Rectal atresia and stenosis: unique anorectal malformations.
BACKGROUND
Rectal atresia/stenosis is a rare disorder in the spectrum of anorectal malformations and is particularly associated with a presacral mass. These patients are born with a normal anal canal but have a stricture or complete atresia located a few centimeters proximal to the dentate line. We present a surgical technique for the management of these patients, as well as their unique clinical concerns and outcomes.


METHODS
We reviewed the records of 14 patients with rectal atresia and 3 with rectal stenosis. We describe a novel technique that we have developed for the preservation of the anterior dentate line that was performed in the last 13 patients.


RESULTS
Rectal atresia/stenosis was associated with a presacral mass in 5 patients (29%). Definitive repair was completed using a circular rectorectal anastomosis in the first 4 patients and an anterior dentate line sparing procedure in the last 13. All patients older than 3 years have demonstrated the ability to have voluntary bowel movements.


CONCLUSIONS
With the largest reported series of rectal atresia/stenosis, we have demonstrated a safe and effective technique for repair. Preoperative evaluation must be thorough because a significant number of these patients will have an associated presacral mass.
Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Miller
M
Hamrick
Bala
B
Eradi
Andrea
A
Bischoff
Emily
E
Louden
Alberto
A
Peña
Marc
M
Levitt
D000015:Abnormalities, Multiple / Q000453:epidemiology; D000714:Anastomosis, Surgical / Q000379:methods; D003125:Colostomy / Q000379:methods; D003248:Constipation / Q000453:epidemiology; D003251:Constriction, Pathologic; D003672:Defecation; D057210:Delayed Diagnosis; D004210:Dissection / Q000379:methods; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007409:Intestinal Atresia / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000601:surgery*; D008297:Male; D009436:Neural Tube Defects / Q000453:epidemiology; D011183:Postoperative Complications / Q000453:epidemiology / Q000209:etiology; D011300:Preoperative Care; D012006:Rectovaginal Fistula / Q000209:etiology; D012007:Rectum / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery*; D012445:Sacrococcygeal Region; D012983:Soft Tissue Neoplasms / Q000453:epidemiology; D013536:Suture Techniques; D013724:Teratoma / Q000453:epidemiology