Objectives:

Hypothalamic hamartomas (HH) are congenital lesions present with gelastic seizures and precocious puberty. Disconnective surgery is a safe and effective treatment method in patients with HH. In this study, we aim to share demographic information, presenting symptoms, surgical complications and surgical outcomes in pediatric patients with HH who were operated in our clinic.

Methods:

In this retrospective study, 12 pediatric patients with HH, who were operated in Acibadem University, School of Medicine, Department of Neurosurgery, Division of Pediatric Neurosurgery and Epilepsy Surgery Clinic between years 2007–2018 were included. All clinical materials, including patient notes, cranial magnetic resonance imaging, electroencephalogram recordings and endocrinological data of patients, were collected. Variables evaluated in the statistical analysis were age, sex, presenting symptom, radiological appearance, treatment outcome. Surgical outcome was evaluated by the Engel classification system. Statistical analysis was performed using SPSS 20.0 software.

Results:

There were eight (66.7) males and four (33.3%) females within the patient cohort. Median age at the time of diagnosis was 3.1 years (4 months–6 years). Median follow-up period was 98.3 months (24–177 months). Presenting symptoms were gelastic seizures in six (50%) patients, precocious puberty in three (25%) patients and both gelastic seizures and precocious puberty in three patients. All patients had disconnective surgery for HH. None of the patients had a postoperative neurological deficit. The seizure outcome in seven (77%) of the nine patients with seizure were Engel class 1 after the surgery. Signs and symptoms of precocious puberty persist after surgery in three (50%) of six patients who had presented with precocious puberty in the preoperative period. There was no statistically significant relationship between age, sex and type of HH (p>0.05). There was also no significant relationship between the type of HH and presenting symptom, operation route or treatment outcome (p>0.05).

Conclusion:

Disconnective surgery is a safe and effective treatment method in pediatric patients with HH. Since most of the patients presents with seizures and/or endocrinological problems, thorough preoperative and postoperative neurological and endocrinological follow-up is recommended.

Keywords: Disconnective surgery, gelastic seizure, Hypothalamic hamartoma, precocious puberty