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.

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.

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.

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.

Asymmetrical Cloacoplasty for the Treatment of Chronic Cloacal Prolapse in Psittaciformes: A Case Series.

Chronic cloacal prolapse is a common condition in psittacine birds, particularly in cockatoos and African grey parrots (Psittacus erithacus). Techniques to permanently reduce the prolapse have been developed and include cloacal sutures, percutaneous cloacopexy, and open circumcostal or rib cloacopexy. Several complications, some of which are life-threatening, have been reported with these tissue reduction techniques. Cloacoplasty surgeries have also been advocated, but most often as an adjunct therapy. Recurrence of cloacal prolapse may occur in traditional symmetrical cloacoplasty techniques since straining pressure remains. In this case series, 5 psittacine birds (2 male and 2 female umbrella cockatoos [Cacatua alba] and 1 male African grey parrot) with chronic recurrent cloacal prolapses were treated with a novel asymmetrical cloacoplasty technique. A semicircular excision at the mucocutaneous junction from the right side with a 2-layer closure reduced the cloacal opening by 50%-75%. A tension-relieving suture was applied to aid in healing of the incision. All 5 cases had successful outcomes with no recurrent prolapses (6 months to 3 years of follow-up), except for 1 case, due to incisional dehiscence. This animal underwent a second surgical procedure that incorporated the tension-relieving suture and has not had a recurrent prolapse in over a year. This asymmetrical cloacoplasty technique provides a simple but effective method of cloacal prolapse treatment and prevention in cases of recurrent and chronic cloacal prolapses, and creates a midline physical barrier not present in symmetrical cloacoplasties. Long-term complications may include cloacolith formation or dystocia in females, and therefore reproductive management of female birds is required.

Surgical repair of traumatic cloaca: a modified technique.

AIM: A modified repair technique for traumatic cloaca caused by obstetric anal sphincter injury was evaluated, and its feasibility and functional outcome were investigated. METHODS: A retrospective review of 23 consecutively enrolled patients diagnosed with traumatic cloaca who underwent the modified repair technique between September 2010 and August 2018 was performed. Demographic, clinical feature, operative and follow-up data were recorded. RESULTS: The patients diagnosed with traumatic cloaca who underwent surgical repair after obstetric anal sphincter injury had a median time from obstetric injury of 24 (12-35) years. The median preoperative Wexner faecal incontinence score was 16 (14-17). The postoperative hospital stay was 6 (6-7) days. The median postoperative Wexner faecal incontinence score decreased to 2 (2-3). The anal resting pressure increased from 9.00 (5.25-11.50) mmHg to 56.00 (55.00-65.75) mmHg (P < 0.01) and the anal squeeze pressure increased from 29.00 (22.50-33.20) mmHg to 110.00 (96.20-121.50) mmHg (P < 0.01) at 2 months after the repair. Sixteen patients completed the Faecal Incontinence Quality of Life Scale questionnaire, and there were significant improvements 1 year after surgical repair in lifestyle (3.10 [2.60-3.70] vs 2.60 [1.90-3.00], P < 0.01), coping/behaviour (3.38 [2.57-3.44] vs 2.33 [1.89-3.00], P < 0.01), depression/self-perception (3.11 [2.27-3.44] vs 2.33 [1.89-3.00], P < 0.01) and embarrassment (3.33 [2.75-3.67] vs 2.33 [2.33-3.00], P < 0.01). No patient presented rectovaginal fistula postoperatively within the median follow-up period of 24 (12-48) months. CONCLUSIONS: The modified repair technique for traumatic cloaca is feasible and achieves good functional outcomes and improved life quality.

Early vaginal replacement in cloacal malformation.

PURPOSE: We assessed the surgical outcome of cloacal malformation (CM) with emphasis on need and timing of vaginal replacement. METHODS: An ambispective study of CM was carried out including prospective cases from April 2014 to December 2017 and retrospective cases that came for routine follow-up. Early vaginal replacement was defined as that done at time of bowel pull through. Surgical procedures and associated complications were noted. The current state of urinary continence, faecal continence and renal functions was assessed. RESULTS: 18 patients with CM were studied with median age at presentation of 5 days (1 day-4 years). 18;3;2 babies underwent colostomy; vaginostomy; vesicostomy. All patients underwent posterior sagittal anorectovaginourethroplasty (PSARVUP)/ Pull through at a median age of 13 (4-46) months. Ten patients had long common channel length (> 3 cm). Six patients underwent early vaginal replacement at a median age of 14 (7-25) months with ileum; sigmoid colon; vaginal switch; hemirectum in 2;2;1;1. Three with long common channel who underwent only PSARVUP had inadequate introitus at puberty. Complications included anal mucosal prolapse, urethrovaginal fistula, perineal wound dehiscence, pyometrocolpos, bladder injury and pelvic abscess. Persistent vesicoureteric reflux remained in 8. 5;2 patients had urinary; faecal incontinence. 2 patients of uterus didelphys are having menorrhagia. One patient succumbed to sepsis at 7 months age. Renal functions remained normal in 16. One patient is undergoing dialysis. CONCLUSION: Early vaginal replacement in CM is feasible. Patients with inadequate introitus may suffer from menorrhagia. A regular follow-up is mandatory.

Diagnosis and Surgical Repair of an Acute Abdominal Wall Hernia and Partial Cloacal Strangulation in a Yellow-Naped Amazon Parrot (Amazona auropalliata).

A 38-year-old female, intact yellow-naped Amazon parrot (Amazona auropalliata) presented with an acute swelling along the ventrocaudal body wall, hematochezia, and tenesmus. Physical examination identified a defect in the ventral body wall. Bimodal pain management was initiated at presentation and hematochezia and tenesmus resolved. Radiographic imaging and contrast fluoroscopy identified a partial cloacal strangulation. An exploratory celiotomy was performed. Adhesions to the body wall were identified and broken down, the cloacal position was corrected, and the abdominal musculature repaired. Contrast fluoroscopy performed 72 hours after surgery confirmed normal positioning of the cloaca. Follow-up examinations documented proper postoperative healing of the hernia repair and maintenance of visibly normal passage of droppings. Although various abdominal hernias have been described in birds, this case demonstrates an unreported variation involving partial cloacal strangulation in a parrot.

Giant fetal hydrometrocolpos associated with cloacal anomaly causing postnatal respiratory distress.

Persistent cloaca is a rare presentation wherein the urethra, vagina, and rectum converge into a common channel with a single perineal opening. Fetal hydrometrocolpos can result if fluid accumulates behind an obstruction of this common channel. A 29-year-old woman (G4P1021) was referred at 36 2/7 weeks of gestation for evaluation of a fetal abdominal cystic mass. Detailed ultrasonography and magnetic resonance imaging showed two symmetric cystic masses, bilateral hydronephrosis, and oligohydramnios. Elective cesarean delivery was performed at 37 0/7 weeks; the baby weighed 4043 g with Apgar scores of 5 and 6 at 1 and 5 min. Intubation was performed for respiratory distress, and the infant was noted to have an imperforate anus; persistent cloaca was diagnosed. Drainage of the hydrometrocolpos improved the infant's breathing remarkably, and extubation was achieved. This child's imaging findings are among the largest ever reported, and resulted in neonatal respiratory distress.

A unique defect - persistent posterior cloaca: An example of staged genito-urinary and digestive tract reconstruction with an alternative vaginal creation using the urinary bladder wall.

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.

Cloacal Prolapse in Raptors: Review of 16 Cases.

Sixteen cases of cloacal prolapse in raptors were reviewed in this study. Colonic prolapse was the most common presentation (56% of cases). Red-tailed hawks ( Buteo jamaicensis ) were overrepresented, comprising 66% of colonic prolapse cases. In cases of colonic prolapse, postsurgical stricture formation was a commonly identified complication after resection and anastomosis of the colon. A novel technique was used in 2 cases of colonic prolapse, in which sterile, semirigid rubber tubing was placed in the distal colon and removed per-cloaca at the end of the procedure; this facilitated a secure, fluid-tight anastomosis while maintaining sufficient intestinal lumen. Oviductal prolapse (31% of cases) was associated with the most guarded prognosis (40% treatment success). Cloacoliths were treated successfully in 2 birds (13% of cases) by minimally invasive per-cloacal manual removal.

Chronic Kidney Disease Stage Progression in Patients Undergoing Repair of Persistent Cloaca.

PURPOSE: Children born with persistent cloaca undergo complex pelvic reconstruction early in life. Long-term risks of bladder dysfunction and chronic kidney disease are well described. We report upper urinary tract outcomes and the risk of chronic kidney disease stage progression in this patient population. MATERIALS AND METHODS: We retrospectively studied a cohort of patients undergoing posterior sagittal anorecto-vagino-urethroplasty at a single institution from 2006 to 2013. Inclusion criteria consisted of complete urological care at our institution. Chronic kidney disease stage was calculated from cystatin C or nuclear medicine glomerular filtration rate. RESULTS: A total of 44 patients met inclusion criteria. Of the patients 12 had undergone vesicostomy or ureterostomy. A total of 19 patients had hydronephrosis, 19 had vesicoureteral reflux and 15 had a tethered spinal cord. Median length of the common channel was 3.5 cm. Median age at posterior sagittal anorecto-vagino-urethroplasty was 7.3 months. Median followup was 5.3 years. A total of 30 patients had neurogenic bladder, of whom 27 required clean intermittent catheterization and 3 had undergone vesicostomy. Of the patients 38 had stage I or II, 5 had stage III and 1 had stage IV chronic kidney disease. During followup no patient with initial stage I to III chronic kidney disease had stage progression. The patient with stage IV chronic kidney disease had a renal allograft placed at age 34 months before needing dialysis. CONCLUSIONS: Early outcomes in patients with stage I to III chronic kidney disease demonstrate that renal function can be maintained despite a high rate of lower urinary tract dysfunction. Aggressive bladder management may help prevent progressive renal injury in this population.

Postoperative complications after reconstructive surgery for cloacal malformations: a systematic review.

The repair of cloacal malformations is most often performed using a posterior sagittal anorecto-vagino-urethroplasty (PSARVUP) or total urogenital mobilization (TUM) with or without laparotomy. The aim of this study was to systematically review the frequency and type of postoperative complication seen after cloacal repair as reported in the literature. A systematic literature search was conducted according to preferred reporting items for systematic reviews and meta-analyses guidelines (PRISMA). Eight records were eligible for this study which were qualitatively analyzed according to the Rangel score. Overall complication rates reported in included studies ranged from 0 to 57 %. After meta-analysis of data, postoperative complications were seen in 99 of 327 patients (30 %). The most common reported complications were recurrent or persistent fistula (n = 29, 10 %) and rectal prolapse (n = 27, 10 %). In the PSARVUP group, the complication rate was 40 % and in the TUM group 30 % (p = 0.205). This systematic review shows that postoperative complications after cloacal repair are seen in 30 % of the patients. The complication rates after PSARVUP and TUM were not significantly different. Standardization in reporting of surgical complications would inform further development of surgical approaches. Other techniques aiming to lower postoperative complication rates may also deserve consideration.

Male cloaca malformation: rare variant of anorectal malformation.

PURPOSE: Experience with male cloaca (MC), a single opening in perineum for passage of urine and meconeum is described. METHODS: Cases of MC were ambispectively studied, prospectively from July 2007 to April 2015 and retrospectively for last three decades. RESULTS: Seven cases of MC were identified, between the ages of newborn-4 years (median 10 days). Two missed cases underwent a colostomy, posterior sagittal anorectoplasty, and urethroplasty. Two cases underwent perineal urethrostomy and anoplasty followed by urethroplasty. In one case, part of the rectal wall was used to form urethral tube and urethrostomy. For three recent cases, posterior sagittal anorectourethroplasty was done with mobilization of rectal pouch and common channel, separation of common wall between the urethra and rectum, urethroplasty varying from 1.5 to 3 cm, perineal body reconstruction, perineal urethrostomy and anoplasty. Follow-up of 6 patients varied from 3 months to 23 years. One case is lost to follow-up. Three patients have completed repair. Complications included a discharging sinus and a urethral fistula in one case each. One patient died while awaiting urethroplasty. Two patients are awaiting formal urethroplasty. CONCLUSION: With familiarity of varying anatomy of MC, early recognition can avoid a neonatal colostomy in selected patients.

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.

Congenital prepubic sinus associated with a urachal remnant: report of a case.

Congenital prepubic sinus is a rare congenital anomaly situated in the midline of the lower abdomen. We report a case of congenital prepubic sinus, closely associated with a urachal remnant. Preoperative magnetic resonance imaging showed clearly that the sinus tracked the urachus caudally. This finding supports the theory that the anomaly is caused by abnormal remnant tissue originating from the cloacal membrane, which tracks the allantois duct caudally along with fetal longitudinal growth.