Congenital Malformations Registry - 1996 Report

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 syndromes.1,2

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 anomalies.3, 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

 090.0 090.9Congenital Syphilis
 658.8Amniotic Bands
 740 - 759*Congenital Anomalies
 760.71Fetal Alcohol Syndrome
 771.0 - 771.2Congenital Infections: including rubella, cytomegalovirus toxoplasmosis and herpes simplex
 *See list of minor and excluded codes

Minor Malformations

 216Benign neoplasm of skin
 228.01Hemangioma of skin
 550Inguinal hernia in males
 553.1Umbilical hernia
 743.65Specified congenital anomalies of lacrimal passages
 744.1Accessory auricle
 744.29Other specified anomalies of ear
 744.3Unspecified anomaly of ear
 744.4Branchial cleft cyst
 744.89Other specified anomalies of face and neck
 744.9Other unspecified anomalies of face and neck
 747.0Patent ductus arteriosis, if birth weight <1500 grams
 747.5Single umbilical artery
 752.41Embryonic cyst of cervix, vagina and external female genitalia
 752.42Imperforate hymen
 752.5Undescended testicle, if birth weight < 2500 grams
 754.61Congenital pes planus
 755.11, 755.13Syndactyly without fusion of bone
 757.2Dermatoglyphic anomalies
 757.32Vascular hamartomas
 757.33Congenital pigmentation anomalies of skin
 757.39Other anomalies of skin
 757.4Specified anomalies of hair
 757.5Specified anomalies of nails
 757.6Specified anomalies of breast
 757.8Other specified anomalies of integument
 757.9Unspecified anomalies of the integument
 750.0Tongue tie
 758.4Balanced autosomal translocation in normal individual
 778.6Congenital 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. New York: Alan R. Liss, 1985:51-53.

  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.