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Appendix 2
Classification of Codes
Congenital malformations have traditionally
been divided into categories of "major" and
"minor". A major anomaly has an adverse effect
on the individual's health, functioning or social
acceptability. A minor anomaly is generally
considered of limited social or medical
significance. While minor anomalies in
themselves do not greatly affect the child, they
can be related to major anomalies or be
indications of certain syndromes1,2.
The division between major and minor is far
from perfect. No standard lists or definitions
exist. We used several sources, including the
practices of other registries, to develop a list of
minor anomalies3, 4, 5. One serious problem in
making this distinction is that some ICD-9-CM
codes include major and minor malformations
under the same code. A more specific coding
scheme that eliminates most of these problems
has been adopted.
Following is a general listing of conditions
included in this report and their classification. A
few codes are not listed since they contain only a
very few cases. Reporting hospitals receive a
CMR Handbook with a complete, detailed list of
reportable anomalies.
| |
Major Malformations |
| 090.0 - 090.9 |
Congenital Syphilis |
| 658.8 |
Amniotic Bands |
| 740 - 759* |
Congenital Anomalies |
| 760.71 |
Fetal Alcohol Syndrome |
| 771.0 - 771.2 |
Congenital Infections: including rubella, cytomegalovirus,
toxoplasmosis and herpes simplex |
| *See list of minor and excluded codes |
| |
Minor Malformations |
| 214 |
Lipoma |
| 216 |
Benign neoplasm of skin |
| 228.01 |
Hemangioma of skin |
| 550 |
Inguinal hernia in males |
| 553.1 |
Umbilical hernia |
| 743.65 |
Specified congenital anomalies of lacrimal passages |
| 744.1 |
Accessory auricle |
| 744.29 |
Other specified anomalies of ear |
| 744.3 |
Unspecified anomaly of ear |
| 744.4 |
Branchial cleft cyst |
| 744.89 |
Other specified anomalies of face and neck |
| 744.9 |
Other unspecified anomalies of face and neck |
| 747.0 |
Patent ductus arteriosis, if birth weight <1500 grams |
| 747.5 |
Single umbilical artery |
| 752.41 |
Embryonic cyst of cervix, vagina and external female genitalia |
| 752.42 |
Imperforate hymen |
| 752.5 |
Undescended testicle, if birth weight < 2500 grams |
| 754.61 |
Congenital pes planus |
| 755.0 |
Polydactyly |
| 755.11, 755.13 |
Syndactyly without fusion of bone |
| 757.2 |
Dermatoglyphic anomalies |
| 757.32 |
Vascular hamartomas |
| 757.33 |
Congenital pigmentation anomalies of skin |
| 757.39 |
Other anomalies of skin |
| 757.4 |
Specified anomalies of hair |
| 757.5 |
Specified anomalies of nails |
| 757.6 |
Specified anomalies of breast |
| 757.8 |
Other specified anomalies of integument |
| 757.9 |
Unspecified anomalies of the integument |
| Exclusions |
| 750.0 |
Tongue tie |
| 758.4 |
Balanced autosomal translocation in normal individual |
| 778.6 |
Congenital hydrocele |
References
- Marden PM, Smith DW, McDonald MJ. Congenital anomalies in the newborn infant including minor
variations. J Pediat 1964; 64:357-371.
- Lippig KA, Werler MM, Caron CI, Cook CA, Holmes LB. Predictive value of minor abnormalities:
association with major malformations. J Pediatr 1987; 110:530-537.
- Merlob P, Papier CM, Klingberg MA, Reisner SH. Incidence of congenital malformations in the newborn,
particularly minor abnormalities. In: Marois, ed. Prevention of physical and mental congenital defects, Part
C: Basic and medical sciences, education and future strategies. Proceedings of a conference of the Institut de
la Vie<.
- Myrianthopoulos NC, Chung CS. Congenital malformations in singletons: epidemiologic survey. Birth
Defects: Original Article Series, 1974; X: 2-3, 51-58.
- Jones KL, Smith's Recognizable Patterns of Human Malformation. 4th ed. Philadelphia: W.B. Saunders Co.,
1988:662-681.
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