Hemispheric surgery and outcomes of Sturge-Weber syndrome patients under 1 year old

doi:10.3877/cma.j.issn.1674-0785.2021.08.004

Abstract

Abstract:

Objective

To analyze the clinical characteristics, hemispheric surgical treatment, and outcomes of Sturge-Weber syndrome (SWS) patients under 1 year old, in order to provide guidance for treatment.

Methods

A total of ten SWS children who underwent hemispheric surgery in Sanbo Brain Hospital from January 2009 to February 2018 were collected. Four patients received anatomical hemispherectomy (AH) and six received modified peri-insular hemispherotomy (MH). The seizure outcome was assessed following the International League against Epilepsy standard (ILAE) and the cognitive outcome was analyzed according to the Denver Developmental Screening Scale-Ⅱ(DDST).

Results

The patients included ranged in age from 3 to 12 months, with an average of (8.10±3.44) months. Six patients met the surgical indications for drug resistant epilepsy with cognitive developmental abnormalities and/or motor dysfunction, and four met the surgical indications for ≥2 times of status epilepticus. After a 2-year follow-up, all of the children achieved ILAE 1 (seizure-free), and 70% of patients achieved cognitive improvement. There was no significant difference in outcomes between AH and MH (cognitive improvement: 83.3% vs 50.0%, P=0.500). MH was safer and less invasive than AH.

Conclusion

Hemispheric surgery is safe and effective for SWS children under 1 year old in terms of both seizures and cognitive function. MH is a modified operation of the traditional AH, and it has similar outcomes with AH but is safer and minimally invasive.

Key words: Sturge-Weber syndrome, Epilepsy, Children, Surgery, Outcome

Shu Wang, Meng Zhao, Junhong Pan, Xiongfei Wang, Chunsheng Zhang, Haowei Ma, Mengyang Wang, Tianfu Li, Jing Wang, Jian Zhou, Changqing Liu, Guoming Luan, Yuguang Guan. Hemispheric surgery and outcomes of Sturge-Weber syndrome patients under 1 year old[J]. Chinese Journal of Clinicians(Electronic Edition), 2021, 15(08): 578-583.

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URL: https://zhlcyszz.cma-cmc.com.cn/EN/10.3877/cma.j.issn.1674-0785.2021.08.004

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Figures/Tables 3

References 22

1	Rihani HT, Dalvin LA, Hodge DO, et al. Incidence of Sturge-Weber syndrome and associated ocular involvement in Olmsted County, Minnesota, United States [J]. Ophthalmic Genet, 2020,41(2): 108-124.
2	Nguyen V, Hochman M, Mihm MJ, et al. The pathogenesis of port wine stain and Sturge Weber syndrome: complex interactions between genetic alterations and aberrant MAPK and PI3K activation [J]. Int J Mol Sci, 2019, 20(9): 2243.
3	Bianchi F, Auricchio AM, Battaglia DI, et al. Sturge-Weber syndrome: an update on the relevant issues for neurosurgeons [J]. Childs Nerv Syst, 2020, 36(10): 2553-2570.
4	Cho S, Maharathi B, Ball KL, et al. Sturge-Weber syndrome patient registry: delayed diagnosis and poor seizure control [J]. J Pediatr, 2019, 215: 158-163.
5	Smegal LF, Sebold AJ, Hammill AM, et al. Multicenter research data of epilepsy management in patients with Sturge-Weber syndrome [J]. Pediatr Neurol, 2021, 119: 3-10.
6	赵惠敏, 石凯丽. 抗癫痫药物对内分泌功能影响的研究进展 [J/CD]. 中华临床医师杂志(电子版), 2019, 13(9): 693-697.
7	滕雪宝, 袁宝强, 雍曾花, 等. 大剂量激素冲击治疗57例难治性癫痫的疗效观察[J/CD]. 中华临床医师杂志(电子版), 2017, 11(7): 1103-1106.
8	Luat AF, Juhász C, Loeb JA, et al. Neurological complications of Sturge-Weber syndrome: current status and unmet needs [J]. Pediatr Neurol, 2019, 98: 31-38.
9	Saxena A, Jones L, Shankar R, et al. Sudden unexpected death in epilepsy in children: a focused review of incidence and risk factors [J]. J Neurol Neurosurg Psychiatry, 2018, 89(10): 1064-1070.
10	Alotaibi F, Albaradie R, Almubarak S, et al. Hemispherotomy for epilepsy: the procedure evolution and outcome [J]. Can J Neurol Sci, 2021, 48(4): 451-463.
11	Javaid U, Ali M H, Jamal S, et al. Pathophysiology, diagnosis, and management of glaucoma associated with Sturge-Weber syndrome [J]. Int Ophthalmol, 2018, 38(1): 409-416.
12	Bourgeois M, Crimmins DW, De Oliveira RS, et al. Surgical treatment of epilepsy in Sturge-Weber syndrome in children [J]. J Neurosurg Pediatr, 2007, 106(1): 20-28.
13	Manacero S, Nunes M L. Longitudinal study of sleep behavior and motor development in low-birth-weight preterm children from infancy to preschool years [J]. J Pediatr (Rio J), 2021, 97(1): 44-51.
14	Mula M, Zaccara G, Galimberti CA, et al. Validated outcome of treatment changes according to International League Against Epilepsy criteria in adults with drug-resistant focal epilepsy [J]. Epilepsia, 2019, 60(6): 1114-1123.
15	Zallmann M, Leventer R J, Mackay M T, et al. Screening for Sturge-Weber syndrome: a state-of-the-art review[J]. Pediatr Dermatol, 2018, 35(1): 30-42.
16	Pinto A L, Chen L, Friedman R, et al. Sturge-Weber syndrome: brain magnetic resonance imaging and neuropathology findings [J]. Pediatr Neurol, 2016, 58: 25-30.
17	Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R, et al. Sturge-Weber syndrome: study of 55 patients [J]. Can J Neurol Sci, 2008, 35(3): 301-307.
18	Luat AF, Juhász C, Loeb JA, et al. Neurological complications of Sturge-Weber syndrome: current status and unmet needs [J]. Pediatr Neurol, 2019, 98: 31-38.
19	Harmon KA, Day AM, Hammill AM, et al. Quality of life in children with Sturge-Weber syndrome [J]. Pediatr Neurol, 2019, 101: 26-32.
20	Luat AF, Behen ME, Chugani HT, et al. Cognitive and motor outcomes in children with unilateral Sturge-Weber syndrome: effect of age at seizure onset and side of brain involvement [J]. Epilepsy Behav, 2018, 80: 202-207.
21	Vasquez A, Farias-Moeller R, Tatum W. Pediatric refractory and super-refractory status epilepticus [J]. Seizure, 2019,68:62-71.
22	McGovern RA, Moosa A, Jehi L, et al. Hemispherectomy in adults and adolescents: Seizure and functional outcomes in 47 patients [J]. Epilepsia, 2019, 60(12): 2416-2427.

编号	年龄（月）	性别	癫痫病程（月）	发作类型	面部血管痣	青光眼	认知发育	MRI
1	12	女	9	CPS	左额面	有	异常	左半球萎缩、异常信号、脑回样强化
2	9	女	7.5	CPS	右眼周	无	异常	右半球萎缩、异常信号、脑回样强化
3	12	男	8.5	CPS、GTCS	右额面	无	异常	右半球萎缩、异常信号、脑回样强化
4	6	男	5	CPS、SE	右额面	有	异常	右半球萎缩、异常信号、脑回样强化
5	6.5	男	6	CPS、GTCS	右额眼周	有	异常	右半球萎缩、异常信号、脑回样强化
6	3	男	2	CPS、SE	左面	无	异常	左半球萎缩、异常信号、脑回样强化
7	4	男	2	CPS、SE	双面	无	异常	右半球萎缩、异常信号、脑回样强化
8	12	女	10	SPS	左面	有	异常	左半球萎缩、异常信号、脑回样强化
9	6	男	5	CPS、SE	右额	无	可疑	右半球萎缩、异常信号、脑回样强化
10	10.5	女	7	CPS	左面	有	异常	左半球萎缩、异常信号、脑回样强化

编号	年龄（月）	性别	癫痫病程（月）	发作类型	面部血管痣	青光眼	认知发育	MRI
1	12	女	9	CPS	左额面	有	异常	左半球萎缩、异常信号、脑回样强化
2	9	女	7.5	CPS	右眼周	无	异常	右半球萎缩、异常信号、脑回样强化
3	12	男	8.5	CPS、GTCS	右额面	无	异常	右半球萎缩、异常信号、脑回样强化
4	6	男	5	CPS、SE	右额面	有	异常	右半球萎缩、异常信号、脑回样强化
5	6.5	男	6	CPS、GTCS	右额眼周	有	异常	右半球萎缩、异常信号、脑回样强化
6	3	男	2	CPS、SE	左面	无	异常	左半球萎缩、异常信号、脑回样强化
7	4	男	2	CPS、SE	双面	无	异常	右半球萎缩、异常信号、脑回样强化
8	12	女	10	SPS	左面	有	异常	左半球萎缩、异常信号、脑回样强化
9	6	男	5	CPS、SE	右额	无	可疑	右半球萎缩、异常信号、脑回样强化
10	10.5	女	7	CPS	左面	有	异常	左半球萎缩、异常信号、脑回样强化

编号	手术类别	侧别	出血量（ml）	术后住院时长（d）	癫痫预后（ILAE分级）	认知发育预后（DDST评价）
1	解剖半球切除术	左侧	1000	22	1	改善
2	解剖半球切除术	右侧	750	15	1	未改善
3	改良环岛半球离断术	右侧	650	14	1	改善
4	解剖半球切除术	右侧	800	12	1	改善
5	解剖半球切除术	右侧	600	13	1	未改善
6	改良环岛半球离断术	左侧	600	12	1	改善
7	改良环岛半球离断术	右侧	500	10	1	未改善
8	改良环岛半球离断术	左侧	450	9	1	改善
9	改良环岛半球离断术	右侧	750	10	1	改善
10	改良环岛半球离断术	左侧	550	9	1	改善

编号	手术类别	侧别	出血量（ml）	术后住院时长（d）	癫痫预后（ILAE分级）	认知发育预后（DDST评价）
1	解剖半球切除术	左侧	1000	22	1	改善
2	解剖半球切除术	右侧	750	15	1	未改善
3	改良环岛半球离断术	右侧	650	14	1	改善
4	解剖半球切除术	右侧	800	12	1	改善
5	解剖半球切除术	右侧	600	13	1	未改善
6	改良环岛半球离断术	左侧	600	12	1	改善
7	改良环岛半球离断术	右侧	500	10	1	未改善
8	改良环岛半球离断术	左侧	450	9	1	改善
9	改良环岛半球离断术	右侧	750	10	1	改善
10	改良环岛半球离断术	左侧	550	9	1	改善

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