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Year : 2020  |  Volume : 68  |  Issue : 7  |  Page : 146--153

Hypothalamic Hamartoma and Endocrinopathy: A Neurosurgeon's Perspective

1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Neurosurgery, Netaji Subhash Chandra Bose Medical College, Jabalpur, Madhya Pradesh, India
4 Department of Neurosurgery, MS Ramaiah Medical College and Hospital, Bengaluru, Karnataka, India
5 Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India
6 Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Dr. P Sarat Chandra
Professor, Department of Neurosurgery, PI and Team Leader COE Epilepsy and MEG Center, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.287681

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Background: The management of hypothalamic hamartomas (HH) rests upon the type of presentation. These are rare congenital benign lesions presenting either with central precocious puberty (CPP), drug refractory epilepsy (DRE) or combination of both. We present here our experience in the management of these lesions from a neurosurgeon's perspective and review the pertinent literature. Objective: To present a series of HH presenting with CPP and DRE managed in the neurosurgery department at our center with an emphasis on the associated endocrine abnormalities. Materials and Methods: A prospective observational study over a period of five years included 16 patients of HH. All patients were evaluated with 3 Tesla Magnetic Resonance Imaging (MRI) brain, complete hormonal workup including gonadotrophins, testosterone (males) and estradiol (females), and video-electroencephalography (VEEG) as a part of epilepsy workup. All these patients were evaluated with postoperative hormonal workup and repeat MRI brain if repeat surgery was contemplated. Results: Among the 16 patients of HH, there were 11 male and 6 female children. All the patients presented with DRE with four of these had associated CPP. All the patients underwent robotic-guided radiofrequency ablation (RFA), with 75% seizure freedom following 1st RFA surgery. Three of the four patients with CPP achieved both clinical and biochemical normalization. One patient had just a marginal reduction in the serum gonadotrophins. One patient was reoperated twice and three underwent RFA thrice. Conclusion: The management of HH should be individualized with DRE taking the precedence requiring early surgery. A multidisciplinary approach is therefore recommended for a successful outcome.


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