A single pediatric surgeon in the Department of Pediatric Surgery of a tertiary hospital managed 29 patients of CPC over a period of 5-year (May 2000 to April 2005). Of these, 18 were males and 11 were females (M: F: 1.6:1). Of these 11 girls, patient-related characteristics were not completely known in two patients and they were excluded from study. A retrospective analysis of various patient-related characteristics with special reference to anatomical characteristics of internal and external genitalia, type of CPC (according to Narasimharao's and Wakhlu's classification) and the level of poucho-genitourinary communication was done in the rest of the nine patients. The preliminary and the definitive surgical management along with their attendant outcome were also studied.
During the same study period, 19 female patients with cloaca were managed, of which 45% had CPC.
Age of presentation varied from 1 day to 8 years. Delayed presentations were seen in those who were decompressing well through the perineal opening.
On examination, eight of the nine patients had single perineal opening. One patient, who had blindly ending pouch, had two perineal openings with normally looking genitalia. Vestibule in all patients with cloaca was short and had receded appearance. In four of these patients, a very short common channel (approximately 0.5 cm long) leading to closely approximated orifices of hypospadiac urethra and vagina could be demonstrated on retraction of the margins of perineal opening [Figure - 1]. Two patients had characteristic clover leaf-like appearance of cloacal opening. One patient had unusual fusion of labia majora posteriorly [Figure - 2].
Type of pouch found at laparotomy
As per the classification given by Narsimha Rao et al . Incidences of various types of pouches were:
Type I CPC - 4 patients
Type II CPC - 3 patients
Type III CPC - 2 patients.
According to Wakhlu's classification, only two patients had partial pouch (short) colon, i.e., normal colon proximal to pouch >8 cm in length.
Level of termination of colonic pouch in genitourinary system
Of the nine patients,thelevel of termination of colonic pouch could be ascertained in seven patients. The colonic pouch was opening in posterior bladder wall in four patients. It terminated as short cloaca in two other patients. Colonic pouch ended blindly in one patient.
Anomalies of internal genitalia could be studied in all patients. In one patient, the internal genitalia were normal. [Figure - 3] shows classical finding of uterine didelphus that was seen in six of our patients. The details of internal genitalia anomalies are shown in [Table - 1].
Correlation of our findings with Chadha's classification of female CPC and Raffensperger's classification of cloaca are shown in [Table - 2].
Flow diagramdepictingdetails of management of individual patients is shown in [Figure - 4]. After preliminary surgery, only five patients have undergone definitive surgery and one patient is waiting for abdomino-posterior sagittal pull through. The patients with short urogenital sinus needed a cutback procedure to expose both the urethral and orifices [Figure - 5].