Abstract
Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage, are often associated with other anomalies, and have a poor functional prognosis. The surgical approach to repairing these defects changed dramatically in 1980 with the introduction of the posterior sagittal approach, which allowed surgeons to view the anatomy of these defects clearly, to repair them under direct vision, and to learn about the complex anatomic arrangement of the junction of rectum and genitourinary tract. Better imaging techniques, and a better knowledge of the anatomy and physiology of the pelvic structures at birth have refined diagnosis and initial management, and the analysis of large series of patients allows better prediction of associated anomalies and functional prognosis. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. With early diagnosis, management of associated anomalies and efficient meticulous surgical repair, patients have the best chance for a good functional outcome. Fecal and urinary incontinence can occur even with an excellent anatomic repair, due mainly to associated problems such as a poorly developed sacrum, deficient nerve supply, and spinal cord anomalies. For these patients, an effective bowel management program, including enema and dietary restrictions has been devised to improve their quality of life.
Highlights
Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts
Orphanet Journal of Rare Diseases 2007, 2:33 http://www.OJRD.com/content/2/1/33 usually treated with a colostomy performed in the newborn period, followed by an abdomino-perineal pullthrough some time later in life, but surgeons lacked objective anatomic guidelines
The surgical approach to repairing these defects changed dramatically in 1980 with the introduction of the posterior sagittal approach, which allowed surgeons to view the anatomy of these defects clearly, to repair them under direct vision, and to learn about the complex anatomic arrangement of the junction of rectum and genitourinary tract [1,2,3,4,5,6]
Summary
The radiologic evaluation of a newborn with imperforate anus includes an abdominal ultrasound to evaluate for urologic anomalies. If the patient's defect is of the type pointing to a good prognosis such as vestibular fistula, perineal fistula, rectal atresia, rectourethral bulbar fistula, or imperforate anus without fistula, one should expect that that child will have voluntary bowel movements by the age of 3 Such children need supervision to avoid fecal impaction, constipation, and soiling. If a patient's defect points to a poor prognosis, such as a high cloaca (common channel greater than 3 cm) or a recto-bladder neck fistula, the parents should be informed of the likelihood that that child will need a bowel management program to remain clean, which should be implemented at the age of 3 or 4. Factors such as the status of the spine, sacrum, and Careful, regular follow-up is necessary in these patients to accurately reassess their prognosis and to avoid problems, which can dramatically impact on their ultimate functional result
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