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Controversies of Sex Re-assignment in Genetic Males with Congenital Inadequacy of the Penis

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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.

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  1. Department of Pediatric Surgery, Sri Ramasamy Memorial (SRM) Medical College, SRM University, Kattankulathur, Chennai, Tamil Nadu, 603203, India

    Venkatachalam Raveenthiran

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Views expressed in this article reflect the author’s understanding of sex re-assignment and they do not represent any official recommendations.

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Raveenthiran, V. Controversies of Sex Re-assignment in Genetic Males with Congenital Inadequacy of the Penis. Indian J Pediatr 84, 700–708 (2017). https://doi.org/10.1007/s12098-017-2412-3

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