Initial Management of Anorectal Malformations in Females
The Colorectal Center at Cincinnati Children's provides a decision-making algorithm for initial management of a female patient with an anorectal malformation / imperforate anus.
Condition
Female Newborn With an Anorectal Malformation / Imperforate Anus |
 |
Procedure
Rule out serious, potentially serious associated defects:
Cardiac echo, Esophagus, Kidney and Abdominal U/S,
Lumbar spine, Sacrum, Spinal U/S (tethered cord) |
| |
Procedure
Perineal inspection |
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Diagnosis
Single Perineal Orifice - Cloaca | Diagnosis
Vestibular Fistula | Diagnosis
Perineal Fistula | Diagnosis
No Visible Fistula
(<10% of cases) |
| | | | |
Procedure
Cross table later x-ray |
| | |
Condition
Rectum below coccyx | Condition
Rectum below coccyx |
| | |
Treatment
Colostomy
- Drain hydrocolpos
- Urinary diversion (if necessary) | Treatment
Colostomy or Primary Repair | Treatment
Anoplasty or Dilation | Treatment
Colostomy
or Primary Repair | Treatment
Colostomy |
Perineal inspection in female babies with an anorectal malformation / imperforate anus is even more valuable than in male patients.
The presence of a cloaca (single perineal orifice), which is easy to diagnose by simple inspection, means that this baby has a very serious condition.
Her chances of having an associated urological defect are as high as 90 percent, and an emergency urological evaluation is required.
In addition, that patient will need a colostomy and sometimes a vesicostomy, a vaginostomy, or some other sort of urinary diversion to be done at the same time the colostomy is opened.
One to six months after this procedure, provided the baby is doing well and growing well, the patient is subjected to the recommended treatment of a persistent cloaca which is a posterior sagittal ano-recto-vagino-urethroplasty / PSARPVP, with or without a laparotomy.
If the patient has a vestibular fistula, it is recommended to open a protective colostomy, and four to eight weeks after that, once we have ruled out the presence of associated important defects, the patient can be subjected to a posterior sagittal anorectoplasty.
In cases of healthy, full-term babies, the primary repair can be performed without a protective colostomy, provided the surgeons have experience with such approach.
Vestibular Fistula Perineum |
Sometimes these vestibular fistulas are widely patent, and the patient does not have symptoms of distal obstruction. If that is the case, the patient can be allowed to grow and develop without having to open a colostomy.
However, eventually these patients will need a colostomy prior to the PSARP, not so much for decompression, but for the purpose of protection, to avoid infection after the main repair. In that case, the colostomy can be done on an elective basis two weeks before the main pull-through.
Patients with a cutaneous / perineal fistula do not require a previous colostomy, and can be treated with a "minimal posterior sagittal anoplasty" during the newborn period.
Cutaneous / Perineal Fistula |
Those patients that do not have a fistula communicating with the genitalia or the perineum need a cross-table lateral firm of the pelvis, taken with the patient in prone position, and the management of these babies must follow the same principles already mentioned for male babies. This last condition (imperforate anus without a fistula) is very rare in female patients.
Real recto-vaginal fistulae occur in less than 1% of all female babies born with an anorectal malformation. That is the reason why such defect is not mentioned in the algorithm.
In addition, most female patients born with vestibular fistulae or with a cloaca are misdiagnosed and mislabeled as "recto-vaginal fistula." This misdiagnosis frequently results in erroneous treatments with significant problems for the patients.
Recto-Vaginal Fistula |
For more information or to request an appointment, please contact the Colorectal Center at Cincinnati Children's.
