Ectopic anus: a type of anorectal malformation where the anus is slightly anterior to (in front of) its normal position
PSARP: posterior saggital anorectoplasty, a definitive method of surgically correcting anorectal malformations
Fecal retention: the failure to completely evacuate/empty the rectum on a regular basis; this term is used in contrast to constipation, since many patients with fecal retention chronically pass small amounts of soft or liquid stool around a large, retained fecal mass
Normal continence: regular, voluntary passage of a bowel movement that completely empties the rectum without accidents or soiling.
Social continence: regular, complete emptying of the rectum using a bowel program so that fecal retention, soiling and accidents are avoided.
What is Anorectal Malformation ?
During early development of a baby in the womb, the urinary tract, the reproductive tract and the digestive tract all exit the pelvis through a common opening. Normally, separation of these structures occurs. In females there is complete separation of the three systems so that the urinary tract opening is farthest forward (the urethra), the reproductive tract exits in the middle (the vagina) and the digestive tract exits farthest back (the anus). In males the urinary tract and the reproductive tract exit through a single opening (the male urethra) in front, while the digestive tract exits through the anus. An anorectal malformation is a condition in which the rectum fails to separate properly during development and does not migrate into its proper position on the perineum. The anus and rectum lie in front of their normal location and are not located properly in the sphincter muscles of the pelvis. Anorectal malformations occur as a spectrum of abnormalities that ranges from very mild conditions in which the anus is situated only a few millimeters in front of its normal location, to severe anomalies with no visible anus. In the more severe abnormalities, there may be an abnormal emptying of rectum into the genitourinary tract, and associated malformations of the spine, genitalia, and kidneys.
Diagnosis of Anorectal Malformation
Anorectal malformations occur in both boys and girls. The diagnosis of an anorectal malformation can usually be made by inspection. When the baby is examined, the anus is absent or abnormally positioned. Ultrasound and x-ray tests may be used to help in the diagnosis. Additional tests will be obtained to look for associated abnormalities. Spine x-rays, kidney and spinal cord ultrasound, and occasionally echocardiography are all routine tests obtained in patients with anorectal malformations.
Treatment of Anorectal Malformation
The nature and severity of the anorectal malformation, as well as the presence of any associated conditions will determine the surgical treatment that is required. Some patients may be treated with a single operation as a newborn, while others will require operations in several stages. For patients with relatively minor abnormalities, a small procedure such as a cut back anoplasty may be all that is required prior to going home. For other patients, initial treatment consists of a colostomy and reconstruction is performed at a later date. A second operation is performed that separates the abnormal communication with the genito-urinary tract, and properly relocates the rectum within the sphincter muscles in the pelvis. Depending on the type of abnormality present, children may have this operation performed utilizing minimally invasive surgical techniques. Alternatively, a PSARP (posterior saggital anorectoplasty) procedure may be performed. Several months after definitive surgery, the colostomy is taken down, re-establishing gastrointestinal tract continuity
Follow Up of Anorectal Malformation
Following reconstruction, patients born with anorectal malformations must be followed frequently by their pediatric surgeons. For the more severe abnormalities, long-term continence issues are the major reasons for follow-up, while in the more mild conditions, patients and their families must work to insure that worsening constipation does not lead to poor continence. Many patients must be managed with bowel program designed to promote regular evacuations of the rectum to avoid ongoing soiling from chronic fecal retention. With attentive management, and rigorous compliance with prescribed regimens, most patients achieve social continence. Many patients will have normal continence. The Pediatric Surgeons of IAPS (Indian Association of Pediatric Surgeons) treat patients with anorectal malformations and provide long-term follow-up and management for patients and their families.
