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.
|090.0 - 090.9||Congenital Syphilis|
|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|
|216||Benign neoplasm of skin|
|228.01||Hemangioma of skin|
|550||Inguinal hernia in males|
|743.65||Specified congenital anomalies of lacrimal passages|
|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|
|752.5||Undescended testicle, if birth weight < 2500 grams|
|754.61||Congenital pes planus|
|755.11, 755.13||Syndactyly without fusion of bone|
|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|
|758.4||Balanced autosomal translocation in normal individual|
- 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<. New York: Alan R. Liss, 1985:51-53.
- 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.