Pneumocephalus associated with benign brain tumor: report of two cases

Two cases of spontaneous pneumocephalus, which developed after ventriculoperitoneal shunt procedures for severe hydrocephalus caused by benign brain tumors, are reported. In both cases there was no previous history of cerebrospinal fluid leakage. Operation revealed that both patients had many small defects of the dura mater and the bone in the middle cranial fossa, which were plugged by necrotic brain tissue. These defects were remote from the sites of the original tumors but may have been produced by long-standing raised intracranial pressure, and they presumably allowed air to enter after intracranial pressure was reduced by shunting. Repair of the defects prevented further intracranial air retention.     

Pneumocephalus following ventriculoperitoneal shunt. Case report.

The authors describe a case of massive pneumocephalus following ventriculoperitoneal shunting for hydrocephalus. After multiple diagnostic and surgical procedures, congenital defects in the tegmen tympani of both temporal bones were identified as the sources for entry of air. A functioning shunt intermittently established negative intracranial pressure and allowed ingress of air through these abnormalities; when the shunt was occluded, air did not enter the skull, and there was no cerebrospinal fluid leakage. Repair of these middle ear defects prevented further recurrence of pneumocephalus.     

Ventriculosagittal sinus shunt placement: technical case report

OBJECTIVE AND IMPORTANCE: Determining an appropriate site for distal catheter placement for ventricular shunting for some hydrocephalic patients can be difficult. We describe a simplification of the technique for sagittal sinus shunt placement using a guidewire. CLINICAL PRESENTATION: A 20-month-old infant with hydrocephalus secondary to Alexander's disease developed erosion of her parieto-occipital ventriculoperitoneal shunt reservoir through an occipital decubitus scalp ulceration. Her hydrocephalus was temporarily treated with a ventriculostomy; however, she developed pneumatosis intestinalis while in the hospital. TECHNIQUE: The patient underwent placement of a ventriculosagittal sinus shunt. The ventricular catheter and shunt valve were placed through a burr hole at Kocher's point, and the distal end of the catheter was placed in the superior sagittal sinus by using the Seldinger technique. CONCLUSION: Ventriculosagittal sinus shunting may be used as an alternative to traditional methods for patients for whom distal shunt placement is problematic. Our technique has the theoretical advantage of reducing the risks of blood loss or air embolism by not requiring a scalpel incision into the sinus.     

Normal pressure hydrocephalus in children and young adults

Normal pressure hydrocephalus (NPH) is generally considered to be a disorder of adult and geriatric patients. We report four patients who are children or young adults with chronic neurological disorders, recent deterioration of their levels of function, normal cerebrospinal fluid (CSF) pressures, and ventricular enlargement. All four patients improved after the placement or revision of a ventriculoperitoneal shunt. Frequent symptoms and signs included irritability (three patients), vomiting (three patients), and abnormal limb posturing (two patients). Correct diagnosis was hampered by two factors: (a) Multiple or prolonged recordings of CSF pressures were invariably well within the normal ranges with respect to age, and (b) the patients had chronic neurological deficits. After ventriculoperitoneal shunting, subjective and objective improvement was seen in all cases. Young patients with large ventricles may benefit from shunting procedures despite low CSF pressures. These patients may be clinically identified by symptoms of new neurological dysfunction in cases of previously static neurological disease or acceleration of slowly progressive neurological dysfunction. Some of these underlying neurological disorders may predispose children and young adults to NPH.     

Treatment of cerebrospinal fluid rhinorrhea by percutaneous lumboperitoneal shunting: review of 15 cases

Fifteen patients with recalcitrant cerebrospinal fluid (CSF) fistula underwent the insertion of a lumboperitoneal shunt. The shunt consists of a two-piece Silastic tube and has been used in a population of 150 patients with communicating hydrocephalus, persistent postoperative meningocele, and benign intracranial hypertension. The spinal catheter is introduced subcutaneously and no flushing device is used. We studied three groups: 9 patients had a history of head trauma, and 7 of these had undergone one or several ineffective direct approaches to the dural leak. Four patients presented with a presumably congenital fistula. Two patients had persistent rhinorrhea due to previous intracranial procedures. Indium-111 cisternography was performed in 10 patients before lumboperitonel (LP) shunting and failed in 2 of those to document the site of leakage. Twelve patients showed cessation of rhinorrhea after LP shunting. In 4 of these, shunt-related complications responded to shunt removal with no further recurrence of rhinorrhea. Two patients underwent revision of the shunt. In 3 patients, the LP shunt failed to control the CSF leak and further intracranial procedures were indicated. The LP shunt provides an attractive and technically simple solution when direct methods of treatment have failed. Additionally, LP shunting should be considered as a primary mode of treatment in elderly patients or when impairment of CSF dynamics is documented by radionuclide cisternography and computed tomographic scanning. When an LP shunt is ineffective, shunt function should be checked by isotopic studies before additional surgery is performed.     

Extreme sensitivity of hearing to decreases of ICP in Menière’s disease

We report the case of a Menière’s disease patient affected by normal pressure hydrocephalus (NPH) who presented a cerebrospinal fluid (CSF) pressure-dependent hearing impairment after shunting. This side-effect was not only reversible and reproducible but occurred at a high opening pressure when the valve setting was lowered by only 0.7 mmHg (10 mmH₂O). This observation suggests that hearing in Menière’s disease might be very sensitive to small reductions of intracranial pressure (ICP) and that these patients should be informed of this potential risk, which can compromise the efficacy of the shunt.     

Shunt migration into the sphenoid sinus: case report

OBJECTIVE AND IMPORTANCE: Shunt catheter migration is a potential complication of cerebrospinal fluid shunting procedures. We report an unusual case of proximal shunt migration into the sphenoid sinus. To our knowledge, there have been no previous reports of shunt migration through the bony structures of the cranial base. CLINICAL PRESENTATION: A 41-year-old man who had had a cyst-to-peritoneum shunt placed 21 years earlier for a temporal lobe arachnoid cyst presented with cerebrospinal fluid rhinorrhea. Neuroradiological imaging revealed migration of the shunt catheter through the medial wall of the middle temporal fossa into the sphenoid sinus. INTERVENTION: The patient underwent shunt removal and repair of the dural defect. Intraoperatively, the proximal catheter tip was found in the sphenoid sinus with dural and bony erosion. The patient made an uneventful recovery. CONCLUSION: We present a unique long-term complication associated with intracranial shunt catheters. We hypothesize that excessive proximal catheter length and chronic cerebrospinal fluid pulsations contributed to migration of the catheter into the sphenoid sinus.     

A novel technique for ventriculovesical shunting of congenital hydrocephalus

PURPOSE: Hydrocephalus is typically treated with a ventriculoperitoneal or ventriculoatrial shunt. However, shunt malfunction, recurrent infection or other co-morbidities occasionally make these shunts inappropriate. As early as 1925 a ureterodural anastomosis was used to divert cerebrospinal fluid into the urinary system. Since then techniques for cerebrospinal fluid urinary diversion have improved. We designed a new technique to drain cerebrospinal fluid into the urinary system surgically while averting some of the problems encountered with previous methods. MATERIALS AND METHODS: We describe a new technique to perform a ventriculovesicular shunt, which we performed on a 17-year-old woman with congenital hydrocephalus. To put this unique operation into perspective we briefly review the history of cerebrospinal fluid urinary shunting. RESULTS: Our results were promising, with no evidence of distal shunt malfunction or infection postoperatively. CONCLUSIONS: Our novel technique of a ventriculovesical shunt with a polyester cuff that provides stabilization and infection control holds promise for patients with hydrocephalus who are not candidates for a ventriculoperitoneal or ventriculoatrial shunt.     

Breast-Related CSF Shunt Complications: Literature Review with Illustrative Case

In this article, we discuss and classify breast-related ventriculoperitoneal (VP) cerebrospinal fluid (CSF) shunt complications, and provide a literature review. Shunt complications related to pre-existing breast implants comprise nearly half of the breast-related shunt complications reported thus far. We present a complication of shunt failure in a 61-year-old woman who had previously undergone mastectomies for breast cancer with implant reconstruction. Following shunting, she developed headaches, fever, and right-sided breast swelling and erythma consequent to breast implant rupture, distal shunt migration, and CSF pseudocyst. This case is unique in that it involved rupture of a breast implant from VP shunt insertion. For complication avoidance, neurosurgeons should be aware of the potential pitfalls in shunting patients with breast implants.     

Frameless stereotactic ventricular shunt placement for idiopathic intracranial hypertension

INTRODUCTION: Cerebrospinal fluid (CSF) shunting effectively reverses symptoms of idiopathic intracranial hypertension (IIH). Lumboperitoneal (LP) shunts have traditionally been used in patients with IIH due to a frequently undersized ventricular system. However, the advent of image-guided stereotaxis has enabled effective ventricular catheter placement in patients with IIH. We describe the first large series of frameless stereotactic ventriculoperitoneal (VP) shunting for patients with slit ventricles and IIH. METHODS: We describe the frameless stereotactic VP shunting technique for IIH in 32 procedures. Outcomes following shunt placement, time to shunt failure, and etiology of shunt failure are reported. RESULTS: A total of 21 patients underwent 32 ventricular shunting procedures (20 VP, 10 ventriculoatrial, 2 ventriculopleural). One hundred percent of shunts were successfully placed into slit ventricles, all requiring only one pass of the catheter under stereotactic guidance to achieve the desired location and CSF flow. There were no procedure-related complications and each ventricular catheter showed rapid egress of CSF. All (100%) patients experienced significant improvement of headache immediately after shunting. Ten percent of ventricular shunts failed at 3 months after insertion, 20% failed by 6 months, 50% failed by 12 months, and 60% failed by 24 months. Shunt revision was due to distal obstruction in 67%, overdrainage in 20%, and distal catheter migration or CSF leak in 6.5%. There were no shunt revisions due to proximal catheter obstruction or shunt infection. CONCLUSIONS: In our experience treating patients with IIH, frameless stereotactic ventricular CSF shunts were extremely effective at treating IIH-associated intractable headache, and continued to provide relief in nearly half of patients 2 years after shunting without many of the shunt-related complications that are seen with LP shunts. Placing ventricular shunts using image-guided stereotaxis in patients with IIH despite the absence of ventriculomegaly is an effective, safe treatment option.     

以听力下降为主要表现的7例神经型布鲁氏菌病患者的临床特点

目的探讨以听力下降为主要表现的神经型布鲁氏菌病患者的临床特点及转归。 方法选取2008年10月至2019年9月于首都医科大学附属北京地坛医院住院的7例神经型布鲁氏菌病合并听力下降的患者作为研究对象,收集其流行病学史、临床表现、病原学特异性检查、脑脊液分析、耳部查体、纯音测听、影像学表现等资料,并结合国内外文献进行分析和讨论。 结果7例患者中,男性5例、女性2例,年龄22～70岁,平均39.29岁。7例患者均有明确的流行病学接触史,早期表现出布鲁氏菌病的典型症状：发热、多汗、肌肉和关节疼痛、乏力、头痛等。听力下降表现为感音神经性聋,出现在发病早期2例、治疗过程中3例、停药后2例。单侧发病者2例,双侧发病者5例。突发性聋者2例,渐进性听力下降5例。听力下降病程小于3个月者4例,听力改善者4例;长于3个月者3例,听力改善者0例。轻、中度患者5例,重度、极重度患者2例。腰椎穿刺检查提示入组患者颅内压轻度升高（平均为197 mmH₂O）,脑脊液中白细胞升高（平均为104个/μl）,且以单核细胞为主（90.7%）,脑脊液蛋白206.1 mg/dl,脑脊液糖1.95 mmol/L,脑脊液氯化物118.7 mmol/L。 结论神经型布鲁氏菌病合并听力损害并不少见,相较于其他典型症状缺乏特异性,极易误诊及漏诊。早发现、早诊断、早期治疗能有效降低相关后遗症。     

Idiopathic normal pressure hydrocephalus: a systematic review of diagnosis and outcome

OBJECTIVE: Patient selection for cerebrospinal fluid diversion is difficult, because idiopathic normal pressure hydrocephalus (INPH) mimics other neurodegenerative disorders and no findings reliably predict outcome. The literature was reviewed to identify diagnostic criteria that predict shunt response and to formulate prognostic expectations. METHODS: MEDLINE was searched, and 44 articles meeting predetermined criteria were included. RESULTS: Clinical series were frequently retrospective with small patient numbers and unstandardized outcome evaluation. Clinical findings suggestive of shunt responsiveness were the complete triad (gait disturbance, urinary incontinence, and dementia) with early gait disturbance. Degree of hydrocephalus was not correlated with clinical improvement. Reduction of the subcortical low-blood flow area was correlated with improvement in three small studies. Clinical response to prolonged cerebrospinal fluid drainage predicted shunt outcome in all cases in two small series. Overall, 59% (range, 24-100%) of patients improved after shunting, and 29% (range, 10-100%) of patients experienced prolonged improvement. Complications occurred in 38% (range, 5-100%) of patients, additional surgery was required in 22% (range, 0-47%) of patients, and there was a 6% (range, 0-35%) combined rate of permanent neurological deficit and death. CONCLUSION: Shunting INPH is associated with an approximately 29% rate of significant improvement and a 6% significant complication rate. Enlargement of the subcortical low-flow area and clinical improvement secondary to prolonged lumbar drainage may provide additive predictive value above clinical and computed tomographic criteria. A multicenter clinical trial that focuses on the value of ancillary tests, defines the clinical course of a patient with a ventriculoperitoneal shunt, and evaluates the cost effectiveness of shunting INPH is needed to better describe outcome from shunting in INPH.     

Ventriculoperitoneal shunt using a continuously variable-resistance valve for management of hydrocephalus, especially for cases mimicking simple brain atrophy]

Twenty-two hydrocephalic patients with a ventriculoperitoneal shunt using a continuously variable flow resistance valve (Orbis-Sigma valve system; Cordis Corporation, USA) were reviewed to discuss usefulness of the shunt system. We divided the cases into two groups according to the demonstration of the cortical sulci in computed tomography (CT) as follows; A: progressive hydrocephalic cases mimicking simple brain atrophy, B: acute or subacute hydrocephalic cases with evidence of increased intracranial pressure. Patients in Group A had begun to present slowly progressive loss of activity, dementia and gait disturbance of various degrees after a long quiescent period following primary central nervous system injury. CT findings of these cases showed they were mimicking simple brain atrophy. Although the period of their deterioration was very long, ranging from 1 month to nine years, clinical signs improved in all cases after the shunt. None showed complications. Group B cases consisted of those with signs of increased intracranial pressure such as consciousness disturbance, nausea and vomiting. These cases showed poor demonstration of the cortical sulci in CT. Eleven out of thirteen cases showed satisfactory clinical improvement, though the size of the ventricle showed a slight decrease following shunt procedure. Only one case showed unilateral subdural effusion. In conclusion, this shunt system is useful for the management of ventricular enlarged cases mimicking simple brain atrophy as well as acute hydrocephalic cases. Troublesome consequences such as subdural effusion and hemorrhage rarely occur even in cases with low cerebrospinal pressure.     

Hydrocephalus: overdrainage by ventricular shunts. A review and recommendations

Selected literature review of the clinical course of patients with ventricular shunts for hydrocephalus shows that the effects of cerebrospinal fluid overdrainage are subdural hematoma, craniosynostosis, slit ventricle syndrome, and low intracranial pressure syndrome. These occur sequentially at different age groups, but approximate averages of incidence and time of occurrence after first shunt reveal an overall incidence of 10%-12% for at least one of these appearing at 6.5 years after shunting. The basic etiology, diagnosis, and variety of treatment modalities available are reviewed, including the need for shunt closing intracranial pressure control. Included is a hydrocephalus program designed to minimize the need for long-term extracranial shunts and to maximize therapeutic intracranial procedures for hydrocephalus.     

Multiple intracranial seeding of craniopharyngioma after repeated surgery--case report

A 17-year-old woman presented with a rare case of intracranial seeding of craniopharyngioma after repeated surgery. She initially presented with secondary amenorrhea and visual impairment. Magnetic resonance imaging revealed a suprasellar mass. Subtotal removal of the tumor was performed. The diagnosis was adamantinomatous craniopharyngioma. Seven months later, the patient underwent a second operation for recurrence of the craniopharyngioma. Subsequently, ventriculoperitoneal (VP) shunting and gamma knife surgery were performed. Twenty-seven months after the first operation, multiple cystic lesions were found in the right frontal and temporal lobes. Positive tumor cytology was observed in the cerebrospinal fluid obtained from the VP shunt chamber. These tumors were subtotally resected. However, the patient died from consecutive tumor recurrence 4 years after the initial diagnosis.     

Large extra-abdominal cyst as a postpartum complication of peritoneal shunt. Case report

An extra-abdominal cyst filled with cerebrospinal fluid was found postpartum in a patient with a ventriculoperitoneal (VP) shunt. No similar complication of VP shunting has been reported before.     

Diagnosis of ventriculoperitoneal shunt infection using [F-18]-FDG PET: a case report

Infection of cerebrospinal fluid (CSF) shunts is a common occurrence and can often be difficult to diagnose using standard analysis of shunt fluid. This article presents the first case report on the diagnosis of a CSF shunt infection on FDG PET scan. A 26-year-old female underwent ventriculoperitoneal shunt placement after developing a pseudomeningocele subsequent to a suboccipital craniectomy for Chiari malformation. Two months later, the patient presented with abdominal pain and non-specific symptoms and was found to have a perisplenic abscess for which she was adequately treated. Failure of her symptoms to solve and an initial negative shunt CSF analysis prompted the search for other sources of infection. An FDG PET scan performed a week later found evidence of increase tracer uptake around the distal tip of the catheter and a repeat shunt CSF analysis showed evidence of CSF infection. FDG PET may be useful in diagnosing shunt related infections in case of high clinical suspicion when standard diagnostic modalities fail to diagnose hardware infection.     

Akinetic mutism responsive to bromocriptine following subdural hematoma evacuation in a patient with hydrocephalus

An 11-year-old girl with obstructive hydrocephalus developed akinetic mutism after treatment for hydrocephalus due to aqueductal stenosis by ventriculoperitoneal (VP) shunting. Bilateral chronic subdural hematomas developed about 2 months after insertion of the VP shunt and were evacuated. Postoperatively, the patient developed akinetic mutism, but her condition improved after administration of bromocriptine. Absence of abnormalities on dopamine transporter single photon emission computed tomography, lack of clinical response to levodopa treatment, and normal homovanillic acid concentration in the cerebrospinal fluid all indicated normal dopamine production. Pressure on the periventricular monoamine projections in the thalamus and hypothalamus without major dopamine deprivation in the striatum may have been the most important factors in the development of akinetic mutism in this patient.     

Symptomatic progressive ventriculomegaly in hydrocephalics with patent shunts and antisiphon devices

The antisiphon device (ASD) was designed to prevent excessive negative intracranial pressure and overdrainage with cerebrospinal fluid shunts. It has been recommended for prevention of slit ventricles and extreme shunt dependency. It was used by the author in 40 children and young adults who had low pressure headaches or were judged to be at risk for the development of subdural hematomas because of extreme hydrocephalus, relatively fixed head size, or tall stature. Nine patients encountered problems with adverse symptoms in spite of shunt patency. Four had alarming neurological symptoms of coma or severe headaches after incorporation of ASDs in ventriculoperitoneal shunt systems. With patients in the horizontal position, shunt patency was documented but computed tomography revealed progressive ventriculomegaly when they were kept erect. Symptoms abated and ventricular size diminished after conversion to proximal medium pressure diaphragm or spring-ball valve systems without ASDs. Because long shunt systems with ASDs require adequate hydrostatic columns to initiate flow when patients are erect, it is theorized that such symptoms could have been prevented by placing ASDs farther downstream from the cerebral ventricles.     

The use of ventriculoperitoneal shunts for uncontrollable intracranial hypertension without ventriculomegally secondary to HIV-associated cryptococcal meningitis

BACKGROUND: The risks associated with implanting a cerebrospinal fluid (CSF) shunt in immunocompromised patients with ongoing CSF infection have historically discouraged surgeons from implanting CSF shunts in patients with HIV and cryptococcal meningitis. However, this patient population often requires frequent lumbar punctures to manage elevated intracranial pressure (ICP) secondary to cryptococcal infection. To date, only 7 cases of ventriculoperitoneal (VP) shunting for the treatment of intracranial hypertension in patients with HIV-associated cryptococcal meningitis have been reported. Few of these reports have included outcomes more than 3 months postsurgery. It remains unclear if VP shunts are an effective long-term treatment of intracranial hypertension in this patient population. CASE DESCRIPTIONS: Two patients with HIV/AIDS (CD4 counts of 8 and 81 cells/mm(3)) presented with altered mental status, visual changes, florid cryptococcal meningitis, and elevated ICP (>500 mm CSF) without evidence of hydrocephalus on computed tomography scan. Both patients experienced rapid reversal of symptoms with external lumbar CSF drainage, and remained lumbar drain-dependent after 2 weeks of amphotericin B and flucytosine therapy. Despite evidence of unresolved cryptococcal meningitis, each patient underwent implantation of a VP shunt without complication and was discharged on lifetime fluconazole therapy. They remained asymptomatic at 12 and 16 months after surgery without evidence of shunt infection or malfunction. CONCLUSION: Patients with intracranial hypertension and HIV-associated cryptococcal meningitis who cannot tolerate cessation of external lumbar CSF drainage or frequent lumbar punctures may be considered for VP shunt placement despite severe immunosuppression and persistent CSF cryptococcal infection.