Skip to main content

Advertisement

Log in

Pediatric functional hemispherectomy: outcome in 92 patients

  • Clinical Article
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Abstract

Background

The revival of epilepsy surgery after the introduction of modern presurgical evaluation procedures has led to an increase in hemispherectomy or hemispherotomy procedures. Since a large part of our pediatric series was done using a newer hemispherotomy technique, we focus mainly on the outcomes after a recently developed hemispherotomy technique (transsylvian keyhole).

Methods

Ninety-six pediatric patients (aged 4 months to 18 years, mean 7.3) were operated on between 1990 and 2009; 92 were available with follow-up.

Results

The most frequent diagnosis was porencephaly in 46 % of all patients. Progressive etiologies were present in 20 % and developmental etiologies in 22 %. At last available outcome (LAO), 85 % of the patients were seizure free (ILAE class 1). Year-to-year outcome was rather stable; usually over 80 % were class 1 for up to 13 years (n = 24). Of 92 assessable patients, 71 were treated with the transsylvian keyhole technique, with 89 % being seizure free. The overall shunt rate was 5.3 % for the whole series and 3 % for the keyhole technique subgroup. Mortality was 1 of 96 patients. Excluding patients with hemimegalencephaly (HME), patients with the shortest duration of epilepsy and the lowest age at seizure onset had the highest rates of seizure freedom. The etiology does influence outcome, with HME patients having the poorest seizure outcome and patients with Sturge-Weber syndrome and porencephaly having excellent seizure control.

Conclusion

Hemispherotomies/functional hemispherectomies are very effective and safe procedures for treating drug-resistant epilepsy with extensive unihemispheric pathology. Etiology and surgery type clearly influence seizure outcome.

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

Similar content being viewed by others

References

  1. Andermann FRTB, Villemure JG (1991) Hemispherectomy: results for control of seizures in patients with hemiparesis. In: Lüders H (ed) Epilepsy surgery. Raven Press Ltd, New York, pp 625–632

    Google Scholar 

  2. Basheer SN, Connolly MB, Lautzenhiser A, Sherman EM, Hendson G, Steinbok P (2007) Hemispheric surgery in children with refractory epilepsy: seizure outcome, complications, and adaptive function. Epilepsia 48:133–140

    Article  PubMed  Google Scholar 

  3. Boshuisen K, van Schooneveld MM, Leijten FS, de Kort GA, van Rijen PC, Gosselaar PH, van Nieuwenhuizen O, Braun KP (2010) Contralateral MRI abnormalities affect seizure and cognitive outcome after hemispherectomy. Neurology 75:1623–1630

    Article  PubMed  CAS  Google Scholar 

  4. Bourgeois M, Crimmins DW, de Oliveira RS, Arzimanoglou A, Garnett M, Roujeau T, Di Rocco F, Sainte-Rose C (2007) Surgical treatment of epilepsy in Sturge-Weber syndrome in children. J Neurosurg 106:20–28

    PubMed  Google Scholar 

  5. Carson BS, Javedan SP, Freeman JM, Vining EP, Zuckerberg AL, Lauer JA, Guarnieri M (1996) Hemispherectomy: a hemidecortication approach and review of 52 cases. J Neurosurg 84:903–911

    Article  PubMed  CAS  Google Scholar 

  6. Cats EA, Kho KH, Van Nieuwenhuizen O, Van Veelen CW, Gosselaar PH, Van Rijen PC (2007) Seizure freedom after functional hemispherectomy and a possible role for the insular cortex: the Dutch experience. J Neurosurg 107:275–280

    Article  PubMed  Google Scholar 

  7. Cook SW, Nguyen ST, Hu B (2004) Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients. J Neurosurg 100:125–141

    Article  PubMed  Google Scholar 

  8. Cukiert A, Cukiert CM, Argentoni M, Baise-Zung C, Forster CR, Mello VA, Burattini JA, Mariani PP (2009) Outcome after hemispherectomy in hemiplegic adult patients with refractory epilepsy associated with early middle cerebral artery infarcts. Epilepsia 50:1381–1384

    Article  PubMed  Google Scholar 

  9. Delalande O, Bulteau C, Dellatolas G, Fohlen M, Jalin C, Buret V, Viguier D, Dorfmuller G, Jambaque I (2007) Vertical parasagittal hemispherotomy: surgical procedures and clinical long-term outcomes in a population of 83 children. Neurosurgery 60:ONS19–ONS32, discussion ONS32

    Article  PubMed  Google Scholar 

  10. Delalande O, Pinard JM, Basevant C, Gauthe M, Plouin P, Dulac O (1992) Hemispherotomy: a new procedure for central disconnection. Epilepsia 33(suppl S3):99–100

    Google Scholar 

  11. Devlin AM, Cross JH, Harkness W, Chong WK, Harding B, Vargha-Khadem F, Neville BG (2003) Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence. Brain 126:556–566

    Article  PubMed  CAS  Google Scholar 

  12. Di Rocco C, Iannelli A (2000) Hemimegalencephaly and intractable epilepsy: complications of hemispherectomy and their correlations with the surgical technique. A report on 15 cases. Pediatr Neurosurg 33:198–207

    Article  PubMed  Google Scholar 

  13. Doring S, Cross H, Boyd S, Harkness W, Neville B (1999) The significance of bilateral EEG abnormalities before and after hemispherectomy in children with unilateral major hemisphere lesions. Epilepsy Res 34:65–73

    Article  PubMed  CAS  Google Scholar 

  14. Gonzalez-Martinez JA, Gupta A, Kotagal P, Lachhwani D, Wyllie E, Luders HO, Bingaman WE (2005) Hemispherectomy for catastrophic epilepsy in infants. Epilepsia 46:1518–1525

    Article  PubMed  Google Scholar 

  15. Hallbook T, Ruggieri P, Adina C, Lachhwani DK, Gupta A, Kotagal P, Bingaman WE, Wyllie E (2010) Contralateral MRI abnormalities in candidates for hemispherectomy for refractory epilepsy. Epilepsia 51:556–563

    Article  PubMed  Google Scholar 

  16. Hamad A, Aengenendt J, Elsharkawy A, Freitag H, Hans V, Pannek H, Sakamoto A, Tuxhorn I, Woermann F, Polster T (2009) Outcome after hemispherectomy—consequences for the process of decision making. Epilepsia 50 (Suppl. 4):136

    Google Scholar 

  17. Handler MH, Zawadzki L, Knupp K, Koh S, Laoprasert P (2009) Paediatric hemispherectomy—a single-institution recent experience. J. Neurosurg-Paediatrics 3:448

    Google Scholar 

  18. Jalin C, Fohlen M, Dorfmueller G, Guttieres E, Bulteau C, Delalande O (2009) Polymicrogyria: electro-clinical and anatomical aspects. A study of 12 children with unilateral polymicrogyria associated with epilepsy with continuous spikes and waves syndrome treated by hemispherotomy. Epilepsies 21:17-33

    Google Scholar 

  19. Jonas R, Nguyen S, Hu B, Asarnow RF, LoPresti C, Curtiss S, de Bode S, Yudovin S, Shields WD, Vinters HV, Mathern GW (2004) Cerebral hemispherectomy: hospital course, seizure, developmental, language, and motor outcomes. Neurology 62:1712–1721

    Article  PubMed  CAS  Google Scholar 

  20. Kawai KS H (2004) Clinical outcome and comparison of surgical procedures in hemispherotomy for children with malformation of cortical. Epilepsia 45(suppl s3):168

    Google Scholar 

  21. Kestle J, Connolly M, Cochrane D (2000) Pediatric peri-insular hemispherotomy. Pediatr Neurosurg 32:44–47

    Article  PubMed  CAS  Google Scholar 

  22. Kossoff EH, Buck C, Freeman JM (2002) Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology 59:1735–1738

    Article  PubMed  Google Scholar 

  23. Kossoff EH, Vining EP, Pillas DJ, Pyzik PL, Avellino AM, Carson BS, Freeman JM (2003) Hemispherectomy for intractable unihemispheric epilepsy etiology vs outcome. Neurology 61:887–890

    Article  PubMed  CAS  Google Scholar 

  24. Koubeissi MZ, Syed TU, Syed I, Jordan J, Alshekhlee A, Kossoff EH (2009) Hemispherectomy-associated complications from the Kids' Inpatient Database. Epilepsy Res 87:47–53

    Article  PubMed  Google Scholar 

  25. Kwan A, Ng WH, Otsubo H, Ochi A, Snead OC 3rd, Tamber MS, Rutka JT (2010) Hemispherectomy for the control of intractable epilepsy in childhood: comparison of 2 surgical techniques in a single institution. Neurosurgery 67:429–436

    Article  PubMed  Google Scholar 

  26. Lam S, Mathern GW (2010) Functional Hemispherectomy at UCLA. In: Cataltepe O, Jallo GI (eds) Pediatric Epilepsy, vol 230-240. Thieme, New York

  27. Limbrick DD, Narayan P, Powers AK, Ojemann JG, Park TS, Bertrand M, Smyth MD (2009) Hemispherotomy: efficacy and analysis of seizure recurrence. J Neurosurg Pediatr 4:323–332

    Article  PubMed  Google Scholar 

  28. McClelland S 3rd, Maxwell RE (2007) Hemispherectomy for intractable epilepsy in adults: the first reported series. Ann Neurol 61:372–376

    Article  PubMed  Google Scholar 

  29. Peacock WJ, Wehby-Grant MC, Shields WD, Shewmon DA, Chugani HT, Sankar R, Vinters HV (1996) Hemispherectomy for intractable seizures in children: a report of 58 cases. Childs Nerv Syst 12:376–384

    Article  PubMed  CAS  Google Scholar 

  30. Pinto A, Takeoka M, Bergin AM, Bourgeois BF, Duffy FH, Sarco D, Black P, Poduri A (2009) Surgery for intractable epilepsy due to unilateral cortical disease: Comparison between anatomical and functional hemispherectomy. Epilepsia 50 (Suppl. 4):148

    Google Scholar 

  31. Ramesha KN, Rajesh B, Ashalatha R, Kesavadas C, Abraham M, Radhakrishnan VV, Sarma PS, Radhakrishnan K (2009) Rasmussen's encephalitis: experience from a developing country based on a group of medically and surgically treated patients. Seizure 18:567–572

    Article  PubMed  CAS  Google Scholar 

  32. Rasmussen T (1983) Hemispherectomy for seizures revisited. Can J Neurol Sci 10:71–78

    PubMed  CAS  Google Scholar 

  33. Schramm J (2002) Hemispherectomy techniques. Neurosurg Clin N Am 13:113–134, ix

    Article  PubMed  Google Scholar 

  34. Schramm J (2011) Hemispheric Disconnection Procedures. In: Winn HR (ed) Youman’s Neurological Surgery, 6th edition, vol Vol 1. Elsevier Inc, Philadelphia, pp 796–806

    Chapter  Google Scholar 

  35. Schramm J, Behrens E, Entzian W (1995) Hemispherical deafferentation: an alternative to functional hemispherectomy. Neurosurgery 36:509–515, discussion 515-506

    Article  PubMed  CAS  Google Scholar 

  36. Schramm J, Kral T, Clusmann H (2001) Transsylvian keyhole functional hemispherectomy. Neurosurgery 49:891–900, discussion 900-891

    PubMed  CAS  Google Scholar 

  37. Schramm J, Behrens E, Entzian W (1992) Hemispherical deafferentation: a modified functional hemispherectomy technique. Epilepsia 33(suppl S3):71

    Google Scholar 

  38. Schramm J, Delev D, Wagner J. Elger CE, von Lehe M (2012) Seizure outcome, functional outcome and quality of life after hemispherectomy in adults. Acta Neurosurg, 154:1603–1612

    Google Scholar 

  39. Sell E, Otsubo H, Snead OC, Smith ML, Kerr E, Rutka J, Weiss SK (2004) Long-term clinical outcome of hemispherectomy in a single epilepsy surgery center. Epilepsia 45:276

    Article  Google Scholar 

  40. Shimizu H (2005) Our experience with pediatric epilepsy surgery focusing on corpus callosotomy and hemispherotomy. Epilepsia 46(Suppl 1):30–31

    Article  PubMed  Google Scholar 

  41. Shimizu H, Maehara T (2000) Modification of peri-insular hemispherotomy and surgical results. Neurosurgery 47:367–372, discussion 372-363

    Article  PubMed  CAS  Google Scholar 

  42. Smith SJ, Andermann F, Villemure JG, Rasmussen TB, Quesney LF (1991) Functional hemispherectomy: EEG findings, spiking from isolated brain postoperatively, and prediction of outcome. Neurology 41:1790–1794

    Article  PubMed  CAS  Google Scholar 

  43. Terra-Bustamante VC, Inuzuka LM, Fernandes RM, Escorsi-Rosset S, Wichert-Ana L, Alexandre V Jr, Bianchin MM, Araujo D, Santos AC, Oliveira dos Santos R, Machado HR, Sakamoto AC (2007) Outcome of hemispheric surgeries for refractory epilepsy in pediatric patients. Childs Nerv Syst 23:321–326

    Article  PubMed  Google Scholar 

  44. Villemure JG, Mascott CR (1995) Peri-insular hemispherotomy: surgical principles and anatomy. Neurosurgery 37:975–981

    Article  PubMed  CAS  Google Scholar 

  45. Villemure JG, Vernet O, Delalande O (2000) Hemispheric disconnection: callosotomy and hemispherotomy. Adv Tech Stand Neurosurg 26:25–78

    Article  PubMed  CAS  Google Scholar 

  46. Villemure JG, Mascott C, Andermann F, Rasmussen T (1989) Is removal of the insular cortex in hemispherectomy necessary. Epilepsia 30(Suppl):728

    Google Scholar 

Download references

Acknowledgement

We thank the following colleagues from the Department of Neurosurgery responsible for keeping up the epilepsy surgery database over 2 decades: Dr. E. Behrens, Dr. H. Clusmann, Dr. T. Kral and Dr. A. Müller-Erkwoh, and the study nurses P. Süßmann and B. Harzheim. Prof. H. Urbach is acknowledged for his long-term neuroradiological expertise. We are grateful to our epileptology colleagues from various other programs who entrusted us with the surgery of patients evaluated elsewhere.

Patient follow-up was supported by grants from the Deutsche Forschungsgemeinschaft (DFG) partly within the transregional collaborative research consortium SFB400 “Molecular basis of CNS disorders” and SFB TR3 “Mesio-temporal lobe epilepsies.”

Conflict of interest

None.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to M. von Lehe.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schramm, J., Kuczaty, S., Sassen, R. et al. Pediatric functional hemispherectomy: outcome in 92 patients. Acta Neurochir 154, 2017–2028 (2012). https://doi.org/10.1007/s00701-012-1481-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00701-012-1481-3

Keywords

Navigation