Anaesthesiology Considerations in Children with DSD

The birth of a baby with ambiguous external genitalia is often a cause of surprise and dismay for the doctors and the family and poses a problem of assignment of sex. Various controversial terms have been used to describe these children and in response to this, the Chicago Consensus recommended a new terminology called Disorders of Sex Differentiation (DSD) which is a more inclusive definition and includes all problems noticed after the child is born in which the genitalia are atypical in relation to the gonads or chromosomes. Management of children with DSD should be individualized and multidisciplinary, and all aspects need to be considered including the psychological implications. These children may present to the anaesthesiologist for a wide plethora of diagnostic and corrective surgical procedures and may require single or multiple anaesthesia exposures. Anaesthetic implications when providing anaesthesia to children with DSD include concerns related to the paediatric age group and the associated congenital syndromes/anomalies which may be present in a large number of these children. Older children often have emotional, psychological or behavioural problems and adequate counselling and allaying of fear and anxiety must be done preoperatively. Children with congenital adrenal hyperplasia constitute almost half the children with DSD and it is important to be able to recognise and manage these children in whom clinical manifestations are mainly due to adrenocortical insufficiency, hyperandrogenism and the adverse effects of glucocorticoid supplementation. Patients with adrenal insufficiency or those receiving long-term corticosteroid therapy are unable to respond normally to surgery or other forms of stress and require “stress dosing” or supplementation with corticosteroids during surgery. The aim of anaesthesia is to maintain hemodynamic stability along with adequate perioperative analgesia which may be achieved with the use of multimodal analgesia along with epidural analgesia or USG-guided nerve blocks.