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.     

Development of a middle fossa arachnoid cyst A theory on its pathogenesis

The progression of congenital arachnoid cysts has seldom been documented. We report the case of a child who was diagnosed with arrested hydrocephalus at the age of 13 months. Neuroimaging studies performed when the girl was 22 months old showed the appearance of an arachnoid cyst in the right middle fossa, while the previously enlarged ventricles seemed to have decreased in size. To the best of our knowledge, the paradoxical expansion of an arachnoid pouch following a reduction in the size of the ventricular system has not previously been documented. We advance the hypothesis that the development of some cases of arachnoid cyst might be pathogenically related to impaired CSF dynamics associated with pre-existing hydrocephalus. We also briefly review the pertinent literature on the formation and evolution of congenital cerebral arachnoid cysts. Received: 4 September 1998     

The use of an adjustable valve to treat over-drainage of a cyst-peritoneal shunt in a child with a large sylvian fissure arachnoid cyst

Introduction The cyst-peritoneal shunt is a recognised surgical alternative in the management of sylvian fissure arachnoid cysts. Shunt overdrainage is well described in literature on ventriculo-peritoneal shunts, but not often appreciated as a complication of cysto-peritoneal shunts.Case report A 5-year-old boy presented with a symptomatic left sylvian fissure arachnoid cyst. This was initially treated by craniotomy and membrane fenestration in the carotid cistern. Recurrence led to insertion of a valveless cyst-peritoneal shunt 5 months later. Initial progress was followed by persistent headaches 18 months after shunt insertion. CT scan revealed a significant reduction in the cyst size, enlargement of the ipsilateral lateral ventricle, collapse of the contra-lateral ventricle and midline shift towards the side of the shunt. These findings were interpreted as over-drainage of the cyst-peritoneal shunt.Result A Codman Medos adjustable valve was inserted, with the intention of gradually increasing the pressure until the midline shift was restored and the contra-lateral ventricle was reconstituted. This was achieved with the valve set at 90 mm H₂O, verified by CT scan. Radiological improvement was associated with dramatic symptomatic improvement.Conclusion Over-drainage of cyst-peritoneal shunts is often not appreciated, especially when the main manifestation is headaches. As it is difficult to predict the required valve pressure setting, it may be advisable to consider the use of an adjustable valve.The material of this paper was presented as a poster at the 31st Annual Meeting of the International Society for Pediatric Neurosurgery, Monaco, 14–18 September 2003     

Huge arachnoid cyst in the posterior fossa: controversial discussion for selection of the surgical approach

Case report We report a case of a huge posterior fossa arachnoid cyst with hydrocephalus. This 15-month-old girl presented with macrocrania and psychomotor retardation. Magnetic resonance (MR) imaging demonstrated ventriculomegaly and a huge cystic lesion in the posterior fossa. A membranectomy was performed microscopically and the patient had a good postoperative course.Outcome Magnetic resonance imaging 1 year after the operation revealed adequate reduction in the size of the ventricle and cyst. Progressive macrocrania discontinued and normal development was obtained.     

Spontaneous disappearance of middle fossa arachnoid cyst after head injury

A case of middle fossa arachnoid cyst is presented, which disappeared after head injury. Five days after the trauma, CT scan revealed subdural fluid collection in addition to an arachnoid cyst in the middle fossa on the same side. As the subdural fluid resorbed, the cyst became smaller and disappeared on the follow-up CT scans without surgical intervention.     

Iatrogenic intradural arachnoid cyst following tethered cord release in a child

Iatrogenic arachnoid cysts represent uncommon complications of intradural spinal procedures. Here we present the case of a 7-year-old girl who was found to have a symptomatic, pathologically proven, intradural arachnoid cyst 3 years following tethered cord release. The patient originally presented with abnormal urodynamics testing and was found to have fatty infiltration in her filum terminale. She underwent sectioning of the filum terminale without complications. The patient presented 3 years later with pain and neurogenic claudication. The patient was successfully treated with subarachnoid cyst fenestration with resolution of her bilateral lower extremity pain. Spinal intradural arachnoid cysts represent an important, though rare, postoperative complication of dural opening.     

蛛网膜囊肿合并慢性硬膜下血肿的治疗及临床分析

目的探讨蛛网膜囊肿合并慢性硬膜下血肿的临床特点及治疗方法。方法采用回顾性病例研究方法,对安徽省立医院神经外科2009年1月至2014年1月所收治的9例确诊为蛛网膜囊肿合并慢性硬膜下血肿患者进行分析,其中男6例,女3例,年龄1.5~60岁,平均23.9岁。临床表现为头痛患者8例;其中6例伴恶心、呕吐,1例伴有肢体无力,1例仅有肢体抽搐;有明确外伤史6例,无明确外伤史3例。结果9例患者中5例行颅骨钻孔硬膜下血肿引流术,5例患者术后均恢复良好,后出院;另2例行开颅硬膜下血肿清除并蛛网膜囊肿切除术,术后痊愈。2例患者行保守治疗,一月后复查头颅CT提示血肿较前缩小,患者无症状,未手术。9例患者分别随访10个月至4年,未再复发硬膜下血肿,生活质量可。结论蛛网膜囊肿合并慢性硬膜下血肿,常见于有头部外伤史的年轻患者。有明显临床症状者需手术治疗,并根据血肿量及是否合并囊内出血等,选择单纯做硬膜下血肿钻孔引流术还是同时开颅清除血肿并处理囊肿。     

Posttraumatic intradiploic arachnoid cyst of the posterior fossa

We report the case of a 5-year-old girl with an enlarging suboccipital mass, a posttraumatic intraosseous arachnoid cyst. Diagnostic work-up revealed that the lesion consisted of an intradiploic arachnoid cyst and an extra-axial occipital pouch that communicated by way of an osseous and dural defect. Surgical repair was undertaken with good results. A search of the current literature has shown only seven previous reports of leptomeningeal cysts situated at the occipital bones, most of them the result of an antecedent skull fracture. A pathogenetic hypothesis is presented comparing the growth of arachnoid intraosseous cysts and the development of meningocencephaloceles. Received: 1 October 1996     

Bobble-head doll syndrome due to a suprasellar arachnoid cyst: endoscopic treatment in two cases

Object We report two cases of bobble-head doll syndrome associated with a large suprasellar arachnoid cyst successfully treated with a minimally invasive endoscopic approach.Methods The clinical history, surgical treatment and results of two children, a 9-year-old boy and a 1-year-old girl, both presenting the clinical features of the bobble-head doll syndrome, are described. As a first procedure, a ventriculo-cystostomy was endoscopically performed in both patients, obtaining either resolution of the symptoms or notable cyst reduction. In the girl, a re-closure of the stoma, with cyst re-expansion, was observed after 18 months. She then underwent a second procedure, a ventriculo-cysto-cisternostomy, with a good result. After 3 years follow-up, the neurological condition of both patients remains good with complete resolution of abnormal head movement.Conclusion In our opinion, endoscopic treatment is the procedure of choice for this condition, as it involves few complications and gives good results.     

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.     

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.     

Acetazolamide and corticosteroid therapy in complicated arachnoid cyst

Case report We report on the successful conservative treatment of a child harbouring a right-temporal arachnoid cyst associated with bilateral hygroma.Treatment Medical treatment was by corticosteroids for several days and acetazolamide (15 mg/kg/die) for 10 months.Outcome Clinical symptoms and signs disappeared completely with partial resolution of the bilateral hygroma at MRI follow-up.     

Epilepsy associated with a cerebellar arachnoid cyst: seizure control following fenestration of the cyst

Objective The role of the cerebellum in the pathogenesis of seizures remains controversial. Cerebellar origin of seizures, albeit rare, has been described in the literature in association with intrinsic lesions of the cerebellum. We present a unique case of a patient with medically intractable, secondary generalized epilepsy, associated with a superior cerebellar quadrigeminal arachnoid cyst. Clinical presentation A 9-year-old child presented with medically refractory secondary generalized epilepsy associated with recurrent headaches since 6 months of age. The child also had moderate intellectual impairment and autism. On the magnetic resonance imaging (MRI) of the head, he was noticed to have a small superior cerebellar arachnoid cyst in the quadrigeminal area that had increased in size slightly. Interictal electroencephalograph (EEG) was unable to localize the site of the epilepsy. Neurological examination was unremarkable. Intervention A suboccipital craniotomy and supracerebellar infratentorial approach to the cyst was performed at 9 years of age. Intraoperative electrocorticography (ECOG) demonstrated epileptic activity from the cerebellar tissue adjacent to the cyst. The cyst was fenestrated, and the cyst wall was sent for histology. Seizure control improved dramatically after fenestration of the cyst. Conclusion This case provides strong evidence that, albeit rare, the cerebellum may be a source of epileptic activity due to compression by a lesion in the posterior fossa. Hence, in cases with intractable epilepsy of unknown supratentorial source, the differential diagnosis should include a posterior fossa lesion. The finding of a posterior fossa lesion in such cases, even if it is small and appears benign, should precipitate a discussion about the possible relationship between the posterior fossa lesion and the epilepsy.     

凝血酶体外诱导模拟脑室出血后大鼠蛛网膜细胞纤维化的实验研究

目的观察凝血酶(thrombin)刺激体外培养大鼠蛛网膜细胞后纤维化程度的变化,探讨其与脑室出血(intraventricular hemorrhage,IVH)后大鼠慢性脑积水形成的关系。方法采用SD大鼠蛛网膜细胞,经不同浓度凝血酶刺激,在体外模拟脑室出血(IVH)后慢性脑积水大鼠的蛛网膜细胞纤维化的病理生理过程。细胞免疫化学法(immunocytochemistry,ICC)鉴定传代培养的SD大鼠蛛网膜细胞标志物细胞角蛋白(CK 8/18)和桥粒蛋白(Desmoplakin)的表达。qRT-PCR与Western Blot检测细胞纤维化因子(Col-I、Col-III、TGF-β1、α-SMA)的表达,观察其纤维化程度,同时检测比较各组细胞的表达差异。结果 (1)培养的蛛网膜细胞标志物Cytokeratin和Desmoplakin表达阳性。(2)凝血酶可诱导蛛网膜细胞纤维化,在50u/ml浓度下,蛛网膜细胞纤维化程度最明显。(3)实验组较对照组细胞纤维化程度明显。结论体外可以稳定培养大鼠蛛网膜细胞。凝血酶诱导的蛛网膜细胞各种纤维化指标明显高于正常组,提示纤维化模型构建成功。构建成功的纤维化蛛网膜细胞有利于下一步深入研究脑室出血后慢性脑积水的机制。     

Choroid plexus cyst of the left lateral ventricle with intermittent blockage of the foramen of Monro, and initial invagination into the III ventricle in a child

A cyst of the choroid plexus of the left lateral ventricle with intermittent blockage of the foramen of Monro and initially with invagination of the III ventricle in a child is described. In a 6-week-old boy a ventriculoatrial shunt was implanted for correction of an active asymmetrical hydrocephalus of unknown origin. When he was 3 months of age a water-soluble contrast CT ventriculography revealed a noncolloid cyst localised predominantly in the upper portion of the III ventricle. At that time the ventricular catheter obstructed with choroid plexus was removed; new bilateral catheters in a parieto-occipital region were implanted. In the course of the next 4 years, first the atrial catheter had to be extracted and then the peritoneal catheter was changed, in both cases because of obstruction. Periods of normal life alternated with periods of transient and intermittent symptoms of increased intracranial pressure, papilloedema, and myoclonic jerks. Repeated computed tomography (CT) and magnetic resonance imaging (MRI) showed stabilised hydrocephalus with an enlarged left lateral ventricle. When the boy was 16 years old MRI revealed a choroid plexus cyst in the left lateral ventricle 2 cm in diameter, with a ball-valve type of obstruction of the foramen of Monro. CT stereoendoscopic resection of the wall of a large cyst filled with cerebrospinal fluid was performed, and two additional adnexal small cysts were coagulated using the bipolar coagulator, Diomed 25 laser and scissors; the symptoms then regressed, except for superior bilateral altitudinal anopsia. Light and electron microscopy of the cyst wall is reported. The cyst was composed of collagenic connective tissue lined with a basal lamina lacking in epithelial cells. The preoperative and postoperative MRI are presented. Choroid plexus cysts localised in the anterior part of lateral ventricles are very rare, and all reported cases have been in male patients. According to the literature our case is only the third ever described in a child. Received: 9 March 1998 Revised: 12 May 1998     

Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus

Introduction The bobble head doll syndrome, mainly affecting children under 10 years of age, is a rare and surgically treatable movement disorder characterized by head bobbing occurring at a rate of 2–3 times/s. Its pathophysiological mechanism is not well known but two main factors are commonly associated with the condition: a dilatation of the third ventricle and, more frequently, a cystic lesion rather than a solid mass in the region of the third ventricle.Illustrative case The illustrative case concerns a child with a third ventricular cystic lesion and hydrocephalus who had experienced abnormal head movements since the age of 1 year as well as ataxia and tremor of the arms. Contrast cranial MRI, at the age of 3, demonstrated enlargement of the third and lateral ventricles, a ballooned cyst inside the third ventricle with compression of all the diencephalic structures, a funnel dilation of the cranial part of the aqueduct, and a cyst in the septum pellucidum. A ventriculoperitoneal shunt (Hakim-Cordis) was placed and the head bobbing, tremor of the arms, and ataxia disappeared immediately.Conclusion The good and immediate clinical result in our case emphasizes the opinion that the reduction of CSF pressure is the best therapeutical option in the bobble head doll syndrome.     

凝血酶体外诱导模拟脑室出血后大鼠蛛网膜细胞纤维化的实验研究

目的 观察凝血酶（thrombin）刺激体外培养大鼠蛛网膜细胞后纤维化程度的变化，探讨其与脑室出血（intraventricular hemorrhage，IVH）后大鼠慢性脑积水形成的关系。方法 采用SD大鼠蛛网膜细胞，经不同浓度凝血酶刺激，在体外模拟脑室出血（IVH）后慢性脑积水大鼠的蛛网膜细胞纤维化的病理生理过程。细胞免疫化学法（immunocytochemistry ，ICC）鉴定传代培养的SD大鼠蛛网膜细胞标志物细胞角蛋白（CK 8/18）和桥粒蛋白（Desmoplakin）的表达。qRT-PCR与Western Blot检测细胞纤维化因子（Col-I、Col-III、TGF-β1、α-SMA）的表达，观察其纤维化程度，同时检测比较各组细胞的表达差异。结果 （1）培养的蛛网膜细胞标志物Cytokeratin和Desmoplakin表达阳性。（2）凝血酶可诱导蛛网膜细胞纤维化，在50u/ml浓度下，蛛网膜细胞纤维化程度最明显。（3）实验组较对照组细胞纤维化程度明显。结论 体外可以稳定培养大鼠蛛网膜细胞。凝血酶诱导的蛛网膜细胞各种纤维化指标明显高于正常组，提示纤维化模型构建成功。构建成功的纤维化蛛网膜细胞有利于下一步深入研究脑室出血后慢性脑积水的机制。     

Neurocognitive and psychological profiles in pediatric arachnoid cyst

Objective  This study aims to investigate whether intracranial arachnoid cysts (AC) compromise neurocognitive function and psychological profiles in pediatric patients, depending on various clinical factors. Methods  We assessed neurocognitive functions and psychological tests in 35 AC patients and 35 healthy control subjects between October 2007 and April 2008. AC patients ranged in age from 3 to 15 (7.94 ± 3.12) years old and control patients from 5 to 13 (8.84 ± 2.17) years old. The location of the AC were temporal (n = 22), frontal (n = 6), suprasellar (n = 4), and posterior fossa (n = 3). Patients underwent neurocognitive and psychological assessments before surgery. To investigate which AC impair neurocognitive function and psychological profile, we assessed intelligence, memory, attention, executive function, behavioral problems, emotional distress, and parenting stress. Results  AC caused some demonstrated impairment by both neurocognitive function and psychological assessments. Left hemisphere AC tended to have more anxiety; mood changes can be detected depending on cyst grade. An incidental finding of AC after trauma is more intelligent, well-reserved executive function. Frontal locations tended to cause more anxiety than temporal locations. Conclusions  Our results imply that intracranial AC impairs some neurocognitive and psychological functions. An incidental finding of AC after trauma was a more intelligent, well-reserved executive function. AC in the left hemisphere, frontal location tended to cause more anxiety. The AC itself did not cause differences in neurocognitive function from the control group. However, parenting stress in the AC group was much higher than in the control group.