Slit ventricle syndrome after cyst-peritoneal shunting for temporal arachnoid cyst in children--a clinical entity difficult to detect on neuroimaging study

Slit ventricle syndrome, known to occur from malfunction of the shunt procedure for hydrocephalus, is reported after cyst-peritoneal shunt for temporal arachnoid cyst. Two children aged 12 and 10 years, who underwent cyst-peritoneal shunting for a large temporal arachnoid cyst at the age of 10 and 5 years, respectively, recently experienced several episodes of severe headache. Prior to admission, repeated CT scans did not reveal any morphological change in either of these two patients. Evidence of high intracranial pressure by lumbar tap revealed shunt malfunction. Both patients became free of neurological complaints and deficits after shunt revision. Despite elevated intracranial pressure due to shunt malfunction, neuroimaging studies showed no morphological changes in slit ventricle syndrome. Delay in both the diagnosis and prompt treatment may result in complete loss of visual acuity and even death. It is important to suspect this complication in patients with persistent elevated intracranial pressure symptoms and signs after any shunting procedure, regardless of unchanged neuroimaging studies. Once this is suspected, lumbar tap may be necessary and the choice of treatment is shunt revision.     

CSF overdrainage in shunted intracranial arachnoid cysts: a series and review

Background  Cerebrospinal fluid (CSF) overdrainage in hydrocephalus is well recognized. Overshunting following cysto-peritoneal (CP) drainage in patients with arachnoid cysts (AC) is rarely documented. Aim  We report five patients with acquired Chiari malformation I and three with posterior fossa overcrowding due to excessive CSF drainage in shunted intracranial ACs. We review our observations and discuss the current knowledge on the pathogenesis and management of this complication. Patients and methods  The medical records of the eight patients were analyzed in regard to clinical manifestation, cyst and shunt characteristics, management, and outcomes. Results  Mean age of the patients was 5.5 years. After an average interval of 5 years, five patients developed symptoms related to hindbrain herniation and three to severe shunt overdrainage following CP shunting. Several management modalities were utilized that achieved a good result in seven instances. Discussion  Some shunted ACs may evolve with overdrainage syndromes. Posterior fossa overcrowding and tonsillar herniation constitute their most severe forms. CSF hypotension, bone changes, venous engorgement, and probably cerebral chronic edema at the posterior fossa constitute the main factors involved in the pathogenesis of this entity. We also review previous instances of acquired Chiari malformation originating after AC shunting. Conclusions  Posterior fossa overcrowding and acquired Chiari I malformation can develop after excessive CSF drainage of intracranial ACs. Overshunting manifestations require prompt recognition and management. Preventive measures consist of making a stringent selection of cases being considered for surgery, avoiding CP drainage, and placing of a programmable valve as initial treatment of intracranial ACs if shunting is considered.     

神经内镜手术治疗颅内蛛网膜囊肿

目的 探讨神经内镜手术治疗颅内蛛网膜囊肿的手术指征、手术方法和预后。方法 回顾性分析26例采用神经内镜手术治疗的颅内蛛网膜囊肿，其中采用神经内镜下囊肿脑池造瘘和囊壁部分剥除18例，囊肿次全切除3例，囊肿脑室造瘘5例(1例尚行脑室腹腔分流术)。术后随访3个月-2年。结果 术前有症状的20例患者中，症状消失11例，改善7例，无变化2例，术后不能改善的症状主要是癫痫。有随访影像学复查结果的16例患者中，囊肿消失6例，缩小7例，无变化3例。结论 神经内镜手术是治疗颅内蛛网膜囊肿有效和微创的方法。     

Slit ventricle syndrome after cyst-peritoneal shunting for the treatment of intracranial arachnoid cyst

Serious complications following installation of a cyst-peritoneal shunt in an 8-year-old boy with asymptomatic arachnoid cyst in the middle cranial fossa are reported. Highly elevated intracranial pressure without association of ventriculomegaly seen in this patient indicates slit ventricle syndrome caused by malfunction of the cyst-peritoneal shunt. This phenomenon is worth recognizing as a possible consequence of cyst-peritoneal shunting for the treatment of intracranial arachnoid cyst.     

Endoscopic management of quadrigeminal arachnoid cysts

Objective  Quadrigeminal cistern arachnoid cysts are rare lesions, accounting for 5% to 10% of all intracranial arachnoid cysts and 9% of all supratentorial localizations. We reviewed the patients with quadrigeminal arachnoid cyst (QAC) who were treated with neuroendoscopic intervention. Materials and methods  Seventeen patients with QAC had been operated on between 2000 and 2007 in our institution. Four patients had undergone shunting prior to neuroendoscopic surgery. There were nine girls and seven boys with age ranging from 7 days to 17 years (mean, 40 months). All patients had hydrocephalus. A wide ventriculocystostomy (VC) and endoscopic third ventriculostomy were performed by using rigid neuroendoscopes. An aqueductal stent was also placed in two of the patients. Psychometric evaluation was administered postoperatively when possible. Follow-up of the patients ranged from 6 to 96 months (mean, 51.8 months). Results  Of the 17 patients, 12 underwent endoscopic procedure as the primary surgery. Five patients had been previously shunted. Macrocrania and psychomotor retardation were the main symptom and sign in all infants with QAC. Older children presented with the symptoms and signs of intracranial hypertension. Of the eight patients who were 6 months old or younger, only one did not need a ventriculo-peritoneal (VP) shunt. Endoscopic procedures were successful in all patients older than 6 months of age (P = 0.005). Conclusion  The patients presenting in their infancy had a psychomotor retardation, and all patients except for one, younger than 6 months of age, needed a VP shunt. Neuroendoscopic procedure is effective particularly in the patients with QAC older than 6 months of age. Presented in part at the 34th Annual Meeting of International Society for Pediatric Neurosurgery, 10–14 September 2006, Taipei, Taiwan.     

儿童蛛网膜囊肿-腹腔分流术后继发裂隙脑室综合征的治疗

目的 探讨颞叶蛛网膜囊肿行囊肿-腹腔分流术后出现的裂隙脑室综合征的早期诊断依据,合理有效的治疗方法以及预防这种并发症的措施.方法 回顾性分析研究了经过本文通信作者治疗的6例颞叶蛛网膜囊肿行囊肿-腹腔分流术后出现裂隙脑室综合征患儿的影像学资料、治疗经过和治疗结果.结果 男4例,女2例.平均发病年龄57.5个月.治疗方法为使用可调压力的抗虹吸分流管泵更换原有分流管泵或联合使用脑室-腹腔分流.6例患者临床症状完全消失,平均随访30个月,未见症状复发.结论 使用可调压力的抗虹吸分流管泵更换原有分流管泵或联合脑室-腹腔分流术对于裂隙脑室综合征是一种有效的治疗措施.避免使用低压的无抗虹吸作用的分流管能够有效地预防蛛网膜囊肿-腹腔分流术后出现裂隙脑室综合征.

Abstract：

Objective To investigate early diagnosis evidences, optimal therapeutic strategies and the prophylactic methods of the slit - ventricle syndrome(SVS) in the temporal lobe arachnoid cysts patients who received the cyst - peritoneal(CP) shunting.Method Six cases of SVS in the temporal lobe arachnoid cysts patients who received the CP shunting were treated by the senior author from Jan 2005 to Jan 2009.The radiological data, treatment process and therapeutic results were reviewed retrospectively.Results There were 4 male and 2 female patients.The mean age of presentation was 57.5 months.The final treatment was to change the original shunt with the programmable valve or combined with the ventricular - peritoneal(VP)shunting.All the symptoms of the patients disappeared totally, and the mean follow - up duration was 30mouths.Conclusions To use programmable shunts to substitute for the original shunt or combined with inserting a VP shunt is an optimal therapeutic method for SVS.The use of low - pressure shunts to treat the ararchoid systs should be abandoned unless dictated by specific indications.     

外侧裂蛛网膜囊肿手术方式分析

目的 探讨外侧裂蛛网膜囊肿手术方式的选择.方法 南方医科大学南方医院神经外科自2003年3月至2008年8月共采用手术治疗外侧裂蛛网膜囊肿患者87例,按手术方式(单纯内镜下手术40例、内镜控制下手术19例、显微镜下囊肿切除术22例)的不同将患者分为单纯内镜组、内镜控制组和显微镜组(分流及混合手术患者6例未计入),回顾性分析患者的临床资料并比较上述3种手术方式的疗效和并发症的发生率.结果 3组患者的年龄、囊肿大小、术后并发症、病情好转和囊肿缩小情况差异无统计学意义(P>0.05),而显微镜组患者的手术时间[(143±36.0)min]、术中出血[(160±39.6)mL]均高于其它2组,差异有统计学意义(P<0.05),单纯内镜组[(97±26.8)min,(15±4.8)mL]和内镜控制组患者[(87±27.6)min,(18±5.7)mL]的手术时间和术中出血量差异无统计学意义(P>0.05).结论 内镜下手术治疗外侧裂蛛网膜囊肿所需时间短,术中出血少,优于显微镜开颅手术.     

Familial intracranial arachnoid cysts

Three siblings with intracranial arachnoid cysts are described, two males and one female. One of the males has symmetric, bilateral, temporoparietal convexity cysts, and the others have singular, unilateral cysts. Three additional siblings in the family and other known relatives are clinically unaffected. As far as we know, this is the second reported case of familial intracranial arachnoid cysts and the first involving three siblings. The significance of these cysts and a review of the literature are presented.     

Congenital arachnoid cyst of the lateral ventricles in children

The authors report a series of three children with symptomatic congenital arachnoid cyst of the lateral ventricles. Presenting symptoms consisted of macrocephally, delay in psychomotor development, and seizures. CT findings were of a well-defined cystic lesion placed in the atrium of the lateral ventricle. One child was treated by direct cyst exposure and cysto-peritoneal shunt. The other two were treated with ventriculo-cystoperitoneal shunts; in one of these, we used a ventriculoscope both for cyst fenestration and for accurate shunt placement. The origin of intraventricular arachnoid cysts seems to be secondary to the displacement of arachnoid cells by the vascular mesenchyma, through the choroid fissure, during the process of choroid plexus development.     

颅内蛛网膜囊肿临床诊断和手术治疗

报告２１例颅内蛛网膜囊肿（ＩＡＣ），对其临床表现、诊断、手术适应证、手术方法选择和术中注意事项进行了讨论。ＩＡＣ约占颅内占位性病变的０．８７％，最常见于外侧裂池。认为：ＣＴ、ＭＲＩ是最可靠的诊断方法。凡囊肿有颅内高压者，充分建立囊腔与周围脑池或腹腔之间的交通是手术成功的关键。     

Proteus syndrome with syringohydromyelia and arachnoid cyst

Introduction Proteus syndrome (PS) is a rarely seen, sporadic disorder with a wide range of abnormalities including asymmetrical overgrowth, skin lesions, dysregulated adipose tissue, and vascular malformations. Brain and spinal malformations are extremely rare; syringohydromyelia and arachnoid cyst have not been reported previously. Materials and methods We present a 5-year-old girl with PS having severe central nervous system (CNS) abnormalities demonstrated by magnetic resonance imaging (MRI) including craniocutaneous lipomatosis, hemimegalencephaly, arachnoid cyst, and syringohydromyelia. A commentary on this paper is available at doi: .     

颅内蛛网膜囊肿的手术方法研究

目的 探讨颅内蛛网膜囊肿的手术治疗方法 . 方法 回顾性分析68例手术治疗的颅内蛛网膜囊肿患者的临床资料,根据手术方式不同,分为4组:囊肿切除术17例(A组).囊肿-脑池沟通术25例(B组),囊肿-腹腔分流术20例(C组),脑立体定向手术6例(D组),对4种手术方式的疗效进行比较. 结果 术后随访3月以上,59例患者症状消失或改善,有效率86.8%.CT证实囊肿消失或缩小者51例(75.0%).术后发生颅内积气2例、颅内出血3例、伤口感染2例.不同术式比较:影像学有效率A组为11/17(64.7%),B组为21/25(84.0%),C组为15/20(75.0%),D组为4/6(66.7%);症状改善率为A组12/17(70.6%),B组22/25(88.0%),C组19/20(95.0%),D组6/6(100%).A组7例患者术后头痛,发生比例较高. 结论 对颅内蛛网膜囊肿应慎重选择手术适应证和手术时机.从安全性和微侵袭角度,绝大多病例应首选囊肿-腹腔分流术.     

A case of intraventricular arachnoid cyst

A rare case of arachnoid cyst in the trigone of a lateral ventricle is reported. The patient was an 8-year-old boy who had had four episodes of convulsions prior to admission. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a cystic lesion containing fluid resembling cerebrospinal fluid. Although he received a cyst-peritoneal shunt, the lesion did not decrease in size. Direct removal of the cyst was then scheduled. The entirc cyst was finally removed, although it was firmly attached to the choroid plexus. The enlarged trigone gradually decreased in postoperative CT. The effectiveness of a cyst-peritoneal shunt is not always satisfactory. We recommend total resection of the cyst without use of a shunt system.     

大脑半球凸面蛛网膜囊肿的内镜手术治疗

目的 探讨神经内镜手术治疗大脑半球凸面蛛网膜囊肿的经验并评价治疗效果.方法 回顾性分析首都医科大学附属北京天坛医院神经外科自2007年9月至2011年7月行神经内镜囊肿-脑室或囊肿-脑池造瘘治疗的13例大脑半球凸面蛛网膜囊肿(均选择邻近脑室或脑池的大脑半球凸面蛛网膜囊肿)患者的临床资料,包括临床症状、影像表现、手术适应证、手术方法、手术并发症以及临床、影像随访结果. 结果 13例患者术后囊肿都有不同程度缩小;术前症状消失9例、改善4例,症状消失或改善率100％;术后发生无症状硬脑膜下积液1例. 结论 神经内镜手术损伤小、效果好,可以作为邻近脑室或脑池的大脑半球凸面蛛网膜囊肿的首选治疗方法.     

颅内蛛网膜囊肿-腹腔分流术后疗效长期随访分析

目的 探讨囊肿-腹腔分流术治疗儿童颅内蛛网膜囊肿的疗效.方法 回顾我院2003年1月至2005年1月62例囊肿分流手术治疗颅内蛛网膜囊肿患者,随访其CT及MRI结果,对其疗效进行了长期随访.结果 62例囊肿中46例(74％)位于侧裂,8例(13％)位于大脑凸面,8例(13％)位于幕下.所有患者接受了蛛网膜囊肿-腹腔分流术.术后平均随访时间6.5年(6-8年),59例患者影像资料显示囊肿大部消失或完全消失,占95％.与分流手术相关的并发症有16例,其中8例出现良性颅高压症状.结论 囊肿-腹腔分流术是一种简单有效、安全的治疗方法,但应严格选择其手术适应证,对术后可能的并发症也应予以充分的重视.     

Posttraumatic intraventricular arachnoid cyst accompanied by pseudomeningoencephalocele in a child

Growing skull fracture (GSF) is a rare complication of head trauma. A posttraumatic intraventricular arachnoid cyst (AC), neither isolated nor accompanied by a GSF has not been reported previously. A seven-year-old girl was admitted after a severe head injury with a separated right parieto-occipital fracture and contusion. She responded well to conservative therapy. Seven weeks after discharge, she was re-admitted with a large parieto-occipital pseudomeningoencephalocele due to herniation of cerebrospinal fluid and neural tissue to the subgaleal space through the widened fracture defect, an extra-axial cyst at the posterior interhemispheric space and an intraventricular cystic mass. She underwent open surgery, and the intraventricular cystic mass was totally removed. The histological findings were consistent with an AC. One week after dural repair, hydrocephalus developed, and a ventriculo-peritoneal shunt was inserted. She did well during two-year follow-up. The present case is unique as an intraventricular AC following head trauma. When an intraventricular cystic lesion is encountered after severe head trauma, the possibility of an AC should be considered; especially with neighboring contused neural tissue and leptomeningeal cyst formation.     

Slit ventricle syndrome successfully treated by a lumboperitoneal shunt

The authors present the case of a 17-year-old man with slit ventricle syndrome, presenting as progressive neurological deterioration after head trauma. Serial computed tomography scans revealed slight ventricular enlargement, suggesting shunt malfunction. Communication between the lumbar subarachnoid space and the lateral ventricles was confirmed by computed tomography cisternography. He underwent a lumboperitoneal shunt, resulting in complete resolution of the symptoms. A lumboperitoneal shunt is considered to be a promising option for the treatment of slit ventricle syndrome. [Neurol Res 1995; 17; 440-442]     

神经内镜治疗颅内蛛网膜囊肿108例临床分析

目的 探讨神经内镜治疗颅内蛛网膜囊肿的效果,为蛛网膜囊肿的治疗提供参考.方法 回顾性分析1998年5月至2010年5月使用神经内镜治疗的108例颅内蛛网膜囊肿患者的临床资料,总结并分析手术适应证的把握及手术效果.结果 108例均成功行造瘘术.短期内症状好转68例(63%,68/108),其中头痛、恶心及呕吐症状缓解40例(95%,40/42);年龄小于12岁74例中症状好转48例(65%,48/74),其余34例中症状好转20例(59%,20/34).术后1周影像学结果示术前合并脑积水16例均明显缓解.手术并发症包括发热、硬膜下积液、皮下积液、动眼神经麻痹、颅内感染,除1例死于术后颅内感染外,其余经处理后治愈出院.94例随访3月,影像学检查示蛛网膜囊肿消失或缩小61例(64.9%,61/94),无变化33例(35.1%,33/94);临床症状较术前改善54例(57.4%,54/94),其中癫痫好转8例(44.4%,8/18);86例术后随访半年,影像学检查示蛛网膜囊肿造瘘术无效46例(53.5%,46/86).结论 神经内镜治疗颅内蛛网膜囊肿长期有效率不高,需严格把握手术适应证.     

神经导航辅助内镜治疗侧脑室蛛网膜囊肿

目的运用神经导航辅助内镜技术手术治疗侧脑室蛛网膜囊肿，探讨两者结合应用对开展神经内镜技术治疗脑室深部病变的意义。方法对10例蛛网膜囊肿病人运用神经导航制定手术计划，设计理想的手术切口和入路，实时、动态地监测内镜的操作轨迹。结果本组均获得良好的治疗效果，内镜下行蛛网膜囊肿开窗术6例；切除术4例，其中次全切除3例，部分切除1例。无出血、感染及神经功能缺失等并发症。结论内镜手术治疗侧脑室内蛛网膜囊肿具有创伤小、疗效好等优点，结合神经导航技术，可以弥补病理情况下脑室内局部解剖变异影响内镜操作定位困难的缺陷，使脑室内复杂手术的精确性更高、更有效和安全。