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
750.0 Tongue tie
758.4 Balanced autosomal translocation in normal individual
778.6 Congenital hydrocele


  1. Marden PM, Smith DW, McDonald MJ. Congenital anomalies in the newborn infant including minor variations. J Pediat 1964; 64:357-371.

  2. 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.

  3. 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<.
  4. Myrianthopoulos NC, Chung CS. Congenital malformations in singletons: epidemiologic survey. Birth Defects: Original Article Series, 1974; X: 2-3, 51-58.

  5. Jones KL, Smith's Recognizable Patterns of Human Malformation. 4th ed. Philadelphia: W.B. Saunders Co., 1988:662-681.