Wang Julei,Huang Tao,Tian Qilong,et al.Clinical analysis of hypothalamic hamartoma in 6 children and literature review[J].Journal of Clinical Pediatric Surgery,2021,20(05):425-429.[doi:10.12260/lcxewkzz.2021.05.006]
显微手术治疗儿童下丘脑错构瘤六例及文献回顾
- Title:
- Clinical analysis of hypothalamic hamartoma in 6 children and literature review
- Keywords:
- Hypothalamic Neoplasms; Hamartoma/SU; Microsurgery/MT; Treatment Outcome; Child
- 分类号:
- R739.4;R726.1;R742.1
- 摘要:
- 目的 探讨显微手术治疗儿童下丘脑错构瘤的适应证、安全性及疗效。方法 回顾性分析空军军医大学第二附属医院2016—2020年经显微手术治疗的6例儿童下丘脑错构瘤临床资料。男5例，女1例，平均年龄4.3岁；2例表现为性早熟，4例表现为痴笑性癫痫，其中1例为Pallister-Hall综合征。6例均经影像学检查确诊。罗氏分型：Ⅱ型5例，Ⅳ型1例。手术方式：经翼点入路5例，经纵裂-胼胝体-穹隆间入路1例。结果 所有病例术后无尿崩、视力视野障碍等相关并发症。术后影像学复查见全切3例，次全切2例，大部分切除1例。随访2个月到4年，4例痴笑性癫痫术后未再次发作。2例性早熟病例身高增长速度较术前明显减缓，骨龄基本同术前，第二性征略有进展。结论 儿童下丘脑错构瘤临床较为罕见，以痴笑性癫痫、中枢性性早熟为主要临床症状，前者手术治疗安全有效，后者宜首选药物治疗。
- Abstract:
- Objective To explore the indication,safety and efficacy of microsurgery for hypothalamic hamartoma in children.Methods Clinical data were retrospectively reviewed for 6 children with hypothalamic hamartoma undergoing microsurgery from 2016 to 2020.There were 5 boys and 1 girl with an average age of 4.3 years.The clinical manifestations included precocious puberty (n=2) and gelastic seizure (n=4),including Pallister-hall syndrome (n=1).All cases were confirmed by imaging examination.The clinical types were Ⅱ (n=5) and Ⅳ (n=1).Operation was performed via a pterional approach (n=5) and an interhemispheric approach (n=1).Results There was no occurrence of diabetes insipidus,visual field disturbance or other related complications.Postoperative imaging re-examinations showed total resection (n=3),subtotal resection (n=2) and partial resection (n=1).During a follow-up period of 2 to 48 months,there was no postoperative recurrence of gelastic seizure.The growth rate of height was significantly slower in precocious puberty children than that before operation.Bone age remained constant and secondary sexual characteristics slightly improved.Conclusion Hypothalamic hamartoma is rare in children and its major clinical symptoms are gelastic seizure and central precocious puberty.Operation is both safe and effective for gelastic seizure while drug treatment should be reserved for precocious puberty.
参考文献/References:
1 罗世琪,李春德,马振宇,等.214例下丘脑错构瘤分型与临床症状[J].中华神经外科杂志,2009,25(9):788-792.DOI:10.3760/cma.j.issn.1001-2346.2009.09.011. Luo SQ,Li CD,Ma ZY,et al.Classifications and clinical manifestations of hypothalamic hamartoma:a report of 214 cases[J].Chin J Neurosurg,2009,25(9):788-792.DOI:10.3760/cma.j.issn.1001-2346.2009.09.011.
2 Alomari SO,Houshiemy MN,Bsat S,et al.Hypothalamic hamartomas:A comprehensive review of the literature-Part 1:Neurobiological features,clinical presentations and advancements in diagnostic tools[J].Clin Neurol Neurosurg,2020,197:106076.DOI:10.1016/j.clineuro.2020.106076.
3 Zhang HW,Li Y,Liu BF,et al.Hypothalamic hamartoma,gray matter heterotopia,and polymicrogyria in a boy:case report and literature review[J].World Neurosurgery,2020,142:396-400.DOI:10.1016/j.wneu.2020.07.098.
4 Kerrigan JF,Ng Y,Chung S,et al.The hypothalamic hamartoma:a model of subcortical epileptogenesis and encephalopathy[J].Semin Pediatr Neurol,2005,12(2):119-131.DOI:10.1016/j.spen.2005.04.002.
5 黎思宇,葛明,孙骇浪,等.儿童下丘脑错构瘤18例临床分析[J].中华实用儿科临床杂志,2019,34(6):462-463.DOI:10.3760/cma.j.issn.2095-428X.2019.06.015. Li SY,Ge M,Sun HL,et al.Clinical analysis of hypothalamic hamartoma in 18 children[J].Chin J Appl Clin Pediatr,2019,34(6):462-463.DOI:10.3760/cma.j.issn.2095-428X.2019.06.015.
6 华刚,谭红平,姜楠,等.SEEG引导下射频热凝毁损术在癫痫外科中的应用[J].立体定向和功能性神经外科杂志,2020,33(2):69-73.DOI:10.19854/j.cnki.1008-2425.2020.02.0002. Hua G,Tan HP,Jiang N,et al.Application of SEEG-guided radiofrequency thermocoagulation during epilepsy surgery[J].Chin J Stereotact Funct Neurosurg,2020,33(2):69-73.DOI:10.19854/j.cnki.1008-2425.2020.02.0002.
7 Ramos CO,Latronico AC,Cukier P,et al.Long-term outcomes of patients with central precocious puberty due to hypothalamic hamartoma after GnRHa treatment:anthropometric,metabolic,and reproductive aspects[J].Neuroendocrinology,2018,106(3):203-210.DOI:10.1159/000477584.
8 Harrison VS,Oatman O,Kerrigan JF.Hypothalamic hamartoma with epilepsy:Review of endocrine comorbidity[J].Epilepsia,2017,58(2):50-59.DOI:10.1111/epi.13756.
9 罗世琪,李春德,马振宇,等.下丘脑错构瘤所致单纯性早熟的显微外科治疗[J].中华神经外科杂志,2000,16(6):341-343.DOI:10.3760/j.issn:1001-2346.2000.06.001. Luo SQ,Li CD,Ma ZY,et al.Microsurgical treatment of hypothalamic hamartoma causing precocious puberty[J].Chin J Neurosurg,2000,16(6):341-343.DOI:10.3760/j.issn:1001-2346.2000.06.001.
10 Ramos CO,Latronico AC,Cukier P,et al.Long-term outcomes of patients with central precocious puberty due to hypothalamic hamartoma after GnRH analog treatment:anthropometric,metabolic and reproductive aspects[J].Neuroendocrinology,2018,106(3):203-210.DOI:10.1159/000477584.
11 Valentin A,Lazaro M,Mullatti N,et al.Cingulate epileptogenesis in hypothalamic chamartoma[J].Epilepsia,2011,52(5):e35-e39.DOI:10.1111/j.1528-1167.2011.03060.x.
12 Wang M,Zhang Y,Xue P,et al.Optimized SEEG-guided radiofrequency thermocoagulation in the treatment of pediatric hypothalamic hamartomas[J].Seizure,2021,86:102-108.DOI:10.1016/j.seizure.2021.01.023.
13 张宏武,刘宝富,高阳旭,等.下丘脑错构瘤所致癫痫的手术治疗[J].中华神经外科杂志,2016,32(9):878-881.DOI:10.3760/cma.j.issn.1001-2346.2016.09.004. Zhang HW,Liu BF,Gao YX,et al.Surgical treatment of epilepsy caused by hypothalamic hamartomas[J].Chin J Neurosurg,2016,32(9):878-881.DOI:10.3760/cma.j.issn.1001-2346.2016.09.004.
14 Wang D,Shan YZ,Bartolomei F,et al.Electrophysiological properties and seizure networks in hypothalamic hamartoma[J].Ann Clin Transl Neurol,2020,7(5):653-666.DOI:10.1002/acn3.51033.
15 Ito S,Kitazawa R,Haraguchi R,et al.Novel GLI3 variant causing overlapped Greig cephalopolysyndactyly syndrome (GCPS) and Pallister-Hall syndrome (PHS) phenotype with agenesis of gallbladder and pancreas[J].Diagn Pathol,2018,13(1):1.DOI:10.1186/s13000-017-0682-8.
备注/Memo
收稿日期:2021-01-04。
基金项目:陕西省重点研发计划（一般项目）（编号：2019SF-058）
通讯作者:屈延,Email:yanqu0123@icloud.com