Cloacal malformations: lessons learned from 490 cases

doi:10.1053/j.sempedsurg.2009.11.012

Seminars in Pediatric Surgery

Volume 19, Issue 2, May 2010, Pages 128-138

https://doi.org/10.1053/j.sempedsurg.2009.11.012 Get rights and content

In this review we describe lessons learned from the authors' series of patients born with the most complex of congenital anorectal problems, cloacal malformations, with the hope to convey the improved understanding and surgical treatment of the condition's wide spectrum of complexity learned from patients cared for over the last 25 years. This includes a series of 490 patients, 397 of whom underwent primary operations, and 93 who underwent reoperations after attempted repairs at other institutions. With regard to the newborn, we have learned that the clinician must make an accurate neonatal diagnosis, drain a hydrocolpos when present, and create an adequate, totally diverting colostomy, leaving enough distal colon available for the pull-through, and a vaginal replacement if needed. A correct diagnosis will avoid repairing only the rectal component. For the definitive reconstruction, all patients in the series were managed with a posterior sagittal approach; 184 of whom also required a laparotomy. The average length of the common channel was 4.6 cm for patients who required a laparotomy and 2.5 cm for those who did not. Hydrocolpos was present in 139 patients (30%). Vaginal reconstruction involved a vaginal pull-through in 308 patients, a vaginal flap in 44, vaginal switch in 48, and vaginal replacement in 90 (33 with rectum, 15 with colon, and 42 with small bowel). A total of 220 underwent total urogenital mobilization, which was first introduced in 1996. Complications included rectal prolapse in 26, vaginal stricture or atresia in 18, urethrovaginal fistula in 13, and urethral atresia in 6. A total of 53% of all cases have voluntary bowel movements. The others are kept clean with a mechanical daily emptying (an enema) as part of a bowel management program. Indications for reoperations included persistent urogenital sinus after initial repair in 39 patients. Fifty-one had problems such as rectal prolapse, stricture, retraction, dehiscence or atresia, 29 had a mislocated rectum, 34 had vaginal stricture, retraction, dehiscence, atresia, or stenosis, 16 had urethrovaginal or rectovaginal fistulae, and 5 had urethral stricture or atresia. The series was divided into 2 distinct groups of patients where common channel measurement was known (n = 400): group A were those with a common channel ≤3.0 cm (n = 225, 56%) and group B had a common channel >3 cm (n = 175, 44%). The separation into these 2 groups has important therapeutic and prognostic implications. Patients in Group A can be repaired posterior sagittally with a reproducible operation. Because they represent most patients, most well-trained pediatric surgeons should be able to repair these types of malformations, and the prognosis is good. Patients in Group B (those with a common channel >3 cm), usually require a laparotomy, have a much higher incidence of associated urological problems, and often require special maneuvers for vaginal reconstruction. Surgeons who repair Group B malformations require special training in urology; the operations are prolonged and technically demanding, and the functional results are not as good as in group A.

Section snippets

Neonatal management

The diagnosis of a cloaca is a clinical one made by identifying a single perineal orifice. The perineum has other important characteristics that help predict the internal anatomy and the final functional prognosis. A “good-looking” perineum consists of a well-formed midline groove and a well-located and clear anal dimple, indicating that the patient has a good sphincter. A “bad-looking” perineum has a single perineal orifice but in addition, a “flat bottom,” with no traces of a sphincter

Definitive reconstruction

Before 1982, the primary surgical treatment of cloacas was not performed with a systematic approach. The related publications were few, with rather poor descriptions of improvised types of operations. Dr Hardy Hendren made significant contributions to the surgical repair of patients born with cloacas.⁴ He initially referred to these defects as “imperforate anus with urogenital sinus.”⁵ His main contributions in cloacal management were in secondary operations and in methods to deal with the

Cloacas with a common channel 1 cm or less (n = 43)

In instances of very short common channel cloaca, we recommend a relatively straightforward procedure that we call a posterior sagittal anorectal vaginoplasty. The urethra is left untouched. We separate the rectum from the vagina as in cases of vestibular fistulas, then, rather than separating the vagina from the urinary tract or performing a total urogenital mobilization, we mobilize only the lateral and posterior walls of the vagina, enough to be able to suture the edges of the vagina to the

Postoperative management

In some instances, we repair these malformations and pull the colostomy down for vaginal replacement at the same time. In those cases, because the patient no longer has fecal diversion, they are maintained with nothing by mouth, and receive parenteral nutrition for 7-10 days. Most of the time, however, the colostomy remains intact, and the patient can eat when their postoperative ileus has resolved. They usually stay in the hospital 2-5 days. A Foley catheter remains in place for 2 to 3 weeks.

Conclusions

The newborn management and definitive reconstruction of cloacal malformations requires knowledge of the broad spectrum of defects. With proper treatment, these patients can undergo an excellent anatomical repair and can be clean and dry.

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Cited by (158)

Sexual and Reproductive Health Concerns in Adults With Cloacal Anomalies: A Qualitative Study
2023, Journal of Pediatric Surgery
Long-term gynecologic data are lacking to inform the care of patients with cloacal malformations. We seek to examine perceived sexual and reproductive health challenges of patients born with cloacal anomalies and characterize the experiences of patients as adults.
Virtual semi-structured focus groups and single-participant interviews were conducted using an online video platform. Retrospective chart review was performed to abstract available demographics and surgical history.
Adult patients were contacted from a database of 143 patients born with cloaca who had been seen at or referred to a tertiary care pediatric colorectal center. Participants were recruited until data collection reached thematic saturation. Twenty patients aged 18–53 years participated in 5 focus groups and 3 single-participant interviews. The hospital IRB determined the research activities to be exempt from IRB review and oversight.
Interviews and focus groups were transcribed and analyzed using the constant comparative method to identify themes regarding sexual and reproductive health and compared with medical and surgical history abstracted from chart review.
Participants reported many perceived barriers to intercourse and intimacy including bowel and bladder continence management. All participants reported discussion of pregnancy and fertility with healthcare providers as important. Other common themes included concerns about independence and transitioning from pediatric to adult providers.
Anorectal malformations are associated with sexual and reproductive health concerns. Patients seek guidance on family building, intimacy, and transition to adult care. Future quantitative study of these topics is needed to develop best practices for counseling and clinical management of these patients.
Level VI.
Prognosis Study.
Bowel Control, Bladder Function, and Quality of Life in Children with Cloacal Malformations
2023, Journal of Pediatric Surgery
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.
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.
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.
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 IV.
A prospective cohort study of assisted bladder emptying following primary cloacal repair: The Children's National experience
2023, Journal of Pediatric Urology
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.
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.
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).
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.
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.
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.
Unique Evaluation and Management Considerations for Adolescents with Late Gynecologic and Colorectal Issues in the Setting of Anorectal Malformations
2023, Journal of Pediatric and Adolescent Gynecology
There is little guidance for managing pubertally identified Mullerian anomalies in patients with anorectal malformations (ARMs). We sought to assess these unique issues.
Retrospective review
Single-institution study
Natal female patients aged 10-25, with an ARM, cloaca, or exstrophy, who presented from 2009 to 2019 with a gynecologic concern were included.
Data collection was performed and included the presenting problem, psychological evaluation, fertility and sexuality concerns, and management strategies for these problems.
The main outcome was unique needs that had to be addressed in the young adult population and the type of colorectal and gynecological procedures needed on representation.
Twelve patients were identified; all had gynecologic concerns. Ten had ARMs, including cloaca (n = 3) and cloacal exstrophy (n = 5). Median age at representation was 14.6 years (IQR = 12.7, 15.3). Colorectal revisions included posterior sagittal anorectoplasty (n = 1), resection of bowel attached to urogenital sinus (n = 1), and appendicostomy revision (n = 1). Gynecologic issues included dysmenorrhea (n = 8), obstructed Mullerian anomaly (n = 6), and introital stenosis (n = 5). Behavioral health concerns (n = 9) and fertility/sexuality concerns (n = 4) were identified. Median time from first visit to reconstruction was 1.5 years (IQR = 0.5, 1.5), providing multiple visits to achieve consensus among patients and providers before intervention, including vaginal or introital repair (n = 5) and hysterectomy of obstructed uterine horns (n = 3).
Goal-directed follow-up is required before surgical management to identify psychological and reproductive issues in patients with ARMs who have gynecologic concerns. Patient input and psychologic consultation are helpful for patients requiring staged reconstruction.
Single institution experience of cloacal malformation
2023, Journal of Pediatric Surgery
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.
Patients were identified from the departmental database from 1994 to 2021. The medical records and operative notes were retrospectively reviewed.
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.
The urinary and faecal continence are the main challenges in the management of cloaca patients. Many require surgical intervention to achieve social continence.
Level IV.
How we select our surgical approach for cloacal reconstruction
2023, Journal of Pediatric Urology
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.

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Cloacal malformations: lessons learned from 490 cases

Section snippets

Neonatal management

Definitive reconstruction

Cloacas with a common channel 1 cm or less (n = 43)

Postoperative management

Conclusions

Semin Neonatol

Semin Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg