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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.
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.
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.
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.
Cloaca is the most complex anorectal malformation affecting girls. Total anatomical correction by the posterior sagittal ano-recto-vaginoplasty (PSARVUP) approach or radical mobilization of the common channel by total urogenital mobilization (TUM) operation in early childhood has been advised not only for cosmetic reasons but also for the feasibility of urethral clean intermittent catheterization (CIC) if needed. It has been observed that the majority of children with cloaca depend on CIC after the above operations, especially when the common channel is long.[1,2] We report a different and possibly unconventional approach to the management of cloaca avoiding dissection of the common channel, a wait and watch policy for the vaginoplasty, and choice of the mitrofanoff channel for intermittent catheterization, when indicated.
Forty-three girls with cloacal malformation were managed in the period 2004–2016 in Christian Medical College, Vellore, PSG Institute of Medical Sciences and Research, Coimbatore, and Coimbatore Medical College hospital under the direct care or supervision of the senior author (SS) and form the subject of this report. Ethical clearance was obtained from the Institutional Review Board. These girls fell into three groups, 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). While Group IIA and IIB children were referred to us mainly for urogenital management, Group I children under our care initially underwent only rectal reconstruction (after initial colostomy) with expectant management of the urogenital apparatus with intervention only when required.
Of the 25 girls in Group I, 7 were newborns while the rest presented at the mean age of 4.2 years, 5 of these without a fecal diversion. The mean age of the 18 Group II (A and B) girls was 9 years (range 2–20 years).
In the total of 43 cases with cloacal malformation, 31 were of the high type. When the original operative findings were not available for review, the classification was done by retrospective review of the radiology.
Totally, 20 of the 43 children required urological management for urinary incontinence and/or retention. This included 12 Group II children (11 with high cloaca) and 8 Group I children (5 with high cloaca). Underlying pathology included structural defects in the urinary tract, lower spine and iatrogenic injury either singly or in combination [Table 1]. Renal insufficiency (chronic renal failure, CRF), hydroureteronephrosis (HUN) with or without vesicoureteral reflux (VUR) and previous urinary diversions were also important management considerations [Table 2].
The genital management included CIC for hydrocolpos in one child, started at the neonatal period, vaginostomy for hydrocolpos in two children, and limited perineal procedure for low vaginae in three children performed during infancy up to 3 years of age. Abdominoperineal pull-through for high vaginae was performed in six, in four of these, partial bowel supplementation were also done. These vaginal reconstructions were performed in the peripubertal period. In six other children, five of these in Group IIB had failed vaginal reconstruction. One of these had to have hysterectomy for pyometra secondary to vaginal stenosis. The remaining 27 girls, 20 in Group I and 7 in Group IIA are awaiting reconstruction and unoperated vaginae are asymptomatic [Table 3].
Three girls with pouch colon remain on permanent ileostomy/colostomy while another girl has a permanent high ascending colostomy as a result of loss of entire transverse and left colon due to surgical misadventure. One child is awaiting reconstruction. The remaining 38 girls have completed anorectal reconstruction, 23 of which have been done in our institution. 16 of the 23 required an abdominal exposure (4 laparoscopy-assisted) in addition to perineal or sacroperineal approaches. In 7 others, the neoanus could be reconstructed by an anterior sagittal anorectoplasty (n = 4) or posterior sagittal anorectoplasty (n = 3) procedures. The pouch colon (n = 7) was excised in 6 but was tubularized after partial excision and used for pull-through in 1. Eight patients subsequently underwent a Malone antegrade continence enema (MACE) procedure for bowel washouts.
The mean duration of postoperative follow-up was 5 years overall and 5 years in the 20 children with specific urological management.
Two children have expired (Group I, high cloaca), both at home. One of these had anorectal reconstruction alone, and she possibly died of intestinal obstruction 1 year after surgery (based on telephonic enquiry). The other was an adolescent girl who had undergone urogenital and rectal reconstruction but was severely depressed after a change of designated sex. She died of unknown cause at home 3 years after surgery.
One girl is lost to follow-up after urorectal reconstruction, and one child is awaiting reconstruction after colostomy but has been included as she is on vaginal CIC for hydrocolpos.
In 31 girls with high cloaca, spontaneous voiding was maintained in 9 of 15 in Group I (1 death), 6 of 11 in Group IIA, and 1 of 5 in Group IIB [Table 3]. 12 of the girls who are voiding spontaneously after reconstruction are more than 5 years old, fully continent, and have stable upper tracts. Peripubertal vaginal reconstruction did not worsen voiding or continence but improved continence in two girls, both with urine storing vaginae. A total of 17 are on CIC, 15 through mitrofanoff channel (1 expired, 1 lost to follow-up), one through reconstructed urogenital sinus, 1 into the hydrocolpos. One girl has permanent ureterostomy. Overall, four children have persistent but stable or improving HUN, while the remaining girls have normal kidneys. Two children have residual CRF with current serum creatinine levels at 1.3 mg/dl and 1.15 mg/dl from initial levels of 3 mg/dl and 2.5 mg/dl, respectively. No child has persistent VUR or urinary incontinence.
Painless menstruation was noted in eight postpubertal girls, three through the cloacal channel (awaiting reconstruction) and five through the reconstructed vagina (including the girl who expired later as noted above). None of the girls are as yet sexually active.
Most of the children are on a bowel management program for fecal cleanliness with washouts through the neoanus or MACE stoma. The children on MACE washouts are very satisfied with this management except one child whose stoma was closed on parental request. Four girls are on permanent ileostomy/colostomy, three of these with pouch colon.
Management of cloaca is complex, involving anatomical and functional correction of the fecal, urinary, and genital passages. Coexistent spinal, renal, alimentary, and Mullerian malformations complicate the outcome of an already complex situation.
Ideally, children with cloaca should be managed by experienced specialist centers. While PSARUVP and TUM operations have become very popular, the surgeon should be aware of potential anatomical and functional complications before undertaking them. The disastrous consequences of attempted and failed surgery for cloaca on the anatomy and function of the urinary tract prompted us to review the need and the nature of urinary management in 43 cases of cloaca in the past 12 years.
There are important lessons to be learned from Group I, IIA, and IIB [Table 3]. While Group IIB children demonstrate the life-threatening complications of failed anatomical repairs, Group IIA illustrates the innate genitourinary issues involved in cloaca after rectal pull-through alone. Group I children, being a nonselected population, demonstrate the natural history of cloaca especially as regards the urogenital apparatus when the cloacal channel is left undisturbed and vaginal reconstruction postponed. In Group I children, urological management was required in only 8 of 25 girls (32%). Although urinary issues were more common in high cloaca (16 of 31), they were also seen in low cloaca (4 of 12).
The etiology of the urinary problems fell into four groups-congenital structural defects, neurogenic bladder caused by congenital spinal pathology, iatrogenic damage, and problems caused by obstructed vagina. Only 8 of the 25 children (32%) primarily managed by us have required CIC including one on vaginal CIC. This is in contrast with majority of children needing CIC (>40%) in the literature.[3,4,5,6,7,8,9] The rest are voiding normally with no deterioration of the upper tracts. Almost all of the eight Group I children needing CIC have major congenital anomalies of the urinary/neural systems, and only in one, the problem has appeared postoperatively. Thus, it is our contention that our policy of doing only the rectal reconstruction in infancy, with watchful expectant management of the urogenital apparatus, interferes less with voiding than attempting total correction in infancy either by PSARUVP or TUM operation. While a “limited” rather than “total” urogenital mobilization is probably harmless, major dissection associated with urogenital mobilization has been associated with deteriorating bladder function in many reports where preoperative and postoperative status has been studied, both clinically and urodynamically.[3,4,8,10]
The pattern of the lower urinary dysfunction in our patients fell into two distinct groups [Table 1]. One group suffered near total urinary incontinence with preserved upper tracts and associated with a small bladder, poorly formed open bladder neck and low placed ureteric orifices. The other group presented with urinary retention with or without incontinence was secondary to spinal pathology or postoperative complications. This latter group was especially prone to HUN, renal failure, and had a greater need for temporary or permanent urinary diversion. While Pena and others stressed mainly on postoperative retention, the group with poorly formed bladder neck has been noted by other authors.[2,5,7,8,10,11] Plain X-ray of the spine, though useful as a prognostic guide, was not always reliable and a spinal magnetic resonance imaging would be a more appropriate investigation.
CIC is often the foundation on which the urinary management of cloaca is built. CIC is required for neurogenic bladder, bladder dysfunction postsurgery, augmented bladders, bladder neck surgery for incontinence, iatrogenic urethral transection, or a bowel neobladder. While some of the above conditions mandate a mitrofanoff channel, in many others, the urethral channel is patent. CIC through the urethra is difficult unless either the meatus is brought to the surface or the vagina is completely separated from the cloacal channel to avoid a vaginal cul-de-sac into which the catheter tends to lodge. This has been an important aim of the urethrovaginal mobilization or separation procedures, even though reports show a greater number of children need CIC after these procedures.[8,10]
We have bypassed this issue, in our 15 children who have needed CIC, by constructing a cosmetically acceptable umbilical mitrofanoff channel, without any attempt at urethrovaginal separation or mobilization. Bladder augmentation is added, when needed. This has possibly reduced the overall need for CIC in our children. We have elected to reconstruct the vagina just before or at puberty unless the vagina was very low and manageable by limited perineal mobilization and skin flap procedure leaving a degree of female hypospadias. Only three of the 42 children had a large hydrocolpos and this could be managed by us with intermittent catheterization of the vagina through the cloacal orifice although the other two had undergone transabdominal vaginostomy in other institutions.[11,15]
While the prepubertal vagina generally remained quiescent in most children except for the occasional hydrocolpos, the onset of puberty unmasked vaginal anomalies unrecognized in the prepubertal period.[3,6,16,17] Vaginal atresia and cervicovaginal atresia affecting one or both of the duplicated system presented with hematometra with or without menstruation through the cloacal channel. None of the high vagina was reconstructed by “PSARVUP” or by total urogenital mobilization. We did not attempt vaginal separation from the urinary apparatus at the “common wall” level but above this level opting to leave some vaginal cuff attached to the cloacal chamber. The dangers inherent in the dissection of the common wall between the bladder and vagina have been emphasized in the literature.[2,11] The vagina then was mobilized and brought to the perineum with or without a small segment of bowel to bridge the gap. Of the five patients who have completed vaginal reconstruction for high vagina and are on current follow-up, four void normally and one is on CIC due to preexisting reasons. In the pubertal patients, the vagina has been found to be capacious, thick walled, well vascularized, and could be mobilized even on the ovarian pedicle.[11,18] Thus, in our experience, postponing vaginal reconstruction till puberty has several advantages if the urinary drainage, when required, could be established with the mitrofanoff channel, and a close watch is maintained on the genital apparatus in the peripubertal period. The dangers of failed reconstruction are many and potentially lethal. While the functional deterioration of bladder function is reported even in experienced centers, structural damage is more serious and under reported. Urethral transection, failure of vaginal pull-through, severe stenosis of neovagina with pyometra, and loss of uterus were some of the complications seen in our patients who had been operated elsewhere.
There were five children in whom urinary diversions (four vesical, one ureteral) were performed – in two of them for congenital uropathy and in three for iatrogenic complications. The timely diversions, which prevented renal failure in four out of these five children, have been also supported in literature.
Psychological issues are extremely important in the management of cloaca, especially in situations where a girl child is socially disadvantaged. One girl with large pseudophallus had been mistakenly reared as a boy, and sex reversal with colovaginoplasty was performed by us when she presented at puberty. She had a probable suicidal death 3 years after the surgery.
Management of cloaca remains a complex challenge. We report our experience with early rectal reconstruction and postponement of vaginal pull-through till near puberty. Intermittent catheterization through mitrofanoff channel was used in children who required bladder management with or without other urinary reconstructive surgery. Only eight of the 25 children (32%) managed primarily by us were catheter-dependent with this approach. Vaginal reconstruction near puberty was performed avoiding dissection of the common wall between the vagina and bladder-urethra as well as avoiding total urogenital mobilization.
There are no conflicts of interest.