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Adolfo Correa-Villase ˜nor,* Janet Cragan, James Kucik, Leslie O’Leary, Csaba Siffel, ... - page 3 / 8





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743.0, 743.1


Congenital cataract






744.01, 744.23

744.01, 744.21

745.0 745.10, .11, .12, .19 745.2 745.4 745.5 745.60, .61, .69 746.01, 746.02 746.1 746.2 746.3 746.7 747.0 747.10

745.00–745.01 745.10–745.19 745.20–745.21, 746.84 745.40–745.490 745.51–745.59 745.60–745.69 746.00–746.01 746.10 746.20 746.30 746.70 747.00 747.10–747.19

749.00–749.04 749.0–749.2 748.0

749.00–749.09 749.10–749.29 748.00

Birth defects

ICD-9 CM codes

CDC-BPA codes

Central Nervous System Anencephaly Spina bifida without anencephaly Hydrocephalus without spina bifida Encephalocele Microcephaly Eye

740.0–740.1 741.0, 741.9 w/o 740.0, 740.1 742.3 w/o 741.0, 741.9 742.0 742.1

740.00–740.10 741.00–741.99 w/o 740.00–740.10 742.30–742.39 w/o 741.00–740.99 742.00–742.09 742.10


Table 1 A sample of Defects in the Metropolitan Atlanta Birth Defects Program Reported to the National Birth Defects Prevention Network, and Their International Classification of Diseases 9th Revision, Clinical Modification Codes, and Centers for Disease Control-British Pediatric Association Codes

Ear Anotia/microtia Cardiovascular Common truncus Transposition of great arteries Tetralogy of Fallot Ventricular septal defect Atrial septal defect Endocardial cushion defect Pulmonary valve atresia and stenosis Tricuspid valve atresia and stenosis Ebstein anomaly Aortic valve stenosis Hypoplastic left heart syndrome Patent ductus arteriosus Coarctation of the aorta Orofacial Cleft palate without cleft lip Cleft lip with and without cleft palate Choanal atresia

Source: CDC (2000).


menstrual period, estimated date of delivery, and birth weight;

5. Outcome information (e.g., stillbirth, neonatal death, and age at death); and

6. Hospital and physician information to facilitate fol- low-up procedures.

and completeness of diagnosis, as well as for defect coding. This clinical review also assists in the classification of cases into patterns of associated defects (isolated, sequences, syndromes with recognized cause, and multiples defects) (Spranger et al., ‘82).

Coding and Classification of Birth Defects

For each affected infant, information is collected on up to 24 individual defects. These defects are coded by trained abstractors using a modified British Pediatric Association (BPA) six-digit code (BPA, ‘79) that is more detailed than the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM codes) (WHO, ‘79). Table 1 provides a sample of the major defects monitored by MACDP and reported to the National Birth Defects Pre- vention Network along with two coding schemes, ICD- 9-CM (codes 740.0 to 759.9) and the BPA system. This list represents a sample of the more than 100 major defects monitored by MACDP. In an effort to improve on the anatomic specificity and pathogenetic classification of de- fects, we are currently developing a new birth defect six- digit code that will be based on the ICD-10 code.

All incoming case abstract forms are evaluated routinely by a clinical geneticist or dysmorphologist for accuracy

Quality Control Procedures

To evaluate the completeness and accuracy of MACDP, we have conducted several projects, including reabstrac- tion of records, reviews of new computerized discharge summary indices, linkages with prenatal records, and spe- cial projects. One of these special projects made use of capture-recapture methods to evaluate the sensitivity of case ascertainment by MACDP, which was estimated to be 87% at 1 year after birth and 95% 2 years after birth (Honein and Paulozzi, ‘99).

Data Analysis and Dissemination

The frequency of birth defects is measured as prevalence at birth, expressed as the number of affected infants per 1,000 live births. Data on major birth defects are analyzed quarterly for changes in birth defects rates. Such changes are monitored by statistical evaluation of the difference between observed and expected numbers of specific de-

Birth Defects Research (Part A) 67:617–624 (2003)

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