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In This Article

  • Summary
  • Abstract
  • Introduction
  • Protocol
  • Representative Results
  • Discussion
  • Acknowledgements
  • Materials
  • References
  • Reprints and Permissions

Summary

Hypothalamic hamartomas are rare, non-neoplastic congenital malformations mainly arising from the inferior hypothalamus or tuber cinereum. Surgical treatment is one of the most effective options, and the surgical approach must be precisely determined for each patient. Here, we describe the full-endoscopic technique for resecting hypothalamic hamartomas.

Abstract

Hypothalamic hamartomas (HH) are rare developmental anomalies of the inferior hypothalamus that often cause refractory epilepsy, including gelastic seizures. Surgical resection is an effective method to treat drug-resistant epilepsy and endocrinopathy in a suitable patient group. Open surgery, endoscopic surgery, ablative procedures, and stereotactic radiosurgery can be utilized. In this study, we aimed to describe the full-endoscopic approach for HH resection. The technique involves the use of an intraoperative ultrasonography (USG) system, a 30° rigid endoscope system that has an outside diameter of 2.7 mm with two working channels, a stylet that has an outer diameter of 3.8 mm, a monopolar coagulation electrode, a fiberoptic light guide, and the endovision system. Microforceps and monopolar electrocautery are the two main surgical instruments for HH removal. The protocol is easy to apply after a particular learning curve has been passed and shorter than open surgical approaches. It leads to less blood loss. Full-endoscopic surgery for HH is a minimally invasive technique that can be applied safely and effectively with good seizure and endocrinological outcomes. It provides low surgical site pain and early mobilization.

Introduction

Hypothalamic hamartomas (HHs) are non-neoplastic, heterotrophic tissues that contain neuronal and glial tissue in an abnormal distribution. Incidence rates of HHs are 1 in 50,000-1,000,000 people with male predominance1. HHs present different clinical symptoms, such as precocious puberty, cognitive impairment, behavioral changes, and various types of seizures, most characteristically, gelastic seizures. Mostly gelastic seizures, as well as other seizure types, are extremely refractory to antiepileptic drugs (AEDs)2,3.

Based on their morphology and relation to....

Protocol

The study protocol was approved by the institutional review board of Istanbul University Faculty of Medicine. Informed consent was obtained from patients for this study.

1. Preoperative procedures

NOTE: Preoperative evaluation is similar to any other medically intractable epilepsy patient. Routine scalp electroencephalography (EEG) monitoring and video-EEG monitoring, interictal and ictal single photon emission computed tomography (SPECT), magnetic re.......

Representative Results

An example of a patient treated by a full-endoscopic approach for HH resection has been presented. The preoperative MRI, intraoperative endoscopic view, and postoperative MRI have been shown in Figure 1, Figure 2, and Figure 3. There was minimal blood loss during the procedure, so it could not be measured. The procedure is short for a surgeon experienced in neuroendoscopy. For the represented case, the operation dur.......

Discussion

In 2003, Delalande classified HHs into four subtypes. Type 1 HHs are small peduncular lesions attached to the tuber cinereum, type 2 HHs are lesions protruding to the third ventricle, type 3 lesions are the combination of type 1 and type 2 HHs, and type 4 HHs are large lesions with a broad attachment to both mammillary bodies and hypothalamus and have an extension to the interpeduncular cistern8. Depending on the location of the HH, various open surgery approaches have been described. For HHs near.......

Acknowledgements

There is no funding source for this study.

....

Materials

NameCompanyCatalog NumberComments
Burr-hole probe of intraoperative ultrasound systemHitachiUST-52114PAloka Linear UST-52114P, Frequency Range: 8 – 3 MH, Scan Angle: 90° FOV
Fiberoptic light guideRiwoSpine80663523180663523 fiber light cable Ø 3.5 mm, TL 2.3 m,
8095.09 adaptor endoscope side,
8095.07 adaptor projector side
Intraoperative Ultrasound systemHitachiHitachi Arietta 70, Tokyo, Japan
MicroforcepsRiwoSpine89240.3023
Monopolar-coagulating electrodeRiwoSpine8922095000
Rigid neuroendoscopeKarl Storz892109205130° Hopkins pediatric telescope, outside diameter 2.7 mm
Sheath for the telescopeKarl Storz8922095103.8 mm outside diameter with two working channels

References

  1. Kerrigan, J. F., et al. Hypothalamic hamartoma: Neuropathology and epileptogenesis. Epilepsia. 58, 22-31 (2017).
  2. Gascon, G. G., Lombroso, C. T. Epileptic (Gelastic) Laughter. Epilepsia. 12, 63-76 (1971).
  3. Berko....

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Hypothalamic HamartomaHH ResectionRefractory EpilepsyGelastic SeizuresFull endoscopic SurgeryIntraoperative UltrasonographyRigid EndoscopeSurgical InstrumentsMinimally Invasive TechniqueElectrocauteryBlood LossSeizure OutcomesEndocrinological Outcomes

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