儿童Sturge-Weber综合征继发癫痫的外科治疗

目的 总结儿童Sturge-Weber综合征继发癫痫的外科治疗经验。方法 回顾性分析2016年1月至2018年6月在我科住院并接受外科手术治疗的8例儿童Sturge-weber综合征继发癫痫患者临床资料,其中男2例,女6例。年龄5个月至4岁,平均年龄26.25个月,3岁以下婴幼儿7例,3岁以上1例。病程时间为2 d至2年,平均8.90个月。对8例患儿的病程、发作类型、影像学、脑电图、手术时机、手术方式、术后癫痫控制情况、并发症等做详细分析。8例患儿均进行详尽的术前评估,包括症状学,影像学,脑电图,认知功能评估等。结果 8例患儿均进行切除手术,其中多脑叶病变切除术3例,功能性大脑半球切除术5例。术后颅内感染2例,皮下积液2例,对侧肢体肌力下降5例,3例与术前相仿。术后随访6～30个月,所有患儿均无癫痫发作,按Engel分级判断疗效,EngelⅠ级8例,无死亡病例,对侧肢体肌力下降的5例患儿均在术后3个月恢复至术前水平。认知功能评估提示8例患儿均有不同程度改善。结论 Sturge-Weber综合征是儿童常见的神经皮肤综合征,癫痫是其常见表现,药物治疗效果有限,积极早期外科干预是有效的治疗方...     

儿童鞍区肿瘤术后高钠血症与脑损害的临床研究

目的　研究儿童鞍区肿瘤术后高钠血症脑损害的发病机制及其预防和治疗。方法　对 1997年 4月至 2 0 0 2年 10月期间北京天坛医院鞍区肿瘤术后发生高钠性脑损害的患儿进行回顾性分析。结果　 4 5 0例儿童鞍区肿瘤术后 111例出现单纯性高钠血症 ,其中 14例 (12 6 1% )发生高钠性脑损害 ,死亡 4例 (3 6 0 % )。血钠高于 16 0mmol/L时 37 5 %患儿发生高钠性脑损害 ,危险性显著增加。结论　高钠性脑损害是儿童鞍区肿瘤术后最主要的并发症之一和致死的主要原因。     

儿童后颅窝髓母细胞瘤42例临床分析

目的：为提高对儿童后颅窝髓母细胞瘤的诊治水平。临床资料：４２例中男女之比为２：１，平均年龄７．３岁，病变位于小脑蚓部者３６例，位于小脑半球６例，均有明显颅内压增高，３９例伴重度醒阻性脑积水，３１例出现小脑功能障碍。治疗结果：全部病例均行手术治疗，除３例死于术手呼吸循环衰竭外，其余３９例恢复良好，术后即行颅脊柱放疗及化疗，随访至今，尚无一例死亡。结论：对儿童后颅窝髓母细胞瘤应采取以手术为主，辅以术后     

经小脑延髓裂入路显微手术治疗儿童第四脑室肿瘤

目的 探讨经小脑延髓裂(CMF)人路显微手术治疗儿童第四脑室肿瘤的方法,提高手术治疗效果.方法 对17例2～14岁原发第四脑室肿瘤患儿,采用枕下正中切口、横窦下颅骨开窗骨瓣成形及经小脑延髓裂入路显微切除肿瘤,其中广泛型CMF切开12例,外侧壁型切开3例,外侧隐窝切开2例;硬脑膜严密缝合或修补,骨瓣复位并固定.结果 肿瘤全切除15例,次全切除2例,病理结果髓母细胞瘤9例,室管膜瘤5例,星形细胞瘤3例,无围手术期死亡发生.术后15例患儿症状体征均明显改善或消失,无新发共济失调、震颤、肌张力降低及脑积水、小脑缄默综合征等并发症.复发2例,均为髓母细胞瘤,2年后死亡1例.结论 经小脑延髓裂人路能较好地显露儿童第四脑室肿瘤,可减少因切开小脑蚓部和向侧方牵拉损伤小脑所导致的并发症发生.     

儿童原发性膜性肾病16例病理特点与远期预后

目的 了解儿童原发性膜性肾病(IMN)病理特点及其远期预后。方法 回顾性收集1979至2010年6月复旦大学附属儿科医院肾脏风湿科经病理诊断为IMN的连续病例为研究对象,分析一般情况、临床表现、病理特点、治疗和随访情况,探讨其远期预后。结果 16例IMN患儿进行分析,占同期肾穿刺活检病例（1 710例）的0.94%。男10例,女6例,年龄2～12岁,平均（5.2±2.6）岁。①临床表现以肾病综合征为主（11例,68.8%）,无症状性蛋白尿5例（31.2%）,伴有高血压2例(12.5%）,起病时伴有肾功能不全2例（12.5%）。②14/16例行肾组织电镜检查,其中Ⅰ期6/14例（42.9%）,Ⅰ~Ⅱ期6/14例（42.9%）,Ⅱ期1/14例（7.1%）,Ⅱ~Ⅲ期1/14例（7.1%）。病理学检查均未见肾小管萎缩、间质纤维化等肾小管间质损伤。③未达到大量蛋白尿标准的7例患儿予随访观察,其中1例病情进展予激素和免疫抑制剂治疗;达到大量蛋白尿标准的9例患儿均予足量激素(2 mg·kg-1·d-1)治疗,其中5例激素依赖或耐药加用免疫抑制剂治疗。至2010年6月,2例失访,14例IMN患儿随访12~91个月,平均（34.0±18.7）个月,在起病后3~16个月均达完全缓解,无一例进展至慢性肾脏疾病;2例起病时伴肾功能不全的患儿肾功能均恢复。结论 儿童IMN临床表现以肾病综合征为主,小年龄、病理分期较轻且不伴肾小管间质损伤患儿的预后相对较好。     

儿童真菌感染继发噬血细胞性淋巴组织细胞增生症 3 例分析

目的提高对儿童真菌感染继发噬血细胞综合征的临床认识。方法总结3例确诊为真菌感染继发噬血细胞综合征患儿的相关资料。结果 3例患儿,男2例,女1例,年龄1岁7个月到3岁1个月,均以反复发热为主诉,2例伴咳嗽,1例伴呕吐,体检发现有肝脾及浅表淋巴结肿大。通过病原学检查确诊为真菌感染的时间均是入院后5天,在有效抗真菌治疗无效后进一步诊断为噬血细胞性淋巴组织细胞增生症。患儿血常规检查早期表现为白细胞计数升高,而血小板及血红蛋白不同程度降低。在抗真菌治疗基础上,2例按照HLH-2004方案进行化疗,1例仅增加地塞米松治疗,3例在治疗过程中均使用丙种球蛋白。最终3例患儿均好转出院。结论儿童真菌感染继发噬血细胞综合征临床少见,在抗真菌治疗基础上增加免疫抑制能改善患儿预后。     

儿童恙虫病继发噬血细胞综合征的临床特点

目的探讨儿童恙虫病继发噬血细胞综合征的临床特点。方法回顾分析1例恙虫病继发噬血细胞综合征患儿的临床资料,并检索国内外期刊数据库,收集32例儿童恙虫病继发噬血细胞综合征的临床资料进行分析。结果 9个月女婴,因发热9天伴抽搐1次入院,入院第3天根据其符合HLH-2004诊断标准8条中的5条,即发热、脾肿大、血细胞减少、血清铁蛋白升高、低纤维蛋白原血症或高脂血症,并有特征性焦痂及血恙虫病抗体阳性,确诊为恙虫病继发噬血细胞综合征。确诊后予阿奇霉素抗感染治疗,3天后体温降至正常,复查各项指标逐渐改善。文献检索到32例(不含本文1例)恙虫病继发噬血细胞综合征患儿,均为亚洲人,男、女各16例,年龄为2个月～11岁;24例患儿有焦痂,30例发现脾大,30例血白蛋白降低,几乎所有患儿都有血细胞减少和血清铁蛋白水平升高。31例患儿病初被误诊。27例采用适当的抗生素治疗,16例联合使用丙种球蛋白,21例使用激素,1例进行了依托泊苷化疗。多数患儿治疗24小时至4天未再发热,5例死亡。结论恙虫病继发噬血细胞综合征常常伴有血清白蛋白降低,需要适当的抗生素及免疫调节治疗,多数预后良好。     

孤立肾并肾盂输尿管连接部梗阻的手术疗效分析

目的了解孤立肾合并肾盂输尿管连接部梗阻(UPJO)的手术治疗时机对于肾功能的影响。方法回顾性分析2006年1月至2018年1月首都医科大学附属北京儿童医院和北京儿童医院顺义妇儿医院收治的孤立肾合并UPJO 23例患儿临床资料。其中男16例,女7例。首诊年龄为1 d~15岁8个月,平均2岁2个月。全组患儿平均保守治疗时间2年9个月。应用SPSS 20.0软件进行统计学分析。结果23例患儿均接受离断性肾盂成形术(A-H术),手术时年龄10个月~16岁4个月,平均4岁11个月。<1岁者2例,分别为10个月、11个月。术后保留双J管2个月,术后3个月时复查静脉肾盂造影(IVP),显示肾盂积水较前无明显变化或不同程度减轻;3例术前IVP 40 min仍不显影者,术后显影明显改善,在10~20 min时显影。随访1年1个月~10年,平均随访3年9个月,泌尿系统超声提示肾盂扩张程度较术前有不同程度减轻。结论孤立肾并UPJO在严密观察下的密切随访及保守治疗至6个月以后是安全的,首选手术方式为肾盂离断成形术。     

Heineke-Mikulicz幽门成形术在儿童胃流出道梗阻良性病变中的应用

目的探讨Heineke-Mikulicz幽门成形术(Heineke-Mikulicz pyloroplasty, HMP)在儿童胃流出道梗阻良性病变中的应用指征及手术疗效。方法回顾性分析广州医科大学附属广州市妇女儿童医疗中心胃肠外科2015年8月至2021年1月收治的11例因良性胃流出道梗阻病变接受HMP患儿临床资料。其中4例为消化性溃疡致幽门或十二指肠球部狭窄;3例为胃壁肿物(2例为异位胰腺, 1例为胃重复畸形);2例为嗜酸性粒细胞性胃肠炎致幽门狭窄;1例为先天性肥厚性幽门狭窄幽门环肌切开术后幽门再梗阻;1例为化学性损伤致胃窦近幽门处狭窄。收集所有患儿年龄、手术情况及术后恢复情况。结果 11例患儿中, 男5例、女6例;平均年龄5岁1个月。均成功施行HMP, 其中9例行开放手术, 2例行腹腔镜手术。手术时间(162±92)min, 术中出血量(10±3)mL, 术后住院时间(12±4)d。11例共行14次手术。1例化学性损伤患儿出院时需半流质饮食, 其余10例恢复至正常饮食。术后随访(38±23)个月, 术后6个月随访有2例饱食后偶有轻微腹痛, 1例偶有胃潴留, 均经保守治疗好转;术后...     

儿童肾盏憩室的鉴别诊断与疗效分析

目的探讨儿童肾盏憩室的鉴别诊断与诊疗方法。方法回顾性分析本院泌尿外科2009年1月至2017年2月收治的44例肾囊性占位病变患儿临床资料,其中7例诊断为肾盏憩室,总结分析7例肾盏憩室患儿的影像学检查结果、治疗方法以及转归等情况。结果 7例患儿中,女性3例,男性4例;右侧5例,左侧2例;年龄4个月至10岁,平均年龄6.4岁。7例患儿中,3例因腹痛就诊,4例无临床症状,为体检时彩超发现。憩室直径1.4~6.2 cm,平均直径3.8 cm。4例患儿予随访观察,1例经抗感染治疗后腹痛缓解好转出院,2例予腹腔镜手术治疗。随访时间为3个月至8年。5例保守治疗患儿定期随访憩室大小,未见明显变化;2例腹腔镜手术患儿术后恢复良好,无并发症发生,憩室无复发。结论儿童肾盏憩室易与单纯性肾囊肿相混淆,CT增强后延迟扫描对诊断肾盏憩室有较高的检出率。临床应严格把握手术指证。     

Posterior fossa syndrome: identifiable risk factors and irreversible complications

Cerebellar mutism was first described by Rekate et al. in 1985 as a transient condition which occurs after posterior fossa operations in children. Posterior fossa syndrome (PFS) and cerebellar mutism are often used interchangeably in the literature. In our experience, we found cerebellar mutism to be a reversible component of a persistent neurologic syndrome. The cause and identifiable risk factors have not been clearly elucidated in the literature. To further characterize PFS, we reviewed 253 children with posterior fossa tumors who underwent surgical resection. We documented 20 cases of PFS (8%), 12 males and 8 females. Age ranged from 1.5 to 13 years (mean = 6.5). Of the 20, 16 were medulloblastoma, 3 ependymoma and 1 astrocytoma. There was a 21 % incidence (16/76) of PFS in medulloblastoma of the posterior fossa. The incidence for ependymoma was 13% (3/24) and 1% (1/102) for astrocytoma. All 20 cases (100%) had brainstem involvement by the tumor. The most frequent postoperative findings included mutism, ataxia, 6th and 7th nerve palsies and hemiparesis. Mutism had a latency range of 1-7 days (mean = 1.7) and a duration of 6-365 days (mean = 69.2, median = 35). Although mutism resolved in all cases, the remaining neurologic complications which characterized our findings of PFS were rarely reversible. We describe potential risk factors for developing PFS after surgery with hopes of making neurosurgeons more aware of potential problems following the removal of lesions in this area. Early recognition of PFS would further promote patient and family understanding and coping with this syndrome.     

Posterior fossa syndrome after a vermian stroke: a new case and review of the literature

The posterior fossa syndrome (PFS) is a well-known clinical consequence of posterior fossa surgery that has only been reported in a limited number of cases with a nontumoral etiology. It consists of transient cerebellar mutism, behavioral abnormalities and personality changes. We describe a 12-year-old child who developed transient cerebellar mutism associated with behavioral and emotional symptoms following rupture of a vermis arteriovenous malformation (AVM). Following the stroke, the girl experienced a 24-hour symptom-free interval. After that, she became mute and her emotional state was characterized by severe anxiety, irritability and withdrawal. After 3 days, mutism resolved and dysarthria became apparent. Two weeks after stroke, the AVM was surgically removed and the postoperative course was uneventful. This case is the first reported in which the PFS occurred after focal nonsurgically induced cerebellar damage.     

Cerebellar mutism after posterior fossa surgery

A large midline posterior fossa medulluloblastoma and a cerebellar arteriovenous malformation with associated clot were moved with postoperative mutism. Mutism was immediate and resolved into an ataxic dysarthria with residual mild dysarthria at 3 months. The literature is reviewed, and the clinicopathological and neurophysiological data of cerebellar function in speech are discussed. Acute injury to the midportion of the cerebellum with or without dentate nuclear involvement can cause a spectrum of speech disturbances. The more widespread the injury to the midportion of the cerebellum with dentate nuclei involvement the greater the risk of developing postoperative mutism.     

Posterior fossa dermoid cysts causing cerebellar abscesses

Dermoid cysts are uncommon tumors, and posterior fossa dermoid cysts may rarely cause abscess formation or formation of daughter abscesses within the cerebellum. At present, there are only 16 cases with posterior fossa dermoid cysts causing cerebellar abscesses reported in the literature. Two cases, 22 and 14 months old, with posterior fossa dermoid cysts and dermal sinus causing multiple cerebellar abscesses are reported. In the first one, there was also marked hydrocephalus. Retrospective examination of the patients revealed pinpoint-sized dimples in their suboccipital regions. Both patients were treated with antibiotics and underwent posterior fossa surgery. In the patient with marked hydrocephalus, ventriculoperitoneal shunting was performed after treatment of the infection. Both patients were neurologically normal, and there were no complaints, except a light learning difficulty in the patient with ventriculoperitoneal shunting, 133 and 34 months after surgery, respectively. Early detection of congenital dermal abnormalities along the craniospinal axis by routine examination of newborns is highly important before development of serious complications. Because surgery is the only effective treatment modality for these lesions, radical excision should be performed in all cases to avoid tumor recurrence. However, subtotal excision may be performed in selected cases, because the cyst capsule may adhere firmly to vital structures.     

MUTISM AFTER SURGICAL REMOVAL OF A CEREBELLAR TUMOR: Two Case Reports

The authors report two pediatric cases of transient mutism that occurred after surgical removal of a medulloblastoma and a pilocytic astrocytoma of the vermis and discuss the pathophysiology of this syndrome. Transient mutism has been described for the first time quite recently, even in cases where these tumors were also surgically removed before. Perhaps improvement in imaging and in surgical techniques made neurosurgeons more daring and some interventions that were judged impossible are routinely performed today. If this is the case, postoperative transient cerebellar mutism might be considered the price that must be paid in order to cure more patients with cerebellar tumors.     

Mutism after surgical removal of a cerebellar tumor: two case reports

The authors report two pediatric cases of transient mutism that occurred after surgical removal of a medulloblastoma and a pilocytic astrocytoma of the vermis and discuss the pathophysiology of this syndrome. Transient mutism has been described for the first time quite recently, even in cases where these tumors were also surgically removed before. Perhaps improvement in imaging and in surgical techniques made neurosurgeons more daring and some interventions that were judged impossible are routinely performed today. If this is the case, postoperative transient cerebellar mutism might be considered the price that must be paid in order to cure more patients with cerebellar tumors.     

Severe cerebellar mutism after posterior fossa tumor resection

Cerebellar mutism is an infrequent but important complication after posterior fossa surgery in children. Dysarthria, irritability and ataxia are among the signs and symptoms of this disorder, which are usually mild and self-limiting. However, in severe cases, there can be impairment of higher-level cognitive functions, affecting the child's future personal and social relations. This disorder has been described in many other situations and consequently pediatricians should be familiar with its symptoms, physiopathology, diagnosis, degrees of severity, treatment, and prognosis, since a multidisciplinary approach is required. We present the case of a 5-year-old boy who underwent surgery for a low-grade ependymoma in the fourth ventricle; 48 hours after surgical resection, the boy developed irritability, cranial nerve involvement and stereotyped movements in the context of active hydrocephalus. His symptoms progressively improved 6 weeks after the intervention. We review the literature on cerebellar mutism and discuss the physiopathology of this disorder, which seems to confirm that the cerebellum not only acts as a simple coordinator of motor function, but also plays an important role in higher-level cognitive functions, such as language.     

Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up

INTRODUCTION: Mutism after posterior fossa tumour resection is generally said to be transient. Our experience suggested that speech did not usually normalise, and that mutism was associated with neurologic deficits that did not recover fully. METHODS: Children with mutism after posterior fossa tumour resection, and alive more than 2 years post-operatively, were reviewed retrospectively. Charts were reviewed and parents contacted to ascertain details about mutism, associated neurologic deficits, and the most recent speech and neurologic status. RESULTS: There were 7 children, with follow-up ranging from 2.5 to 13.1 years (mean 6.8 years). Tumours were midline, with 4 astrocytomas and 3 medulloblastomas. Mutism was noted immediately after post-operative extubation in all patients. Speech reappeared 1-15 weeks post-operatively, except for 1 patient, who remained mute at 2.5 years. Speech returned to normal in only 1 patient. Mutism was always accompanied by new or worsened cerebellar ataxia, which resolved incompletely in the long term. Sixth nerve palsies occurred in 3 and recovered incompletely. Seventh nerve paresis occurred in 2 and recovered completely. CONCLUSION: Mutism after posterior fossa tumour resection is associated with other neurologic deficits, particularly ataxia. Whereas speech usually returns, contrary to general opinion, speech rarely normalises. Other associated deficits rarely resolve completely. These findings have significant implications for counselling of family and patients.     

Novel use of zolpidem in cerebellar mutism syndrome

Cerebellar mutism syndrome (CMS) is a complication of posterior fossa surgery seen primarily in pediatric patients after resection of medulloblastoma. CMS is characterized by mutism, ataxia, hypotonia, and irritability. Currently, there is no therapy of proven efficacy. Zolpidem, although primarily used as a sedative, has been shown to alleviate mutism and promote arousal in similar neurologic and psychiatric disorders. Here, we describe a child with severe CMS in whom zolpidem seemed to increase arousal, accelerate the resolution of mutism, and decrease emotional lability. Our report suggests that clinicians should consider using zolpidem for patients with CMS.     

Transient eye closure after posterior fossa tumor surgery in children.

Transient cerebellar eye closure following posterior fossa tumor surgery in children has recently been observed. This phenomenon is characterized by a transient inability to open the eyes postoperatively with complete resolution within 4-6 days. Presented are four cases of transient cerebellar eye closure after posterior fossa tumor surgery in children. Possible mechanisms are reviewed with regard to historical works of cerebellar stimulation in man, and anatomical structures and pathways which might be involved.