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Single-center experience with pediatric Cushing's disease
Adam S Kanter1, Alfa O Diallo, John A Jane
1Department of Neurological Surgery, University of Virginia Health System, Charlottesville, Virginia 22908, USA.
Journal of Neurosurgery
|November 24, 2005
Summary
Pediatric Cushing's disease management involves a multidisciplinary approach. Surgical interventions, including selective adenomectomies, achieve high remission rates, with some patients requiring further treatment for recurrence.
Area of Science:
- Pediatric Endocrinology
- Neurosurgery
- Oncology
Background:
- Pediatric Cushing's disease presents significant diagnostic and therapeutic challenges despite advancements in treatment.
- This study reviews the clinical presentation, diagnosis, treatment, and outcomes of pediatric Cushing's disease.
Purpose of the Study:
- To evaluate the effectiveness of surgical and multimodal therapeutic strategies for pediatric Cushing's disease.
- To analyze clinical outcomes, complications, and long-term remission rates in pediatric patients.
Main Methods:
- Retrospective review of 33 pediatric patients diagnosed with Cushing's disease.
- Analysis of clinical symptoms, diagnostic evaluations (MRI, Inferior Petrosal Sinus Sampling), and surgical procedures (selective adenomectomy, subtotal hypophysectomy).
- Assessment of complications, disease recurrence, and long-term remission rates.
Main Results:
- Common symptoms included weight gain, growth delay, round facies, and hirsutism.
- Preoperative MRI accurately predicted pituitary lesions and lateralization in most cases.
- 91% of patients achieved clinical remission, with 76% after initial surgery and 15% after adjuvant therapy; 9% had persistent disease, and 12% experienced recurrence.
Conclusions:
- Pediatric Cushing's disease necessitates a multidisciplinary diagnostic and multimodal therapeutic strategy.
- Surgical management, often combined with other treatments, leads to high rates of long-term remission.