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.     

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.     

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.     

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.     

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]     

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     

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: .     

Short-term dexamethasone treatment for symptomatic slit ventricle syndrome

Objective The objective was to report our positive experience of using dexamethasone to treat 13 patients with symptomatic slit ventricle syndrome (SVS).Methods Thirteen SVS patients who received dexamethasone during acute episodes were studied. The etiology for hydrocephalus was prematurity and intraventricular hemorrhage in 9 patients and neonatal meningitis, chorioamnionitis, Dandy–Walker variant, and congenital in 1 case each. The shunt was inserted at 1.8±1.0 months of age and SVS was diagnosed at 4.9±3.2 years of age.Results All patients reported relief and shorter duration of symptoms with dexamethasone. Surgical intervention was decided upon and carried out within 11±8 months of SVS diagnosis in 9 out of 13 patients. The other 4 are being monitored and continue to receive dexamethasone when needed.Conclusions Dexamethasone appears to be a useful treatment in acutely increased intracranial pressure caused by SVS. It can provide temporary relief during the decision-making process of whether and when to perform surgery.A commentary on this paper is available at     

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.     

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.     

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.     

Cranial disjunction and visual failure in a slit ventricle syndrome with patent shunt

A 6.5-year-old child who received a shunt at 3 weeks of age for triventricular hydrocephalus related to his congenital toxoplasmosis developed symtoms of intracranial hypertension and papilloedema. Computed tomographic scan demonstrated slit ventricles. The shunt device was shown to be patent on isotope transit study. Spontaneously the cranial sutures widened and headaches disappeared, but loss of vision occurred and did not reverse despite optic nerve sheath fenestration. We suspect that a rapid drop in intracranial pressure played a role in the pathogenesis of our patient's blindness. This possible complication should be taken into account when calvarial expansion is planned in a patient with an intracranial hypertension syndrome with papilloedema in the presence of slit ventricles and a patent shunt.     

以慢性硬膜下血肿为首诊的儿童颅内蛛网膜囊肿五例并文献复习

目的 探讨儿童颅内蛛网膜囊肿相关性的慢性硬膜下血肿的病因机制及治疗方法.方法 回顾性分析福建医科大学附属第一医院2006年1月至2012年4月5例以慢性硬膜下血肿为首诊患儿的临床资料,男4例,女1例,年龄4 ～11岁,平均7.2岁,均以典型颅高压表现首诊,其中1例并发癫痫,另1例并发健侧肢体无力;发病前3个月内明确颅脑损伤史1例,无明确外伤史4例.结果 5例患儿术后颅高压及脑神经功能损害症状均消失.4例张力性囊肿的患儿,血肿均在3d内引流干净,最短为术后1d.其中3例于术后3周-2个月内二期行囊肿一腹腔分流术,术后随访半年-2年,血肿无复发,蛛网膜囊肿均近全消失;而另1例合并囊肿腔内血肿的患儿引流6d后血肿大部消失,症状好转,术后4个月CT复查发现囊肿消失.结论 颅内蛛网膜囊肿是儿童发生慢性硬膜下血肿的重要病因;硬膜下血肿的形成可能是机体为修复蛛网膜囊肿壁裂口而启动一系列局部炎性反应促动过程的“副产品”;治疗上首选颅骨钻孔血肿外引流术.     

Intracranial pressure monitoring using a programmable pressure valve and a telemetric intracranial pressure sensor in a case of slit ventricle syndrome after multiple shunt revisions

A case of hydrocephalus showing slit ventricle syndrome after multiple shunt revisions was treated with a programmable pressure valve, and intracranial pressure was monitored with a telemetric sensor. High pressure setting produced positive and normal values of intracranial pressure in this patient. We report our experiences.     

The influence of the semax nootropic agent on the behavioral signs of the withdrawal syndrome and on the craving for alcohol in white rats and the determination of its clinical efficacy and safety in patients with alcohol dependence

The influence of a course of medical treatment with the Semax nootropic peptide (50 and 200 μg/kg) on alcohol motivation and general behavior was studied in rats preliminarily alcoholized over six months. Semax was also included in the scheme of complex therapy of human alcohol dependence in clinical practice. Experiments with rats showed an increase in the locomotor and exploratory activity of rats and a decrease in the degree of depressive-like components. This effect compensated the behavioral changes observed in the control group exposed to alcohol deprivation. At the same time, the behavior of test groups under anxiogenic conditions showed increased anxiety. Semax in a dose of 200 μg/kg caused a significant increase in alcohol motivation, whereas its lower dose induced no proalcohol effect. Clinical trials demonstrated the safety (the absence of side effects and fairly high tolerance) of Semax in the course of alleviating the acute withdrawal syndrome and its efficacy for the recovery of mnestic functions (which was similar to the efficacy of Pyracetam). Semax did not change the frequency of cases of pathological craving for alcohol, but increased the overall intensity of behavioral reactions, which made the craving for alcohol stronger. These results allowed determination of safe course doses of Semax (to 50 μg/kg) under conditions of chronic alcoholization and inclusion of this nootropic agent in a scheme of complex therapy for patients with alcohol dependence. The mechanisms underlying the individual differences in the effects of Semax should be studied further. Original Russian Text ? M.L. Lovat’, M.A. Vinnikova, N.A. Kozyreva, E.A. Kushnir, R.D. Platonova, I.P. Ashmarin, 2008, published in Neirokhimiya, 2008, Vol. 25, Nos. 1–2, pp. 49–56