Of the 360 reported cases of CPC in English literature, 26.67% have been known to occur in female subjects. The relative incidence of CPC in female subjects in our study was 37.93%. Though complex, CPC in female has received less attention. This intricacies of this entity were addressed previously only by few authors.,
In our study, out of nine patients of CPC, eight had cloaca. Thus, the incidence of associated cloaca in female subjects with CPC was as high as 89%. The incidence quoted for concomitant presence of cloaca in female subjects with CPC in previously available literature ranges from 40 to 58%.,,
The converse is also true. We have noted a 45% incidence of concomitant CPC in our patients presenting with cloaca. This suggests that at least in this part of the world, one should strongly suspect and rule out the presence of CPC in a case of cloaca. This is extremely relevant in view of the sitting a diverting colostomy in these patients. Similarly, if CPC is suspected on a plain roentgenogram in a female neonate with anorectal malformation, the possibility of one or the other subtype of cloaca should be kept in mind.
Of the eight patients with cloaca with CPC, one patient had type 5 cloaca (short cloaca) and another had type 1c cloaca (cloaca with septate vagina) (Raffensperger's classification). In the other four patients, there was ultra-short UGS and the colon opened in the posterior wall of bladder through a fistulous opening. In two patients as the fistulous communication of bowel with genitor-urinary tract was not known, hence, we cannot classify them according to Raffensperger's classification.
Previous authors have described the fistulous communication in females with CPC as colo-claocal, colo-vaginal, colo-vesical and colo-vestibular.,,, But most of them have not mentioned the details of internal anatomy. Wakhlu et al. have casually mentioned cloaca and mullerian duct anomalies as separate entities in their 25 cases of CPC in female subjects. Malformation of the mullerian ductal structures including all variety of duplex system, two separate uteri and vagina opening into a common channel or septate uterovaginal canal are usual accompaniments of cloaca. So, it needs to be clarified whether the authors are talking about the same 10 patients of cloaca or different subset of patients. It is not surprising to note these discrepancies, as 'cloaca' is as such a neglected entity in this part of the world. A quick glance of search engines on internet would show gross paucity of literature about cloaca from India.
Chadha et al. have detailed the various anatomical characteristics of CPC in female subjects and categorized these patients into three separate categories based on their level of fistula opening and type of CPC. The patients with CPC associated with persistent cloaca were grouped as Group A; Group B were the girls with Type I/II CPC with colo-vesical or colo-vestibular fistulas while type III/IV CPC with colo-vestibular fistulas were categorized as Group C. A thorough review of the literature revealed these descriptions to be in close semblance with the descriptions given for one or the other variant of cloaca, so there is nothing new described as far as type B is concerned. The description given in Chadha's category B was exactly similar to the description given for Raffensperger's cloaca type 9. The finding of colon opening into urethra-vaginal septum described as characteristic feature seen only in females with CPC, has already been described by Hendren as one of the variant of cloacal anomaly years back.
Chadha et al. have quoted colo-vestibular fistula to be present in 60% subjects, but no patient in present series had colo-vestibular fistula. Most other authors have also quoted a far lower incidence of this entity (0-16%).,, We tend to believe that Chadha's type C variant where the rectovestibular fistula has been described in association with type III and IV CPC, the anatomy in the vulva is that of an ultra- short cloaca or Raffensperger type 9 cloaca.
We suspect that in most if not all of these cases of CPC in female subjects, one or the other type of cloaca is always present. In the light of this statement, we suggest a simplistic approach to treat it. Put most simplified, management of such patients is a management of two different anomalies- management of CPC and management of cloaca.
Treatment of cloaca, whether in single stage as proposed by Pena or a staged management where initially the bowel is pull throughed and urogenital sinus is left untouched, is well described in English literature and any further discussion is beyond the scope of this manuscript.
With regard to management of CPC, majority believes in an initial 'minimalist' management of CPC in the form of proximal diversion. But then there is a large series published recently that recommends single-stage treatment of CPC. Even if the traditional staged approach is followed, controversy prevails around preservation of colon and subsequent colorrhaphy. Colorrhaphy was first described by Trussler, and it was appreciated and adopted by many in 1990s. But last few years have seen the upsurge of an opposite view as few authors have reported re-dilatation of tubularized pouch., There is a mention about disorganized muscular arrangement that causes hypoperistalsis. Gangopadhyay et al. has recommended colorraphy of only 5 cm of distal pouch in cases of complete pouch colon. On the other hand, pouch excision in our experience has resulted in intractable diarrhea and severe perineal excoriation [Figure - 6]. This issue is important for male subjects too but in females, excoriation results in severe vulvitis, vaginitis, and urethritis with its attendant morbidity. We believe that management of constipation with regular bowel washes is much easier than the management of severe excoriation and diarrhea. Rattan et al has recently mentioned a technique of 'pouch colon patch graft' that seems worthy of a trial.
As individual experience from different centers is miniscule, there is urgent need to have a national registry about the entity. Only collective efforts from many centers can make us wiser about the embryology, anatomical details and management of this unique entity. Our hypothesis based on our small experience that the cloaca is present in most cases of female subjects of CPC needs to put to be test.