Cloacal Reconstruction Research Articles

Transperineal perineoplasty with anal sphincteroplasty: cloacal reconstruction for 4th degree obstetric perineal tear with failed previous repair

We describe the case of a patient with anal sphincter injury and fecal incontinence related to obstetric trauma, who was previously treated in another center with primary repair on 2 occasions and with recurrence of the tear, developing cloacal deformity. Transperineal perineoplasty with sphincter restoration was performed with overlapping technique, with immediate postoperative recovery of fecal continence, and during the 3-year follow-up, fecal continence was maintained.

How we select our surgical approach for cloacal reconstruction

During cloacal reconstruction, we consider both common channel (CC) and urethral length when deciding between total urogenital mobilization (TUM) and a urogenital separation (UGS). Our concern about TUM in the case of a short urethra is resultant bladder neck incompetence, while conversion to UGS after completing the TUM dissection may result in urethral injury. Use of an interposition flap harvested from the ischiorectal fossa is another measure we use to prevent potential complications.

Does a standardized operative approach in cloacal reconstruction allow for preservation of a patent urethra?

BackgroundCloacal malformations are recognized as a particularly challenging congenital condition to manage and they present with a wide spectrum of anatomical configurations making surgical repair very complicated. Urethral necrosis or urethral loss is a known and devastating complication of cloacal repair. The surgical repair of these malformations has evolved over time and historically only common channel (CC) length was measured. More recently, it has been advocated that the urethral length and the CC are both important in determining surgical repair. The purpose of our study is to evaluate if this surgical approach allows for preservation of a patent urethra. MethodsA prospective database of all cloaca patients maintained with IRB approval (IRB# STUDY00000721) was retrospectively reviewed. We included any girl with cloacal malformation who underwent primary repair at our institution between May 2014 and December 2019. Standardized preop evaluation with endoscopy and 3-dimentional imaging to assess urethral length and CC length. These measurements were used to determine operative approach. Girls with CC < 1 cm undergo posterior sagittal anorectoplasty and introitoplasty (PSARP + I), those with CC measuring 1–3 cm and urethra > 1.5 cm undergo total urogenital mobilization (TUM) and those with CC > 3 cm or urethra < 1.5 cm undergo urogenital separation (UGS). Postoperative urethral patency was determined at the time of cystoscopy and exam under anesthesia (EUA) 4–6 weeks postoperatively by visualizing a viable and healthy urethra that is catheterizable. ResultsA total of 59 patients met inclusion criteria with a median age of 11.6 months. Four girls underwent PSARP + I, 19 girls had a TUM and 36 girls underwent a UGS. All of the girls who had PSARP +I (n = 4) or TUM (n = 19) had a viable and patent urethra that was catheterizable at the cystoscopy and EUA 4–6 weeks postoperatively. Of the 36 girls who had UGS, all but 2 (5.6%) had a viable and patent urethra that catheterized without problems. Overall, 97% of girls in this cohort had a patent urethra after cloacal repair using this surgical protocol. ConclusionsThe use of a standard protocol that considers urethral and common channel length for cloacal repairs results in a viable and patent urethra in 97% of patients. Level of evidenceLevel II.

Preliminary Use of Indocyanine Green Fluorescence Angiography and Value in Predicting the Vascular Supply of Tissues Needed to Perform Cloacal, Anorectal Malformation, and Hirschsprung Reconstructions.

Reconstructive techniques for cloaca, anorectal malformations (ARM), and Hirschsprung disease (HD) may require intestinal flaps on vascular pedicles for vaginal reconstruction and/or colonic pull-throughs. Visual assessment of tissue perfusion is typically the only modality used. We investigated the utility of intraoperative indocyanine green fluorescence angiography (ICG-FA) and hypothesized that it would be more accurate than the surgeon's eye. Thirteen consecutive patients undergoing cloacal reconstruction (9), HD (3), and ARM repair (1) underwent ICG-FA laser SPY imaging to assess colonic, rectal, vaginal, and neovaginal tissue perfusion following intraoperative visual clinical assessment. Operative findings were correlated with healing at 6 weeks, 3 months, and 1 year postoperatively. ICG-FA resulted in a change in the operative plan in 4 of the 13 (31%) cases. In three cases, ICG-FA resulted in the distal bowel being transected at a level (>10 cm) higher than originally planned, and in one case the distal bowel was discarded, and the colostomy used for pull-through. ICG-FA correctly identified patients who might have developed a complication from poor tissue perfusion. Employing this technology to assess rectal or neovaginal pull-throughs in cloacal reconstructions, complex HD, and ARM cases may be a valuable technology.

Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation

BackgroundA subset of patients with cloacal malformations requires vaginal replacement during their primary reconstruction, increasing the surgical complexity. Identifying factors which predict the need for vaginal replacement would facilitate operative planning. MethodsWe retrospectively reviewed patients who underwent primary cloacal reconstruction at our Center (2014–2018) and assessed the length of the common channel, urethra, and vagina. The presence of hydrocolpos at birth, Müllerian anomalies, sacral ratio, and tethered cord were also assessed between patients who did and did not require vaginal replacement. Results50 patients were identified. 17/50 patients (34%) underwent a total urogenital mobilization (TUM), and none required vaginal replacement. 33/50 (66%) patients underwent a urogenital separation. 19/33 (58%) required vaginal replacement. This group had a shorter vagina (4.2 cm vs 6.6 cm, p < 0.01). There was no difference in urethral or common channel length, number of cervices, sacral ratio, presence of a vaginal septum, hydrocolpos, or tethered cord between those who did and those who did not require vaginal replacement. ConclusionsUrethral and common channel lengths were used to successfully determine the operative plan (TUM or urogenital separation) to reconstruct cloacal malformations. The need for urogenital separation and a shorter vaginal length were predictive of the need for vaginal replacement. Level of evidenceLevel IV. Type of studyCase series with no comparison groups.

The use of rotational fluoroscopy and 3-D reconstruction in the diagnosis and surgical planning for complex cloacal malformations

IntroductionCloacal malformations, a confluence of the urinary tract, vagina and rectum into a single common channel, has a broad and complex anatomic spectrum requiring an imaging tool for visualization, measurement, and surgical planning for the reconstruction of these structures. We evaluated the role of 3-D fluoroscopy for this purpose, as it offers a combination of spatial correlation with precise anatomic measurements. MethodsWe examined our imaging protocol for patients with a cloacal malformation and report our experience with rotational fluoroscopy and 3-D reconstruction in 16 consecutive patients referred for cloacal reconstruction. The length of the common channel (CC), the length of the urethra from the bladder neck to the common channel, and the height (and existence or absence) of a vagina or vaginas were determinants of the surgical procedures used for the repair. ResultsWe performed 16 consecutive 3-D cloacagrams (age range 4 months to 9 years) using a new protocol (Figure 1) that provided the following data which helped with surgical planning: Gynecologic: 3 cases with a single vagina, 5 cases with a duplicated Mullerian system (3 of which were asymmetric) and 2 cases with high vaginas requiring vaginal replacement. Colorectal: Four had a high rectum requiring an abdominal approach, and 6 had a rectum reachable via a posterior sagittal approach. Urologic: Two ectopic ureters requiring reimplantation, 3 patients had vesicoureteral reflux (1 bilateral, 2 unilateral), 1 patient had no bladder, and 7 had a normal sized bladder. Common channel length and urethral length were demonstrated in all cases and used to decide between a total urogenital mobilization or a separation of vagina(s) from the common channel, urogenital separation. ConclusionThe 3-D cloacagram can help predict the surgical plan for urologic, gynecologic, and colorectal components of the cloacal repair. It can predict the CC length as well as the length of the urethra. It helps with predicting the need for vaginal replacement and whether an abdominal approach is needed for the rectum. Its effectiveness is based on the ability to adequately distend structures and see their distal most extent, an advantage over other modalities such as MRI. Added benefits (particularly from the 3D view) include a better spatial understanding of the defect and the diagnosis of concomitant urological abnormalities such as vesicoureteral reflux and ectopic ureters. Disadvantages to this procedure include the need for general anesthesia and a higher exposure to radiation. Level of evidence3.

Urethral length in female infants and its relevance in the repair of cloaca

Aim of the StudyThe goals of urinary reconstruction in urogenital sinus and cloacal repair include: (1) positioning of the bladder neck above the urogenital diaphragm to maximize future urinary continence, and (2) creating a visible urethra that can be catheterized if needed. A recent algorithm in cloacal reconstruction proposed a urethral length of 1.5 cm as the key determinant in deciding whether to perform a total urogenital mobilization or a urogenital separation, the hypothesis being that a 1.5 cm length urethra is needed for the patient to remain dry. We wondered if the normal female urethral length correlated with this empiric technical determinant. MethodsWe reviewed voiding cystourethrograms of healthy female patients between ages 6 and 36 months and measured the patient's urethral length. ResultsNinety-one children were included. The mean urethral length for patients age 6–12 months was 2.50 cm, age 12–24 months was 2.31 cm, and age 24–36 months was 2.59 cm. There was no difference between the urethral length in the three groups (p = 0.38). Of 91 patients, 87 (96%) had a urethral length >1.5 cm. ConclusionA urethra of at least 1.5 cm was present in the majority of normal control patients. We believe therefore that for urogenital sinus and cloacal repair, surgeons can extrapolate that patients need a 1.5 cm urethra at the end of the reconstruction. Additional follow-up is needed to determine if this urethral length as an independent factor maintains dryness in the long term after cloacal repair. Type of StudyCase Series. Level of EvidenceIII.

The Mullerian Black Box: Predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment

IntroductionMost patients with a cloacal malformation have a Mullerian anomaly. We sought to examine our patients with a cloacal malformation to determine the proportion of them we felt we knew their Mullerian anatomy and which proportion we felt would most benefit from longitudinal assessment to define their anatomy, reproductive potential, and risk of outflow tract obstruction after puberty. We also compared the preoperative assessment of reproductive anatomy (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) and intra-operative abdominal findings (at the time of primary cloacal reconstruction or subsequent abdominal procedures) to see how these correlated with and which preoperative assessment tool was most predictive of intraoperative anatomy. We also sought to confirm what we expected to be a lack of ovarian pathology. MethodsA single site retrospective chart review was performed on all patients with a cloacal anomaly seen between May 2014 and September 2017. Preoperative assessment (pelvic ultrasound, MRI, cloaca gram, and vaginoscopy) and operative reports (both primary reconstruction and later abdominal procedures) were reviewed to ascertain Mullerian and ovarian anatomy. Results30 of 36 (83%) of patients had defined Mullerian anatomy after preoperative assessment (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) with or without the addition intraoperative assessment of Mullerian structures obtained during laparoscopy or laparotomy. 19/30 (63%) had duplication of their Mullerian structures. 25/36 (69%) had intraoperative assessment of Mullerian anatomy during laparoscopy or laparotomy. In this group, preoperative assessment with pelvic ultrasound correlated in 4/8 patients (50%), MRI correlated in 3/4 patients (75%), cloacagram in 10/15 patients (67%), and vaginoscopy in 23/25 patients (92%). 14/36 (39%) patients were found to require longitudinal assessment to define anatomy, reproductive potential or risk of outflow tract obstruction after puberty. Patients with ovarian findings described at the time of laparoscopy or laparotomy had no evidence of ovarian pathology. ConclusionsThe majority of patients with cloaca in our series (83%) had their Mullerian anatomy defined by either preoperative assessment and/or findings at the time of laparoscopy or laparotomy. Duplication of the vagina and uterus was the most commonly described Mullerian anatomy (63%) in our series. Vaginoscopy appears to be superior to pelvic ultrasound, MRI, and cloacagram in predicting Mullerian anatomy. Fourteen of our 36 (39%) patients will require longitudinal assessment follow for reproductive potential and/or risk of outflow tract obstruction after puberty as their Mullerian anatomy is not known. There was no evidence of ovarian pathology in any cloaca patient.While we felt as though we could define Mullerian anatomy in most of our patients, any opportunity for intraoperative assessment of Mullerian anatomy should be utilized and therefore teams who are involved in the management of these patients must have a systematic and collaborative method established to ensure that Mullerian structures are thoroughly evaluated intra-operatively and documented in a standardized fashion. Type of studyRetrospective Chart review. Level of EvidenceIII.

Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning

BackgroundCloacal malformations represent a uniquely complex challenge for surgeons. The surgical approach to date has been based on the common channel (CC) length with two patient groups considered: less than or greater than 3cm, which we believe is an oversimplification. We reviewed 19 patients, referred after surgery done elsewhere. Eight had postoperative urinary complications, 3 had constant urinary leakage and had been left after surgery with a urethra <1cm, .5 with an original 3 to 5cm common channel, who had undergone total urogenital mobilization (TUM), experienced peri-operative urethral loss needing a vesicostomy, and later, a Mitrofanoff. These patients together with a review of the cloacal and urological literature led us to design a new algorithm where urethral length is a key determinant for care. MethodsWe prospectively collected data on 31 consecutive cloaca patients referred to our team (2014 to 2016) and managed according to this new protocol. The CC length, urethral length, surgical technique employed, and initial outcomes were recorded. ResultsOf 31 primary cases, CC length was 1 to 3cm in 20, 3 to 5cm in 9, and greater than 5cm in 2. In the 1 to 3cm and the 3 to 5cm groups, a urethra less than 1.5cm led us to perform an urogenital separation. We only performed a TUM if the urethra was greater than 1.5cm. Using this protocol, we performed a urogenital separation in 1 of 20 in the 1 to 3cm CC group, 6 of 9 in the 3 to 5cm CC group, and 2 of 2 in the greater than 5cm CC group. Seven patients underwent separation, who with the previous approach, would have had a TUM. Thus far, no urinary leakage or urethral loss has occurred in any patient, but follow-up is less than 3years. ConclusionUrethral length appears to be a vitally important component in cloacal reconstruction. A short urethra left after repair can lead to urinary leakage. A TUM done under the wrong circumstances can lead to urethral loss. We describe a new technical approach to cloacal repair which considers urethral length but recognize that long term urological outcomes will need to be carefully documented. Type of studyClinical cohort study with no comparative group. Level of evidenceLevel 4.

Post-operative strictures in anorectal malformation: trends over 15years.

For decades, paediatric surgeons have employed the standard posterior sagittal anorectoplasty (PSARP) approach to deal with patients with anorectal malformations (ARM). In recent years, we noted an apparent increase in the incidence of anal stricture after surgical repair of ARM following the introduction of laparoscopic pull-through and techniques aiming to preserve the internal sphincter-the internal sphincter sparing approach (ISSA). We decided to analyse our data to find out if these new trends had added to the problem of post-operative strictures. All patients with ARM at our institution from January 2000 to December 2015 were identified. A retrospective case note review was carried out. Data collected included patient demographics, type of ARM, operative details, and post-operative outcomes. 114 patients were identified. Ten patients were excluded. Of the remaining 104 children, 48 (46%) were female. Median age was 8.3 (range 1.2-16.8) years. Types of ARM were as follows: perineal fistula (15 patients), anterior stenotic anus (12), imperforate anus without fistula (10), vestibular fistula (32), rectourethral (bulbar) fistula (11), rectourethral (prostatic) fistula (14), rectovesical fistula (7), and cloaca (3). Twenty-seven patients with a perineal fistula or anterior stenotic anus underwent perineal procedures that were variably described by the different operating surgeons. The majority (15 patients) had an anoplasty, 5 had anal transposition, 5 had limited PSARP, and 2 patients had ISSA. Two patients with a cloacal anomaly underwent open cloacal reconstruction. Of the remaining 75 patients, 45 had a PSARP approach, 6 had a laparoscopic-assisted pull-through, and 18 had ISSA. Four girls with vestibular fistula had anal transposition and two boys with imperforate anus without fistula had anoplasty. 15 (14%) children developed anal stricture. Stricture incidence differed according to operation type. PSARP was the most commonly performed procedure, with only 6% developing a stricture. In contrast, 30% of ISSA patients and 50% of children who had laparoscopic pull-through developed a stricture. Strictures also occurred in 11 and 12% of children having anal transposition and anoplasty, respectively. The laparoscopic-assisted pull-through involves tunnelling the sphincter muscle complex. We found that often the tunnels were not wide enough, resulting in narrowing not just at the ano-cutaneous junction but also at the deeper level. 50% developed strictures. We have modified our technique by ensuring that the tunnels are generous enough to allow the rectum to be pulled through without any resistance. ISSA unfortunately resulted in 30% of our patients developing strictures. This approach, started in 2004, was, therefore, abandoned in 2013. The standard Pena's PSARP, with or without a laparotomy, has stood the test of time. Any modification of this approach must be carefully thought through and audited meticulously. Strictures can cause significant morbidity, which may need several revisions, and the resulting redo anoplasties run the risk of sphincter damage, ironically which the newer modifications of ISSA were trying to conserve.

Cloacal reconstruction after a complex treatment of perineal haemangioma in a variant of PELVIS syndrome.

BackgroundPELVIS is an acronym defining the association of perineal hemangioma, malformations of external genitalia, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag. Eleven cases have been reported according to the Orphanet data. Acronyms of LUMBAR and SACRAL syndrome have been used and most probably represent a spectrum of the same entity. Very little is known about the success and timing of cloacal reconstruction after the treatment of hemangioma. We present a variant of PELVIS syndrome and discuss the possibilities and optimal timing of surgical reconstruction.Case presentationFemale infant was born with persistent cloaca and multiple hemangiomas of genitals, perineal area and left thigh. Colostomy was performed after birth. In order to treat hemangioma and to make the reconstruction of cloaca possible, corticosteroid treatment orally and multiple laser treatments were performed alternating Nd:YAG laser and pulsed dye laser therapy. Cystoscopy confirmed hemangiomatosis in the mucosa of the common channel, bladder neck and septate vagina. Oral propranolol treatment was started at the age of 18 months and continued for 1 year. It induced rapid improvement of hemangiomas. Two more pulsed dye laser treatments were performed to remove residuals of hemangiomas from the perineum and genital area. Posterior sagital reconstruction by separation of the rectum, mobilization of urogenital sinus and vaginal reconstruction was performed with no major bleeding at the age of 4 years. Postoperatively, after a period of progressive rectal dilatation colostomy was closed. Girl is now 6 years old, dry day and night without residual urine and normal upper tracts. Rectal calibration is normal, fecal continence is still to be evaluated but constipation is easily manageable. CT of the spine and the perineum showed sacral dysplasia and spina bifida with lumbo-sacral lipoma and tethering of terminal filum without neurological deterioration at the moment but requiring close neurological monitoring.ConclusionsLarge perineal hemangiomas are commonly associated with extracutaneous abnormalities. Successful reconstructive surgery is possible after significant reduction of hemangioma by complex treatment.

Urogenital function after cloacal reconstruction, two techniques evaluated

ObjectiveCurrent surgical techniques for cloacal reconstruction are posterior sagittal anorecto vagino urethroplasty (PSARVUP) and posterior sagittal anorectoplasty (PSARP) with total urogenital mobilization (TUM). The aim of this study was to explore the results of reconstructive cloaca surgery in the Netherlands and evaluate urogenital function after PSARVUP and TUM. Patients and methodsMedical records from five pediatric surgical departments in the Netherlands were studied for patients with cloacal malformations treated between 1985 and 2009. Forty-two patients were eligible, and patients with short common channels were categorized into PSARVUP and TUM groups. Groups were compared using Fisher's exact test. ResultsMedian age at time of surgery was 9 months (range 1–121). In 24 patients (57%) a PSARVUP was done, in 18 patients (43%) TUM. Median follow-up was 142 months (range 15–289). At follow-up spontaneous voiding was seen in 29 patients (69%). Clean intermittent catheterization (CIC) was needed in 14 patients (33%); a urinary diversion was created in 10 patients (24%). In total 32 patients (76%) were dry with no involuntary loss of urine per urethra. Recurrent urinary tract infections were seen in 23 patients (55%). When comparing PSARVUP and TUM groups in our series of patients with short common channels, there were no differences in urological outcome. Normal menstruation was present in 11 of the 20 patients who reached puberty (55%). ConclusionUrogenital functional outcome after reconstructive surgery for cloacal malformations was similar in PSARVUP and TUM groups in patients with short common channels. A thorough urological follow-up is needed to establish the long-term bladder function and urinary incontinence results to prevent long-term risks of recurrent UTI. Albeit without differences between PSARVUP and TUM groups, 45% of the patients present with abnormal or absent menstruations. Gynecological follow-up is mandatory in all patients with cloacal malformations 6 months after the first sign of puberty.

Early versus late reconstruction of cloacal malformations: The effects on postoperative complications and long-term colorectal outcome

IntroductionPatients with a cloacal malformation generally undergo reconstructive surgery within the first years of life. However, the ideal age for surgery has rarely been mentioned. The aim of this study was to report differences in outcome between early (<6months) and late repair of cloacal malformations. MethodsCharts of patients with a cloacal malformation treated in 5 pediatric surgical centers between 1985 and 2009 were retrospectively studied for associated anomalies, postoperative complications, and colorectal and urological outcome. ResultsForty-two patients were eligible for this study, giving a mean exposure of less than 1 patient yearly per center. Forty-five percent of the patients had a short common channel (>3cm), and 14% had a long common channel. Length of common channel was missing in 41% of the patients. Median age of the cloacal reconstruction was 9months (range 1–121months). Twelve patients (29%) underwent an early surgical repair (within the first 6months of age; median 3months), and 30 (71%) patients underwent a late repair (after 6months of age; median 14months). Eighteen postoperative complications (<30days) had been documented in 15 patients (35%), with significant more perineal wound dehiscences in patients with an early repair (42% vs. 10%, p=0.031). There were no differences in complication rate between patients with short and long common channels. Mean follow-up was 142months (range 15–289). At the last follow-up, 10 patients (24%) had voluntary bowel movements. Fourteen patients (33%) had complaints of soiling, 25 (60%) were constipated, with no differences between the early and late repair groups. Patients in the late repair group as well as the group of patients with a short common channel were more frequently able to void spontaneously. ConclusionsPostoperative complications are common in patients with cloacal malformations. Early repair is associated with more wound dehiscences, however, without affecting long-term functional outcome. All centers had limited annual exposure of less than 1 patient. In these clinical settings, ideal age of cloacal reconstruction seems to be between 6 and 12months. In general, centralized care for these complex malformations may be the crucial factor for reducing postoperative complications and better long-term outcome.

Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys

This report describes a girl with congenital pouch colon (CPC), uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervix uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract.

Urethroplasty and vaginoplasty by a single small bowel interposition graft: a novel technique for cloacal reconstruction

Despite the advances in surgical management of anorectal malformations, repair of cloacal malformations remains a challenging condition for many pediatric surgeons. Posterior cloaca is present where urogenital sinus deviates posteriorly to form a cloaca with rectum. Often, achieving adequate urethral and vaginal length can be challenging. We describe a novel technique where a loop of small bowel on a single mesenteric blood supply could be constructed in 2 functional tubular structures: one for urethral and the other for vaginal replacement.

Congenital pouch colon in females

From January 1995 to March 1998, congenital pouch colon (CPC) with anorectal agenesis was diagnosed in ten girls who were classified into four groups based on the length of normal colon proximal to the colonic pouch. Of six girls with little or no normal colon, one had a cloacal anomaly while five had a colovesical or colovestibular fistula along with a completely bifid uterus and cervix and a septate vagina. Initial surgery consisted of ligation of the fistula and subtotal pouch excision with tubularization of the remaining colon in four girls, ileostomy after excision of a gangrenous pouch in one, and window colostomy in another. None of the patients had a sacral abnormality or associated major malformation. Definitive surgery using the posterior sagittal approach consisted of a pull-through of the tubularized colon in three girls (including one in whom one-stage cloacal reconstruction was performed), the ileum in one, and the proximal colon in another. The four girls with a longer length of normal colon had an associated vestibular fistula. In these, a colostomy was constructed just proximal to the pouch with definitive surgery in two patients consisting of excision of the pouch and pull-through of the proximal colon. Definitive surgery was well tolerated in all seven patients. The embryogenesis of this condition and the related genitourinary tract abnormalities are discussed.

Construction of female neourethra using a flipped anterior bladder wall tube

Two cases in which a neourethra was constructed using a flipped anterior bladder wall tube are reported. One girl had lost her urethra secondary to a major urethrovaginal injury from blunt pelvic trauma, and in the other, the urethra had sloughed after cloacal reconstruction. Both patients had a closed bladder neck/proximal urethra and were received with a suprapublic cystostomy. A neourethra was constructed from an anterior bladder wall flap based distally. The flap was tubularized and flipped caudally to the site of the original external urethral meatus. Both girls are continent—one voids normally and the other is managed by clean intermittent catheterization. The flipped anterior bladder wall tube technique for construction of a female neourethra is suitable in patients with an absent urethra, particularly when the bladder neck/proximal urethra is closed.