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Media Gallery

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

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Table 1. Krickenbeck Classification of Anorectal Malformations

Major Clinical Groups	Rare/Regional Variants
Perineal (cutaneous) fistula	Pouch colon
Rectourethral fistula Prostatic Bulbar	Rectal atresia/stenosis
Rectovesical fistula	Rectovaginal fistula
Vestibular fistula	H fistula
Cloaca	Others
No fistula
Anal stenosis

Table 1. Krickenbeck Classification of Anorectal Malformations

Major Clinical Groups	Rare/Regional Variants
Perineal (cutaneous) fistula	Pouch colon
Rectourethral fistula Prostatic Bulbar	Rectal atresia/stenosis
Rectovesical fistula	Rectovaginal fistula
Vestibular fistula	H fistula
Cloaca	Others
No fistula
Anal stenosis

Table 2. Numerical Scoring of Chances of Having Fecal Continence for Patient With Anorectal Malformation

Type of ARM	Score
Perineal fistula	1
Anal stenosis	1
Rectal atresia	1
Rectovestibular fistula	1
Rectobulbar fistula	1
ARM without fistula	1
Cloaca < 3 cm common channel	2
Rectoprostatic fistula	2
Rectovaginal fistula	2
Rectobladderneck fistula	3
Cloaca ≥3 cm common channel	3
Cloacal exstrophy	3
Spine	Score
Normal termination of conus (L1-2)	1
Normal filum appearance	1
Abnormally low termination of conus (below L3)	2
Abnormal fatty thickening of filum	2
Myelomeningocele	3
Sacrum	Score
Sacral ratio ≥0.7	1
Sacral ratio ≥0.4, ≤ 0.69	2
Hemisacrum	2
Sacral hemivertebrae	2
Presacral mass	2
Sacral ratio < 4	3
ARM = anorectal malformation. Individual scores for ARM type, spine, and sacrum are added together. Total scores are interpreted as follows: 3 or 4 - Good potential for continence 5 or 6 - Fair potential for continence 7, 8, or 9 - Poor potential for continence

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Author

Richard J Wood, MD, MBChB, MRCS(Eng), Dip PEC(SA), FCPaedSurg(SA) Chief, Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Attending Pediatric Surgeon, Nationwide Children's Hospital; Associate Professor of Clinical Surgery, Ohio State University College of Medicine

Richard J Wood, MD, MBChB, MRCS(Eng), Dip PEC(SA), FCPaedSurg(SA) is a member of the following medical societies: American Medical Association, American Pediatric Surgical Association, British Association of Paediatric Surgeons, College of Paediatric Surgeons of South Africa, Colorectal Team Overseas, Hirschsprung Disease Research Collaborative, International Pediatric Endosurgery Group, Pan-African Pediatric Surgical Association, Pediatric Colorectal and Pelvic Learning Consortium, Pediatric Trauma Society, Royal College of Surgeons of England, Royal Society of Medicine, South African Association of Paediatric Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Marc A Levitt, MD Chief of Colorectal and Pelvic Reconstructive Surgery, Children’s National Hospital; Professor of Surgery and Pediatrics, George Washington University School of Medicine and Health Sciences; Consulting Surgeon, Seattle Children’s Hospital

Marc A Levitt, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, Society of American Gastrointestinal and Endoscopic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Chief Editor

Eugene S Kim, MD, FACS, FAAP Director, Division of Pediatric Surgery, Vice Chair, Department of Surgery, Cedars Sinai Medical Center; Professor of Surgery and Pediatrics, Division of Pediatric Surgery, Division of Hematology, Oncology, Blood and Marrow Transplantation, Keck School of Medicine of the University of Southern California

Eugene S Kim, MD, FACS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Surgical Association, Association for Academic Surgery, Children's Oncology Group, Society of Laparoscopic and Robotic Surgeons, Society of University Surgeons, Texas Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

Robert Kelly, MD

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, Southern Medical Association

Disclosure: Nothing to disclose.

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