Issues and concerns regarding surgery of the sexual-reproductive anatomy during infancy and early childhood are discussed using four actual examples. A case of a 46, XX infant with 21 hydroxylase deficiency congenital adrenal hyperplasia (CAH) with atypical (ambiguous) genitalia is discussed regarding timing and potential harms and benefits of surgery. We present the perspective of balancing the child's rights to bodily autonomy and right to an open future versus parents' decision making authority regarding what they perceive as their child's future best interests. The second case is a newborn with complete androgen insensitivity syndrome and we discuss the harms, benefits and timing of gonadectomy. The third case examines the physical and psychological impact of penile shaft hypospadias, raising the question of whether surgery is justified to prevent what may or may not be considered a permanent disability. The fourth case involves an adult woman with classic CAH, born with a urogenital sinus and clitoromegaly, who never had genital surgery and is now requesting vaginoplasty, but not clitoral reduction. The primary message of this article, as the previous articles in this series, is to encourage patient-family centered care that individualizes treatment guided by shared decision making.The healthcare of individuals with differences of sex development (DSD) is complex and determined by patient, family, social and legal considerations, particularly the need for full disclosure and patient/family participation in management decisions.

There is evidence that approaches to surgical interventions have been shifting, especially avoiding clitoral surgery for those with mild to moderate enlargement, with the best example being the type and timing of female genitoplasty. Furthermore, it is apparent that the approach differs among countries based on cultural values, medical policy, resources, openness, and peer-group support. This is the 4th part of this series of articles reviewing care of individuals with DSD. In this article, we review and discuss specific clinical scenarios that highlight the surgical complexities in DSD management and illustrate the need for individualizing decisions regarding surgery for each patient rather than simply applying an algorithm for a given diagnosis. This is not intended to be a research article but the use of specific cases to provide a perspective regarding the care of patients with an emphasis upon individualizing care considering each individuals specific characteristics rather than applying an algorithm based upon diagnosis because of the variation of findings within a specific genetic diagnosis and also each person's personality, temperament and environment.

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Mishita
Jornal co-ordinator
Journal of General Surgery Reports