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Outcome of bilateral adrenalectomy in congenital adrenal hyperplasia: one unit's experience
Cara Megan Ogilvie1, Gill Rumsby, Tom Kurzawinski
1Department of Endocrinology, University College London Hospitals, UK.
Insights
Bilateral adrenalectomy may benefit selected women with congenital adrenal hyperplasia (CAH) seeking fertility, but carries risks for those managing hyperandrogenism. Further data is needed for broader recommendations.
Area of Science:
- Endocrinology
- Surgical Management
- Reproductive Health
Background:
- Congenital adrenal hyperplasia (CAH) management is complex, with bilateral adrenalectomy being a controversial therapeutic option.
- This study reviews outcomes in five adult CAH patients who underwent adrenalectomy at a tertiary clinic.
Observation:
- Two patients underwent adrenalectomy for fertility, achieving successful outcomes without adrenal crises.
- Three patients had adrenalectomy for unsuppressible hyperandrogenism and obesity, experiencing improved appearance but significant complications like adrenal insufficiency and pigmentation.
Findings:
- Adrenalectomy for fertility in CAH demonstrated positive outcomes.
- Symptomatic management via adrenalectomy led to complications, including adrenal insufficiency and hyperpigmentation.
- Mean follow-up was 4.2 patient years, highlighting the need for cautious application of this procedure.
Implications:
- Bilateral adrenalectomy could be a viable option for specific CAH patients desiring fertility.
- The risks associated with adrenalectomy for symptom control in CAH warrant careful consideration and further investigation.
- More extensive research is necessary before widespread adoption of adrenalectomy for CAH management.
Objective:
The use of bilateral adrenalectomy in the management of congenital adrenal hyperplasia (CAH) is controversial. We set out to review the outcome of 5 cases of CAH who have undergone adrenalectomy in our unit.
Design:
A retrospective case note review and subject interview of the experience of adrenalectomy in the setting of a tertiary adult CAH clinic.
Methods:
Subjects who had undergone adrenalectomy were reviewed at a routine clinic visit with particular reference to clinical and biochemical outcomes after adrenalectomy.
Results:
Two subjects underwent surgery for the sole indication of desire for fertility with successful outcome and without subsequent adrenal crises. Three women suffered from the common clinical management problem of unsuppressible hyperandrogenism and worsening obesity. Whilst the outcome of improved appearance and weight loss was achieved in these subjects, all three experienced significant complications including pigmentation and acute episodes of adrenal insufficiency.
Conclusion:
We present a mean follow-up of 4.2 patient years and conclude that this procedure may be suitable for selected women with CAH. Outcomes for those pursuing fertility were positive; however, complications were noted in those women for whom the procedure was performed for symptom control. Accepting that the number of subjects is small, it is clear that more data are required before widely recommending this procedure.
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