Secondary amenorrhea caused by hydrocephalus due to aqueductal stenosis : report of two cases

Amenorrhea is rarely presented as a manifestation of endocrinological disturbances in patients of chronic hydrocephalus. We describe two cases of secondary amenorrhea caused by hydrocephalus due to aqueductal stenosis. Two female patients of age 30 and 20 yr presented with amenorrhea and increasing headache. Magnetic resonance images revealed marked, noncommunicating hydrocephalus without any tumorous lesion. In one patient, emergent extraventricular drainage was necessary because of progressive neurological deterioration. Each patient underwent surgical intervention for the hydrocephalus-ventriculoperitoneal shunt and endoscopic third ventriculostomy. Both resumed normal menstruation continuing so far with further normal menstrual bleeding. These two cases and others reported in the literature indicated that the surgical intervention for hydrocephalus resolves amenorrhea in all the cases of amenorrhea due to hydrocephalus. The suspected role of the surgery is the correction of increased intracranial pressure, which is an important pathogenetic factor in the development of amenorrhea.     

Endoscopic ventricular anatomy

Neurosurgical endoscopy enables in situ exploration of the dilated ventricular system, primarily for therapeutic rather than diagnostic purposes. Ventriculocisternostomy in patients with obstructive hydrocephalus is the most widely performed endoscopic procedure. Perfect knowledge of the intraventricular anatomy is necessary for proper endoscopic navigation so the operator always recognizes the position of the endoscope and the anatomic structures encountered. Endoscopic anatomy in this situation is different from normal anatomy because of the hydrocephalus. Anatomic landmarks must be reassessed. We present here this "new" anatomy.     

Risk factors and outcomes of cerebrospinal fluid overdrainage in HIV-negative patients with cryptococcal meningitis after the ventriculoperitoneal shunting procedure

Purpose

Shunt procedures used to treat cryptococcal meningitis complicated with hydrocephalus and/or increased intracranial pressure (IICP) could result in cerebrospinal fluid (CSF) overdrainage, thereby presenting therapeutic challenges.

Utility of ventricular access in an acute deterioration after endoscopic third ventriculostomy

Endoscopic third ventriculostomy (ETV) has now been accepted widely as a safe procedure for treatment of non-communicating hydrocephalus. Despite its learning curve, most of the neurosurgeons have understood its technical details, benefits, and risks and have started to practice it to perfection. The benefit of shunt independence with minimal risks offers a remarkable advantage which has made this procedure widely popular. However, late closure of stoma leading to morbidity and even death has been reported off late. We report a case of a 7-year-old girl with hydrocephalus due to tectal glioma who deteriorated after 7 months following a successful procedure. She developed a cardio-respiratory arrest and was resuscitated with aspiration of cerebrospinal fluid from the ommaya reservoir kept during the primary surgery. Keeping all the patients under strict surveillance for stoma patency is mandatory and in addition, ommaya reservoir in certain high-risk patients may be a useful option for achieving quick ventricular access by medical and nonmedical personnel in case of deterioration. This case is the first reported case of acute deterioration after ETV from India. Previously, 14 such cases have been reported worldwide and only 2 of them have survived.     

A randomized trial of ceftriaxone versus trimethoprim-sulfamethoxazole to prevent ventriculoperitoneal shunt infection.

BACKGROUND AND PURPOSE: Shunt infection represents a particularly morbid condition, which can also result in mortality. In order to decrease the high morbidity and mortality rates, prevention is an essential step. The purpose of this study was to compare the prophylactic use of ceftriaxone and trimethoprim-sulfamethoxazole (SXT) for the prevention of ventriculoperitoneal (VP) shunt infection. METHODS: In this prospective, single-institution, randomized clinical trial, 107 children with hydrocephalus and an indication for shunting were randomly assigned to prophylaxis with ceftriaxone (n = 50) or SXT (55), each administered as a single dose during anesthesia and two divided doses postoperatively. Patients were followed up for at least one year. RESULTS: The mean age of patients was 15 months, and 85% were aged 6 months or younger. During the first postoperative year, meningitis occurred in 13.5% of patients receiving ceftriaxone and 14.5% of the SXT group, with no statistically significant difference between the groups. Younger age, presence of cerebrospinal fluid leakage and aqueductal stenosis as a cause of hydrocephalus showed significant correlation with meningitis occurrence on univariate analysis. However, only the latter 2 factors were associated with meningitis on multivariate analysis. The risk of shunt infection did not correlate with the gender of the patient, time of VP shunt surgery, or duration of hospitalization for shunting. CONCLUSION: Ceftriaxone and SXT showed similar efficacy in preventing shunt infection. Cerebrospinal fluid leakage before or after VP shunt placement and aqueductal stenosis were independent risk factors for meningitis after VP shunt.     

Marked calvarial thickening and dural changes following chronic ventricular shunting for shaken baby syndrome

We report the case of a 23-year-old woman who was abused at the age of 5 months. She suffered from complications frequently associated with shaken baby syndrome, such as hydrocephalus secondary to subarachnoid hemorrhage. The patient underwent a procedure to place a ventriculoperitoneal shunt 3 weeks after her presentation with signs of abuse. The ventricular shunt remained in place throughout her life, and the patient received multiple revisions. She also was noted to have a markedly thickened calvarium on both radiographs and computed tomographic scan at 6 years old. She died following an episode of grand mal status epilepticus. An autopsy was performed and her skull was found to be thickened circumferentially. Histologic examination revealed increased cancellous space with normal trabecular bone. It is hypothesized that intracranial hypotension resulting from chronic ventricular shunting lead to her thickened calvarium, a condition previously reported as hyperostosis cranii ex vacuo. Dural changes seen microscopically corroborate this hypothesis.     

Assessment of cerebrospinal fluid outflow resistance

The brain and the spinal cord are contained in a cavity and are surrounded by cerebrospinal fluid (CSF), which provides physical support for the brain and a cushion against external pressure. Hydrocephalus is a disease, associated with disturbances in the CSF dynamics, which can be surgically treated by inserting a shunt or third ventriculostomy. This review describes the physiological background, modeling and mathematics, and the investigational methods for determining the CSF dynamic properties, with specific focus on the CSF outflow resistance, R _out. A model of the cerebrospinal fluid dynamic system, with a pressure-independent R _out, a pressure-dependent compliance and a constant formation rate of CSF is widely accepted. Using mathematical expressions calculated from the model, along with active infusion of artificial CSF and observation of corresponding change in ICP allows measurements of CSF dynamics. Distinction between normal pressure hydrocephalus and differential diagnoses, prediction of clinical response to shunting and the possibility of assessment of shunt function in vivo are the three most important applications of infusion studies in clinical practice.     

神经内窥镜下侧脑室和第三脑室手术的应用解剖学研究

目的：研究神经内窥镜下侧脑室、第三脑室的解剖结构和影像学特点,为临床开展神经内窥镜脑室手术提供解剖学依据。方法：在10例成人尸头和25例脑积水第三脑室底造瘘手术病人神经内镜下观察侧脑室、三脑室在内镜下的解剖结构,并录象作记录和研究之用。结果：额角入路可以观察到侧脑室额角和侧脑室体部,且可以通过室间孔进入第三脑室。室间孔的Y形结构是脑室内观察和定位的重要标志。枕角、三角区和颞角入路分别可观察到侧脑室相应部位和脉络丛。结论：侧脑室额角入路是观察侧脑室和三脑室的最常用入路,观察侧脑室范围最大,也是第三脑室底造瘘的最佳入路,枕角入路便于对侧脑室三角区和侧脑室体后部病变的观察和治疗,三角区和颞角入路很少用到．     

脑室腹腔分流装置流量测算方法

目的 提供一种预算患脑室腹腔分流装置实际流量的方法；方法 根据流体力学的相似原理，首先测定水通过患拟使用的分流装置的压力－流量关系，然后利用腰椎穿刺测定患颅内压及脑脊液粘度；利用流体力学相似准则推导公式，计算出脑脊液实际流量；结果 准确测算在一定体温、颅内压、脑脊液粘度的条件下脑脊液在分流装置中的流量，为术前选择分流装置及术后控制分流量提供客观依据；结论 此测算方法为进一步研究脑脊液粘度、蛋白含量、流量之间关系提供方法；并在一定条件下指导分流术前选管和术后分流管的管理。     

Functioning periods and complications of 246 cerebrospinal fluid shunting procedures in 208 children.

To investigate the functioning periods and the causes of cerebrospinal fluid shunt failures, 246 shunts inserted in 208 children from October 1985 to August 1992 at the authors'' institute were retrospectively reviewed. The mean age at shunt insertion was 4.0 years and the reasons for the shunting procedures were congenital hydrocephalus (47.6% per procedure), tumor-associated hydrocephalus (21.1%), postmeningitic hydrocephalus (11.8%), congenital cyst (10.6%) and others (8.9%). All except 2 were shunted to the peritoneal cavity. Forty-five operations for shunt failure were done (18.3%) during the follow-up period (mean, 32 months). The functioning (shunt survival) rates at 6, 12, 24 and 36 months after surgery were 91.6%, 86.6%, 83.6% and 82.9%, respectively. The complications were mechanical malfunction (12.2%), infection (4.1%), subdural fluid collection which required drainage (1.6%) and migration (0.4%). About half of the mechanical malfunctions and infections which had occurred during the follow-up period were within 6 months and 2 months after surgery, respectively. There was no shunt-related mortality. These chronological data should be considered in the planning of follow-up schedules.     

Growth and development in thanatophoric dysplasia

Two cases of prolonged survival of thanatophoric dysplasia are presented, in which ventilatory support was initiated in the neonatal period because of respiratory distress. Both patients required a ventriculoperitoneal shunt for hydrocephalus and had decompression of the posterior fossa. The history of each patient has been characterized by profound developmental delay and dramatic growth failure.     

Clinical and bacteriological features of relapsing shunt-associated meningitis due toAcinetobacter baumannii

A case is reported of a patient who developedAcinetobacter baumannii meningitis ten days after a ventriculoperitoneal shunt had been placed for control of elevated intracranial pressure. Intravenous antimicrobial therapy was instituted with imipenem and tobramycin after shunt removal and insertion of a ventriculostomy tube. Following the placement of a ventriculoatrial shunt, clinical and microbiological relapse occurred that was eventually cured after complete removal of the ventricular drainage system and a second course of systemic antibiotics. Relapse was confirmed by modern molecular typing techniques including plasmid DNA fingerprinting and analysis of total genomic DNA by pulsed-field gel electrophoresis.     

Recurrent ventriculoperitoneal shunt pseudocyst in a nine-year-old girl

Summary A case of a ventriculoperitoneal shunt pseudocyst in a 9-year-old girl diagnosed using computed tomography is reported. Two attempts at relocation of the shunt failed to prevent reaccumulation of cerebrospinal fluid. A ventriculoatrial shunt was then performed with a successful outcome. Such pseudocysts should be part of the differential diagnosis of abdominal processes in patients with ventriculoperitoneal shunts. If relocation of the shunt outside the pseudocyst is unsuccessful, a ventriculoatrial shunt is generally indicated.     

Choroid plexus hyperplasia and chromosome 9p gains

Choroid plexus hyperplasia leading to communicating hydrocephalus is a rare disorder with only 24 patients reported so far in the literature. Furthermore, genetic information is only available for six of these cases: In one patient the condition was associated with trisomy 9p, in one patient with trisomy 9 mosaicism and in three patients with tetrasomy 9p. Here, we describe four additional patients with choroid plexus hyperplasia leading to various levels of hydrocephalus, and gain of the entire chromosome 9p region: Three with trisomy 9p and one with tetrasomy 9p. The three patients with trisomy 9p were siblings. Normal karyotypes were identified in the lymphocytes of the parents. Likely one of the parents is a mosaic for a cell line with trisomy 9p in the gonads. We demonstrate the importance of correctly diagnosing choroid plexus hyperplasia as the cause of hydrocephalus in patients with chromosome 9p gain since ventriculoperitoneal shunting is likely to fail due to intolerable formation of ascites.     

Immune reactions associated with silicone-based ventriculo-peritoneal shunt malfunctions in children

The implantation of ventriculo-peritoneal (VP) shunting systems is the most commonly performed neurological procedure in children with hydrocephalus. Although the overall complication risk is low, the cumulative risk of shunt failure is high and unfortunately results in a high prevalence of revision surgeries. In this study, we explored the concept that some pediatric patients may develop an immune response to either the proteins attached to the silicone implant surface or to the biomaterial itself, and that this reaction may contribute to VP shunt failure in some individuals. The data displays that the sterile shunt malfunction group had a higher rate of protein deposition and increased levels of autoantibodies to the extracted surface proteins as compared to individuals with functioning shunting systems. The precise nature of the shunt-bound proteins that serve as antigens in this experiment have not yet been determined. The data also indicated that some individuals develop antibodies to polymeric substances that cross-react with partially polymerized acrylamide. The detection of significant amounts of shunt-bound protein, antibody responses to these proteins and to polymeric substances suggest that an immunological response to these proteins may play a role in the mechanism behind sterile shunt malfunctions.     

“Anemone' Cell (Villiform) Tumor of the Brain

At age 31, the patient developed hydrocephalus secondary to a brain lesion that had been present for 12 years. A ventriculoperitoneal shunt was performed, but a stereotactic biopsy did not establish a definitive diagnosis at that time. At age 34 the patient experienced spontaneous intratumoral and ventricular hemorrhage, which prompted radical surgical resection of the lesion. Histologic and im-munocytochemical findings established a diagnosis of ependymoma; review of the ultrastructure of the tumor from the biopsy performed when the patient was 31 years old revealed the characteristics of an “anemone” cell neoplasm.     

持续腰大池置管脑脊液压力检测对Tap试验阴性iNPH患者的诊疗价值

目的探讨腰大池置管持续测定脑脊液压力对腰椎穿刺脑脊液放液试验(Tap试验)阴性的特发性正常压力脑积水(iNPH)患者的诊疗价值。方法选择82例Tap试验阴性的iNPH患者作为研究对象,持续12 h行腰大池置管脑脊液压力测定后行脑室—腹腔分流术。根据颅内压波幅与颅内压相关系数(RAP)值分为2组,RAP≥0.6的34例患者为高RAP组,RAP<0.6的48例患者为低RAP组。比较2组手术前后无障碍直线折返计时行走测试(TUGT)时间、简易精神评价测量表(MMSR)评分和排尿评分,评估2组患者步态不稳、认知障碍、排尿障碍改善率及治疗效果。结果术后1个月,高RAP组TUGT时间较术前及低RAP组明显缩短,MMSR评分较术前及低RAP组明显提高,排尿评分较术前及低RAP组明显降低,差异有统计学意义(P<0.05)。高RAP组术后1个月步态不稳、认知障碍、排尿障碍改善率高于低RAP组,差异有统计学意义(P<0.05)。高RAP组患者脑室—腹腔分流术后疗效总有效率高于低RAP组,差异有统计学意义(P<0.05)。结论Tap试验阴性的iNPH患者经持续12 h腰大池置管脑脊液压力测定再筛查,以RAP≥0.6作为行脑室—腹腔分流术的指标具有较高的诊疗价值。     

Infection of central nervous system by motile Enterococcus: first case report

A 66-year-old man with four indwelling ventriculoperitoneal shunts for multiloculated hydrocephalus from a complicated case of meningitis a year before developed shunt infection based on a syndrome of fever, drowsiness, and cerebrospinal fluid neutrophil pleocytosis in the background of repeated surgical manipulation to relieve successive shunt blockages. The cerebrospinal fluid culture, which yielded a motile Enterococcus species, was believed to originate from the gut. This isolate was lost in storage and could not be characterized further. The patient improved with vancomycin and high-dose ampicillin therapy. He relapsed a month later with Enterococcus gallinarum shunt infection, which responded to high-dose ampicillin and gentamicin therapy. This is probably the first case report of motile Enterococcus infection of the central nervous system.     

Abdominal actinomycosis associated with a sigmoid colon perforation in a patient with a ventriculoperitoneal shunt

Abdominal actinomycosis causing hydronephrosis in a patient with a ventriculoperitoneal shunt is very rare. A 27- year-old female patient was admitted complaining of lower abdominal pain. She had undergone ventriculoperitoneal shunt surgery 10 years ago. Abdominal Ultrasonography and a CT scan demonstrated an inflammatory mass in the lower left quadrant of the abdomen causing obstructive hydroureter and hydronephrosis. Laparotomy revealed a diffusely infiltrating mass involving the small bowel, mesentery, and sigmoid colon, and a 1cm perforation in the sigmoid colon. Actinomycosis was diagnosed upon histological examination. After treatment with antibiotics and surgery, the patient's condition improved.     

不同手术路径脑室-腹腔分流术对小儿脑积水的临床疗效分析

目的比较经侧脑室额角穿刺和经侧脑室枕角穿刺行脑室-腹腔分流术对小儿脑积水的临床疗效。方法回顾性分析徐州医科大学附属医院2014年5月至2018年6月收治的58例诊断为脑积水并行脑室-腹腔分流术患儿的临床资料,根据手术路径不同分为额角穿刺组(32例)和枕角穿刺组(26例)。比较2组患儿术后常见并发症的发生情况;术后随访1年,按照格拉斯哥昏迷评分(GCS)评价手术疗效。结果与枕角穿刺组相比,额角穿刺组术后并发症较少,堵管发生率较低,差异有统计学意义(P<0.05)。术后1年,2组患儿GCS评分明显高于术前,差异具有统计学意义(P<0.05)。结论与经侧脑室枕角穿刺比较,经侧脑室额角穿刺路径的脑室-腹腔分流术可降低相关术后并发症的发生率,其临床疗效优于经枕角穿刺的分流术式。