cm) allowed evaluating functional results in "low" ARM series with extensive fistula resection. METHODS: One hundred four specimens were collected from 52 patients (32 recto-perineal and 20 recto-vestibular fistulas) during the posterior sagittal anorectoplasty (PSARP). The distal 3 cm of aberrant anorectal canals (fistulas) was systematically resected and divided longitudinally. One portion was fixed for immuno-histochemical stainings (PGP 9.5, S-100, NSE), H&E, and tricromic stainings. The frozen sections of the second portion were incubated for enzyme histochemical stainings (AChE, etc.). The follow-up of 42 of 52 ARM was postoperatively evaluated at 3-8 years of age, and the assessment of the outcome after PSARP repair was in line with Krickenbeck's 2005 meeting parameters. RESULTS: Muscle coat was abnormal in all cases (100%), showing aspect and absence of organization into the circular and longitudinal layers. The connective tissue was found to be irregular and abnormally represented in 100% of cases. Abnormal vascularization was detected in 5 cases (9.6%). All vestibular (100%) and 71.8% of perineal fistulas showed different degrees of enteric nervous system (ENS) anomalies. In the series of 42 patients followed up at least after 3 years of age, 40 cases (95.2%) showed postoperative good continence without use of laxatives (according to Krickenbeck's 2005 criteria). CONCLUSION: Every wall component of the distal rectum can be affected by different structural abnormalities in "low" ARMs. Pediatric surgeons should take into consideration the implications of these structural abnormalities during radical treatment. The resection of a significant portion of the distal fistula seems to permit better functional results.

A newborn with caudal duplication and duplex imperforate anus.

There are case reports of duplication of the colon, rectum, anus, urinary system, lower genital tract, and external genitalia, spinal anomalies, and abdominal wall defects. However, it is rare to encounter a single newborn with all of the mentioned abnormalities, which have been defined as the caudal duplication syndrome (CDS). Herein, we present a newborn with an omphalocele, duplex external genitalia (with duplex labia minora and labia majora), duplex urethral orifices, duplex vaginal orifices, and duplex anal dimple with imperforate anus and rectovestibular fistula on both sides. Exploration revealed duplex appendix, colon duplication, duplex uterus (continuing with tuba and ovaries on both sides), duplex rectum, malrotation of the intestines, with the cecum located in the middle of the abdomen, defect in the intestinal mesentery, and internal herniation of the small intestines through this defect. The intestines were operatively reduced and the defect repaired.

Modified transanal repair of congenital H-type rectovestibular fistula: a technique to avoid recurrence.

Congenital H-type rectovestibular fistulas are rare in the spectrum of anorectal malformations. Repair is associated with recurrence rates of up to 30%, using perineal repair, vestibuloanal pull-through or anterior anorectoplasty. The rarity of the malformation has limited experience with the surgical approach; hence, the rate of wound breakdown. We describe the use of a modification of the transanal approach for the repair in a 7-month-old female patient.

Treatment of adults with unrecognized or inadequately repaired anorectal malformations: 17 cases of rectovestibular and rectoperineal fistulas.

STUDY OBJECTIVE: To analyze all cases of congenital rectovestibular and rectoperineal fistulas diagnosed and treated later in life, and to describe presenting complaints, treatment, and outcome. DESIGN: Retrospective cohort study. SETTING: Pediatric surgery departments of 3 major referral centers in the US and Europe. PARTICIPANTS: Seventeen women with untreated or inadequately treated rectovestibular or rectoperineal fistulas. INTERVENTIONS: Analyses of all eligible patients: charts were analyzed for the classification of the malformation, main complaints, continence, sexual function, indications for surgery, associated anomalies, surgical procedure, complications, and outcome. MAIN OUTCOME MEASURES: Patients' complaints, continence, constipation, and sexual function. RESULTS: Major complaints at time of diagnosis were fecal incontinence, and concerns for hygiene and cosmesis. All patients were repaired by a posterior sagittal approach. In all but 1 patient the complaints disappeared or improved after surgery. CONCLUSIONS: Anorectal malformations in females are congenital malformations mostly seen and treated in early childhood. If unrepaired or inadequately repaired the patient, when reaching adulthood, can suffer from significant morbidity. Surgical treatment is similar as in childhood and has an excellent clinical outcome.

Use of an endorectal mucosal advancement flap to treat H-type rectovestibular fistula in patients with a normal anus.

The combination of an H-type rectovestibular fistula (RVF) and a normal anus is a rare type of anorectal malformation, resulting in abnormal communication between the normal rectum and vestibule in girls. Among the surgical methods used to treat this condition are simple fistula resection and extensive perineal dissection, but postoperative fistula recurrence is frequent. We utilized the endorectal mucosal advancement flap technique on three patients with H-type RVFs and a normal anus, all of whom had favorable outcomes.

H-type rectovaginal fistula in a patient with bilateral single ectopic ureters.

Congenital H-type rectovaginal fistulas and single ectopic bilateral ureters are each rare malformations. We describe a baby girl with a congenital rectovaginal fistula diagnosed 2 years after correction of single ectopic bilateral ureters. To our knowledge, this is the first association of these entities. Repair of fistula was complicated by recurrence, requiring a second procedure. The recommended operation for this anomaly requires separating the suture lines on the vagina and rectum. The practice of simply oversewing and then buttressing the suture lines is probably not sufficient.

[Congenital H-type anorectal fistula: two case reports]. / Kongenitale anorektale H-Fisteln: Zwei Fallberichte.

The congenital H-type fistula between the anorectum and the urogenital tract without anal atresia is an extremely rare entity in the spectrum of anorectal anomalies. We report about two young girls, who had a congenital fistula between the left labium/vulva and the anus (=H-type fistula). After the management of the local infection and the abscess, the two patients underwent an anterior sagittal anorectovaginoplasty (ASARVP). Up to now there was no relapse of the fistula or the abscess in both cases. Thus ASARVP can be used as a simple method for congenital H-type fistulas with excellent functional and anatomical results.

Congenital pouch colon associated with anorectal malformation: report of 2 cases.

Congenital pouch colon associated with anorectal malformation is an unusual anomaly reported most frequently in Asian countries. Pediatric surgeons must be familiar with this anomaly and the pre- and postoperative problems of these patients. The aim of this report is to bring attention to congenital pouch colon associated with anorectal malformation by discussing experiences with 2 patients.

Anterior sagittal anorectoplasty for anovestibular fistula.

Anterior sagittal anorectoplasty (ASARP) was used for the definitive correction in 107 cases of anovestibular fistula (AVF) between 1996 and 2005. These cases were subjected to three different types of treatment regimes during the same period. Majority of the cases (78) were operated in one stage where postoperatively an early oral feed was started (A). Cases were discharged in 2-4 days. In the second group (B), there were ten cases who were also operated in one stage but with prolonged fasting of 9-10 days postoperatively. Nineteen cases (C) were operated under cover of colostomy during the same period. In the immediate postoperative period, among the group A, one case had a major wound disruption requiring a colostomy and a redo surgery. Three cases had subcutaneous leak. In seven cases there was premature dehiscence of mucocutaneous or skin sutures. In groups B and C, there were no significant complications in the immediate postoperative period. In the follow-up period, out of 107 cases, 63 (58.8%) had constipation at the end of 3 months. However, at the end of one year, only 24.3% (26 cases) cases had constipation. Regarding fecal continence, 86 cases (90.5%) were totally continent. Seven had history of occasional soiling and in two cases, soiling was more frequent. As far as repair or correction of AVF or vestibular anus is concerned, we feel that anterior sagittal approach is more suitable as it requires less pelvic dissection. Separation of posterior vaginal wall from rectum, which is considered, is the most important step of the operation, takes place under direct vision. We also feel that AVF can be repaired in one stage with an early postoperative oral feed, provided we are meticulous in pre and postoperative bowel management. It reduces hospital stay and the cost of treatment. This provides a good option to cases who are not able to afford prolonged hospitalization (fasting) or are not willing for a colostomy.

One-stage correction of recto-vestibular fistula by trans-fistula anorectoplasty (TFARP).

BACKGROUND: The present article details a new technique for the repair of recto-vestibular fistula. MATERIALS AND METHODS: Twenty-five patients with recto-vestibular fistula, between 13 days and 4 years of age underwent surgical correction by trans-fistula ano-recto-plasty (TFARP). The technique, described in detail, involves mobilization of the fistula and the rectum through the fistula and creation of a new anus in the anatomically normal site by preserving both the perineal skin bridge (skin between the neo-anus and the posterior fourchette) and the levator muscle. RESULTS: The mean operating time was 85 min, and the mean hospital stay was 5 days. Moderate anal stenosis developed in 1 patient and was treated successfully by anal dilatations using Hegar dilators. A diverting colostomy was not required in any patient, and none of the patients developed rectal prolapse. Eleven patients who are now 3 years of age or older have voluntary bowel movements with good fecal continence scores. The 14 neonates and infants, who are still too young to be evaluated for continence, have symmetric anal contraction to stimulation and strong squeeze on digital rectal examination. The average number of bowel movements per day was three to five, without the need for any laxative or enema. CONCLUSIONS: Trans-fistula ano-recto-plasty is a simple surgical procedure that does not divide the levator muscle or the perineal body. Preservation of these structures contributes significantly toward improvement of the aesthetic appearance of the perineum and of fecal continence.

Double gastric outlet obstruction with epidermolysis bullosa in a neonate.

We report a 2-day-old baby who had double gastric outlet obstruction, rectovaginal fistula and associated epidermolysis bullosa. She recovered after excision of diaphragms, pyloroplasty and pelvic colostomy.