Initial Management in Males with Anorectal Malformations / Imperforate Anus
The chart below illustrates a decision-making algorithm for the initial management of male patients. In about 80 percent of the cases, the physical exam (perineal inspection) and urinalysis of a newborn male baby with anorectal malformation / imperforate anus will allow us to obtain enough clinical evidence to determine whether the patient needs a colostomy or not.
Condition
Male Newborn Absent an Anal Orifice |
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Procedure
Perineal Inspection: Fistula?, Midline groove?, Anal Dimple?, Flat bottom? |
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Studies
Spine, Sacrum, Kindey U/S, Urinalysis, Cardiac echo,
Rule out esophageal atresia (within 20 to 24 hours) |
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Re-Evaluation
And Cross Table Lateral Film |
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Diagnosis
Perineal Fistula | Diagnosis
Rectal gas seen below coccyx and no associated defects | Diagnosis
Flat bottom
Abnormal sacrum
Associated defects
Rectal gas above coccyx |
| | | |
Treatment
Anoplasty | Treatment
Consider Posterior Sagittal Anoplasty / PSARP without colostomy or with colostomy | Treatment
Colostomy |
Babies with anorectal malformations / imperforate anus are not distended at birth, in addition, 90 percent of them have the distal rectum surrounded by striated muscle, which has tone and therefore, the rectum is collapsed.
There is no imaging technically accurate enough to elucidate the height of the malformation at this stage. In fact, when imaging diagnostic tests are performed during the first hours of life, the result is always a misdiagnosed "high defect" with serious negative therapeutic implications. Approximately 16 to 24 hours later:
- The abdomen becomes distended
- The bowel intraluminal pressure increases enough to destend the funnel-like sphincter
- The most distal rectum becomes distended
- Meconium may pass through the fistula
Therefore, a colostomy is such an important step because it allows for a future high pressure distal colostogram, which can define the anatomy accurately.
Low Defects – No Colostomy Required
The presence of a perineal subepithelial midline raphe fistula through which we can see meconium coming out, the presence of a "bucket-handle" type of defect, as well as anal stenosis, are all defects that are easily detectable by inspection and all of them are considered "low."
The treatment of these defects does not require a previous protective colostomy. The child may be treated during the newborn period with a and is considered a "minimal" posterior sagittal anoplasty.
In male patients with a different type of malformation, four to eight weeks after the colostomy is done, the patient can be subjected to a posterior sagitttal anorecrectoplasty / PSARP. During those weeks, we observe the normal growth of the baby to be sure that there are no other associated defects that require priority.
For more information or to request an appointment, please contact the Colorectal Center at Cincinnati Children's.
