The postoperative renal function of persistent cloaca patients treated by posterior sagittal anorecto-urethro-vaginopalsty: results of a nationwide survey in Japan.

PURPOSE: We investigated the postoperative renal function in persistent cloaca (PC) patients who underwent posterior sagittal anorecto-urethro-vaginopalsty (PSARUVP) and factors influencing the renal functional outcomes. METHODS: A questionnaire survey was distributed to 244 university and children's hospitals across Japan. Of the 169 patients underwent PSARUVP, 103 patients were enrolled in the present study. Exclusion criteria was patients without data of renal prognosis. RESULTS: The present study showed that renal anomalies (p = 0.09), vesicoureteral reflux (VUR) (p = 0.01), and hydrocolpos (p = 0.07) were potential factors influencing a decline in the renal function. Approximately half of the patients had a normal kidney function, but 45.6% had a reduced renal function (Stage ≥ 2 chronic kidney disease: CKD). The incidence of VUR was significantly higher in the renal function decline (RFD) group than those in the preservation (RFP) group (p = 0.01). Vesicostomy was significantly more frequent in the RFD group than in the RFP group (p = 0.04). Urinary tract infections (p < 0.01) and bladder dysfunction (p = 0.04) were significantly more common in patients with VUR than in patients without VUR. There was no association between the VUR status and the bowel function. CONCLUSIONS: Prompt assessment and treatment of VUR along with bladder management may minimize the decline in the renal function.

Cloacal Dysgenesis Sequence in a Preterm Neonate.

BACKGROUND Cloaca malformations result from a disproportion of apoptosis, cell growth, and maturation. The range of cloacal malformations is extensive and diverse, with a lack of a straightforward classification system. Cloacal dysgenesis sequence (CDS), also known as urorectal septum malformation sequence, is a rare cloaca variant described as the absence of a perineal orifice. Prenatal magnetic resonance imaging and antenatal ultrasounds may reveal a cloacal malformation; however, many patients are not diagnosed with cloacal malformation until birth. CASE REPORT We present a case of a female neonate delivered by a 23-year-old G2P1T1A0L0 mother who had received comprehensive prenatal care. During pregnancy, bilateral multicystic dysplastic kidneys were identified prenatally, leading to the in utero placement of a vesicoamniotic shunt. The physical exam revealed a distended abdomen with reduced abdominal musculature and laxity, ascites, a vesicoamniotic shunt in place, absent urethra, ambiguous genitalia with no vaginal opening, no perineal opening, and clubfoot. Abdominal radiograph showed findings consistent with significant abdominal ascites. An exploratory laparotomy was performed that included diverting colostomy, mucous fistula creation, tube vaginostomy, removal of the vesicoamniotic shunt, and suprapubic tube placement. The patient recovered well from this operation with no complications. CONCLUSIONS CDS is an uncommon condition in pediatric patients, and although sonographic findings can reveal urinary tract abnormalities, prenatal imaging might not always identify CDS. Our case underscores the uniqueness of the case and the significance of early detection and immediate medical and surgical intervention.

Fetal ascites in cloacal malformations-a red flag.

INTRODUCTION: Cloacal malformation is a rare anomaly that remains a diagnostic challenge prenatally, despite the current advances in ultrasonography and MRI. This condition can in some, present with isolated ascites or with other findings, such as a pelvic cyst or upper urinary tract dilatation. In a minority, the ascites may be progressive, questioning the role of antenatal intervention. METHODS: We report on ten patients that have been identified from our Cloaca database between 2010 and 2022. RESULTS: The presence of ascites was associated with extensive bowel adhesions and matting, leading to a challenging initial laparotomy and peri-operative course. CONCLUSIONS: Antenatal finding of ascites in newborns with cloacal malformations should raise a red flag. The surgeon and anaesthetist should be prepared for the operative difficulties secondary to bowel adhesions and the higher risk of haemodynamic instability at the initial surgery. An experienced team at initial laparotomy in such patients is vital. LEVEL OF EVIDENCE: II.

Posterior sagittal anorecto-urethro-vagino-plasty in the late period was associated with the long-term bowel function in patients with persistent cloaca: results of a nationwide survey in Japan.

PURPOSE: After conducting a nationwide survey of persistent cloaca (PC), we assessed whether or not the timing of definitive anorectoplasty affects the long-term bowel function of patients with PC. METHODS: Patient information was obtained via questionnaire, and a total of 169 PC patients who underwent posterior sagittal anorectourethrovaginoplasty (PSARUVP) were enrolled in this study. Patients were classified into 2 groups based on their operative period, which was analyzed by the area under the receiver operating characteristic curve: the early group (EG) underwent anorectoplasty at ≤ 18 months old (n = 106), and the late group (LG) underwent anorectoplasty at > 18 months old (n = 63). The bowel function was evaluated using the evacuation score of the Japan Society of Anorectal Malformation Study Group. We also examined the postoperative results of vaginoplasty. RESULTS: The total evacuation score was significantly higher in the EG than in the LG (5.2 ± 1.7 vs. 4.2 ± 1.8, p = 0.003). The frequency of bowel movement and the constipation scores were significantly higher in the EG than in the LG (1.4 ± 0.6 vs. 1.2 ± 0.7, p < 0.05, 2.4 ± 1.0 vs. 2.1 ± 1.0, p < 0.05, respectively). Postoperative vaginal stenosis was observed in 18 cases (10.7%), of which 16 could be reconstructed transperineally. CONCLUSION: PSARUVP should be performed in early infancy and facilitate vaginal reconstruction.

Perinatal diagnosis of congenital urogenital sinus abnormality.

Anomalies of the urogenital sinus, which is a transient feature of the early human embryological development, are rare birth defects. Urogenital sinus abnormalities commonly present as pelvic masses, hydrometrocolpos, or ambiguous genitalia and most commonly occur within the context of congenital adrenal hyperplasia. Anomalies of the urogenital sinus requires surgical repair. We experienced a case of a female newborn with congenital urogenital sinus abnormality in which the early diagnosis helped us to prevent complications by decompressing the vagina soon after birth. Antibiotic prophylaxis was sufficient to avoid infections and to decompress the genitourinary system, thus allowing a deferred elective surgery to correct the sinus.

Partial urogenital mobilization in cloacal malformation: is it a viable option?

INTRODUCTION: Total Urogenital Mobilization (TUM) has been the standard surgical approach for the urogenital complex in Cloacal Malformations (CM) since its inception in 1997. Partial Urogenital Mobilization (PUM) in CM remains an under-utilized or under-reported option. The main anatomical difference between TUM and PUM is the division of the pubo-urethral ligaments. OBJECTIVE: We explored the feasibility of PUM in a select subset of our patients with CM and report early outcomes. STUDY DESIGN: We retrospectively reviewed prospectively collected data of all our CM patients who had primary reconstruction at our centre from 2012 to 2020. We included in our review the patients who underwent PUM. Mullerian abnormalities, spinal cord involvement, common channel length (CC), urethral length (UL), surgical reconstruction, and outcomes including urinary continence, recurrent UTI, ultrasound and preoperative DMSA/MAG3, cystovaginoscopy post-reconstruction, and post-void residuals were noted. RESULTS: Fifty-three patients had primary reconstruction, and of these, eleven had a common channel less than 3 cm. Of the eleven, only one underwent TUM. In the PUM group, two underwent filum untethering (20%). Mullerian duplication was noted in 5 patients (50%). The median CC length = 1.6 cm (range = 1.5cm-2.7 cm), and median UL = 1.5 cm (range = 1.5cm-2.5 cm). Follow-up ranged from 9 to 134months (median = 63months). Post-reconstruction all had a separate urethral and vaginal opening on examination and cysto-vaginoscopy. The continence outcomes are summarized in Fig.1. DISCUSSION: Although TUM is the most common solution for the urogenital complex in CM, a subset would be suitable for PUM, and this option is under-utilized or under-reported in literature. We presume that many who had TUM probably only needed a PUM, and therefore could report better outcomes from a bladder function aspect. It is important to differentiate the two, and outcomes should be appropriately categorized. Our default approach is a PUM in all CM with less than 3 cm common channel. Only the lateral and posterior aspects of the urogenital complex are mobilized and if the urethra did not reach a satisfactory level for easy intermittent catheterization, then we proceed to a TUM dividing the pubo-urethral ligaments. PUM avoids the potential complications related to dividing the pubo-urethral ligament in TUM. It may also avoid the need for CIC which is encountered in patients who undergo TUM. CONCLUSION: PUM is a viable alternative in cloacal malformations with good outcomes in those with a common channel under 3 cm. This of course requires appropriate patient selection and accurate categorization of interventions to understand the true outcomes.

A prospective cohort study of assisted bladder emptying following primary cloacal repair: The Children's National experience.

INTRODUCTION/BACKGROUND: Although the combination of bladder dysfunction and upper tract anomalies puts patient with cloaca at risk for renal disease, the rarity of this condition makes it difficult to study empirically. As a high-volume center, we uniquely capture bladder function outcomes following our growing number of cloacal repairs. OBJECTIVE: 1) Describe the rates of incomplete bladder emptying following primary cloacal repair (at 2-3 months after repair and last follow up), and 2) identify clinical factors associated with assisted bladder emptying. STUDY DESIGN: We performed a prospective cohort study of patients undergoing primary cloaca repair by our Children's National Colorectal Center team between 2020 and 2021. The primary outcome was assisted bladder emptying at 2-3 months postoperatively and last visit. Covariables included preoperative characteristics (cloacagram measurements), ARM complexity (moderate = common channel [CC] <3-cm, severe = CC ≥ 3-cm), vesicoureteral reflux (VUR) status, sacral ratio (good ≥0.7, intermediate 0.7-0.4, poor ≤0.4), spinal cord status, means of preoperative bladder emptying, and operative details (age at repair, repair type, & concomitant laparotomy). RESULTS: Eighteen participants were eligible. A majority had moderate cloaca (78%), VUR (67%), spinal cord abnormalities (89%), and good sacral ratios (56%). Preoperatively, 10 patients were diapered for urine and 8 had assisted bladder emptying. Surgical repairs were performed at a median age of 8 months (range 4-46). Nine (50%) patients underwent urogenital separation (UGS), eight (44%) total urogenital mobilization, and 1 (6%) perineal sparing posterior sagittal anorectoplasty with introitoplasty. Exploratory laparotomy was performed in 7 (39%) patients. At 2-3 months, 7 patients were voiding and 11 required assisted bladder emptying. Median length of long-term follow up was 12 months (range 5-25), and 8 patients were voiding and 10 required assisted bladder emptying. Postoperative need for assisted bladder emptying was significantly associated with assisted bladder emptying preoperatively, a shorter urethra and increasing common channel length, UGS and exploratory laparotomy. Spinal cord imaging findings were not associated. DISCUSSION: Bladder emptying following cloaca repair is likely a result of congenital function and surgical effects. Indeed, increasingly cloaca complexity requiring UGS and laparotomy was associated with both pre- and post-operative assisted bladder emptying. The lack of association with spinal cord imaging may reflect a divergence between anatomy and function. CONCLUSION: Approximately half of patients required assisted bladder emptying in this study. Associated factors included urethral and common channel length, the need for assisted bladder emptying preoperatively, the type of surgical approach and additional laparotomy. Being diapered with seemingly normal voiding prior to surgery did not guarantee normal bladder function postoperatively.

Clinical features of patients who underwent anoplasty for cloacal exstrophy and their functional outcomes: the results of a nationwide survey in Japan.

PURPOSE: This study was performed to clarify the clinical features of cloacal exstrophy (CE) patients who underwent anoplasty and their functional outcomes based on a nationwide survey in Japan. METHODS: A questionnaire survey was conducted and data were obtained from 229 CE patients. After the exclusion of non-surviving patients and unknown data, 195 patients were enrolled. We compared the patient characteristics of the no anoplasty group (NAP group) to the anoplasty group (AP group). The defecation function of patients who underwent anoplasty was evaluated. RESULTS: The AP group had significantly lower rates of omphalocele (p = 0.045) and spinal defects (p = 0.003) than the NAP group. Of the 195 patients enrolled in this study, only 17 (8.7%) underwent anoplasty. Of the 17 patients who underwent anoplasty, 7 (41.1%) eventually had a permanent stoma after anoplasty [after ileostomy: n = 3 (60.0%), after colostomy: n = 4 (44.4%)]. Regarding soiling, 4 patients (66.7%) showed the absence of soiling, 2 (33.3%) showed accidental soiling, and no patients showed frequent soiling. CONCLUSION: In our study, spinal defects may have affected the determination of anoplasty. Anoplasty may improve the quality of life, because defecation can be kept clean, but indications, including colon function, may be carefully considered.

Bowel Control, Bladder Function, and Quality of Life in Children with Cloacal Malformations.

INTRODUCTION: Long-term outcomes of cloacal malformations remain unclear. We evaluated postoperative bowel control, bladder function and quality of life in patients under 18 years of age with cloaca. MATERIALS AND METHODS: This was a multi-center cross-sectional observational study accomplished by the Nordic Pediatric Surgery Research Consortium. Patients with a cloacal malformation, 4-17 years of age, were eligible. Data including patient characteristics, surgical procedures, and complications were retrieved from case records. Established questionnaires with normative control values evaluating bowel function, bladder function, and health-related quality of life (HRQoL) were sent to the patients and their caregivers. The study was approved by the participating center's Ethics Review Authorities. RESULTS: Twenty-six (67%) of 39 eligible patients with median age 9.5 (range, 4-17) years responded. Twenty-one (81%) patients had a common channel ≤3 cm. Imaging confirmed sacral anomalies in 11 patients and spinal cord abnormalities in nine. Excluding patients with stoma (n = 5), median bowel function score was 12 [7-19], and 5 patients (20%) reported a bowel function score ≥17, approaching normal bowel control level. Bowel management increased proportion of socially continent school-aged children to 52%. Six (23%) patients had a permanent urinary diversion or used clean intermittent catheterization (CIC), while majority (70%) of the remaining patients were urinary continent. The reported HRQoL was comparable to healthy Swedish children. CONCLUSION: Whilst well-preserved spontaneous bowel control was rare, a majority of patients were dry for urine without any additional procedures. Few patients experienced social problems or negative impact on HRQoL due to bladder or bowel dysfunction. LEVEL OF EVIDENCE: Level IV.

Single institution experience of cloacal malformation.

INTRODUCTION: The aim of this study is to report on the outcomes of patients born with cloacal malformation, managed at a single institution more than the last 28 years. The focus of this study is the long term renal and colorectal outcomes. METHODS: Patients were identified from the departmental database from 1994 to 2021. The medical records and operative notes were retrospectively reviewed. RESULTS: Twenty-one patients fulfilled the inclusion criteria. Eleven long common channel (LCC) and ten short common channel (SCC) cloacae patients were identified. Median age at the time of primary reconstruction was 11 months in both groups. In the LCC group, seven (63.6%) patients underwent a Total Urogenital Mobilisation (TUM), and 4 (36.4%) required a vaginal replacement. 6/11 (54.5%) of patients required drainage of a hydrocolpos. In the SCC group, four patients required a TUM, two patients underwent mobilisation of the rectum and vagina alone, and three underwent rectal mobilisation alone. Two patients have required renal transplant for congenital renal dysplasia, and two have developed chronic renal failure associated with the sequalae of vesicoureteric reflux. Eleven (52.3%) of the patients manage their bowels with an antegrade continent enema (ACE), and two of the LCC cloaca are defunctioned with a colostomy. Clean intermittent catheterisation is performed by 12 (57%) of the patients, either per urethra or via a Mitrofanoff channel. CONCLUSION: The urinary and faecal continence are the main challenges in the management of cloaca patients. Many require surgical intervention to achieve social continence. LEVEL OF EVIDENCE: Level IV.

An Alternative Approach to Single-Stage Cloaca Repair: Image-Guided Anorectoplasty with Delayed Vaginoplasty.

BACKGROUND: Cloacal anomalies occur when a fetus's rectum, vagina, and urethra fail to separate and result in 1 common channel at birth. They are commonly managed by complex reconstruction in the first year of life. This manuscript presents an alternative approach to management in patients with absent or nondilated Mullerian structures. CASE: Image-guided, combined endoscopic and laparoscopic surgery (CELS) was used to perform an anorectal pull-through at 5 months of age on a patient with persistent cloaca and no definite vaginal or uterine structures seen on MRI and endoscopy. Urogenital reconstruction is delayed until adolescence. SUMMARY AND CONCLUSION: We hypothesize that performing a minimally invasive anorectoplasty on patients with complicated anatomy and low risk for hydrocolpos could potentially result in improved urologic function and better psychosocial outcomes. Delaying vaginoplasty will enable determination of the function of remnant Mullerian structures and allow the patient to direct the augmentation approach.

Laparoscopic management of common cloaca: Current status.

AIM: This study reviewed the literature on the laparoscopic management of cloaca. METHODS: A Medline and Embase search was performed for "laparoscopy" and "cloaca." Articles for which full-text in English was not available, duplicate articles, and review articles were excluded. Demographic characteristics, duration of follow-up, length of common channel, postoperative complications, and functional outcomes were analyzed. RESULTS: The database search retrieved 81 articles after excluding unrelated articles and identified new articles through cross-referencing 14 articles (72 patients) for this review. The rectal pouch was situated below the pubococcygeal (PC) line in three patients. In all other patients, the rectum was located above the PC line. Only the rectal component of the malformation was repaired laparoscopically in 80% (58/72). Fourteen patients underwent laparoscopic mobilization of the rectum and urogenital component. The length of the common channel was more than 3 cm in all these fourteen patients. The most common complication was rectal prolapse (n = 11). Functional evaluation by Krickenbeck scoring system was reported in 32 patients, of which 6/32 (18.75%) had fecal soiling > Grade 2. DISCUSSION: Until recently, laparoscopy for the common cloaca was almost exclusively used in patients with low urogenital sinus with high rectal pouch. Moreover, only the rectal component was repaired laparoscopically. Recently, laparoscopic rectal mobilization and urogenital separation was described for patients with common channel length ≥3 cm. It has been reported that laparoscopic vaginal mobilization is easy and more complete by this technique and may avoid vaginal replacement in most of these patients with the long common channel. However, only two studies have reported this technique, and its reproducibility and long-term results are still awaited. Another interesting observation was the increasing use of urethral length along with common channel length in determining the appropriate procedure for the patients with common cloaca. Recent studies propose that the urogenital separation technique be preferred over urogenital mobilization in patients with the short urethra. Nonetheless, we still don't have long-term comparative data to demonstrate that the functional outcomes are better with this new algorithm. We conclude that the persistent cloaca needs an individualized approach, and laparoscopy can be utilized to mobilize the high rectum and is also helpful for the urogenital separation in patients with common channel length >3 cm. However, at present, there is no conclusive evidence to support that laparoscopic repair has a better functional outcome than the open approach.

Laparoscopic Resurrection of an Old Technique: A New Approach for Total Urogenital Separation and Rectal Pull-Through in Patients with Long Common Channel Cloacal Malformation.

Background and Aims: Before the significance of urethral length was highlighted in patients with cloacal malformation, total urogenital mobilization using a posterior sagittal approach was recommended for common channel (CC) length <3 cm, those >3 cm it was followed by urogenital separation. However, many urologists are advocating that the urethral length rather than length of the CC should influence the choice of operation. It is also recommended that total urogenital mobilization should be avoided in patients with short urethral length as intraoperative decision to shift to urogenital separation will risk devascularization of the urethra, advocating total urogenital separation (TUS) from the start; the later technique was deemed difficult using open approach. We describe our experience with laparoscopic TUS and rectal pull-through in patients with cloacal malformation. Methods: Six patients were operated for a period of 3 years from December 2017 to July 2021; they underwent laparoscopic TUS and rectal pull-through. Preoperative investigations included cystoscopy, genitogram, and MRI pelvis and abdominal ultrasound. IRB approval has been obtained from research ethical committee at Cairo University. Results: Six female patients born with single perineal opening had colostomy at birth. Age during the second operation ranged from 1 to 4 years. Length of the CC ranged between 2 and 5 cm. Proximal urethral length ranged between 0.5 and 1.5 cm and vaginal depth >3 cm. Average operative time was 4.25 hours. Postoperative period was 1-5 days and uneventful. On the long-term follow-up. No patient developed urethrovaginal fistula and one patient developed vaginal stenosis. All patients had no urinary problems, dry over 4-hour interval, voiding spontaneously, and had normal kidney functions. Conclusions: Laparoscopic urogenital separation, as well as vaginal and rectal pull-through for cloacal malformation, is feasible in cloacal malformation providing anatomical repair.

Efficacy of vesicostomy for refractory metabolic acidosis in persistent cloaca.

Persistent cloaca involves fusion of the bladder, vagina, and rectum into a single duct called the common duct. Although its pathogenesis remains unclear, it has been associated with hyperchloremic metabolic acidosis. Herein, we present the case of a neonatal girl with high-confluence type variant of persistent cloaca treated with vesicostomy (Blocksom) for refractory metabolic acidosis. She was diagnosed with persistent cloaca before birth; colostomy was performed and a urinary catheter was placed in the bladder. Voiding cystourethrography on day 19 after birth showed that most of the contrast material leaked into the rectum; hence, the urinary catheter was removed. On day 27, hyperchloremic metabolic acidosis was detected and treatment with oral sodium bicarbonate was initiated; however, the infant showed no response. Because hyperchloremia occurred after removal of the urinary catheter, continuous urine retention in the colon through the common duct was believed to have caused the progression of hyperchloremic metabolic acidosis through transporters in the intestinal mucosa. As reinstallation of a urinary catheter was technically difficult, vesicostomy was performed on day 29, after which the metabolic acidosis improved. This report suggests vesicostomy as an effective treatment for refractory hyperchloremic metabolic acidosis associated with high-confluence type persistent cloaca.

Cysto-Vaginoscopy of a 3D-Printed Cloaca Model: A Step toward Personalized Noninvasive Preoperative Assessment in Patients with Complex Anorectal Malformations.

INTRODUCTION: For the classification of the complexity of cloacal malformations and the decision on the operative approach, an exact anatomical assessment is mandatory. To benefit from using three-dimensional (3D)-printed models in preoperative planning and training, the practicability of these models should be guaranteed. The aim of this study was to evaluate the quality and feasibility of a real-size 3D-printed cloaca model for the purpose of cysto-vaginoscopic evaluation. MATERIALS AND METHODS: We performed a 3D reconstruction and printed a real-size, rubber-like 3D model of an infant pelvis with a cloacal malformation and asked invited pediatric surgeons and pediatric urologists to perform a cysto-vaginoscopy on the model and to complete a brief questionnaire to rate the quality and feasibility of the model and to indicate whether they would recommend the model for preoperative planning and training. RESULTS: Overall, 41 participants rated the model quality as good to very good (M = 3.28, standard deviation [SD] = 0.50, on a scale from 1 to 4). The model was rated as feasible for preoperative training (M = 4.10, SD = 0.75, on a scale from 1 to 5) and most participants (85.4%) would recommend the model for preoperative training. The cysto-vaginoscopy of the model was considered as a valid training tool for real-life cases and improved the confidence on the anatomy of a cloaca. CONCLUSION: The results of our study indicate that patient-specific 3D-printed models might be a useful tool in the preoperative evaluation of complex anorectal malformations by simulation of cysto-vaginoscopy with an excellent view on anatomical structures to assess the whole spectrum of the individual cloacal malformation. Our model might be a valuable add-on tool for specialty training in pediatric colorectal surgery.

Reconsidering Diagnosis, Treatment, and Postoperative Care in Children with Cloacal Malformations.

Cloacal malformations are among the most complex types of anorectal malformation and are characterized by the urological, genital, and intestinal tracts opening through a single common channel in the perineum. Long-term outcome is affected by multiple factors, which include anatomical variants of the malformation itself, associated anomalies, and the surgical approach. Reconsidering these variables and their influence on "patient important" function might lead to strategies that are more outcome-driven than focused on the creation of normal anatomy. Key outcomes reflect function in each of the involved tracts and the follow-up needed should therefore not only include the classical fields of colorectal surgery and urology but also focus on items such as gynecology, sexuality, family-building, and quality of life as well as other psychological aspects. Involving patients and families in determining optimal treatment strategies and outcome measures could lead to improved outcomes for the individual patient. A strategy to support delivery of personalized care for patients with cloacal malformations by aiming to define the best functional outcomes achievable for any individual, then select the treatment pathway most likely deliver that, with the minimum morbidity and cost, would be attractive. Combining the current therapies with ongoing technological advances such as tissue expansion might be a way to achieve this.

Laparoscopic Rectal and Vaginal Pull-Through in the Same Setting in Cloaca with Common Channel of More Than 3 cm Separation.

Aim of the Study: This study aims at evaluating the feasibility and outcome of a laparoscopically assisted rectal and vaginal pull-through procedure in the same setting for cases of long common channel in a cloaca >3 cm in length. Methods: I have 4 cases with a cloaca with long common channel >3 cm in length diagnosed by cloacogram and cystoscopy. As for the ages of our cases, two of them were 1 year old, and the other 2 cases were 2 years old. Laparoscopic rectal and vaginal pull-through in the same setting was performed in all cases. The operative time was 3 hours. A tension-free anastomosis of the rectum in anal complex was carried out; also anastomosis of vagina to the perineum was realized after laparoscopic mobilization of the vagina, separation from the bladder neck at the confluence and pull-through. This is to avoid perineal or perirectal dissection. Results: Frequent anal and vaginal dilation after 2 weeks from operation was carried out. Our cases have not demonstrated any stricture, or urethrovaginal fistula. This procedure resulted in a good cosmetic and unimpaired functional outcome. It has been conducted also to avoid excessive perineal dissection, and reduce risk of urinary incontinence by evading the occurrence of urethral sphincter damage. Conclusion: Laparoscopic-assisted rectal and vaginal pull-through is a new approach for long common channel cloaca that avoids perineal dissection, reducing dissection, and risk of urinary incontinence. This technique should be used and implemented on a wide scale.

Single perineal opening with 'H-type' cecovesical fistula and blind-ending foreshortened distal colon: a new variant of persistent cloaca.

Persistent cloaca is a rare and severe variety of anorectal malformation, which is more common in females and includes a spectrum of abnormalities. The urinary tract, genital tract and rectum open into a common channel, which exteriorises as a single perineal opening. We are reporting a patient with a novel variation in the classical anatomy of the cloaca. The child has a short blind-ending colon with a cecovesical fistula associated with mullerian agenesis and lipomyelomeningocoele. The child is being managed in a stepwise approach and she has completed the anal reconstruction. Here, we discuss this novel variation in anatomy and challenges in its management.

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

BACKGROUND: Cloacal 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. METHODS: A 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. RESULTS: A 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. CONCLUSIONS: The 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 EVIDENCE: Level II.

Long-term urological assessment and management of cloaca patients: A single tertiary institution experience.

PURPOSE: Preserving renal function and achieving urinary continence are the most important urological goals in the management of cloaca patients. Many prognostic factors have been described, such as the length of the common channel (CC) and urethra, the presence of spinal dysraphism, and associated urological anomalies. The aim of this study was to analyze urinary continence status and need for reconstructive procedures and their correlation with urological risk factors in a series with a long-term follow-up. MATERIAL AND METHODS: The institutional database of patients with anorectal malformations was reviewed. Patients with cloaca who underwent cloacal reconstructive surgery at our institution between January 1995 and May 2015 and who had a minimum postoperative follow-up of 5 years with complete urological care were included. Urologic and spinal anomalies, length of the CC, renal function, urodynamic study patterns, continence status, and urologic reconstructive surgeries were assessed. A CC was defined as long CC when its measure was longer than 3 cm in the cloacogram and then confirmed by cystoscopy. Descriptive statistical analysis was performed. RESULTS: Fifty-five cloaca patients with a mean follow-up of 12 years (5-20) were included. A long CC was documented in 38 patients (69%). The sacral ratio (SR) was <0.4 in the AP projection in 30 (54.5%). Urodynamic evaluation revealed an inadequate detrusor contraction pattern in 65.4% of the cases. A CC >3 cm and SR <0.4 were significantly correlated with this urodynamic finding. A total of 50 patients (91%) achieved urinary continence, but only 30.9% had volitional voiding and 56% needed major urological reconstructive surgeries. Eighteen patients (32.7%) were in stage 2 or more of chronic kidney disease (<90 ml/min/1.73 m2) at the last follow-up visit. CONCLUSION: Cloacal management requires a multidisciplinary and long-term follow-up. Early assessment of prognostic urological factors and accurate stratification of each patient are essential to avoid renal impairment and achieve urinary continence in the future. The length of the common channel and the presence of spinal dysraphism were correlated with the presence of neurovesical dysfunction specifically an abnormal bladder contraction efficiency. A significant number of these patients will need catheterization and reconstructive urinary tract surgeries to attain urinary continence. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level 3. Retrospective comparative study.