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Neurosurgical treatment of hypothalamic hamartomas causing precocious puberty
1Department of Neurosurgery, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pennsylvania.
Insights
Surgical resection of hypothalamic hamartomas effectively treats precocious puberty in children. Complete tumor removal led to the complete regression of pubertal symptoms and normalization of hormone levels.
Area of Science:
- Pediatric Endocrinology
- Pediatric Neurosurgery
- Pediatric Oncology
Background:
- Precocious puberty in children can be caused by hypothalamic hamartomas.
- Early diagnosis and treatment are crucial for managing this condition.
Observation:
- Five children (3 girls, 2 boys) with precocious puberty underwent surgical hamartoma resection.
- Hamartomas were located below the tuber cinereum and varied in size and attachment.
- Surgical approach involved a right subtemporal route with hypothalamic transection.
Findings:
- Complete regression of precocious puberty symptoms was observed in all patients post-surgery.
- Hormone levels (testosterone, LH, FSH) normalized to prepubertal ranges after resection.
- No recurrence of precocious puberty was noted during a mean follow-up of 5.0 years.
Implications:
- Complete resection of hypothalamic hamartomas is a curative treatment for associated precocious puberty.
- This surgical approach offers a long-term solution, potentially allowing normal pubertal development later.
- Highlights the importance of neurosurgical intervention for specific pediatric endocrine disorders.
Abstract:
Five children, three girls and two boys, were treated for precocious puberty secondary to hypothalamic hamartoma by resection of the hamartoma. The patients' ages at onset of pubertal development ranged from 6 to 19 months. The hamartomas ranged in size from 6 to 10 mm; four were pedunculated, one was sessile, and all were located below the tuber cinereum. The hamartomas were excised via a right subtemporal approach, with transection at the inferior surface of the hypothalamus; two were adherent posteriorly to the basilar artery and brain stem, and the adhesions were divided. Postoperatively, three children exhibited a transient oculomotor paresis and one other child required eye-muscle surgery. The symptoms and signs of precocious puberty completely regressed postoperatively in all patients. Preoperative hormone assays of testosterone, luteinizing hormone, and follicle-stimulating hormone were within the pubertal range in all five children; postoperative assays fell to prepubertal levels. The children have been followed for 0.5 to 10.5 years (mean 5.0 years) postoperatively, without evidence of recurrence of precocious puberty. One child has begun spontaneous puberty at a normal age. It is concluded that complete resection of hypothalamic hamartomas causing precocious puberty is curative.