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.     

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.     

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.     

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.     

Intracranial symptomatic giant arachnoid cyst of the interhemispheric fissure presenting with frontal lobe syndrome

Symptomatic interhemispheric arachnoid cysts (ACs) are extremely rare lesions seen more frequently in the elderly. Only 13 cases of symptomatic interhemispheric ACs in the elderly have previously been reported. Herein, we describe a 70-year-old female with symptoms of vertigo, memory and behavior disturbances. Magnetic resonance imaging (MRI) revealed a giant arachnoid cyst (AC) on the interhemispheric fissure, which was exerting a mass effect and displacement on the corpus callosum and anterior cerebral vessels, with a massive midline shift. The patient was treated with a cysto-peritoneal shunt. Reports to date have reviewed the pathology, clinical presentation, radiology and surgical management of interhemispheric symptomatic arachnoid cysts in elderly patients.     

Surgical treatment of middle cranial fossa arachnoid cyst in children with atypical symptoms--report of three cases

We report three cases of symptomatic arachnoid cyst in the middle cranial fossa who presented with atypical symptoms including speech delay, vomiting and personality changes. They were improved by a cyst-peritoneal shunt operation. In many patients with a small or middle sized arachnoid cyst in the middle cranial fossa, their symptom tends to be so trivial that surgery is not the choice of treatment. The present cases suggest that peculiar symptoms caused by a limbic system lesion may be improved by treating the arachnoid cyst in the middle cranial fossa.     

Brain glucose utilisation in acquired childhood aphasia associated with a sylvian arachnoid cyst: recovery after shunting as demonstrated by PET.

Regional brain glucose utilisation was investigated with PET and fluorodeoxyglucose (FDG) in a case of epileptic aphasia (Landau-Kleffner syndrome) associated with a left sylvian arachnoid cyst. CT and MRI had failed to disclose any mass effect of the cyst on surrounding brain structures. Sequential metabolic measurements showed a comparable pronounced hypometabolism in cortical regions around the cyst, involving speech areas, and suggested mild but chronic compression of the developing brain. After placement of a cyst-peritoneal shunt system, significant metabolic improvement occurred in all cortical regions, especially the inferior frontal gyrus and the perisylvian area, with predominant residual deficit in the left superior temporal gyrus. These findings were correlated with a pronounced increase in word fluency and slower progress in verbal auditory comprehension. This report suggests that PET is able to evaluate the functional disturbances associated with expanding arachnoid cysts, and to follow the neurological improvement after drainage.     

Symptomatic, expansive middle cranial fossa arachnoid cyst treated by a cysto-peritoneal shunt Case report and evaluation of treatment options

We report a symptomatic space-occupying arachnoid cyst in a 16-year-old boy. During the last two years, he was frequently absent from school because of headaches. Due to symptoms of nausea, fever and headache the patient was primarily referred to a surgical unit, where appendectomy was performed, revealing a normal appendix. Postoperatively, the patient complained of severe headache. Computed tomography (CT) of the head demonstrated a large left-sided middle cranial fossa arachnoid cyst. The patient was operated with insertion of a cysto-peritoneal shunt. A total regress of the clinical symptoms was seen at follow-up examination 3 months after shunting.     

The treatment of large supratentorial arachnoid cysts in infants with cyst-peritoneal shunting and Hakim programmable valve

OBJECTIVE: This retrospective case series examines 7 infants with large supratentorial arachnoid cysts who underwent cyst-peritoneal shunting and insertion of a Hakim programmable valve. Comparing pre- and postoperative clinical data, neuroradiological and regional cerebral blood flow (rCBF) findings we evaluated the efficacy of the surgical procedure. METHODS: Infants, ranging in age from 1 to 55 days (mean age 29.5 days), were assessed pre- and postoperatively by neurological examination, developmental profile and neuroimaging. RESULTS: Post procedure, all patients showed a significant reduction in the cyst/brain ratio on neuroimaging (p<0.001), 6 had a normal developmental profile (p<0.001) and 5 cases showed a significant amelioration of clinical symptoms and neurological signs. Two patients underwent preoperative SPECT scans, which showed hypoperfusion in the area surrounding the cyst; this decreased rCBF also improved post shunting. CONCLUSIONS: Large supratentorial arachnoid cysts in infants can be successfully treated with cyst-peritoneal shunting and insertion of a Hakim programmable valve. This is the first study specifically aimed at evaluating the long-term results of these conditions.     

Treatment of a pontine neuroepithelial cyst by placement of a cystocisternal grommet

We describe an 8-year-old girl who presented with cranial nerve compression due to a brainstem cyst adjacent to the fourth ventricle and describe the first documented insertion of a grommet to form a conduit between a neuroepithelial cyst and ventricle. The patient presented with diplopia and headaches and was found to have the cystic lesion in the right pons. The patient underwent craniotomy, aspiration and fenestration with subsequent recurrence 8 months later. Definitive treatment involved insertion of a grommet. Surgical treatment of symptomatic neuroepithelial cysts can achieve full resolution of neurological deficits. Insertion of a grommet, as distinct from a shunt or fenestration procedure, has the potential to provide long-term resolution of these symptoms without recurrence.     

可调压式分流管治疗脑积水

目的探讨使用可调压式分流管行脑室-腹腔分流术治疗脑积水的临床效果。方法采用可调压式分流管行脑室.腹腔分流术治疗脑积水18例。其中高压性脑积水6例;等压性脑积水10例;等压性脑积水伴穿通畸形2例。术前根据患者头颅CT及MRI提示脑积水类型设定阀门压力,术中测量颅内压并根据分流管末端滴速重新调整阀门压力。术后一周复查头颅CT了解脑积水缓解情况。出院后随访1-11月,根据头颅CT所示脑积水缓解情况再行阀门压力调整。结果脑积水症状缓解16例(88.9%);症状缓解不满意2例(11.1%)。18例均无出血、颅内感染及死亡。结论使用可调压式分流管行脑室一腹腔分流术,可在术前、术中、术后调整阀门压力,缓解脑积水症状,分流效果优于传统不可调压式分流管。     

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.     

Endoscopic treatment of suprasellar and third ventricle-related arachnoid cysts

OBJECTIVE: To evaluate the role of neuroendoscopy in the treatment of rare suprasellar and other third ventricle-related arachnoid cysts. METHODS: A review of supratentorial midline arachnoid cysts treated by endoscopic fenestration. RESULTS: Ten cases were identified. The median age of these ten patients was 7.5 years. In three a cysto- or ventriculoperitoneal shunt had previously been inserted. Six cysts were located in the suprasellar/prepontine area, one was a combined suprasellar/middle fossa cyst, and three were complex cysts arising mainly in the quadrigeminal cistern and extending into the third ventricle. Successful endoscopic fenestration to both the ventricular system and the basal cisterns ("bipolar" fenestration) was achieved in all cases. In seven cases a cystoventriculostomy and cystocisternostomy was performed, while in three a cystoventriculostomy was combined with a third ventriculostomy, achieving additional communication to the subarachnoid space. The previously inserted shunts were removed at the end of the procedure. The median follow-up period was 17 (range 1-30) months. The clinical outcome was excellent in all cases, despite only marginal reduction in the cyst size and persistence of ventriculomegaly in some cases. No further treatment to the cyst was required during the period of follow-up. CONCLUSION: Third ventricle-related arachnoid cysts can be satisfactorily and safely treated by endoscopy. This approach leaves the patient shunt independent.     

Evaluation of the management of arachnoid cyst of the posterior fossa in pediatric population: experience over 27 years

Introduction The necessity of surgical treatment of the arachnoid cyst in general and posterior fossa arachnoid cyst (PFAC) in particular is sometimes controversial. Surgery is warranted in symptomatic patients. In this study, we evaluated our experience throughout 27 years in the management of patients with PFAC. Materials and methods The study was designed with special emphasis on clinical features, surgery, and outcome. We investigated a total of 112 arachnoid cysts. Of them, 23 patients were symptomatic for PFAC. We assessed clinical characteristic and outcome for all patients. We obtained information from medical records and outpatient charts on age, sex, presenting symptoms, associated abnormalities, psychomotor status, modality of treatment, complications and follow-up in 23 patients (9 boys, 14 girls, ages 1 day–6 years) who had been admitted for evaluation and treatment of PFAC at Kobe Children’s Hospital between 1978 and 2004. Results The mean follow-up period was 93 months (range 5–313 months). More than half of the patients had a history of increased head size and signs of intracranial pressure as presenting symptoms. One-third of the patients had associated abnormalities and six patients (26%) presented hydrocephalus. Computed tomography cisternography displayed delayed filling of the cyst in 11 patients (48%). All patients were treated surgically; the total number of surgical procedures was 55 with a surgical rate of 2.4 per patients. The marsupialization and marsupialization with cyst-peritoneal (C-P) shunt were the most common open surgical procedure in 14 and 5 patients, respectively. Endoscopic cysternostomy was performed in three patients. In our series, 25 (45%) of 55 surgical procedures included shunt malfunction and removal. The marsupialization of the cyst wall was successful in 9 of 14 operated patients (64%); the other five patients needed additional C-P or ventriculoperitoneal (V-P) shunt. Marsupialization with cyst-peritoneal shunt was successful in only one of five patients (20%), and for the other four patients, additional C-P or V-P shunt was necessary. No mortality is reported. Eight patients presented minor surgical morbidities that were resolved conservatively. Discussion The relatively good results using marsupialization or endoscopic cisternostomy as surgical procedure and the high incidence of shunt malfunction buttresses our use of both operations as a first-line surgery at present. Surgical procedure that does not include shunting decreases the ratio of recurrent operation because this kind of complication develops over time and should be consider as a second-line procedure.     

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。     

Electroconvulsive Therapy in a Depressed Patient with an Intracranial Arachnoid Cyst: A Brain Magnetic Resonance Imaging Study

We describe a depressed man with an asymptomatic arachnoid cyst of the left sylvian fissure who was successfully treated with ECT. Magnetic resonance imaging of the brain before and after the course of therapy revealed no change in the cyst nor any injury to the surrounding brain parenchyma.     

蛛网膜下腔顺向交通术治疗颅内蛛网膜囊肿

目的探讨颅内蛛网膜囊肿(intracranial arachnoid cyst,IAC)的有效治疗方法。方法采用蛛网膜下腔顺向交通术治疗IAC29例,并进行分析。结果28例患者术后临床症状消失或缓解,术后3~12月复查CT或MRI,囊肿消失或明显缩小;1例枕大池蛛网膜囊肿,术后1.5年囊肿未见缩小,临床症状无明显改善,改行囊肿-腹腔分流术,治愈。结论蛛网膜下腔顺向交通术是目前治疗IAC的有效方法,手术应采用显微神经外科技术。     

Pseudotumor syndrome in treated arachnoid cysts

We report three patients with arachnoid cysts treated by cyst-peritoneal shunting in whom intracranial hypertension occurred during episodes of shunt malfunction. In one case this was associated with re-expansion of the arachnoid cyst, whilst in the other two cases this did not occur. The similarities between these two cases and patients with pseudotumor cerebri suggest a common pathogenic mechanism — specifically, a disturbance of the cerebrospinal fluid circulation.     

Hypothalamic hamartoma associated with an arachnoid cyst.

A hypothalamic hamartoma associated with an arachnoid cyst in an 8-year-old boy is reported herein. He presented with precocious puberty, and neuroimaging studies demonstrated a solid mass in the prepontine cistern and a huge arachnoid cyst in the left cranial fossa. The mass appeared isointense to the surrounding cerebral cortex on T1-weighted magnetic resonance images, hyperintense on T2-weighted images, and was not enhanced after administration of Gd-DTPA. The patient underwent a left frontotemporal craniotomy and a cyst-peritoneal shunt was inserted. Histological features of the cyst wall and the mass were characteristic of an arachnoid cyst and hamartoma, respectively. While a hypothalamic hamartoma associated with an arachnoid cyst is rare, such a case may help clarify the geneses of both anomalous lesions.     

Primary intracranial arachnoidal cysts

Sixty-seven cases (41 males and 26 females) of arachnoidal cysts in children under 11 years are reported. About 53% of cases were diagnosed before 1 year of life. Thirty-one (42.2%) were supratentorial (interhemispheric 9, temporal fossa 10, convexity 5, sylvian fissure 3, supra- and/or retrosellar 4); 31 (46.2%) infratentorial (supra- and/or retrocerebellar 22, foramen of Magendie 3, quadrigeminal cistern 5, pontocerebellar 1); 5 (7.5%) supra-and infratentorial. Macrocephaly was the presenting symptom in 48 cases (71.5%). Associated features were frequent: cranial asymmetry in 24; aqueductal stenosis in 10; agenesis of corpus callosum in 8; deficient cerebellar lobullation in 4; Chiari I malformation in 2; neurofibromatosis type 1 with dysgenetic zones of the brain in 1; arteriovenous malformation in 1. Diagnosis was made at autopsy in six cases in the days before computed tomography and magnetic resonance: three patients had a cyst in the supra-and retrocerebellar midline; two had a cyst in the quadrigeminal cistern and the sixth was a rare case with the cyst passing from the posterior fossa to the left lateral ventricle through a hole in the basal surface of the brain. Small and some middle-sized cysts were not treated. Big and some middle-sized cysts were usually treated by cysto- and/or ventriculoperitoneal shunts. Arachnoidal cysts of the quadrigeminal cistern usually present with aqueductal stenosis and have to be treated with ventriculoperitoneal shunt. Craniotomy and fenestration of the cysts were performed in some cases with good results. The average mental level of these children is usually moderately low.