Acta Neurochirurgica

, Volume 154, Issue 11, pp 2017–2028 | Cite as

Pediatric functional hemispherectomy: outcome in 92 patients

  • J. Schramm
  • S. Kuczaty
  • R. Sassen
  • C. E. Elger
  • M. von LeheEmail author
Clinical Article



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).


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.


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.


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.


Epilepsy surgery Pediaric Hemispherectomy Outcome 



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



  1. 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 625–632Google Scholar
  2. 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 48:133–140PubMedCrossRefGoogle Scholar
  3. 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 75:1623–1630PubMedCrossRefGoogle Scholar
  4. 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 106:20–28PubMedGoogle Scholar
  5. 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 84:903–911PubMedCrossRefGoogle Scholar
  6. 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 107:275–280PubMedCrossRefGoogle Scholar
  7. 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 100:125–141PubMedCrossRefGoogle Scholar
  8. 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 50:1381–1384PubMedCrossRefGoogle Scholar
  9. 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 ONS32PubMedCrossRefGoogle Scholar
  10. 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):99–100Google Scholar
  11. 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 126:556–566PubMedCrossRefGoogle Scholar
  12. 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 33:198–207PubMedCrossRefGoogle Scholar
  13. 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 34:65–73PubMedCrossRefGoogle Scholar
  14. 14.
    Gonzalez-Martinez JA, Gupta A, Kotagal P, Lachhwani D, Wyllie E, Luders HO, Bingaman WE (2005) Hemispherectomy for catastrophic epilepsy in infants. Epilepsia 46:1518–1525PubMedCrossRefGoogle Scholar
  15. 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 51:556–563PubMedCrossRefGoogle Scholar
  16. 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):136Google Scholar
  17. 17.
    Handler MH, Zawadzki L, Knupp K, Koh S, Laoprasert P (2009) Paediatric hemispherectomy—a single-institution recent experience. J. Neurosurg-Paediatrics 3:448Google Scholar
  18. 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 21:17-33Google Scholar
  19. 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 62:1712–1721PubMedCrossRefGoogle Scholar
  20. 20.
    Kawai KS H (2004) Clinical outcome and comparison of surgical procedures in hemispherotomy for children with malformation of cortical. Epilepsia 45(suppl s3):168Google Scholar
  21. 21.
    Kestle J, Connolly M, Cochrane D (2000) Pediatric peri-insular hemispherotomy. Pediatr Neurosurg 32:44–47PubMedCrossRefGoogle Scholar
  22. 22.
    Kossoff EH, Buck C, Freeman JM (2002) Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology 59:1735–1738PubMedCrossRefGoogle Scholar
  23. 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 61:887–890PubMedCrossRefGoogle Scholar
  24. 24.
    Koubeissi MZ, Syed TU, Syed I, Jordan J, Alshekhlee A, Kossoff EH (2009) Hemispherectomy-associated complications from the Kids' Inpatient Database. Epilepsy Res 87:47–53PubMedCrossRefGoogle Scholar
  25. 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 67:429–436PubMedCrossRefGoogle Scholar
  26. 26.
    Lam S, Mathern GW (2010) Functional Hemispherectomy at UCLA. In: Cataltepe O, Jallo GI (eds) Pediatric Epilepsy, vol 230-240. Thieme, New YorkGoogle Scholar
  27. 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 4:323–332PubMedCrossRefGoogle Scholar
  28. 28.
    McClelland S 3rd, Maxwell RE (2007) Hemispherectomy for intractable epilepsy in adults: the first reported series. Ann Neurol 61:372–376PubMedCrossRefGoogle Scholar
  29. 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 12:376–384PubMedCrossRefGoogle Scholar
  30. 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):148Google Scholar
  31. 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 18:567–572PubMedCrossRefGoogle Scholar
  32. 32.
    Rasmussen T (1983) Hemispherectomy for seizures revisited. Can J Neurol Sci 10:71–78PubMedGoogle Scholar
  33. 33.
    Schramm J (2002) Hemispherectomy techniques. Neurosurg Clin N Am 13:113–134, ixPubMedCrossRefGoogle Scholar
  34. 34.
    Schramm J (2011) Hemispheric Disconnection Procedures. In: Winn HR (ed) Youman’s Neurological Surgery, 6th edition, vol Vol 1. Elsevier Inc, Philadelphia, pp 796–806CrossRefGoogle Scholar
  35. 35.
    Schramm J, Behrens E, Entzian W (1995) Hemispherical deafferentation: an alternative to functional hemispherectomy. Neurosurgery 36:509–515, discussion 515-506PubMedCrossRefGoogle Scholar
  36. 36.
    Schramm J, Kral T, Clusmann H (2001) Transsylvian keyhole functional hemispherectomy. Neurosurgery 49:891–900, discussion 900-891PubMedGoogle Scholar
  37. 37.
    Schramm J, Behrens E, Entzian W (1992) Hemispherical deafferentation: a modified functional hemispherectomy technique. Epilepsia 33(suppl S3):71Google Scholar
  38. 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, 154:1603–1612Google Scholar
  39. 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 45:276CrossRefGoogle Scholar
  40. 40.
    Shimizu H (2005) Our experience with pediatric epilepsy surgery focusing on corpus callosotomy and hemispherotomy. Epilepsia 46(Suppl 1):30–31PubMedCrossRefGoogle Scholar
  41. 41.
    Shimizu H, Maehara T (2000) Modification of peri-insular hemispherotomy and surgical results. Neurosurgery 47:367–372, discussion 372-363PubMedCrossRefGoogle Scholar
  42. 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 41:1790–1794PubMedCrossRefGoogle Scholar
  43. 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 23:321–326PubMedCrossRefGoogle Scholar
  44. 44.
    Villemure JG, Mascott CR (1995) Peri-insular hemispherotomy: surgical principles and anatomy. Neurosurgery 37:975–981PubMedCrossRefGoogle Scholar
  45. 45.
    Villemure JG, Vernet O, Delalande O (2000) Hemispheric disconnection: callosotomy and hemispherotomy. Adv Tech Stand Neurosurg 26:25–78PubMedCrossRefGoogle Scholar
  46. 46.
    Villemure JG, Mascott C, Andermann F, Rasmussen T (1989) Is removal of the insular cortex in hemispherectomy necessary. Epilepsia 30(Suppl):728Google Scholar

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • J. Schramm
    • 1
  • S. Kuczaty
    • 2
  • R. Sassen
    • 2
  • C. E. Elger
    • 2
  • M. von Lehe
    • 1
    Email author
  1. 1.Department of NeurosurgeryBonn University Medical Center, University of BonnBonnGermany
  2. 2.Department of EpileptologyBonn University Medical Center, University of BonnBonnGermany

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