Indeterminate sex and pseudohermaphroditism (Q56)

ICD-10 code Q56 covers conditions involving indeterminate sex and pseudohermaphroditism, which are congenital anomalies where the physical or genetic sex characteristics are ambiguous or atypical. These codes classify disorders like hermaphroditism and male or female pseudohermaphroditism, helping guide medical documentation and diagnosis.

This section includes specific codes to distinguish between different forms of sex development disorders. For example, Q56.0 refers to hermaphroditism, also known as ovotesticular disorder of sex development or "false hermaphrodite," where individuals have both ovarian and testicular tissue. Q56.1 codes male pseudohermaphroditism, linked to various conditions such as partial gonadal dysgenesis and adrenal enzyme deficiencies in genetically male (46,XY) individuals. Similarly, Q56.2 pertains to female pseudohermaphroditism, describing cases involving ambiguous genitalia or sex development disorders in genetically female (46,XX) individuals, sometimes associated with maternal tumor influences. Other codes like Q56.3 and Q56.4 cover unspecified pseudohermaphroditism and indeterminate sex, respectively, to capture less defined presentations such as ambiguous genitalia. Medical coders seeking the ICD-10 code for ambiguous genitalia or related disorders will find the Q56 category essential for accurate classification.