Pediatric Imperforate Anus Surgery Workup

  • Author: Marc A Levitt, MD; Chief Editor: Marleta Reynolds, MD   more...
 
Updated: Apr 9, 2012
 

Imaging Studies

  • Imaging studies performed in the newborn period include the following:
    • The radiologic evaluation of a newborn with imperforate anus includes abdominal ultrasonography to evaluate for urologic anomalies. In patients with persistent cloaca, a distended vagina (hydrocolpos) may be identified.
    • Plain radiography of the spine may reveal spinal anomalies, such as spina bifida and spinal hemivertebrae.
    • Plain radiography of the sacrum in the anterior-posterior and lateral projections may demonstrate sacral anomalies, such as a hemisacrum and sacral hemivertebrae. In addition, the degree of sacral hypodevelopment may be assessed, and a sacral ratio can be calculated by measuring the distances between key bony structures (see the image below).Calculation of the sacral ratio. Calculation of the sacral ratio.
    • Spinal ultrasonography in the newborn period and up to age 3 months (at which point the sacrum ossifies) may be performed to find evidence of a tethered spinal cord and other spinal anomalies.
    • Cross-table lateral radiography may help demonstrate the air column in the distal rectum in the small percentage of patients in whom clinical evidence does not delineate the likely anorectal anomaly in 16-24 hours.
  • Imaging studies performed after the newborn period include the following:
    • High-pressure distal colostography is performed on an outpatient basis, after the colostomy has been created.
      • Hydrosoluble contrast material is injected into the distal stoma to demonstrate the precise location of the distal rectum and its likely urinary communication.
      • Hydrostatic pressure under fluoroscopic control is required. A Foley catheter is placed in the mucous fistula, and the 3-cm3 balloon is inflated and pulled back to occlude the stoma during contrast injection.
      • The hydrostatic pressure must be high enough (manual syringe injection) to overcome the muscle tone of the striated muscle mechanism that surrounds the rectum and keeps it collapsed. This is the best way to demonstrate a rectourinary communication and determine the rectum's true height.
      • The contrast material usually fills the proximal urethra and bladder through the fistula.
      • The injection is continued until the child voids, and pictures are taken during micturition to reveal, in a single picture, the sacrum, rectum height, perineum, fistula location, bladder, vesicoureteral reflux (if present), and urethra.
      • When colostography is performed correctly, voiding cystography and cystoscopy are not necessary.
      • Colostography is vital in determining the anatomy to plan definitive repair. In 10% of patients, the fistula is at the level of the bladder neck; in these instances, during the main repair, the surgeon knows that the rectum can be found only through the abdomen, and a combined posterior sagittal and abdominal or laparoscopic approach is used.
      • The anorectal defect of imperforate anus without fistula may also be demonstrated with this radiologic evaluation. Occurring in approximately 5% of patients, imperforate anus without fistula has a good functional prognosis and is common in individuals with Down syndrome.
    • In most newborn girls with anorectal malformations (except for those with persistent cloaca), distal colostography is not necessary because the fistula is clinically evident.
  • If the spine was not evaluated with ultrasonography in the newborn period, MRI is necessary after age 3 months to exclude the presence of tethered cord and other spinal anomalies.
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Contributor Information and Disclosures
Author

Marc A Levitt, MD  Associate Professor of Surgery, University of Cincinnati College of Medicine; 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  Founding Director, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center

Alberto Pena, MD, is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, American Society of Colon and Rectal Surgeons, Pacific Association of Pediatric Surgery, and Royal College of Surgeons of England

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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.

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, 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, Northwestern University, The Feinberg School of Medicine; Head, Department of Surgery and Surgeon in Chief, Head, Division of Pediatric 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.

References

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Newborn boy with imperforate anus.
Newborn girl with imperforate anus.
Cross-table lateral radiograph of a patient in which the air column in the distal rectum can be observed close to the perineal skin.
Perineum of a newborn with persistent cloaca. Note the single perineal orifice.
Hemisacrum with presacral mass.
Absent lumbosacral vertebrae, a severe vertebral anomaly.
Tethered cord.
Calculation of the sacral ratio.
Ultrasonography demonstrating hydronephrosis in a newborn with imperforate anus.
Cystography of a neurogenic bladder.
Multicystic kidney.
Mercaptotriglycylglycine (MAG-3) renal scan in a patient with a multicystic kidney and imperforate anus.
Vesicoureteral reflux.
Distal colostography in a patient with imperforate anus and a rectourethral fistula.
Newborn with imperforate anus and a rectoperineal fistula.
Newborn with imperforate anus and a bucket-handle malformation (usually associated with a rectoperineal fistula).
Diagram of imperforate anus and rectourethral fistula.
Augmented-pressure distal colostography demonstrating rectourethral fistula only when adequate pressure is used. Note the flat rectum on the left, which represents compression of the distal rectum in the funnel-like sphincteric mechanism.
Diagram of an imperforate anus and rectovestibular fistula.
Imperforate anus and rectovestibular fistula in a newborn.
Recommended colostomy with divided stomas, the proximal stoma in the descending colon.
Operative view of a posterior sagittal anoplasty in a newborn with rectoperineal fistula.
Positioning for posterior sagittal approach.
Posterior sagittal incision.
Electrical stimulator used to show sphincteric contractions.
Electrical stimulator probe used to show sphincteric contractions. Used with electrical stimulator shown in Image 25.
Posterior sagittal incision showing the parasagittal fibers.
Schematic diagram of the anatomy and the repair of a rectourethral anorectal malformation.
Posterior sagittal repair of a rectovestibular fistula.
Closure of the posterior sagittal incision.
 
 
 
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