Synchronous bilateral adrenalectomy for Adreno CorticoTropic Hormone (ACTH)-independent Cushing's syndrome in children: multidisciplinary management
Authors' conclusion
Pediatric CS must be referred to a pediatric center with medical and surgical expertise. Shared decision-making regarding SBA must be prompt to allow rapid cure of CS, even if it implies lifelong steroid replacement. This avoids long-term complications of hypercortisolism, particularly cognitive decline. Robot-assisted SBA is feasible and safe, even in infants when performed by an experienced robotic team.
Study context
- Population
- Children with ACTH-independent Cushing's syndrome (CS) who underwent synchronous bilateral adrenalectomy (SBA), tertiary reference center, 1992–2022
- Participants
- 10 children
- Exposure
- McCune-Albright syndrome vs Primary pigmented nodular adrenocortical disease
- McCune-Albright syndromen = 6
- Primary pigmented nodular adrenocortical diseasen = 4
- Outcome
- Presentation, management and outcomes
Methodological detailssingle-centre, tertiary reference center
- Single-centre
- Tertiary reference center
Main finding
As reported in the abstract
- Infants
- 7
- Infants: failure to thrive with anorexia
- 7
- Infants: cushingoid facies
- 7
- Infants: arterial hypertension
- 6
- Infants: hypotonia
- 5
- Infants: hyperglycemia
- 4
- Infants: liver dysfunction
- 4
- Infants: hypercalcemia with nephrocalcinosis
- 4
- Prepubertal children (linear growth arrest with obesity, cushingoid facies, hirsutism)
- 3
- SBA by robot-assisted laparoscopy
- 6
- SBA by laparoscopy
- 2
- SBA by laparotomy
- 2
Observational data: associations, not effects.
Safety
Post-operative complications and follow-up events · median follow-up 4.8 years
- Post-operative complication (seroma in the adrenal bed)
- 1
- Deaths
- 0
- Acute adrenal crisis
- 0
Original publication
Synchronous bilateral adrenalectomy for ACTH-independent Cushing's syndrome in children: multidisciplinary management. Eur J Endocrinol. Published 16 December 2024. doi:10.
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