Male pseudohermaphroditism, not elsewhere classified
ICD-10 Q56.1 is a billable code used to indicate a diagnosis of male pseudohermaphroditism, not elsewhere classified.
Male pseudohermaphroditism is a condition characterized by the presence of male chromosomes (typically XY) and male gonads (testes) but with ambiguous or female-typical external genitalia. This condition arises due to various factors, including hormonal imbalances during fetal development, which can lead to incomplete virilization of the external genitalia. Common associated congenital malformations include hypospadias, where the urethral opening is located on the underside of the penis, and cryptorchidism, where one or both testes fail to descend into the scrotum. Additionally, ambiguous genitalia may present, complicating the clinical picture. Uterine malformations can also be observed in some cases, particularly when there are underlying chromosomal abnormalities. Accurate diagnosis often requires a multidisciplinary approach, including genetic testing and endocrinological evaluation, to determine the underlying cause and guide management. Treatment may involve surgical correction of genital anomalies and hormone therapy to promote the development of secondary sexual characteristics.
Detailed physical examination notes, including descriptions of genitalia and any associated anomalies.
Evaluation of newborns with ambiguous genitalia, management of hypospadias repair, and follow-up for cryptorchidism.
Consideration of psychosocial aspects and parental counseling regarding the condition and its implications.
Genetic testing results, family history, and any syndromic associations.
Genetic counseling for families with a history of intersex conditions and evaluation for chromosomal abnormalities.
Understanding the genetic basis of the condition and implications for future pregnancies.
Surgical correction of undescended testis in a patient with cryptorchidism.
Operative report detailing the procedure and indication.
Pediatric urology may be involved in the surgical management.
Surgical intervention for hypospadias in a male patient.
Preoperative assessment and operative report.
Collaboration with pediatric urology for optimal outcomes.
Male pseudohermaphroditism involves male chromosomes and gonads but ambiguous external genitalia, while true hermaphroditism includes both ovarian and testicular tissue, leading to a different clinical presentation and management approach.