Posterior cloaca: A rare complex anomaly (original) (raw)
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An Anatomical Classification – a New Paradigm for Management of Urinary Dysfunction in the Female
International Urogynecology Journal, 1999
A new classification specifies anatomical defects in the anterior, middle and posterior zones of the vagina as causing urinary dysfunction. An external musculoelastic mechanism stretches the vagina to open and close the outflow tract. The same pelvic floor muscles provide a peripheral control mechanism for micturition. The stretched vagina prevents the filling bladder from activating the stretch receptors. Vaginal laxity may weaken the transmission of muscle forces, interfering with the mechanical process of opening and closure. Laxity may also destabilize the peripheral control mechanism, a neurological process, causing bladder control to swing between the open and closed modes, i.e. bladder instability. Specific symptoms, signs and urodynamic tests were arranged into a pictorial algorithm as a practical guide for locating the three zones of anatomical defects. It was possible to reinterpret almost all the definitions and descriptions of the International Continence Society in terms of this classification, and to explain how vaginal laxity may cause premature activation of the micturition reflex (detrusor instability), stress incontinence and abnormal emptying (dribble, overflow). This convergence in anatomical and urodynamic (ICS) concepts explains many previously unexplained phenomena, and potentially opens up an entirely new approach to management, non-surgical strengthening of specific ligaments, or surgical reinforcement thereof with ambulatory ‘keyhole’ methods which do not require catheterization.
Annals of Pediatric Surgery
Background Persistent urogenital sinus is a rare cloacal anomaly in which the urinary and genital tracts cannot be differentiated during embryonic development. However, the presence of concomitant recto-urogenital sinus or recto-vaginal fistula is much rarer. In the literature, only two cases with accompanying fistula have been presented so far. Case presentation We present the diagnosis and treatment management of a persistent urogenital sinus case with recto-vaginal fistula. We also aimed to reveal the difference between our patient, who is the 3rd case in the literature, and the other two and the described cloacal anomalies. By performing total urogenital sinus mobilization and fistula repair, the three systems were separated from each other, and both a functional and cosmetic appearance was obtained. Conclusion It should be kept in mind that there may be different variants other than the defined cloacal malformations. In patients with cloacal malformation, cystoscopy and rectosc...
International Journal of Urology, 2017
We present a case of a girl with an extremely rare, posterior type of persistent cloaca, which was associated with other abnormalities, including an undeveloped vulva and vagina, agenesis of the right kidney, secondary obstructive megaureter, unicornate uterus, persisted tailgut, sacral bone hypoplasia, and pubic symphysis hypertrophy. An operative approach was as follows: (i) colostomy and ureterocutaneostomy; (ii) creation of an ileal conduit with antirefluxing uretero-ileal anastomosis, and then creation of a continent catheterizable ileal reservoir; (iii) anastomosis of sigmoid colon to rectal stump; and (iv) vaginal and external genital reconstruction. Because of abnormal anatomical conditions where the uterus was situated adjacent to the open, incompetent bladder neck, we decided to create a vagina using the bladder wall instead of the bowel segment.
Purpose: Retrospective reviews suggest that the functional outcomes of surgery of the urogenital sinus have often been unsatisfactory and to our knowledge the long-term results of newer surgical techniques have yet to be evaluated. A precise understanding of pelvic fetal neuroanatomy is germane for optimizing surgical correction of the urogenital sinus. Materials and Methods: The pelves of 10 human female fetuses were serially sectioned. Masson's trichrome staining and immunochemistry for the neuronal marker S100 (Dako Corp., Carpinteria, California) along with anatomical computer reconstruction allowed 3-dimensional analysis of the nerves in relation to the pelvic structures as an animated motion picture. Results: Two types of neuronal structures were identified. 1) A dense perivisceral foil of branching nerves closely surrounded the pelvic organs. The localization of most nerves was on the external faces of the viscera with a limited fraction in the rectovaginal and urethrovaginal septa. This innervation was from the anterior cephalad periurethral area to the posterior caudal perirectal area. 2) A significant amount of nerves surrounded the cephalad urethra on its anterior and posterior faces. Conclusions: Based on these anatomical data during surgical repair of a urogenital sinus we would advocate minimal mobilization of the lateral faces of the vagina, avoiding dissection of the proximal urethra above the pubic bone and electing a vaginal flap in severe cases.
Frontiers in pediatrics, 2014
To evaluate postoperative urinary continence in patients with congenital adrenal hyperplasia (CAH) with intermediate (IT) and high urogenital sinus (UGS) who underwent a UGS mobilization maneuver. We called IT to those that although needing an aggressive dissection to get to the vagina, still have enough urethra proximal to the vaginal confluence. Very low variants are excluded from this analysis. Dissection always started in the posterior wall of the UGS with an aggressive separation from the anterior rectal wall. If the wide portion of the vagina was reached dissection stopped and the UGS opened ventrally widening to the introitus. Nineteen patients were treated using this maneuver (Group 1). When more dissection was required the anterior wall of the UGS was dissected and carefully freed from the low retropubic space. Then the UGS was opened either ventrally or dorsally. Thirty three patients required this approach (Group 2). Combined procedures were used in three patients with hi...
Urological management of cloacal anomalies
International Journal of Urology, 2001
Background: A cloacal anomaly results from incomplete urorectal division and is frequently associated with genitourinary abnormalities. Experience of the urological reconstruction of this entity is reported. Methods: Nine girls with cloacal malformation have been treated at the Osaka Medical Center in the past 9 years. Seven patients were detected by prenatal ultrasonography as having: megacystis (two patients); hydronephrosis (two patients); or hydrometrocolpos (three patients). Two patients underwent prenatal shunt placement between the enlarged bladder and the amniotic space. Results: For neonatal urinary diversion, four patients received cystostomy. Six of seven patients with associated hydrocolpos required intermittent catheterization to decompress an enlarged vagina. Vesicoureteral reflux was detected in 12 ureters of seven patients. Antireflux surgery was indicated in four patients before definitive repair. Definitive reconstruction was performed on eight patients. The posterior sagittal approach was used in all patients. Vaginal reconstruction was done utilizing a perineal skin flap (one patient), a tubularized vaginal flap (three patients), distal rectum (three patients) and total urogenital mobilization (one patient). Postoperatively, urethrovaginal fistula was created in one patient and complete occlusion was seen in one patient. Conclusion: The anatomical variety of this entity determines the management options from in utero. It involves not only the creation of three perineal orifices, but also a continent, catheterizable urethra under the stabilization of renal function. Accomplishment of the definitive repair requires the combined expertise of experienced pediatric surgeons and pediatric urologists.
Modified Posterior Sagittal Transanorectal Approach in Repair of Urogenital Sinus Anomalies
Urology, 2007
To describe the operative details and results of a modified posterior sagittal transanorectal approach for the reconstruction of urogenital sinus (UGS) anomalies. TECHNICAL CONSIDERATIONS Six children with UGS anomalies underwent surgery using this technique. In a prone jack-knife position, a midline incision was continued to the puborectalis muscle. A plane of dissection was created circumferentially around the rectum separating it from the underlying UGS. Circumferential transanal mucosectomy and a transanal dissection was carried proximally for 5 to 10 cm. The mucosal tube with the serosal wall was resected, exposing the proximal part of the UGS. The posterior and anterior sphincters, anus, and perineal body were then divided in the midline, completely exposing the UGS. Reconstruction of the urethra and vagina was done. At completion of UGS reconstruction, an endoanal pull through of the rectal tube and a low coloanal anastomosis were performed. The muscle complex and perineal body were closed in layers. The modified technique of posterior sagittal transanorectal approach allowed excellent exposure in all 6 patients. None developed any complications related to suture line leak. Fecal and urinary continence was preserved in patients who were continent before the operation.