`Q99.1` 46, XX true hermaphrodite

Billable ICD-10-CM code, FY2026. Exempt from present-on-admission (POA) reporting.

MS-DRG index

Defines the logic of DRG(s) 729-730 in MDC 12.

Type 1 Excludes: E28.3 Primary ovarian failure ("pure gonadal dysgenesis (Q99.1)")
Type 1 Excludes: Q56 Indeterminate sex and pseudohermaphroditism ("46,XX true hermaphrodite (Q99.1)")
Type 1 Excludes: Q56 Indeterminate sex and pseudohermaphroditism ("pure gonadal dysgenesis (Q99.1)")
Type 1 Excludes: R62 Lack of expected normal physiological development in childhood and adults ("gonadal dysgenesis (Q99.1)")

Code First: D84.81 Immunodeficiency due to conditions classified elsewhere ("chromosomal abnormalities (Q90-Q99)")
Type 1 Excludes: E25 Adrenogenital disorders ("chromosomal abnormalities (Q90-Q99)")
Type 2 Excludes: P04 Newborn affected by noxious substances transmitted via placenta or breast milk ("congenital malformations (Q00-Q99)")
Type 1 Excludes: Q56 Indeterminate sex and pseudohermaphroditism ("pseudohermaphroditism with specified chromosomal anomaly (Q96-Q99)")
Type 1 Excludes: Z15 Genetic susceptibility to disease ("chromosomal anomalies (Q90-Q99)")

Anomaly, anomalous (congenital) (unspecified type) › chromosomes, chromosomal › sex › gonadal dysgenesis (pure)
Dysgenesis › gonadal (due to chromosomal anomaly) › pure
Hermaphrodite, hermaphroditism (true) › 46,XX with streak gonads
Hermaphrodite, hermaphroditism (true) › 46,XY with streak gonads
Karyotype › 46,XX › hermaphrodite (true)
Karyotype › 46,XY › hermaphrodite (true)
Swyer syndrome
Syndrome › Swyer