Anorectal Malformation with Rectovestibular Fistula and Vaginal Agenesis: Usage of Rectovestibular Fistula as a Neovagina Followed by PSARP with Preservation of the Anal Sphincter

AbstractAnorectal malformation with rectovestibular fistula associated with vaginal agenesis is rare. We report on a child in whom this combination was diagnosed at the age of 1 year. After creation of a divided descending colostomy, we chose to leave the rectum-rectovestibular fistula to function as a neovagina, while the sigmoid colon was relocated via modified posterior sagittal anorectoplasty. The colostomy was closed 6 months after the main surgery. After a follow-up of 3 years, the patient is continent for stool and urine. The introitus of the neovagina appears wide and easy to explore. We conclude that our surgical approach may be a good option for these children.

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Menstrual, Sexual, and Obstetrical Outcomes after Vaginal Replacement for Vaginal Atresia Associated with Anorectal Malformation

European Journal of Pediatric Surgery ◽

10.1055/s-0036-1593610 ◽

2016 ◽

Vol 27 (06) ◽

pp. 495-502 ◽

Cited By ~ 6

Author(s):

Clare Skerritt ◽

Alejandra Vilanova Sánchez ◽

Victoria Lane ◽

Richard Wood ◽

Geri Hewitt ◽

...

Keyword(s):

Anorectal Malformation ◽

Outcome Data ◽

Current Evidence ◽

Vaginal Agenesis ◽

Vaginal Reconstruction ◽

Term Outcome ◽

Long Term Outcome ◽

Vaginal Atresia ◽

Rectovestibular Fistula

Background The authors of this article became aware of significant differences in the management of two females with a rectovestibular fistula and associated vaginal agenesis. In one patient, a sigmoid colovaginoplasty was performed at the time of the posterior sagittal anorectoplasty (PSARP), and the other underwent repair of the rectovestibular fistula with a primary PSARP, but the surgeons elected to delay the timing of vaginal reconstruction. We decided to review the literature, to establish if recommendations could be made to optimize the management of these children based on current evidence. Methods A literature review was conducted to determine the management and long-term outcomes in patients with an anorectal malformation and associated vaginal atresia. Specific gynecological outcomes assessed were menstrual egress and adequacy of the vaginal replacement for penile–vaginal intercourse. Results Eighty-eight cases were included in the review. Age at diagnosis had a bimodal distribution: 0 to 5 years, 56%; >10 years, 37%. Vaginal atresia was recognized before the operation in 45 patients. Types of vaginal atresia encountered were: (a) distal vaginal atresia (n = 17), (b) vaginal agenesis with absent Mullerian development (n = 47), (c) vaginal agenesis with variable Mullerian development (n = 17), and (d) cervico-vaginal agenesis (n = 7). Types of vaginal replacement used were sigmoid colovaginoplasty (n = 26), distal rectal fistula as neovagina (n = 30), terminal ileum (n = 5), vaginal pull-through (n = 9), and others/unknown (n = 8). Two patients followed a perineal dilatation program and nine patients await reconstruction. Median follow-up was 18 months (6 weeks to 31 years). Long-term menstrual outcomes were reported in 18 (21%) patients. Sexual function was reported in 10 (11%) patients. Three pregnancies were reported but none resulted in live births. Conclusion Vaginal atresia is often missed in association with ARMs. Management should be in collaboration with pediatric gynecologists. Due to a lack of long-term outcome data, no definite conclusion can be drawn for the best technique, tissue, or timing of vaginal replacement. The opportunity to perform vaginal replacement in conjunction with the rectal repair may be worth considering because of a shallower pelvis, nonscarred tissue planes, and the excellent surgical exposure.

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Surgical and functional outcome of sigmoid colon-vaginoplasty in Mayer-Rokitansky-Kuster-Hauser syndrome

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20174421 ◽

2017 ◽

Vol 6 (10) ◽

pp. 4441 ◽

Cited By ~ 1

Author(s):

Pradyumna Pan

Keyword(s):

Sigmoid Colon ◽

Adolescent Females ◽

Sexually Active ◽

Vaginal Agenesis ◽

Vaginal Length ◽

Short Recovery Time ◽

Sigmoid Vaginoplasty ◽

Good Cosmetic ◽

Complications Rate

Background: The objective of this study is to report our experience with sigmoid vaginoplasty in adolescent female patients of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome with emphasis on the effectiveness of surgery, complications, sexual and functional outcomes.Methods: A retrospective study of adolescent females with vaginal atresia and Mayer-Rokitansky-Kuster-Hauser syndrome. The sigmoid segment was used for vaginoplasty in all the case.Results: Eleven females were studied over a period of 15 years. Postoperative complications were perineal rash in 3, ileus in 3, and minor wound infection in 1 patient and urinary tract infection in one patient. Nine patients were on regular follow-up. All the neovaginas were patent and functional. The postoperative mean vaginal length was 12.1 cm (range 11 cm to 14 cm). One of the patients had mild vaginal stenosis which responded to vaginal dilation, one had mucosal prolapse of the neovagina, one had excessive mucus discharge, during follow-up visits. Out of the 11patients, 8 patients are sexually active and 7 reported that they were engaging in satisfactory sexual activity with mild or no pain, and with good mucosal sensitivity. Only one experienced dyspareunia and three patients had not participated in sexual activity.Conclusions: Sigmoid vaginoplasty is a safe and acceptable procedure for vaginal agenesis with good cosmetic results and acceptable complications rate. Sigmoid colon vaginoplasty is the treatment of choice because of its large lumen, thick walls resistant to trauma, adequate secretion allowing lubrication, not demanding prolonged dilatation and short recovery time.

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Fecal Incontinence after Posterior Sagittal Anorectoplasty for Anorectal Malformation: A Single-Center Study

Scientifica ◽

10.1155/2018/8297617 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4

Author(s):

Manoochehr Ghorbanpoor ◽

Behzad Dehvan ◽

Siavash Rahimi ◽

Azar Pirdehghan

Keyword(s):

Fecal Incontinence ◽

Anorectal Malformation ◽

Fecal Continence ◽

Posterior Sagittal Anorectoplasty ◽

Medical Sciences ◽

High Type ◽

Single Center Study ◽

The Status ◽

The Difference

Background. Fecal incontinence is one of the worst functional complications of posterior sagittal anorectoplasty for treatment of anorectal malformation. Objectives. In this study, we aimed to identify the prevalence of fecal incontinence in patients with the diagnosis of high or low anorectal malformation who underwent three-stage posterior sagittal anorectoplasty surgery in our center. Patients and Methods. Children with the diagnosis of anorectal malformation who underwent posterior sagittal anorectoplasty at the Department of Pediatric Surgery of Besat Hospital, Hamadan University of Medical Sciences, Iran, from 2012 to 2016 were enrolled in the study. Parents or guardians were recruited and asked to fill the study questionnaire including the Templeton and Ditesheim Scoring System to assess the status of fecal continence of the patients. Results. Thirty-four patients including 10 (29.4%) males were enrolled in the study. High type of anorectal malformation was diagnosed in 23 (67.6%) patients. The overall mean scores of fecal continence were 4.57 ± 0.84 (range 1.5–5) after a mean follow-up time of 50.7 (range 22.5–69.8) months. Good fecal continence was observed in 91.3% of patients with low type compared to 72.8% of patients with high type of anorectal malformation; however, the difference was not significant (P=0.13). Conclusion. Posterior sagittal anorectoplasty surgery in patients with anorectal malformation may result in acceptable fecal continence.

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Anorectal malformation with long perineal fistula: one of a special type

Scientific Reports ◽

10.1038/s41598-021-81056-3 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Sen Li ◽

Jun Wang

Keyword(s):

Anorectal Malformation ◽

Bowel Function ◽

Anorectal Malformations ◽

Contrast Enema ◽

Posterior Sagittal Anorectoplasty ◽

Function Score ◽

Perineal Fistula ◽

Definitive Surgery ◽

Inhibitory Reflex

AbstractThe anorectal malformation with long perineal fistula is a rare anomaly in the spectrum of anorectal malformations. Aim of the study is to describe the series of patients with anorectal malformation with long perineal fistula and compare the outcome with patient with standard perineal fistula. From March 2012 to January 2019, 7 patients who suffered from anorectal malformation with long perineal fistula were retrospectively reviewed. Three were operated on primarily by our department, and 4 cases were re-operated after a perineal anoplasty repair performed elsewhere. Four were operated by laparoscopy assisted anorectoplasty, and 3 cases were repaired by posterior sagittal anorectoplasty. The follow-up outcomes were compared with 71 cases of normal perineal fistula (NPF) in the same period. 7 cases have been followed up for 0.5–4 years (M = 2.57 ± 1.26) after definitive surgery. Their bowel function score was lower than normal perineal fistula (SPF = 12, range: 5–18; NPF = 18.5, range: 18–20). Four cases underwent anorectomanometry. The incidence of rectoanal inhibitory reflex was lower in the special type group. (p = 0.14). Three cases of contrast enema using barium: 2 cases of colorectal dilatation and thickening changes, 1 case showed no obvious abnormalities. Anorectal perineal fistula should be examined by distal colostogram at preoperation. This should be altered in: When suspecting a case of anorectal malformation type long perineal fistula a preoperative contrast enema could give insight of the anatomy befor performing a anoplasty.

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National Medical Research Center for Children’s Health

Medical Council ◽

10.21518/2079-701x-2021-11-118-123 ◽

2021 ◽

pp. 118-123

Author(s):

E. A. Okulov ◽

A. V. Dotsenko ◽

E. I. Dyakonova ◽

S. P. Yatsyk ◽

S. P. Yatsyk

Keyword(s):

Surgical Treatment ◽

Postoperative Period ◽

Anorectal Malformation ◽

Diagnostic Methods ◽

Functional Study ◽

Perineal Ultrasound ◽

Reliable Determination ◽

Intestinal Tube ◽

Rectovestibular Fistula

Abstract Introduction. Anorectal malformations are one of the most numerous groups of proctologic pathology in children. The incidence is 1 per 5,000 live births. Perineal ultrasound, distal colostography, and MRI of the pelvic organs and sacrococcygeal region are used to clarify the anatomico-topographic features of the malformation and identify concomitant malformations (presacral masses, distal spinal cord pathology). This article presents a clinical case of surgical treatment of an 11-year-old girl who underwent primary surgery for anorectal malformation with rectovestibular fistula in infancy. In the postoperative period for 10 years, the parents practically did not carry out rehabilitation therapy. These factors led to persistent decompensation of the lower intestine in the form of a giant megarectum, which required repeated anorectoplasty with resection of pathologically dilated parts of the intestinal tube. The article presents description of the repeated surgery with intraoperative electromyoidentification of the perineal muscles, data of follow-up 9 months after the repeated anorectoplasty with instrumental diagnostic methods (irrigography, functional study of the closing apparatus of the rectum).Discussion. This clinical observation illustrates complications following surgical treatment of anorectal malformation with rectovestibular fistula. Lack of intraoperative electromyoidentification of the muscles during primary anorectoplasty often resulted in ectopia of the neoanus relative to the center of the perineal muscle center. This circumstance was one of the causes of persistent chronic constipation in the postoperative period.Conclusion. The use of electromyostimulation during primary proctoplasty for reliable determination of the sphincter-levator complex topography is mandatory. Long sequential rehabilitation in the postoperative period including neoanus bougienage, provision of regular full defecation, physiotherapeutic treatment, as well as regular follow-up examination in the in-patient department is also an integral part of the treatment.

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Magnetic resonance imaging of the anal sphincter and spine in patients with anorectal malformations after posterior sagittal anorectoplasty: a late follow-up cross-sectional study

Pediatric Surgery International ◽

10.1007/s00383-020-04774-1 ◽

2020 ◽

Author(s):

Thomas Bjørsum-Meyer ◽

Peter Christensen ◽

Gunnar Baatrup ◽

Marianne Skytte Jakobsen ◽

Jon Asmussen ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Anal Sphincter ◽

Anorectal Malformations ◽

Cross Sectional Study ◽

Posterior Sagittal Anorectoplasty ◽

Sectional Study ◽

Resonance Imaging ◽

Cross Sectional

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Anorectal Malformation: Paediatric Problem Presenting in Adult

Case Reports in Surgery ◽

10.1155/2015/625474 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Rahulkumar N. Chavan ◽

Bhargav Chikkala ◽

Cinjini Das ◽

Somak Biswas ◽

Diptendra Kumar Sarkar ◽

...

Keyword(s):

Case Report ◽

Anorectal Malformation ◽

Abdominal Distension ◽

Definitive Treatment ◽

Posterior Sagittal Anorectoplasty ◽

Conventional Procedure ◽

Satisfactory Outcome ◽

Vestibular Fistula ◽

Anal Transposition

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.

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Angiosarcoma of sigmoid colon with intraperitoneal bleeding: case report and literature review

Annals of The Royal College of Surgeons of England ◽

10.1308/147870811x591017 ◽

2011 ◽

Vol 93 (6) ◽

pp. e91-e93 ◽

Cited By ~ 4

Author(s):

Tse-Hua Lo ◽

Mu-Shiun Tsai ◽

Tzu-An Chen

Keyword(s):

Sigmoid Colon ◽

Alimentary Tract ◽

Surgical Excision ◽

Sigmoid Colectomy ◽

Epithelioid Cells ◽

Spindle Cells ◽

Choice Of Treatment ◽

Intraperitoneal Bleeding ◽

Immunohistochemical Studies

Primary angiosarcomas arising from the alimentary tract are rare and only a few cases have been reported in the literature. We report a case of an angiosarcoma of the sigmoid colon with intraperitoneal bleeding but not rectal bleeding. A 21-year-old female patient received a laparotomy and a mass lesion over the sigmoid colon was found with active bleeding. A sigmoid colectomy was performed as a curative resection. Grossly, the sigmoid colon contained a kidney shaped, hemorrhagic tumour from the submucosal layer extension to the antimesenteric side. Intraluminally, the mucosa of the colon was intact. Microscopic examination revealed a high grade angiosarcoma composed of fascicles of spindle cells and solid sheets of epithelioid cells. Immunohistochemical stains revealed a positive result for CD31 and the endothelial nature of the malignancy was confirmed. Smooth muscle antigens, desmins, cytokeratins AE1/AE3 and CD117 were all negative. The patient is still alive without evidence of recurrence or metastasis at a three-year follow-up appointment. Owing to the availability of immunohistochemical studies, some atypical sarcomas would now be correctly classified as angiosarcomas. Since no optimal adjuvant treatment is effective, curative surgical excision is still the best choice of treatment.

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Posterior Cloacal Variant with Clitorolabial Transposition and a Rectoperineal Fistula

European Journal of Pediatric Surgery Reports ◽

10.1055/s-0041-1728724 ◽

2021 ◽

Vol 09 (01) ◽

pp. e46-e49

Author(s):

Niveshni Maistry ◽

Giulia Brisighelli ◽

Chris Westgarth-Taylor

Keyword(s):

Operative Intervention ◽

Posterior Sagittal Anorectoplasty ◽

Surgical Team ◽

Ureteric Reimplantation ◽

Long Term Follow Up

AbstractWe present a case and discuss the management of a posterior cloacal variant not as yet described in the literature. A 5-week-old infant presented to our institution with a posterior cloacal variant and transposition of the clitoris and labia. After initial radiological investigations, staged operative intervention was performed over a 1-year period. This included an initial laparotomy (with drainage of hydrocolpos and formation of a colostomy), a left ureteric reimplantation and a posterior sagittal anorectoplasty due to a rectoperineal fistula. The child is under continued long-term follow-up by our specialist pediatric surgical team.

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