35th ESPU Joint Meeting in Vienna, Austria

S26: CLOACA

ESPU Meeting on Friday 5, September 2025, 11:10 - 12:00


11:10 - 11:13
S26-1 (OP)

LUTO-CLOACA - A NEW ENTITIY OF PERSISTENT CLOACA WITH PRENATAL VESICOAMNIOTIC SHUNTING

Ina MEMETAJ-LANG, Lena GINDNER and Dr. Thomas BOEMERS
Childrens Hospital Cologne, Amsterdamer Street, Pediatric surgery und pediatric urology, Cologne, GERMANY

PURPOSE

Since the application of vesicoamniotic shunting (VAS), we have encountered severe forms of cloacal malformations associated with the prenatal appearance of Lower Urinary Tract Obstruction (LUTO plus 46XX karyotype). This patient group is very heterogeneous in terms of anatomical findings. However, there appears to be a specific subgroup of these patients with a distinct anatomical constellation. In our opinion  thix subgroup represents a new entity of cloacal malformations. The aim of this presentation is to describe the anatomical features and clinical implications of this new entity of cloacal malformation. 

MATERIAL AND METHODS

We conducted a retrospective analysis of our patients with cloacal malformations who underwent prenatal intervention (VAS). We analyzed the timing of VAS, the distinct anatomical findings, including endoscopy, imaging and the the type of operative reconstruction performed.

RESULTS

A total of 11 patients were identified. Eight patients exhibited classic anatomical features of cloacal malformation. In three patients, we found a significantly different anatomical constellation. This specific group demonstrated a hypoplastic common channel leading into the bladder, while the rectum opened in the middle of the upper part of the trigonum. The vaginae opened laterally to the rectum. In all three patients, the pelvic opening was very narrow, allowing only one of the three pelvic organ systems to be pulled through.

CONCLUSIONS

The implementation of VAS in female patients with LUTO challenges us, as this new entity of cloacal malformation is not amenable for complete surgical reconstruction. Appropriate prenatal counseling, as well as treatment in specialized centers is crucial for these difficult patient group. Parents have to be aware of the problems encountered after birth, best before the decision for VAS is made.


11:13 - 11:16
S26-2 (OP)

IS NEWBORN HYDROCOLPOS ASSOCIATED WITH THE FUTURE USE OF ASSISTED BLADDER EMPTYING AMONG PATIENTS WITH CLOACAL ANOMALIES?

Diego GONZALEZ 1, Natalie EWING 2, Butool HISAM 3, Christopher CORBETT 3, Thomas O. XU 4, Christina P. HO 3, Hans G. POHL 3, Andrea BADILLO 4, Marc LEVITT 4 and Briony K. VARDA 3
1) George Washington University School of Medicine and Health Sciences , Medicine, Washington, USA - 2) George Washington University School of Medicine and Health Sciences, Medicine, Washington, USA - 3) Children's National Hospital, Urology, Washington, USA - 4) Children's National Hospital, Colorectal Surgery, Washington, USA

PURPOSE

Is newborn hydrocolpos associated with the future use of assisted bladder emptying among patients with cloacal anomalies? 

MATERIAL AND METHODS

We identified all patients with a history of cloaca who presented at a single institution from 2019 to 2024. The outcome was the use of ABE. Hydrocolpos at birth was the exposure variable. Covariables included cloaca characteristics, genitourinary and spinal abnormalities, and repair type. Descriptive statistics were calculated and logistic regression performed.  

RESULTS

71 patients with a median age of 5.4 years (range 1 – 26) were included. 35 (49%) had a complex cloaca (common channel ≥3-cm), 43 (61%) had vesicoureteral reflux (VUR), 36 (51%) had a spinal cord abnormality, and 58 (82%) had a sacral ratio >0.4. 24 (34%) had hydrocolpos at birth. ABE was used by 45 (63%) patients at last follow up. ABE use was greater in patients with complex cloaca (30 [86%] vs. 15 [42%], p < 0.001), a history of urogenital separation (27 [82%] vs 18 [47%], p < 0.001) and VUR (32 [74%] vs 13 [46%] p = 0.03). 37 (90%) patients who used ABE pre-repair continued it post-operatively. On adjusted analysis, only complex cloaca increased the odds of ABE use (OR 6.7 [CI 2.1-24.3], p = 0.002). Hydrocolpos was not associated with ABE (p = 0.7).  

CONCLUSIONS

Cloacal complexity independently increased the likelihood of ABE use after cloacal repair. Hydrocolpos was not associated.

Future Use of Assisted Bladder Emptying Among Patients with Cloaca (median age 5 years-old)

 Adjusted Association (p < 0.05) 

 Unadjusted Association (p < 0.05) 

 Not Associated (p > 0.05) 

Cloaca complexity  

(common channel≥3-cm) 

Cloaca complexity

(common channel≥3-cm)  

Spinal cord abnormality  
Vesicoureteral reflux  Hydrocolpos 

Use of Assisted bladder emptying prior to cloacal repair 

History of spinal cord detethering 
Type of cloacal repair  Sacral Ratio 
Demographic variables

11:16 - 11:19
S26-3 (OP)

IS THE LENGTH OF COMMON CHANNEL A PREDICTOR FOR UROLOGICAL ANOMALIES IN CLOACAL MALFORMATIONS?

Francesca PALMISANI 1, Sophie LANGER 2, Francesca GALBIATI 3, Leila HARTFORD 4, Anna MORANDI 3, Renate FARTACEK 2, Ernesto LEVA 3 and Giulia BRISIGHELLI 5
1) Medical Universtity of Vienna, Department of Pediatric Surgery, Vienna, AUSTRIA - 2) Medical University of Vienna, Department of Pediatric Surgery, Vienna, AUSTRIA - 3) Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Department of Pediatric Surgery, Milano, ITALY - 4) Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Pediatric Colorectal and Pelvic Reconstruction Centre, Department of Pediatric Surgery, Johannesburg, SOUTH AFRICA - 5) Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Department of Pediatric Surgery, Johannesburg, SOUTH AFRICA

PURPOSE

Prognosis of cloacal malformations is strongly impacted by the presence of associated renal anomalies and the risk of chronic renal failure. 

In accordance with the concept of caudal regression’s anomalous cloacal septation, it has been hypothesized that a longer common channel (CC) increases the risk of urological issues. However, this remains unproven. 

This study aims to determine if CC length predicts the risk of urological anomalies in cloacal malformations.

MATERIAL AND METHODS

We conducted a retrospective review of patients with cloacal malformations treated between 2015 and 2024 at pediatric colorectal clinics in three international centers: Chris Hani Baragwanath Academic Hospital (Johannesburg, South Africa), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (Milan, Italy) and the Medical University of Vienna (Vienna, Austria). We evaluated radiological, surgical and clinical data, stratifying patients by CC length ( > or < 3 cm).

RESULTS

A total of 72 patients were identified, with 26 excluded due to incomplete data. Of the remaining 46 patients, 21(46%) had a CC > 3 cm,while 25(54%) had a short CC. Overall, 31 patients (68%) presented at least one renal anomaly, with hydronephrosis (58%) being the most common. Anatomical anomalies, including vesicoureteral reflux, single or ectopic kidneys, duplex systems and multicystic-dysplastic disease, were observed in 18 patients (40%). The distribution of anomalies was equal in both groups (9 each for long and short CC), with no statistically significant association (p = 0.55).

CONCLUSIONS

In this study, CC length did not predict urological anomalies. Further research is essential to better understand the pathogenesis of these associations.


11:19 - 11:22
S26-4 (OP)

OUTCOMES AND MANAGEMENT OF MULLERIAN ANOMALIES IN FEMALE PATIENTS WITH COMPLEX CONGENITAL BIRTH DEFECTS OF CLOACAL EXSTROPHY AND ANORECTAL MALFORMATION

Nora BROADWELL, Geneva PANTOJA, Juliet ALEXANDER, Alisha PAZ, Aidan BOYNE, Catherine NGUYEN, Andrea BALTHAZAR and Niccolo Maria PASSONI
Texas Children's Hospital, Pediatric Urology, Houston, USA

PURPOSE

Females with cloacal exstrophy (CE) and cloacal anomalies (CA) have high rates of mullerian abnormalities leading to outflow tract obstruction (OTO) of menses, invasive surgeries, and/or chronic pain. This study aims to discuss long-term outcomes of mullerian structures in a single major institution with a high volume of CE and CA patients.

MATERIAL AND METHODS

A retrospective database was reviewed for CE and CA patients. Data on mullerian anatomy at birth, hormone suppression, and surgical procedures were evaluated.

RESULTS

91 females (46XX) were included with median age of 14.5 [0.17, 30.5] years. Of those, 68.1% had duplicated uteri. Vaginal anatomy was duplicated in 52.7% with 18.7% having complete atresia. 23.1% of patients underwent a hysterectomy (41.2% CE, 18.9% CA). Hysterectomy was performed prior to menarche in 52.4% and post menarche in 47.6%. Hormone suppression was used in 24.2% (22/91) of the entire cohort, with 45.2% (10/22) of those patients proceeding to hysterectomy. In those with hormone suppression alone (54.5%, 12/22), either vaginoplasty to relieve obstruction or hysterectomy of obstructed horn is planned for the future once the patient is ready to participate in surgical discussion. All hysterectomies were performed on patients with duplicated uteri. Reasons for pre-menarchal hysterectomy included nonfunctional or noncommunicating uteri, little chance of safe pregnancy, and/or family desire for minimal surgical intervention. Post-menarchal hysterectomy reasons included pelvic pain secondary to hematocolpos, desire to discontinue hormone suppression and/or need for vaginostomy, and/or vesicouterine fistula.

CONCLUSIONS

This study demonstrates high rates of surgical removal of mullerian structures in CE and CA patients. Further study would be beneficial for early identification of patients at risk of requiring hysterectomy while maintaining fertility potential in those with low risk of mullerian complications.


11:22 - 11:38
Discussion
 

11:38 - 11:41
S26-5 (OP)

COMPARATIVE OUTCOMES OF PSARVUP AND TUM IN CLOACAL MALFORMATIONS: A RETROSPECTIVE STUDY

Mohamed ABDELMALAK 1 and Saber WAHEEB 2
1) Nile of Hope Hospital, Pelvic reconstruction surgery, Alexandria, EGYPT - 2) Alexandria university, Pediatric surgery, Alexandria, EGYPT

PURPOSE

To evaluate the technical feasibility and long-term urological outcomes of posterior sagittal anorecto-vagino-urethroplasty (PSARVUP) and total urogenital mobilization (TUM) in patients with cloacal malformations.

MATERIAL AND METHODS

A retrospective study of 30 cloaca patients who underwent definitive repair at two tertiary centers. Preoperative evaluations included cystoscopy, distal colostogram, and MRI to assess common channel and urethral lengths. Patients underwent PSARVUP (n=18) or TUM (n=12) based on surgical preference. Postoperative outcomes were reviewed over three years, with assessments of urinary tract infections (UTIs), voiding patterns, continence, and urodynamic parameters.

RESULTS

TUM was associated with shorter operative times and better cosmetic outcomes but frequently disrupted the bladder neck-pelvic floor relationship, leading to higher incontinence rates and dependence on clean intermittent catheterization (CIC). PSARVUP, though more complex, preserved anatomical relationships, resulting in longer urethral length, improved spontaneous voiding rates (66.7% vs. 25%), and reduced UTI rates.

Urinary Function: Spontaneous voiding rates were higher in PSARVUP (66.7% vs. 25%), with fewer patients requiring CIC.

Complications: UTI rates were lower in PSARVUP, but vesicourethral fistulas were more common. TUM had higher rates of vaginal/urethral stenosis.

Urodynamics: PSARVUP patients exhibited better bladder compliance and detrusor function.

Anatomy: PSARVUP better preserved bladder neck position and functional urethral length.

CONCLUSIONS

PSARVUP offers superior functional urological outcomes in complex cloaca cases, particularly those with short urethral length, due to its ability to preserve anatomical structures. TUM provides faster recovery but should be limited to patients with favorable anatomy. Long-term, multicenter studies are needed to refine surgical approaches.


11:41 - 11:44
S26-6 (OP)

RENAL OUTCOMES IN PATIENTS WITH CLOACAL MALFORMATION: HOW AND WHY DOES CKD STAGE PROGRESS IN THESE PATIENTS?

Carla RAMIREZ-AMOROS 1, Maria SAN BASILIO 1, Ricardo MEJIA 1, Virginia AMESTY 2, Francisco De Borja NAVA HURTADO DE SARACHO 2, Roberto LOBATO 2, Susana RIVAS 2, Alejandra VILANOVA-SANCHEZ 1, Marta MELGOSA 3, Pedro LOPEZ-PEREIRA 2 and Maria Jose MARTINEZ URRUTIA 2
1) Hospital La Paz, Paediatric surgery, Madrid, SPAIN - 2) Hospital La Paz, Paediatric urology, Madrid, SPAIN - 3) Hospital La Paz, Paediatric nephrology, Madrid, SPAIN

PURPOSE

Patients with cloaca have a high risk of CKD. However, data on the progression and management of their affected renal function is limited. We summarised these findings in a tertiary reference centre.

MATERIAL AND METHODS

Retrospective review of renal and continence outcomes of cloaca patients treated from 1980-2022. We compared the presence of possible risk factors in patients who had undergone renal transplantation and those who had not. We also reviewed renal function with eGFR at 1 year, after potty training and at adolescence.

RESULTS

Of the 37 patients from the study period, we had registered eGFR in 16 patients, with a mean age of 12.25±7.16 years. Of these, 25% had undergone renal transplantation. No significant differences were found between transplanted and non-transplanted groups in type of cloaca (classical vs. posterior), length of common channel (<3 vs. >3 cm), hydrocolpos, hydronephrosis, ectopic/solitary kidneys, ureteral ectopia, bladder agenesis or spinal anomalies. Transplanted patients had significantly higher rates of dysplasia (75% vs. 8%) and VUR (75% vs. 41%).

At 1 year, CKD staging was: I (62.5%), II (18.7%), III (2.5%), IV (6.2%). After potty training, 1 patient worsened from stage I to II, 3 from II to III, 1 from III to IV and the stage IV patients had been transplanted. Of the 8 patients who had reached puberty, CKD had worsened in 62.5%, with 1 escalating from stage I to II, 1 from II to III and 3 requiring a transplant (coming from stage II, III and IV).

43.7% patients achieved contience and the rest remain dry with CIC, in 12.5% though urethra and 43.7% with Mitrofanoff, of which 25% required bladder neck closure and 6% a cervicouretroplasty.

CONCLUSIONS

Cloaca patients are at risk of CKD, especially those with renal dysplasia and VUR. CKD stages II and III after potty training predict deterioration at puberty. These patients often require surgery to achieve dryness.


11:44 - 11:47
S26-7 (OP)

PATIENTS BORN WITH CLOACAL MALFORMATIONS ARE AT-RISK FOR PROGRESSIVE RENAL DISEASE STARTING AT A YOUNG AGE.

Briony VARDA 1, Butool HISAM 1, Melanie BOWSER 2, Christopher CORBETT 3, Allison MAYHEW 4, Melissa MEYER 5, Christina HO 1, Hans POHL 1, Andrea BADILLO 4 and Marc LEVITT 4
1) Children's National Hospital, Urology, Washington, USA - 2) Children's National Hospital, Colorectal, Washington, USA - 3) Children's National Medical Center, Urology, Washington, USA - 4) Children's National Hospital, Colorectal & Pelvic Reconstruction, Washington, USA - 5) Children's National Hospital, Nephrology, Washington, USA

PURPOSE

We aimed to identify patients born with cloaca “at-risk” for progressive renal disease and identify associated factors. 

MATERIAL AND METHODS

Patients with cloaca repaired primarily and followed prospectively (2020-2025) were included. “At-risk” for renal disease (solitary kidney, renal dysplasia/atrophy, VUR grades 3-5, UTD SFU grades 3-4, and/or eGFR<90) was the outcome. Unadjusted analysis was used to identify associated factors. Sub-group analysis was performed for CKD stage ≥2 (among patients >1 year-old with cystatin C and creatinine). 

RESULTS

Fifty patients with median age 0.8 years were included. 33 (66%) were “at-risk”, of whom a majority had concurrent urinary tract anomalies (91%). Median GFR among patients “at-risk” was 70 (1-87; n = 22). Associated factors included: common channel (CC) ≥3-cm (85% vs. 53%, p = 0.02), urethral length (median 1.2 vs. 2.2; p <0.001), poor sacral ratio (100%), and assisted bladder emptying prior to repair (79% vs. 50%, p = 0.03). Among 33 patients with eGRF, 20 (61%) had CKD ≥ 2. Associated factors included: CC ≥3-cm (92% vs. 40%, p <0.01), urethral length (median 0.95 vs. 2.3, p <0.001), rectal fistula above PC line (77% vs. 30%, p = 0.02), uterovaginal anomalies (p < 0.05 for all), high-grade UTD (86% vs. 44%, p = 0.02) and renal atrophy (100% vs. 50%, p = 0.02).  

CONCLUSIONS

Most patients with cloaca were “at-risk” for progressive renal disease. This was likely driven by cloacal complexity and attendant upper urinary tract and uterovaginal anomalies; findings affirm the need for longitudinal CKD and bladder function screening. 


11:47 - 12:00
Discussion