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Paeds Casesendocrinology-diabetes-and-growth

Paeds Cases · endocrinology-diabetes-and-growth

Counsel parents given a new finding of atypical genitalia — OSCE

OSCE communication and shared-planning station: breaking the news of a disorder of sex development to the parents of a two-day-old infant with atypical genitalia, explaining that no gender or surgical decision will be made that day, naming the work-up, addressing the fear and the shame honestly, and building a shared plan with the multidisciplinary team.

osce communication and shared decision-making
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Target exams

MRCPCH ClinicalRACP DCERCPSC Pediatrics

Target exams

MRCPCH ClinicalRACP DCERCPSC Pediatrics
Prompt
The parents of a two-day-old infant, noted at birth to have clitoromegaly and posterior labial fusion, are frightened and tearful. They have heard a nurse say the word 'intersex' and have read online that their child may never have a normal life. They do not understand why the team will not tell them whether their baby is a boy or a girl, and they are asking for the genitalia to be surgically corrected immediately. The 17-hydroxyprogesterone is pending and the karyotype is not yet back. Counsel them.

Candidate brief

You have eight minutes to counsel the parents of a two-day-old infant noted at birth to have atypical genitalia. The work-up is pending and no gender or surgical decision will be made today. Use a structured, honest, empathic approach that addresses the fear and the shame, names the work-up, and builds a shared plan with the multidisciplinary team. [1] [5]

Key teaching and communication objectives

Acknowledge and validate the parents' fear and their confusion before delivering information, and allow silence. Explain in plain language that their baby's external appearance does not yet tell the full story, that the team is running a karyotype and a hormone panel to understand it properly, and that no decision about gender or about surgery will be made that day or under pressure. Name that this is a known, nameable condition and that the team has cared for many families through it. [1]

Address the word the parents heard directly and without alarm. Explain that the older term 'intersex' is no longer used because it carried stigma and implied a person between two sexes, and that the team uses 'a disorder of sex development' — a condition in which the chromosomal, gonadal, or anatomical sex developed atypically — because it is accurate and respectful. Invite the parents to name the baby, and frame the conversation around the person rather than the diagnosis. [3]

Address the request for immediate surgery honestly. Explain that the team does not perform cosmetic genital surgery in infancy without the child's eventual consent, because the studies that followed operated adults showed measurable harm to sensation, function, and satisfaction, and because the child has the right to decide about their own body. Offer that medically necessary surgery is performed on its own merits, and that the cosmetic question waits until the child can participate. [3]

Address the online information honestly. Explain that the condition is variable, that many children grow up healthy and well, and that the team will build a structured plan across childhood and adolescence. Name that the most common cause — which the team is working to confirm or exclude — is a hormone condition that is well understood and treatable, and that if that is the cause the appearance may change with treatment. Outline the immediate next steps — completing the work-up, convening the multidisciplinary team, and a clear follow-up appointment — and name the coordinator who will hold the plan together. [5] [11]

References

  1. [1]Hughes IA, Houk C, Ahmed SF, Lee PA, et al. Consensus statement on management of intersex disorders. Arch Dis Child, 2006.PMID 16624884
  2. [3]Lee PA, Nordenström A, Houk CP, et al. Global Disorders of Sex Development Update since 2006: Perceptions, Approach and Care. Horm Res Paediatr, 2016.PMID 26820577
  3. [5]Ahmed SF, Achermann JC, Arlt W, et al. Society for Endocrinology UK guidance on the initial evaluation of an infant or an adolescent with a suspected disorder of sex development (Revised 2015). Clin Endocrinol (Oxf), 2016.PMID 26270788
  4. [11]Speiser PW, Arlt W, Auchus RJ, et al. Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab, 2018.PMID 30272171