Cases Of Cloaca Research Articles

Long-term (> 10years) bowel function of anorectal malformations: a retrospective single-center study.

To describe the long-term bowel function of anorectal malformation (ARM) patients and explore the potential influence factors. ARM patients with follow-up data > 10years were included. Cases of cloaca, Currarino syndrome, and VACTERL syndrome were excluded. Rintala score and PedsQL 4.0 were used to assess bowel function score (BFS) and quality of life (QoL). Based on the results, patients were divided into satisfactory group with BFS ≥ 17 and unsatisfactory group with it < 17. Comparisons between the groups were made. Among the 81 patients were 44 males and 37 females. Follow-up time was 138 (126,151) months. 16 (19.75%) patients had associated anomalies. 23 (28.40%) patients had reoperations, and fistula recurrence was the most common reason. BFS of the patients was 20 (18,20). QoL score was 100 (100,100), which correlated positively with BFS (r = 0.648, P < 0.001). The satisfactory and the unsatisfactory groups had 69 and 12 cases, and their BFS were 20 (20,20) and 11 (8,15) respectively, which had statistical difference (P < 0.001). Total QoL score and psycho-social health score of the unsatisfactory group were lower (P < 0.001). Only reoperations were statistically different between the groups (P < 0.001). Long-term (> 10years) bowel function of ARM patients is good in this study. Defecation problems have negative impacts on QoL and mainly affects their psycho-social health. Primary anorectoplasty is extremely important. Reoperations, which are most commonly seen in recto-urethral fistula recurrence, adversely affect the outcome.

POST OPERATIVE FUNCTIONAL OUTCOME FOR ANORECTAL MALFORMATIONS IN INFANT AND CHILDREN IN ERBIL

Background/aims: Outcomes of anorectal malformations have greatly improved but many children still experience some form of urinary or fecal incontinence despite optimal management. The aim is to evaluate the functional outcome in infants with anorectal malformation for different surgical procedure including traditional and recent operations and outcome for different type of anorectal malformation. Subjects and Methods : An analytical study include 28 cases of anorectal malformation (16 males and 12 females ) operated by different technique in Erbil city during 2010 - 2016. Cases of cloaca and early deaths were excluded from the study. Results : In male Rectoperineal fistula (25% ) and in female Rectovestibular fistula (25% ) were the most common anomalies. the most common procedure were primary anorectoplasty for perineal fistula in both male and female infants in 10 cases ( 35.71% ) followed by transfistula anorectoplasty for rectovaginal fistula in 7 cases (25%) and PSARP for rectobladder neck and urethral fistula for male infants in 6 cases. The most common functional complication was constipation in 2 (7.15%) cases and only one case (3.57%) developed fecal incontinence . 89.29 percent of the patients had voluntary bowel movements and were totally continent. Conclusion : Despite significant developments in the understanding of the pathophysiology and surgical techniques, the results of surgery of ARM remain far from perfect. An optimal operation restores normal anatomy and preserves all potential sphincter structures. Functional complications, especially treatable ones, such as constipation, should be detected and treated early to achieve an optimal outcome.

Persistent cloaca: persistence of the challenge

BackgroundPersistent cloaca or cloacal anomalies represent a special category of anorectal anomalies affecting the female sex with a reported incidence of about one in 25,000 live birth.The study included 34 cases of cloaca that were managed at our unit between 2003 through 2017. We retrospectively reviewed patients’ records that included clinical presentation, investigations, operative data, and follow-up notes.Anatomically, we stratified cloaca into three types according to the level of urogenital confluence. A low confluence (type 1) was defined by being at or below the level of the lower border of pubic symphysis with a short common channel (11 cases). A high confluence (type 3) was defined by being at or above the level of the upper border of pubic symphysis (9 cases). Between the low and high types, we defined an intermediate type (type 2) where the urogenital confluence was behind the mid-portion of pubic symphysis (14 cases).ResultsRenal anomalies were common association: solitary kidney in seven, pelvic kidney in two, and urinary tract dilatation (hydroureteronephrosis) in 12 cases. At follow-up, chronic renal insufficiency was detected in seven casesThe prognosis for urinary continence was excellent in low confluence (type 1) cloaca. On the other hand, urinary incontinence was common among type 3 (high confluence) cloaca (62.5%).ConclusionRenal anomalies represent a common association with cloaca and a major cause of morbidity. Efforts should be directed to preserve renal function during the initial management, and to preserve the continence potential following the definitive repair.Level of evidenceThis is a case series with no comparison group (level IV).

Urogenital Management in Cloaca: An Alternative Approach.

Introduction:In the management of cloaca, there is concern that dissection of the urogenital sinus in early childhood with the aim of total anatomical correction is hazardous. Avoiding such mobilization and providing mitrofanoff channel, when needed, till peripubertal period reduces complications and is technically easier.Materials and Methods:Forty-three cases of cloaca were managed in the period 2004–2016. Case records and radiology were reviewed retrospectively. The follow-up evaluation was done by looking into voiding history, bowel movements, and menstruation history.Results:There were three groups of children, namely, those with no reconstruction done elsewhere except a diverting fecal stoma (Group I, n = 25), those who had undergone anorectal correction elsewhere with no attempt at urogenital reconstruction (Group IIA, n = 13), and those with attempted bowel and genitourinary reconstruction elsewhere (Group IIB, n = 5). The Group I children (one still awaiting reconstruction) underwent early rectal reconstruction followed by expectant management of the urogenital apparatus. The 18 referred cases had multiple problems, chiefly urogenital, of congenital or iatrogenic origin. While urinary reconstruction included bladder augmentation, ileal neobladder, bladder neck closure, and ureteric reimplantation, the foundation of urinary management was intermittent catheterization through mitrofanoff stoma and the avoidance of any dissection of the cloacal common channel. Surgery on the genital tracts included drainage of hydrocolpos, perineal surgery for low vaginae and abdominoperineal vaginoplasty for high vaginae in the peripubertal period with or without bowel supplementation. Spontaneous voiding was maintained in 17 of 25 (68%) Group I girls (including one death later from intestinal complications), 7 of 13 (54%), Group IIA girls, and 1 of 5 (20%) Group IIB girls. Painless menstruation was noted in eight postpubertal girls, three through the cloacal channel (awaiting reconstruction) and five through the reconstructed vagina. Most of the children are on a bowel management program for fecal cleanliness with washouts through the neoanus or Malone's stoma.Conclusion:We report a nonconventional approach to cloaca based on avoiding dissection of or around the common channel for urethrovaginal reconstruction, opting for mitrofanoff stoma for intermittent catheterization, when needed, and late vaginal reconstruction. We believe this approach has reduced the overall need for intermittent catheterization.

Achieving Full Anatomical Correction in Girls with cloacal anomalies: A Necessity or an Overdoing?

Purpose: To highlight a different concept for the urogenitoplasty in the management of cloaca and its possible implications on functional and cosmetic outcome.Patients and Methods: The study included six cases of cloaca who were recently managed during the period 2011 through 2015. Regarding urogenitoplasty, two cases with low urogenital confluence had a short common channel and were managed by introitoplasty in one and partial urogenital sinus mobilization in the other. One case with high distended vagina communicating close to the bladder neck was managed by vaginal pull-through, while keeping the common urogenital sinus to act as urethra. In between, three intermediate cases were also managed by introitoplasty, while keeping them with the common urogenital sinus uncorrected.Results: The follow-up period ranged from 2 to 6.5 years. Apart from the hypoplastic external genitalia, all cases included in the study had minimal perineal scaring compared with old cases that underwent extensive perineal ‘urogenital’ dissection. Urinary and stool incontinence was observed in one case that was associated with caudal regression syndrome. Another case that underwent vaginal pull-through had poor evacuation of the urinary bladder; this was managed by anticholinergics and clean intermittent catheterization. The rest of cases had voluntary bowel movements and spontaneous voluntary micturition.Conclusion: Considering the potential risk and complications of separating the vagina from the urethra in some cases of cloaca, it might be better from the functional and cosmetic point of view to perform just introitoplasty while keeping these girls with the common urogenital sinus uncorrected.

Hypermethylation of SHH in the pathogenesis of congenital anorectal malformations

ObjectiveThis study sought to examine promoter methylation and expression of the identified sonic hedgehog (SHH) gene in terminal rectal tissues of children with congenital anorectal malformations (ARMs). MethodsTissue samples from the terminal rectum of pediatric patients with ARMs (five cases each of high and intermediate malformation — two cases of rectovesical fistula, two cases of rectourethral prostatic fistula, one case of cloaca with >3cm common channel, four cases of rectourethral bulbar fistula and one case of imperforate anus without fistula, respectively, and ten cases of low malformation — five cases of perineal fistula and five cases of vestibular fistula, respectively), and patients with non-gastrointestinal tract malformation (six cases, anal fistula) were collected and divided into three groups: high-intermediate ARM (ARMhi-int), low ARM (ARMlo), and control (Cont.). Real-time RT-PCR was used to detect mRNA expression levels of the verified differentially methylated gene SHH, and bisulfite genomic sequencing was performed to evaluate DNA methylation in the SHH promoter region. ResultsThe average methylation levels of the SHH promoter were significantly higher in ARMhi-int (0.850±0.030, P=0.0036) and ARMlo (0.540±0.053, P=0.0087) groups than in Cont. group (0.280±0.032). SHH mRNA expression levels were lower in ARMhi-int (0.340±0.015, P=0.0065) and ARMlo (0.530±0.042, P=0.0156) groups than in Cont. group (0.870±0.046). The average methylation levels of the SHH promoter were higher in ARMhi-int group than in ARMlo group (0.850±0.030 vs. 0.540±0.053, P=0.0095), while SHH expression was significantly reduced in ARMhi-int group compared to ARMlo group (0.340±0.15 vs. 0.530±0.042, P=0.0252). The methylation levels of the SHH promoter in ARMhi-int group were negatively correlated with SHH gene expression (r=−0.89, P<0.01). ConclusionsThe SHH gene, which plays a major role in the development of the anorectum and enteric nervous system, is hypermethylated at its promoter, and this is correlated with low levels of SHH gene expression. This epigenetic modification may therefore be responsible for the observed changes in SHH expression, which could in turn underlie the pathogenesis of congenital ARMs.

A collective review of cases with imperforate anus managed in a teaching hospital

Objectives: The current study aimed to review the cases of imperforate anus, their demographic distribution, associated anomalies, results of management, and defining cases that require further management. Materials and Methods: A total of 123 cases of different types of imperforate anus were managed over a period of 20 years. Results: The patient group comprised 70 males and 53 females. Recto-vestibular fistula was the commonest type seen (27%), followed by ano-perineal fistula (20%). Only two cases of cloaca (1.6%) and one iatrogenic recto-vaginal fistula were seen. The commonest associated anomaly was hypospadius (79.6%) in males, followed by hydronephrosis and abnormal sacrum in both sexes. Most cases were managed by posterior sagittal anorectoplasty (72%). Voluntary bowel control and fecal continence were found in 100% of cases with ano-perineal fistula, in 93.3% of cases with recto-vestibular fistula, and in 63% of those with recto-urethral fistula. Fecal soiling was found in 12 patients; three of them had sacral anomalies and they were offered Malone appendicostomy. Eleven patients had mucosal prolapse; in two of them the prolapse was complete. Five patients possessed mislocated anus but kept clean by daily enemas and diet control. Conclusions: Although the number of cases with imperforate anus treated in our hospital was relatively small, the outcome of management was favorable and matching with reports from specialized centers. It is advisable to keep those patients under regular follow-up and updating their contact information frequently for further assistance.

Total urogenital sinus mobilization in the repair of cloacal anomalies and congenital adrenal hyperplasia

Purpose: Urogenital sinus mobilization has facilitated markedly the vaginal reconstruction in cases of cloacal anomalies. Application of the same technique in cases of congenital adrenal hyperplasia has resulted in cosmetic and functional improvement. Methods: Total urogenital sinus mobilization was used as a part of the repair in 9 patients: 6 with congenital adrenal hyperplasia (4 high and 2 mid vaginal confluence), and 3 with cloacal anomalies; sinus mobilization to the level of the symphesis pubis allowed the vagina to reach the perineum in all cases. Their age ranged from 6 to 8 months, and follow-up ranged from 6 months to 2 years. In the former group, the mobilized sinus was split dorsally and used as an anterior vaginal flap. Cases of cloaca needed weekly dilatation early postoperatively. Results: All patients are below 3 years of age, so urinary control assessment is not yet objective; however, all mothers reported dry intervals. Examination 6 months postoperatively showed wide vagina and excellent cosmetic appearance in all cases. Conclusions: Total urogenital mobilization provides an easier way for vaginal reconstruction. The improved cosmetic appearance and the absence of vaginal stenosis provided by this technique is a major advantage in the management of these difficult surgical situations. J Pediatr Surg 36:1656-1658. Copyright © 2001 by W.B. Saunders Company.