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One Hundred Three Consecutive Patients With Anorectal Malformations and Their Associated Anomalies
Sechin Cho, MD;
Shawn P. Moore, MD;
Tony Fangman, MD
Arch Pediatr Adolesc Med. 2001;155:587-591.
Objective A long-term retrospective analysis of 103 infants with anorectal malformations
(ARMs) was conducted to describe any associated congenital anomalies and surgical
classifications.
Design Retrospective medical record review.
Setting This case series was conducted on all infants with ARMs born at, or
referred to, any of 3 major medical centers in Wichita, Kan, for close to
a 22-year period.
Patients The 103 infants in this study represent a consecutive sample of patients
with ARMs. Patients were separated into 2 groups: isolated ARMs without associated
anomalies (n = 30), and ARMs with associated anomalies (n = 73). The male-female
ratio was 2:1.
Main Outcome Measures Patients with associated anomalies were further classified into groups
of ARMs with minor anomalies; major anomalies; chromosomal abnormalities;
and malformation syndromes, associations, or sequences. Only anomalies that
occurred more than once were reported. Malformations were also classified
according to major organ systems.
Results The incidence of ARMs in our study was approximately 1 in 2500 live
births. Additional anomalies were found in 71% of infants with ARMs. Associated
anomalies by major organ system included genitourinary anomalies (49%), musculoskeletal
anomalies (43%), craniofacial anomalies (34%), cardiovascular anomalies (27%),
gastrointestinal anomalies (18%), respiratory anomalies (13%), and central
nervous system anomalies (12%). The most common chromosomal abnormalities
were trisomies (8%), and ARMs were associated with VATER complex (vertebral
defects, anal atresia, tracheoesophageal fistula with esophageal atresia,
and radial and renal anomalies) in 11 cases (11%) and VACTERL (vertebral,
anal, cardiac, tracheal, esophageal, renal, and limb anomalies) in 4 cases
(4%).
Conclusions Patients with ARMs have a high incidence of associated congenital anomalies.
Evaluation of the most commonly affected organ systems in these infants is
essential because it is these associated anomalies that account for most of
the morbidity and mortality that is associated with this condition.
From the Department of Pediatrics, Kansas University School of Medicine–Wichita.
Corresponding author and reprints: Sechin Cho, MD, Department of
Pediatrics, Kansas University School of Medicine–Wichita, 1010 N Kansas
St, Wichita, KS 67214 (e-mail: sechincho{at}compuserve.com).
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Trisomy 13 is associated with anorectal malformations
Lewis and Lander
Arch. Dis. Child. 2007;92:185-185.
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