DOI: 10.1093/neuped/wuag026.526 ISSN: 2977-4454

ID #1169 Clinical Characteristics and Management of Pediatric Hypothalamic Hamartoma in Indonesia: A Single-Center Case Series

Ludi Dhyani Rahmartani, Ferry Liwang, Mohamad Yanuar Amal, Ghaisani Fadiana

Abstract

Background and Aims

Hypothalamic hamartoma (HH) is a rare, congenital, non-neoplastic malformation arising from the tuber cinereum, classically associated with central precocious puberty (CPP), gelastic seizures, and developmental delay. In low- and middle-income countries, limited access to neuroimaging and specialized services contributes to underdiagnosis, and data on pediatric HH in Indonesia are lacking. This study aimed to describe the clinical characteristics, diagnostic features, and management of pediatric HH in Indonesia.

Methods

We conducted a retrospective review of children (<18 years) diagnosed with HH who presented to a tertiary referral hospital in Jakarta, Indonesia, between January 2023 and February 2025. Clinical presentation, radiological findings, and treatment modalities were extracted from medical records.

Results

Four children were identified, three of whom were male. The mean age at symptom onset was 29.0 ± 15.2 months, with a mean age at diagnosis of 46.5 ± 13.8 months. All patients presented with central precocious puberty; two had gelastic seizures, and all exhibited developmental delay. Neuroimaging demonstrated pedunculated hypothalamic hamartomas in all cases, with a mean lesion size of 10.3 ± 4.4 mm. All patients received gonadotropin-releasing hormone (GnRH) agonist therapy. Surgical intervention was not performed due to technical and resource-related limitations. Following GnRH agonist treatment, the mean bone age–chronological age discrepancy improved from 6.58 years to 3.58 years.

Conclusion

This case series highlights the presence of pediatric hypothalamic hamartoma in Indonesia and underscores ongoing diagnostic and therapeutic challenges in resource-limited settings. While GnRH agonist therapy effectively controlled pubertal progression, access to surgical management remains limited. Larger studies with longer follow-up are needed to optimize diagnostic pathways and develop feasible multidisciplinary treatment strategies.

More from our Archive