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Citation
Tags
HERO ID
7379432
Reference Type
Journal Article
Subtype
Review
Title
Controversies of Sex Re-assignment in Genetic Males with Congenital Inadequacy of the Penis
Author(s)
Raveenthiran, V
Year
2017
Is Peer Reviewed?
Yes
Journal
Indian Journal of Pediatrics
ISSN:
0019-5456
EISSN:
0973-7693
Volume
84
Issue
9
Page Numbers
700-708
Language
English
PMID
28687949
DOI
10.1007/s12098-017-2412-3
Web of Science Id
WOS:000412925400011
Abstract
Sex assignment in 46XY genetic male children with congenital inadequacy of the penis (CIP) is controversial. Traditionally, children with penile length less than 2 cm at birth are considered unsuitable to be raised as males. They are typically re-assigned to female-sex and feminizing genitoplasty is usually done in infancy. However, the concept of cerebral androgen imprinting has caused paradigm shift in the philosophy of sex re-assignment. Masculinization of the brain, rather than length of the penis, is the modern criterion of sex re-assignment in CIP. This review summarizes the current understanding of the complex issue. In 46XY children with CIP, male-sex assignment appears appropriate in non-hormonal conditions such as idiopathic micropenis, aphallia and exstrophy. Female-sex re-assignment appears acceptable in complete androgen insensitivity (CAIS), while partial androgen insensitivity syndrome (PAIS) patients are highly dissatisfied with the assignment of either sex. Children with 5-alpha reductase deficiency are likely to have spontaneous penile lengthening at puberty. Hence, they are better raised as males. Although female assignment is common in pure gonadal dysgenesis, long-term results are not known to justify the decision.
Keywords
Disorders of sex development; Ambiguous genitalia; Androgen insensitivity syndrome; Sex re-assignment; Penile length; Micropenis; Aphallia; Penile agenesis; Exstrophy; Sexual function; Intersex disorders
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