eMedicine Specialties > Pediatrics: Surgery > General Surgery

Imperforate Anus: Surgical Perspective: Follow-up

Author: Marc A Levitt, MD, Assistant Professor of Surgery and Pediatrics, University of Cincinnati; Associate Director, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center
Coauthor(s): Alberto Pena, MD, Consulting Surgeon, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center
Contributor Information and Disclosures

Updated: Aug 10, 2007

Outcome and Prognosis

When evaluating the results of treatment of anorectal defects, patients cannot be grouped into the traditional high, intermediate, and low categories. For instance, within the traditional high group, malformations have different treatments and carry different prognoses (eg, rectoprostatic fistula compared with rectobladder neck fistula). Both of these would be considered high in the traditional nomenclature; however, the malformations are so different they should not be grouped together. An anatomic classification is of more clinical value.

The functional results of repair of anorectal anomalies have improved significantly since the advent of the posterior sagittal approach. However, the results of this approach are difficult to compare with those of other methods because terminology and classification are inconsistent.

Approximately 75% of all patients with anorectal malformations have voluntary bowel movements. Approximately 50% have occasional soiling accidents. Episodes of soiling are usually related to constipation; when constipation is treated properly, the soiling commonly disappears. Approximately 40% of all patients have voluntary bowel movements and no soiling.

About 25% of patients with anorectal malformations have fecal incontinence and must receive a bowel management regimen to keep clean (see Bowel Management).

Apart from the anorectal anomaly, the status of the sacrum, spine, and muscles greatly affects a patient's fecal continence. Even with a perfect reconstruction, a patient with a poor sacrum may not achieve bowel control.

Bowel control must be evaluated when the child is older than 3 years. The need for laxatives, medications, special diet, and enemas must be considered when assessing outcomes.

Patients with low defects (eg, rectoperineal fistula, rectal atresia) have excellent outcomes. Girls with vestibular fistulas have very good outcomes, except for a tendency to develop constipation. Approximately 60% of boys with rectourethral fistulae and typical sacra have good outcomes. More than 80% of patients with persistent cloaca and a typical sacrum have bowel control. Patients with very high malformations (eg, rectobladder neck fistula in boys) have poor outcomes, even with a typical sacrum.

The sacrum is a good predictor of outcome. Patients with a typical sacrum are much more likely to have fecal continence. Patients with a hypodeveloped sacrum are much more likely to be incontinent. A sacral ratio has been developed to allow for a more objective assessment of the sacrum (see Image 8). Thus far, no patients with a sacral ratio less than 0.3 have achieved continence. A hypodeveloped sacrum is also a good predictor of associated spinal problems, such as tethered cord.

A child's outcome may be predicted more accurately with the knowledge obtained from a large patient series. Parents can be realistically informed of their child's potential for bowel control, even in the newborn period. This avoids a great deal of frustration later in life. Establishing the functional prognosis early is vital to avoid raising false expectations in the parents.

Once the diagnosis of the specific defect is established, the functional prognosis can be predicted. The status of the spine, sacrum, and perineal musculature are all factors that affect the counseling given to the parents.

If a given defect carries a good prognosis, such as vestibular fistula, perineal fistula, rectal atresia, rectourethral bulbar fistula, or imperforate anus without fistula, expect the child to have voluntary bowel movements by age 3 years. Such children require supervision to avoid fecal impaction, constipation, and soiling.

Certain defects indicate a poor prognosis, such as a high cloaca (common channel >3 cm) or a rectobladder neck fistula. Parents should be informed that the child may require a bowel management program to remain clean. The program should be implemented at age 3-4 years (see Bowel Management).

Patients with rectoprostatic fistulas carry an almost equal chance of voluntary bowel movements or incontinence. Toilet training should be attempted at age 3 years, and, if unsuccessful, a bowel management program should be initiated. Each year, during summer vacation, bowel control should be attempted, and, if unsuccessful, the bowel management should be restarted. As the child grows older and more cooperative, the likelihood of achieving bowel control improves.

Urinary incontinence occurs in boys with anorectal malformations only when they have an extremely defective or absent sacrum or when the basic principles of surgical repair are not followed and important nerves are damaged during the operation. The vast majority of boys have urinary control. This is also true for girls, not including the group with persistent cloaca (see Cloacal Malformations). Careful, regular observation is necessary in these patients to accurately reassess their prognosis and to avoid problems that can dramatically affect their ultimate functional results.

Future and Controversies

Potential methods of evaluation for anorectal malformations, including prenatal diagnosis and genetic karyotyping to reveal familial disposition, remain controversial.

 


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Follow-up: Imperforate Anus: Surgical Perspective
Multimedia: Imperforate Anus: Surgical Perspective
References
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References

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Further Reading

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Keywords

imperforate anus, anorectal malformations, anorectal anomaly, persistent cloaca, rectoperineal fistula, bucket-handle malformation, recto–bladder neck fistula, rectobladder neck fistula, bowel control, anoplasty, colostomy, rectobulbar urethral fistula, rectoprostatic urethral fistula, rectovesical fistula, imperforate anus without fistula, rectal atresia, rectovestibular fistula, rectovaginal fistula, urinary fistula, urinary tract malformation, colostography, rectovesical defect, rectoperineal, tethered cord, sacral defect, spinal defect, descending colostomy with separated stomas, fecal diversion, megarectum, posterior sagittal approach, constipation, megasigmoid

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Contributor Information and Disclosures

Author

Marc A Levitt, MD, Assistant Professor of Surgery and Pediatrics, University of Cincinnati; Associate Director, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center
Marc A Levitt, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, and Society of American Gastrointestinal and Endoscopic Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Alberto Pena, MD, Consulting Surgeon, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Robert Kelly, MD, Chairman, Department of Surgery, Departments of Surgery and Pediatrics, Children's Hospital of the King's Daughters; Associate Professor, Eastern Virginia Medical School
Robert Kelly, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society of Abdominal Surgeons, Medical Society of Virginia, Norfolk Academy of Medicine, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Deborah F Billmire, MD, Associate Professor, Department of Surgery, Indiana University Medical Center
Deborah F Billmire, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Phi Beta Kappa, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Pediatric Oncology Group, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
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