Skip to main content

Advertisement

Log in

Orbitozygomatic resection for hypothalamic hamartoma and epilepsy: patient selection and outcome

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

This study aims to examine the outcomes of ten patients after orbitozygomatic (OZ) pterional surgery in cases of refractory epilepsy caused by hypothalamic hamartomas (HH).

Methods

Ten patients with HH and treatment-resistant epilepsy (mean age 18.3 years, range 0.7 to 42.7) underwent HH resection with an OZ approach (n = 8) or an OZ approach combined with a transventricular endoscopic approach (n = 2). Follow-up for the patients ranged from 0.5 to 6.2 years (mean 3.1). Outcomes were prospectively monitored with the use of a proprietary database.

Results

Four patients (40%) are seizure-free, and four (40%) have had greater than 50% reduction in seizures. One patient had no significant change in seizure frequency, and one patient died unexpectedly 2.8 years after surgery. Six patients had total or near-total HH resection (98–100% of HH lesion volume). Of these, four of six (66%) were seizure-free, and two had at least greater than 50% reduction in seizures. Residual complications include diabetes insipidus (n = 1), poikilothermia (n = 1), visual field deficit (n = 1), and hemiparesis (n = 1). Eight families (80%) reported improved quality of life.

Conclusions

Patients with treatment-resistant epilepsy and tumors with an inferior or horizontal plane of attachment to the hypothalamus should continue to be approached from below. Those with both intrahypothalamic and parahypothalamic components may require approaches from above and below, either simultaneously or staged. For appropriately selected patients, the success of controlling seizures with an OZ is comparable to results utilizing transcallosal or transventricular approaches. The likelihood of controlling seizures appears to correlate with extent of resection.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Valdueza JM, Cristante L, Dammann O, Bentele K, Vortmeyer A, Saeger W, Padberg B, Freitag J, Herrmann HD (1994) Hypothalamic hamartomas: with special reference to gelastic epilepsy and surgery. Neurosurgery 34:949–958

    Article  CAS  PubMed  Google Scholar 

  2. Ng YT, Rekate HL, Prenger EC, Wang NC, Chung SS, Feiz-Erfan I, Johnsonbaugh RE, Varland MR, Kerrigan JF (2008) Endoscopic resection of hypothalamic hamartomas for refractory symptomatic epilepsy. Neurology 70:1543–1548

    Article  PubMed  Google Scholar 

  3. Feiz-Erfan I, Horn EM, Rekate HL, Spetzler RF, Ng YT, Rosenfeld JV, Kerrigan JF III (2005) Surgical strategies for approaching hypothalamic hamartomas causing gelastic seizures in the pediatric population: transventricular compared with skull base approaches. J Neurosurg 103:325–332

    PubMed  Google Scholar 

  4. Arita K, Ikawa F, Kurisu K, Sumida M, Harada K, Uozumi T, Monden S, Yoshida J, Nishi Y (1999) The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. J Neurosurg 91:212–220

    Article  CAS  PubMed  Google Scholar 

  5. Delalande O, Fohlen M (2003) Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification. Neurol Med Chir (Tokyo) 43:61–68

    Article  Google Scholar 

  6. Kerrigan JF (2007) Hypothalamic hamartoma. Semin Pediatr Neurol 14:49–50

    Article  Google Scholar 

  7. Luo S, Li C, Ma Z, Zhang Y, Jia G, Cheng Y (2002) Microsurgical treatment for hypothalamic hamartoma in children with precocious puberty. Surg Neurol 57:356–362

    Article  PubMed  Google Scholar 

  8. Regis J, Scavarda D, Tamura M, Villeneuve N, Bartolomei F, Brue T, Morange I, Dafonseca D, Chauvel P (2007) Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Semin Pediatr Neurol 14:73–79

    Article  PubMed  Google Scholar 

  9. Ng YT, Rekate HL, Prenger EC, Chung SS, Feiz-Erfan I, Wang NC, Varland MR, Kerrigan JF (2006) Transcallosal resection of hypothalamic hamartoma for intractable epilepsy. Epilepsia 47:1192–1202

    Article  PubMed  Google Scholar 

  10. Palmini A, Paglioli-Neto E, Montes J, Farmer JP (2003) The treatment of patients with hypothalamic hamartomas, epilepsy and behavioural abnormalities: facts and hypotheses. Epileptic Disord 5:249–255

    PubMed  Google Scholar 

  11. Rosenfeld JV, Harvey AS, Wrennall J, Zacharin M, Berkovic SF (2001) Transcallosal resection of hypothalamic hamartomas, with control of seizures, in children with gelastic epilepsy. Neurosurgery 48:108–118

    Article  CAS  PubMed  Google Scholar 

  12. Kahane P, Tassi L, Hoffmann D, Francione S, Gratadou-Juery G, Pasquier B, Munari C (1994) Crises dacrystiques et hamartome hypothalamique: a propos d'une observation vidéo-stéréo-EEG. Epilepsies 6:259–279

    Google Scholar 

  13. Likavec AM, Dickerman RD, Heiss JD, Liow K (2000) Retrospective analysis of surgical treatment outcomes for gelastic seizures: a review of the literature. Seizure 9:204–207

    Article  CAS  PubMed  Google Scholar 

  14. Palmini A, Chandler C, Andermann F, Costa Da Costa J, Paglioli-Neto E, Polkey C, Rosenblatt B, Montes J, Martinez JV, Farmer JP, Sinclair B, Aronyk K, Paglioli E, Coutinho L, Raupp S, Portuguez M (2002) Resection of the lesion in patients with hypothalamic hamartomas and catastrophic epilepsy. Neurology 58:1338–1347

    CAS  PubMed  Google Scholar 

  15. Rosenfeld JV, Freeman JL, Harvey AS (2004) Operative technique: the anterior transcallosal transseptal interforniceal approach to the third ventricle and resection of hypothalamic hamartomas. J Clin Neurosci 11:738–744

    Article  PubMed  Google Scholar 

  16. Harvey AS, Freeman JL, Berkovic SF, Rosenfeld JV (2003) Transcallosal resection of hypothalamic hamartomas in patients with intractable epilepsy. Epileptic Disord 5:257–265

    PubMed  Google Scholar 

  17. Akai T, Okamoto K, Iizuka H, Kakinuma H, Nojima T (2002) Treatments of hamartoma with neuroendoscopic surgery and stereotactic radiosurgery: a case report. Minim Invasive Neurosurg 45:235–239

    Article  CAS  PubMed  Google Scholar 

  18. Lemole GM Jr, Henn JS, Zabramski JM, Spetzler RF (2003) Modifications to the orbitozygomatic approach. Technical note. J Neurosurg 99:924–930

    Article  PubMed  Google Scholar 

  19. Kanaan IN (2005) Minimally invasive approach to management of pituitary adenomas. Minim Invasive Neurosurg 48:169–174

    Article  CAS  PubMed  Google Scholar 

  20. Gore PA, Nakaji P, Deshmukh V, Rekate HL (2006) Synchronous endoscopy and microsurgery: a novel strategy to approach complex ventricular lesions. Report of three cases. J Neurosurg 105:485–489

    Article  PubMed  Google Scholar 

  21. Ng YT, Rekate HL (2007) Endoscopic resection of hypothalamic hamartoma for refractory epilepsy: preliminary report. Semin Pediatr Neurol 14:99–105

    Article  PubMed  Google Scholar 

  22. Kerrigan JF, Ng YT, Chung S, Rekate HL (2005) The hypothalamic hamartoma: a model of subcortical epileptogenesis and encephalopathy. Semin Pediatr Neurol 12:119–131

    Article  PubMed  Google Scholar 

  23. Cascino GD, Andermann F, Berkovic SF, Kuzniecky RI, Sharbrough FW, Keene DL, Bladin PF, Kelly PJ, Olivier A, Feindel W (1993) Gelastic seizures and hypothalamic hamartomas: evaluation of patients undergoing chronic intracranial EEG monitoring and outcome of surgical treatment. Neurology 43:747–750

    CAS  PubMed  Google Scholar 

  24. Rosenfeld JV, Feiz-Erfan I (2007) Hypothalamic hamartoma treatment: surgical resection with the transcallosal approach. Semin Pediatr Neurol 14:88–98

    Article  PubMed  Google Scholar 

  25. Kuzniecky R, Guthrie B, Mountz J, Bebin M, Faught E, Gilliam F, Liu HG (1997) Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy. Ann Neurol 42:60–67

    Article  CAS  PubMed  Google Scholar 

  26. Leal AJ, Monteiro JP, Secca MF, Jordao C (2009) Functional brain mapping of ictal activity in gelastic epilepsy associated with hypothalamic hamartoma: a case report. Epilepsia 50:1624–1631

    Article  PubMed  Google Scholar 

  27. Kahane P, Ryvlin P, Hoffmann D, Minotti L, Benabid AL (2003) From hypothalamic hamartoma to cortex: what can be learnt from depth recordings and stimulation? Epileptic Disord 5:205–217

    PubMed  Google Scholar 

  28. Khan S, Wright I, Javed S, Sharples P, Jardine P, Carter M, Gill SS (2009) High frequency stimulation of the mamillothalamic tract for the treatment of resistant seizures associated with hypothalamic hamartoma. Epilepsia 50:1608–1611

    Article  PubMed  Google Scholar 

  29. Savard G, Bhanji NH, Dubeau F, Andermann F, Sadikot A (2003) Psychiatric aspects of patients with hypothalamic hamartoma and epilepsy. Epileptic Disord 5:229–234

    PubMed  Google Scholar 

  30. Freeman JL, Coleman LT, Wellard RM, Kean MJ, Rosenfeld JV, Jackson GD, Berkovic SF, Harvey AS (2004) MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases. AJNR Am J Neuroradiol 25:450–462

    PubMed  Google Scholar 

  31. Lekovic GP, Gonzalez LF, Feiz-Erfan I, Rekate HL (2006) Endoscopic resection of hypothalamic hamartoma using a novel variable aspiration tissue resector. Neurosurgery 58:ONS166–ONS169

    Article  PubMed  Google Scholar 

  32. Ng YT, Rekate HL (2005) Emergency transcallosal resection of hypothalamic hamartoma for “status gelasticus”. Epilepsia 46:592–594

    Article  PubMed  Google Scholar 

  33. Rekate HL, Feiz-Erfan I, Ng YT, Gonzalez LF, Kerrigan JF (2006) Endoscopic surgery for hypothalamic hamartomas causing medically refractory gelastic epilepsy. Childs Nerv Syst 22:874–880

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

We thank the Neuroscience Publications Department at Barrow Neurological Institute for editing and helping to prepare the manuscript and Maggie Varland MSN, MBA for coordinating the study.

Disclosures

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to John F. Kerrigan.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Abla, A.A., Rekate, H.L., Wilson, D.A. et al. Orbitozygomatic resection for hypothalamic hamartoma and epilepsy: patient selection and outcome. Childs Nerv Syst 27, 265–277 (2011). https://doi.org/10.1007/s00381-010-1250-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-010-1250-7

Keywords

Navigation