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Published in: Acta Neurochirurgica 11/2012

01-11-2012 | Clinical Article

Pediatric functional hemispherectomy: outcome in 92 patients

Authors: J. Schramm, S. Kuczaty, R. Sassen, C. E. Elger, M. von Lehe

Published in: Acta Neurochirurgica | Issue 11/2012

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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.
Literature
1.
go back to reference 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 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
2.
go back to reference 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 48:133–140PubMedCrossRef 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 48:133–140PubMedCrossRef
3.
go back to reference 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 75:1623–1630PubMedCrossRef 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 75:1623–1630PubMedCrossRef
4.
go back to reference 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 106:20–28PubMed 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 106:20–28PubMed
5.
go back to reference 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 84:903–911PubMedCrossRef 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 84:903–911PubMedCrossRef
6.
go back to reference 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 107:275–280PubMedCrossRef 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 107:275–280PubMedCrossRef
7.
go back to reference 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 100:125–141PubMedCrossRef 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 100:125–141PubMedCrossRef
8.
go back to reference 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 50:1381–1384PubMedCrossRef 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 50:1381–1384PubMedCrossRef
9.
go back to reference 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 ONS32PubMedCrossRef 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 ONS32PubMedCrossRef
10.
go back to reference 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 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
11.
go back to reference 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 126:556–566PubMedCrossRef 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 126:556–566PubMedCrossRef
12.
go back to reference 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 33:198–207PubMedCrossRef 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 33:198–207PubMedCrossRef
13.
go back to reference 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 34:65–73PubMedCrossRef 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 34:65–73PubMedCrossRef
14.
go back to reference Gonzalez-Martinez JA, Gupta A, Kotagal P, Lachhwani D, Wyllie E, Luders HO, Bingaman WE (2005) Hemispherectomy for catastrophic epilepsy in infants. Epilepsia 46:1518–1525PubMedCrossRef Gonzalez-Martinez JA, Gupta A, Kotagal P, Lachhwani D, Wyllie E, Luders HO, Bingaman WE (2005) Hemispherectomy for catastrophic epilepsy in infants. Epilepsia 46:1518–1525PubMedCrossRef
15.
go back to reference 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 51:556–563PubMedCrossRef 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 51:556–563PubMedCrossRef
16.
go back to reference 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 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
17.
go back to reference Handler MH, Zawadzki L, Knupp K, Koh S, Laoprasert P (2009) Paediatric hemispherectomy—a single-institution recent experience. J. Neurosurg-Paediatrics 3:448 Handler MH, Zawadzki L, Knupp K, Koh S, Laoprasert P (2009) Paediatric hemispherectomy—a single-institution recent experience. J. Neurosurg-Paediatrics 3:448
18.
go back to reference 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 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
19.
go back to reference 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 62:1712–1721PubMedCrossRef 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 62:1712–1721PubMedCrossRef
20.
go back to reference Kawai KS H (2004) Clinical outcome and comparison of surgical procedures in hemispherotomy for children with malformation of cortical. Epilepsia 45(suppl s3):168 Kawai KS H (2004) Clinical outcome and comparison of surgical procedures in hemispherotomy for children with malformation of cortical. Epilepsia 45(suppl s3):168
21.
go back to reference Kestle J, Connolly M, Cochrane D (2000) Pediatric peri-insular hemispherotomy. Pediatr Neurosurg 32:44–47PubMedCrossRef Kestle J, Connolly M, Cochrane D (2000) Pediatric peri-insular hemispherotomy. Pediatr Neurosurg 32:44–47PubMedCrossRef
22.
go back to reference Kossoff EH, Buck C, Freeman JM (2002) Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology 59:1735–1738PubMedCrossRef Kossoff EH, Buck C, Freeman JM (2002) Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide. Neurology 59:1735–1738PubMedCrossRef
23.
go back to reference Kossoff EH, Vining EP, Pillas DJ, Pyzik PL, Avellino AM, Carson BS, Freeman JM (2003) Hemispherectomy for intractable unihemispheric epilepsy etiology vs outcome. Neurology 61:887–890PubMedCrossRef Kossoff EH, Vining EP, Pillas DJ, Pyzik PL, Avellino AM, Carson BS, Freeman JM (2003) Hemispherectomy for intractable unihemispheric epilepsy etiology vs outcome. Neurology 61:887–890PubMedCrossRef
24.
go back to reference Koubeissi MZ, Syed TU, Syed I, Jordan J, Alshekhlee A, Kossoff EH (2009) Hemispherectomy-associated complications from the Kids' Inpatient Database. Epilepsy Res 87:47–53PubMedCrossRef Koubeissi MZ, Syed TU, Syed I, Jordan J, Alshekhlee A, Kossoff EH (2009) Hemispherectomy-associated complications from the Kids' Inpatient Database. Epilepsy Res 87:47–53PubMedCrossRef
25.
go back to reference 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 67:429–436PubMedCrossRef 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 67:429–436PubMedCrossRef
26.
go back to reference Lam S, Mathern GW (2010) Functional Hemispherectomy at UCLA. In: Cataltepe O, Jallo GI (eds) Pediatric Epilepsy, vol 230-240. Thieme, New York Lam S, Mathern GW (2010) Functional Hemispherectomy at UCLA. In: Cataltepe O, Jallo GI (eds) Pediatric Epilepsy, vol 230-240. Thieme, New York
27.
go back to reference 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 4:323–332PubMedCrossRef 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 4:323–332PubMedCrossRef
28.
go back to reference McClelland S 3rd, Maxwell RE (2007) Hemispherectomy for intractable epilepsy in adults: the first reported series. Ann Neurol 61:372–376PubMedCrossRef McClelland S 3rd, Maxwell RE (2007) Hemispherectomy for intractable epilepsy in adults: the first reported series. Ann Neurol 61:372–376PubMedCrossRef
29.
go back to reference 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 12:376–384PubMedCrossRef 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 12:376–384PubMedCrossRef
30.
go back to reference 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 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
31.
go back to reference 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 18:567–572PubMedCrossRef 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 18:567–572PubMedCrossRef
32.
go back to reference Rasmussen T (1983) Hemispherectomy for seizures revisited. Can J Neurol Sci 10:71–78PubMed Rasmussen T (1983) Hemispherectomy for seizures revisited. Can J Neurol Sci 10:71–78PubMed
34.
go back to reference Schramm J (2011) Hemispheric Disconnection Procedures. In: Winn HR (ed) Youman’s Neurological Surgery, 6th edition, vol Vol 1. Elsevier Inc, Philadelphia, pp 796–806CrossRef Schramm J (2011) Hemispheric Disconnection Procedures. In: Winn HR (ed) Youman’s Neurological Surgery, 6th edition, vol Vol 1. Elsevier Inc, Philadelphia, pp 796–806CrossRef
35.
go back to reference Schramm J, Behrens E, Entzian W (1995) Hemispherical deafferentation: an alternative to functional hemispherectomy. Neurosurgery 36:509–515, discussion 515-506PubMedCrossRef Schramm J, Behrens E, Entzian W (1995) Hemispherical deafferentation: an alternative to functional hemispherectomy. Neurosurgery 36:509–515, discussion 515-506PubMedCrossRef
36.
go back to reference Schramm J, Kral T, Clusmann H (2001) Transsylvian keyhole functional hemispherectomy. Neurosurgery 49:891–900, discussion 900-891PubMed Schramm J, Kral T, Clusmann H (2001) Transsylvian keyhole functional hemispherectomy. Neurosurgery 49:891–900, discussion 900-891PubMed
37.
go back to reference Schramm J, Behrens E, Entzian W (1992) Hemispherical deafferentation: a modified functional hemispherectomy technique. Epilepsia 33(suppl S3):71 Schramm J, Behrens E, Entzian W (1992) Hemispherical deafferentation: a modified functional hemispherectomy technique. Epilepsia 33(suppl S3):71
38.
go back to reference 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 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
39.
go back to reference 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 45:276CrossRef 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 45:276CrossRef
40.
go back to reference Shimizu H (2005) Our experience with pediatric epilepsy surgery focusing on corpus callosotomy and hemispherotomy. Epilepsia 46(Suppl 1):30–31PubMedCrossRef Shimizu H (2005) Our experience with pediatric epilepsy surgery focusing on corpus callosotomy and hemispherotomy. Epilepsia 46(Suppl 1):30–31PubMedCrossRef
41.
go back to reference Shimizu H, Maehara T (2000) Modification of peri-insular hemispherotomy and surgical results. Neurosurgery 47:367–372, discussion 372-363PubMedCrossRef Shimizu H, Maehara T (2000) Modification of peri-insular hemispherotomy and surgical results. Neurosurgery 47:367–372, discussion 372-363PubMedCrossRef
42.
go back to reference 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 41:1790–1794PubMedCrossRef 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 41:1790–1794PubMedCrossRef
43.
go back to reference 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 23:321–326PubMedCrossRef 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 23:321–326PubMedCrossRef
44.
go back to reference Villemure JG, Mascott CR (1995) Peri-insular hemispherotomy: surgical principles and anatomy. Neurosurgery 37:975–981PubMedCrossRef Villemure JG, Mascott CR (1995) Peri-insular hemispherotomy: surgical principles and anatomy. Neurosurgery 37:975–981PubMedCrossRef
45.
go back to reference Villemure JG, Vernet O, Delalande O (2000) Hemispheric disconnection: callosotomy and hemispherotomy. Adv Tech Stand Neurosurg 26:25–78PubMedCrossRef Villemure JG, Vernet O, Delalande O (2000) Hemispheric disconnection: callosotomy and hemispherotomy. Adv Tech Stand Neurosurg 26:25–78PubMedCrossRef
46.
go back to reference Villemure JG, Mascott C, Andermann F, Rasmussen T (1989) Is removal of the insular cortex in hemispherectomy necessary. Epilepsia 30(Suppl):728 Villemure JG, Mascott C, Andermann F, Rasmussen T (1989) Is removal of the insular cortex in hemispherectomy necessary. Epilepsia 30(Suppl):728
Metadata
Title
Pediatric functional hemispherectomy: outcome in 92 patients
Authors
J. Schramm
S. Kuczaty
R. Sassen
C. E. Elger
M. von Lehe
Publication date
01-11-2012
Publisher
Springer-Verlag
Published in
Acta Neurochirurgica / Issue 11/2012
Print ISSN: 0001-6268
Electronic ISSN: 0942-0940
DOI
https://doi.org/10.1007/s00701-012-1481-3

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