Cloacal exstrophy: a single center experience.
OBJECTIVE
Cloacal exstrophy is an exceedingly rare and complex anomaly. The records of 23 patients treated in a tertiary care center with limited infrastructure were analyzed for anatomic types, associated anomalies, surgical procedures adopted, and the outcome.


METHODS
There were 14 males. Seventeen babies were preterm with an average weight of 1.92 kg. The time of presentation, gestational age, birth weight, position of the hemibladders and associated malformations were noted. Reconstruction procedures involved dismantling of the hemibladders and primary turn in, tubularization of the bowel with an end colostomy, and reconstruction of the abdominal wall. Results of the primary surgical repair, bowel function, and outcome of secondary procedures were analyzed.


RESULTS
The position of hemibladders was lateral in 11, upper confluent in 4 and lower confluent in 8. Associated anomalies were noted in 19 patients. Four patients presented late (>5 days). Five died preoperatively, all had major associated anomalies. Four of them were preterm with average weight of 1.4 kg. Two patients refused surgery. Single-stage surgical reconstruction was done in 15 patients. Five patients died postoperatively because of associated anomalies, prematurity, and sepsis. One patient is waiting for surgery. Six patients had follow-up at 3-42 months and are awaiting further reconstruction. Four patients were lost to follow-up.


CONCLUSIONS
Prematurity, late presentation, and sepsis are the major causes of high mortality noted in this series. In our experience, single-stage reconstruction without osteotomy gives satisfactory results.
Assam Medical College & Hospital, Dibrugarh, Assam 786002, India. Electronic address: hemontdut@gmail.com.
Hemonta Kr
HK
Dutta
D000071056:Anorectal Malformations; D001006:Anus, Imperforate / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D001746:Bladder Exstrophy / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery; D002988:Cloaca / Q000002:abnormalities*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D007420:Intestine, Large / Q000002:abnormalities* / Q000601:surgery; D008297:Male; D035583:Rare Diseases; D019651:Plastic Surgery Procedures / Q000379:methods* / Q000401:mortality; D012189:Retrospective Studies; D018570:Risk Assessment; D015996:Survival Rate; D016896:Treatment Outcome
Cloacal exstrophy; Exstrophied bowel; Exstrophy; Hemibladders