Background: Increasing numbers of children and adolescents experiencing gender dysphoria/incongruence are being referred to specialist gender services and there are various published guidelines outlining approaches to clinical care.

Aim: To examine the recommendations about the management of children and/or adolescents (age 0-18) experiencing gender dysphoria/incongruence in published guidelines or clinical guidance. A separate paper examines the quality and development of guidelines.

Methods: A systematic review and narrative synthesis. Databases (Medline, Embase, CINAHL, PsycINFO, Web of Science) were searched to April 2022 and web-based searches and contact with international experts continued to December 2022, with results assessed independently by two reviewers. The Appraisal of Guidelines for Research and Evaluation tool was used to examine guideline quality.

Results: 23 guidelines/clinical guidance publications (1998-2022) were identified (4 international, 3 regional, 16 national). Guidelines describe a similar care pathway starting with psychosocial care for prepubertal children, puberty suppressants followed by hormones for eligible adolescents and surgical interventions as these adolescents enter adulthood. In general, there is consensus that adolescents should receive a multidisciplinary assessment, although clear guidance about the purpose or approach is lacking. There are differing recommendations about when and on what basis psychological and medical interventions should be offered. There is limited guidance about what psychological care should be provided, about the management of prepubertal children or those with a non-binary gender identity, nor about pathways between specialist gender services and other providers.

Conclusions: Published guidance describes a similar care pathway; however, there is no current consensus about the purpose and process of assessment for children or adolescents with gender dysphoria/incongruence, or about when psychological or hormonal interventions should be offered and on what basis.

Prospero registration number: CRD42021289659.