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The management of ventriculoperitoneal (VP) shunt failure is a common problem in neurosurgical practice. On occasion, extraperitoneal sites for CSF diversion are required when shunting to the peritoneal cavity has failed after multiple attempts. The authors report a novel minimally invasive procedure allowing cannulation of the ureter for the purpose of ventriculo-ureteral (VU) shunting. Sixteen years prior to presentation, this 46-year-old woman had contracted tuberculous meningitis and had chronic hydrocephalus, with multiple distal shunt failures in recent months. A percutaneous nephrostomy was used to pass the distal catheter based on intraoperative retrograde pyelography. Following successful placement of the VU shunt, the patient's hydrocephalus stabilized and she returned to her regular functional status. The only long-term complication noted within 36 months of follow-up was a transient episode of electrolyte disturbance and dehydration associated with a diarrheal illness that responded to adequate hydration and salt supplementation. By its minimally invasive nature, this approach offers a reasonable extraperitoneal alternative after multiple distal shunt catheter failures have occurred.

OBJECT Hydrocephalus is one of the commonest complications of tubercular meningitis (TBM), and its incidence is increasing with the HIV epidemic. Literature evaluating the role of ventriculoperitoneal shunts in HIV-positive patients with TBM and their long-term prognosis is scarce. METHODS Between June 2002 and October 2012, 30 HIV-positive patients with TBM and hydrocephalus underwent ventriculoperitoneal shunt placement. Thirty age-, sex-, and grade-matched HIV-negative patients with TBM and hydrocephalus were randomly selected as the control group. Outcome was analyzed at discharge (short-term outcome) and at follow-up (long-term outcome). Univariate and multivariate analyses were performed to look for predictors of outcome; p < 0.05 was considered significant. RESULTS There were no differences in the clinical, radiological, or biochemical parameters between the 2 groups. Short-term outcome was better in the HIV-negative group (76.7% improvement) than in the HIV-positive group (70%). However, the long-term outcome in HIV-positive patients was very poor (66.7% mortality and 76.2% poor outcome) compared with HIV-negative patients (30.8% mortality and 34.6% poor outcome). Seropositivity for HIV is an independent predictor of poor outcome both in univariate and multivariate analyses (p = 0.038). However, in contrast to previous reports, of 5 patients with TBM in good Palur grades among the HIV-positive patients, 4 (80%) had good outcome following shunt placement. CONCLUSIONS The authors recommend that shunt treatment should not be performed in HIV-positive patients in poor Palur grade with hydrocephalus. A trial of external ventricular drainage should be undertaken in such patients, and shunt treatment should be performed only if there is any improvement. However, HIV-positive patients in good Palur grades should undergo VP shunt placement, as these patients have better outcomes than previously reported.

Object: Hemorrhagic origin is unidentifiable in 10%-20% of patients presenting with spontaneous subarachnoid hemorrhage (SAH). While the patients in such cases do well clinically, there is a lack of long-term angiographic followup. The authors of the present study evaluated the long-term clinical and angiographic follow-up of a patient cohort with SAH of unknown origin that had been enrolled in the Barrow Ruptured Aneurysm Trial (BRAT)., Methods: The BRAT database was searched for patients with SAH of unknown origin despite having undergone two or more angiographic studies as well as MRI of the brain and cervical spine. Follow-up was available at 6 months and 1 and 3 years after treatment. Analysis included demographic details, clinical outcome (Glasgow Outcome Scale, modified Rankin Scale [mRS]), and repeat vascular imaging., Results: Subarachnoid hemorrhage of unknown etiology was identified in 57 (11.9%) of the 472 patients enrolled in the BRAT study between March 2003 and January 2007. The mean age for this group was 51 years, and 40 members (70%) of the group were female. Sixteen of 56 patients (28.6%) required placement of an external ventricular drain for hydrocephalus, and 4 of these subsequently required a ventriculoperitoneal shunt. Delayed cerebral ischemia occurred in 4 patients (7%), leading to stroke in one of them. There were no rebleeding events. Eleven patients were lost to followup, and one patient died of unrelated causes. At the 3-year follow-up, 4 (9.1%) of 44 patients had a poor outcome (mRS > 2), and neurovascular imaging, which was available in 33 patients, was negative., Conclusions: Hydrocephalus and delayed cerebral ischemia, while infrequent, do occur in SAH of unknown origin. Long-term neurological outcomes are generally good. A thorough evaluation to rule out an etiology of hemorrhage is necessary; however, imaging beyond 6 weeks from ictus has little utility, and rebleeding is unexpected.

Object: In this study the authors compare the efficacy of endoscopic third ventriculostomy (ETV) versus CSF shunting for resolution of papilledema in hydrocephalus., Methods: This comparative case series study recruited 12 patients (24 eyes) with hydrocephalus who underwent either an ETV (Group 1, 6 patients [12 eyes]) or CSF shunt treatment (Group 2, 6 patients [12 eyes]). A complete ophthalmological examination including retinal nerve fiber layer (RNFL) evaluation by optical coherence tomography was provided for all patients before surgery and in the 1st week, 1st month, and 3rd month postoperatively. The 2 groups were compared for quantitative changes in RNFL thickness and, thereby, resolution of papilledema. Statistical evaluation was performed using the Mann-Whitney U-test with the aid of SPSS version 16.0., Results: The mean preoperative RNFL thickness was 259.7 ± 35.8 μm in Group 1 and 244.5 ± 53.4 μm in Group 2 (p = 0.798). The mean decrease in RNFL thickness was 101.3 ± 38.8 μm, 141.2 ± 34.6 μm, and 162.0 ± 35.9 μm in Group 1 versus 97.0 ± 44.6 μm, 143 ± 45.6 μm, and 130.0 ± 59.8 μm in Group 2 for the postoperative 1st week, 1st month, and 3rd month, respectively. There was no significant difference between the two groups with respect to decrease in RNFL thickness during the 1st week, 1st month, and 3rd month (p = 0.563, p = 0.753, and p = 0.528, respectively)., Conclusions: This is the first study to quantitatively evaluate papilledema in assessing the success of ETV and CSF shunting. The authors' results indicated that ETV is as effective as CSF shunting with respect to decreasing intracranial pressure and resolution of papilledema.

The authors present the case of a 78-year-old man who presented with a subarachnoid hemorrhage due to rupture of an aneurysm at the origin of the persistent primitive olfactory artery (PPOA). Interestingly, the PPOA was originating from the A1 segment of the anterior cerebral artery and coursed anteromedially along the olfactory tract. Moreover, the PPOA in this case had 2 branches: the branch making a hairpin turn and supplying the distal part of the anterior cerebral artery territory (Type 1), and the branch extending to the cribriform plate to supply the nasal cavity (Type 2). To the best of the authors' knowledge, this is a new variant (Type 3) of PPOA associated with a ruptured aneurysm. The clinical implications of this case are discussed in terms of the embryological aspects.

Object: Fluorescence-guided surgery using 5-aminolevulinic acid (5-ALA) has been broadly recognized as a beneficial tool for the resection of glioblastoma multiforme (GBM). Fluorescence in the ventricular walls, which were apparently free of macroscopic tumor or MR imaging enhancement indicative of CSF dissemination, was detected during surgery for GBM. To evaluate the clinical significance of the 5-ALA fluorescence, the authors resected ventricle wall tissue together with the adjacent tumors for pathological examination and then followed up the clinical courses of the patients., Methods: Seven consecutive GBMs located near the lateral ventricle were surgically treated using a fluorescence-guided technique with 5-ALA at the authors' hospital since acquiring instrumentation for the detection of 5-ALA fluorescence in 2007. All of the procedures were performed using a ventricular entry, and 5-ALA fluorescence of the ventricular wall was detected despite the absence of macroscopic tumor invasion of the wall., Results: A pathological examination of the resected ventricular wall tissues revealed tumor cells in 6 of the 7 cases and disruption of the ependymal cell layer in all 7 cases. Delayed communicating hydrocephalus followed surgery in all 7 patients, and ventricular wall enhancements on MR imaging were demonstrated after hydrocephalus in 2 of the patients., Conclusions: Data in this study suggest that 5-ALA fluorescence of the ventricular wall may be predictive of postoperative hydrocephalus associated with CSF dissemination even in cases without evidence of CSF dissemination on MR imaging studies before surgery. The authors also speculate that postoperative radiotherapy covering the whole ventricular system may be a better therapeutic option for these patients.

Object: Complete dural closure is not always possible following posterior fossa surgery, often requiring a graft to secure complete closure. The authors report their experience of using a collagen matrix as an onlay dural graft for repair of a posterior fossa dural defect., Methods: A retrospective analysis was performed in 52 adult patients who had undergone collagen matrix duraplasty for the posterior fossa. Complications directly related to the dural graft, the presence or absence of hydrocephalus, and the role of closed suction wound drainage in relation to postsurgical pseudomeningoceles were analyzed., Results: The indication for posterior fossa surgery was tumors in 32 patients, vascular abnormalities in 9 patients, and spontaneous cerebellar hemorrhage in 11 patients. Closed suction wound drainage was used in 23 patients (44.2%). Forty-eight (92.3%) of 52 patients had a dural defect > 2 cm. Nine (81.8%) of 11 patients with hydrocephalus required ventriculoperitoneal shunts. Complications of the surgery included pseudomeningoceles in 2 patients (3.8%; no closed suction wound drainage); superficial wound infections in 1 patient (1.9%; with closed suction wound drainage); and unexplained eosinophilia in 1 patient., Conclusions: Duraplasty using a collagen matrix is safe and effective in the posterior fossa, and is easy to use and time efficient. Meticulous layered wound closure, the detection and effective control of hydrocephalus, and the use of closed suction wound drainage reduces complications related to collagen matrix duraplasty for the posterior fossa.

Multidetector CT has become widely available and with it the ability to rapidly create detailed reformatted images. Multiplanar images can be created depicting the anatomy in planes other than the traditional axial plane, using isotropic to near-isotropic data. It is important for both clinicians and radiologists to be aware of this capability in order to take advantage of such images. To illustrate the value of this type of imaging, the authors present a case of a third ventricular clot that migrated into the cerebral aqueduct exacerbating hydrocephalus.

Neurocutaneous melanosis (NM) coexisting with the Dandy-Walker complex (DWC) is a rare condition, with fewer than 15 cases reported in the literature. The authors present a case of an infant with NM and DWC suffering from progressive brainstem compression following ventriculoperitoneal (VP) shunt placement for hydrocephalus. This 1-year-old boy with congenital melanocytic nevi had met normal developmental milestones until the age of 11 months, when he began regressing in ambulation and language function. Intractable vomiting had developed 1 week later. Magnetic resonance (MR) imaging of the brain revealed DWC with hydrocephalus, and spinal MR images demonstrated a proliferative process within the meninges, consistent with NM. The patient underwent right frontal VP shunt placement resulting in immediate symptom relief, but 3 weeks later became irritable, increasingly lethargic, unable to pull to stand, and unable to tolerate solid food without choking. Due to these symptoms and intractable vomiting, the patient presented to the authors' institution. Brain MR imaging revealed a new-onset diffuse cystic process with anterior and posterior brainstem compression, marked kinking of the cervicomedullary junction, melanocyte pigmentation of the left temporal lobe, diffuse leptomeningeal enhancement, and no evidence of hydrocephalus. Consistent with these imaging findings, the degree of brainstem involvement upon gross visualization predictably deterred resection attempts beyond those necessary for biopsy. Pathological examination revealed diffuse melanocytosis, and the family decided not to pursue aggressive measures postoperatively. This report indicates the potential for rapid intracranial manifestation of diffuse melanocytosis in NM patients. Although the prognosis is poor, early neurosurgical involvement in these patients may provide tissue diagnosis and the potential for decompression if the process is caught early in its course.

Due to early diagnosis and treatment of hydrocephalus, neurosurgeons rarely are called upon to treat patients with extreme hydrocephalic macrocephaly. Macrocephaly can limit mobility and hygiene. The critical evaluation and surgical correction of the morphological problem of macrocephaly secondary to hydrocephalus is complex. Various techniques such as quadrantal, picket fence, crossbar, and modified rr techniques have been used to reduce the size of the cranial vault to decrease cranial volume while achieving good cosmesis. Limitations of vault reduction cranioplasty include the inability to alter the anteroposterior and lateral diameters of the skull base, the inability to shorten the superior sagittal sinus, and the need to avoid infolding of the brain due to the risk of venous infarcts. Reduction cranioplasty is indicated in the occasional patient whose large head size represents a mechanical or cosmetic problem of sufficient magnitude to seriously interfere with motor development and functioning, with resultant development of pressure sores and difficulties with nursing care. Reduction cranioplasty should be avoided in patients under the age of 3 years.

Object: Benign extracerebral fluid collections are common in infants, but there are unanswered questions regarding presentation and long-term outcome. This study was undertaken to establish head size at birth, head size at presentation, head growth over time, history of presentation, indications for surgery, and long-term results., Methods: Cases of benign extracerebral fluid collections in infancy were retrospectively reviewed. Data pertaining to clinical presentation, progress, and long-term follow-up were evaluated. Patients were divided into two groups on the basis of the presence or absence of increased signal intensity of fluid in the extracerebral space on T1-weighted magnetic resonance images. Group 1 consisted of 39 patients who had benign extracerebral fluid collections without any subdural collections. Group 2 consisted of nine patients who had a chronic subdural hematoma or hygroma without a history of trauma and had generous subarachnoid spaces and/or a history of premature birth. Patients were observed for an average of 49 months., Results: Group 1 included 39 patients (ages at presentation 3-12 months); the most common presentation in this group was macrocephaly (28 cases). Of those Group 1 cases in which data regarding gestational age at birth were available, 15 births were either severely or mildly premature and 14 were full term. Of those Group 1 patients with adequate data, 14 had an occipitofrontal circumference (OFC) in the 50th to 98th percentile at birth and two had OFCs greater than the 98th percentile. Measurements obtained at 24 months of age were available for 18 Group 1 patients: OFC was at approximately the 98th percentile in eight (all eight born at full term) and more than 1 cm larger than the 98th percentile in 10. Head growth in Group 1 patients continued to decrease, approaching the 98th percentile after 24 months of age. Only three of the 39 patients in Group 1 required shunt placement. Of the 33 Group 1 patients for whom long-term follow-up was available, 30 were developing normally, and three had mild developmental delays. Group 2 included nine patients (ages at presentation 3-7 months); the most common presentations were large head (three patients) and seizures (three patients). Data concerning gestational age at birth were available in eight Group 2 cases: birth was severely premature in four and gestation was full term in four. Five Group 2 patients were treated surgically-two with subduroperitoneal (SDP) shunt placement, two with subdural punctures, and one with subdural punctures and SDP shunt placement. Adequate follow-up information was available in eight of nine Group 2 cases; all eight patients were found to be developing normally., Conclusions: Group 1 patients usually had a normal head size at birth, they rarely required shunt placement, their head growth eventually leveled off toward the 98th percentile line, and their outcomes were generally good. Although five of nine Group 2 patients required subdural punctures and/or SDP shunt placement, that group of patients also developed well. Infants with nontraumatic subdural hematomas or hygromas, presumably associated with benign extracerebral fluid collections, can often experience significant resolutions of the hematoma or hygroma within several months without surgical treatment.

Object: A myelomeningocele (MMC) can be associated with paraplegia, bladder and bowel incontinence, Chiari malformation Type II, and hydrocephalus. The coincidence of an MMC and a neoplasm is rare, and only limited reports on the concurrence of a teratoma within an MMC have been published., Methods: A retrospective study was performed using the records of 330 children who underwent operations to correct an MMC at the Children's Hospital Medical Center in Tehran between January 2001 and June 2005. The postoperative histopathological assessments in 15 (4.5%) of these patients revealed evidence of a teratoma inside the MMC. The age of these patients at admission ranged from 2 days to 2 years (median 30 days). Neurological findings were normal in all patients except for three with lower-extremity weakness or paralysis. Hydrocephalus was detected in six patients. Physical appearance of the teratoma in all cases included cystic or solid soft-tissue masses in the dorsal midline area, covered with abnormal rudimentary skin. Pedunculated skin tags, a primitive genitalia-like appendage, dermal sinus, a human tail, and cutaneous stigmata such as color changes, hemangioma, dysplastic skin, and tufts of hair around the mass were occasionally observed., Conclusions: The special feature of a protruding fingerlike appendage or intraoperative detection of a cystic portion of an MMC without direct connection to the spinal canal can be possible signs of teratoma concurrent with an MMC. Teratoma inside an MMC is a benign neoplasm, without any recurrence after standard surgery for an MMC.

Accessory human tails are uncommon malformations consisting of meningeal and neural structures and possibly occult spinal dysraphism (pseudotails). The thoracic location of a lipomeningocele constituting a pseudotail is very rare. The authors report the case of a young boy harboring a thoracic dorsal appendage consisting of a lipomeningocele and a dermoid cyst that was complicated by the presence of diastematomyelia, tethered spinal cord, syringomyelia, and mild hydrocephalus. The preoperative moderate ventricular dilation was decompressed by the surgical repair of the spinal malformation and required a specific treatment. The authors discuss the rare association of these clinical entities and conclude that complete spine and brain neuroimaging studies are necessary for the correct surgical planning of this kind of malformation.

Although the Codman Hakim programmable valve (CHPV) system is popular, several problems arising from its design have been described. The authors report an additional cause of shunt failure associated with the use of the CHPV system. A 7-year-old girl who had previously undergone placement of a ventriculoperitoneal shunt with the CHPV system was admitted in a comatose state. Radiography did not demonstrate any breakage or change in the opening pressure of the valve. Manual pumping of the valve resulted in neurological improvement and shrinkage of the girl's ventricles. However, ventriculomegaly was again observed several hours later, and an emergency valve revision was performed. The manufacturer's examination of the extracted valve revealed a crack on the surface of the hard plastic housing covering the valve chamber, which appeared to be suggestive of some type of blunt trauma. In addition, the flat spring that transmitted resistance from the pressure control cam to the valve ball was deformed in such a manner that it caused excessive pressure against the valve ball. Because of this deformity, the actual opening pressure in this patient had reached a maximum level of 226 mm H2O instead of the adjusted pressure of 60 mm H2O. In patients with the CHPV system whose medical history is difficult to ascertain, the possibility of damage to the valve spring and the need for a prompt revision surgery should be considered. The authors conclude that placement of the CHPV system may be contraindicated in patients with a known habit of head banging.

Object: Only limited data exist addressing the long-term outcome of patients with ganglionic hemorrhage complicated by intraventricular hemorrhage (IVH) and hydrocephalus that requires an external ventricular drain (EVD). The aim of this study was to compare the long-term outcome of patients with pure ganglionic hemorrhage and those requiring an EVD due to additional IVH and hydrocephalus., Methods: For this study, 116 patients with supratentorial ganglionic hemorrhage and occlusive hydrocephalus were screened. To avoid any bias the authors excluded all patients with nonprimary intracerebral hemorrhage as well as those who received no treatment. Forty patients with IVH and subsequent hydrocephalus were examined, and 40 more patients with pure ganglionic hematomas were matched with regard to age, sex, Glasgow Coma Scale score, need for mechanical ventilation, and, in particular, hematoma volume. Outcome analysis was performed using the Barthel Index and the modified Rankin Scale (mRS). The mean hematoma volume was 25.3 +/- 15.2 ml. The overall long-term outcome was unfavorable (mRS Score 4-6) in 59% of all patients. Good outcome (mRS score < 4) was observed in 25 (62.5%) of 40 patients with hematoma volumes less than 25 ml, compared with eight (20%) of 40 who had hematoma volumes greater than 25 ml (p < 0.05, chi-square test). The need for an EVD was not associated with a worse long-term outcome in patients with comparable hematoma volumes. In contrast, the duration of treatment in the intensive care unit was longer for patients with EVDs than for those who had pure ganglionic hematomas (16 [range 5-29] days compared with 8 [range 2-19] days; p < 0.05, Mann-Whitney U-test), regardless of hematoma volume., Conclusions: The long-term outcome of treated patients with supratentorial ganglionic hemorrhage with ventricular involvement and occlusive hydrocephalus is comparable to that of patients with similar hematoma volumes but no IVH.

The authors describe a 58-year-old man with sudden onset of a unilateral tremor caused by a midbrain lesion that affected the substantia nigra and the cerebellothalamic pathway. There were also clinical and neuroimaging signs of a communicating chronic hydrocephalus. The patient was severely handicapped by this tremor, which was a typical Holmes tremor with rest, posture, and intention components. Parkinson disease or multiple-system atrophy as causes for the tremor could be ruled out by DaTSCAN and 123I iodobenzamide and single-photon emission computerized tomography (SPECT), respectively. The tremor was completely supressed by temporary and permanent cerebrospinal fluid release after ventriculoperitoneal shunt placement, without any additional medication, for a period of 6 months. Afterward, the tremor returned, and the patient had to be treated by a stereotactic electrode implantation in the contralateral ventralis intermedius nucleus, which led to complete tremor suppression during the 1.5-year follow-up period. In this case report, the authors present the clinical description and the electrophysiological, SPECT, and magnetic resonance imaging data of a rare combination of symptoms and their surgical treatment.

In an effort to identify critical gaps in the prevailing knowledge of hydrocephalus, the authors formulated 10 key questions. 1) How do we define hydrocephalus? 2) How is cerebrosinal fluid (CSF) absorbed normally and what are the causes of CSF malabsorption in hydrocephalus? 3) Why do the ventricles dilate in communicating hydrocephalus? 4) What happens to the structure and function of the brain when it is compressed and stretched by the expanding ventricles? 5) What is the role of cerebrovenous pressure in hydrocephalus? 6) What causes normal-pressure hydrocephalus? 7) What causes low-pressure hydrocephalus? 8) What is the pathophysiology of slit ventricle syndrome? 9) What is the pathophysiological basis for neurological impairment in hydrocephalus, and to what extent is it reversible? 10) How is the brain of a child with hydrocephalus different from that of a young or elderly adult? Rigorous answers to these questions should lead to more effective and reliable treatments for this disorder.

Object: In this study the authors conducted a retrospective evaluation of the effectiveness of endoscopic aqueductoplasty, performed alone or accompanied by placement of a stent, in the treatment of an isolated fourth ventricle (IFV) in seven patients afflicted with loculated hydrocephalus after a hemorrhage or infection., Methods: Seven children with symptomatic IFV and membranous aqueductal stenosis underwent endoscopic aqueductoplasty alone or combined with placement of a stent in the cerebral aqueduct. The mean age of the patients at the time of surgery was 10 months. The mean duration of follow up was 26 months. In all patients a supratentorial shunt had already been implanted, and in five patients neuroendoscopy had already been performed because other isolated compartments had been present inside the ventricular system. Aqueductoplasty alone was performed in three patients and aqueductoplasty and aqueductal stent placement in four. A precoronal approach was performed in five patients and a suboccipital approach in two. Signs and symptoms of intracranial hypertension resolved in all cases. Stent placement was successful in all five cases, resulting in clinical and neuroimaging-confirmed improvements in the IFV. Restenosis of the aqueduct occurred in two patients in whom stents had not been placed. In one of these patients restenosis was managed by an endoscopic procedure, during which the aqueduct was reopened and a stent implanted; in the other patient a shunt was placed in the fourth ventricle. Hydrocephalus was controlled by a single shunt in six cases (86%) and by a double shunt in one case., Conclusions: Endoscopic placement of a stent in the aqueduct is more effective in preventing the repeated occlusion of the aqueduct than aqueductoplasty alone and should be indicated as the initial treatment in each case of compatible anatomy.

Object: The authors set out to evaluate the feasibility and effectiveness of preoperative chemotherapy in treating high-risk medulloblastomas., Methods: Between 1997 and 2000, 21 children with high-risk medulloblastomas (M > or = 2 and/or T3b/T4 according to the Chang classification) were treated consecutively in a pilot study. The protocol began with treatment of the hydrocephalus and confirmation of the diagnosis. Tumor surgery was performed either after conventional chemotherapy (eight patients) or after subsequent high-dose chemotherapy (HDCT; 11 patients). Two children with early leptomeningeal progression died before surgery. Craniospinal irradiation was applied to children older than 5 years of age, whereas younger children received local irradiation only. Hydrocephalus was present in 17 children and was treated with ventriculocisternostomy in 13 and shunt insertion in four. A biopsy procedure was performed with a stereotactic frame in 10 children, an open surgery was performed in four, an endoscope was used during the ventriculocisternostomy in three, and the diagnosis was made based on cerebrospinal fluid cytological analysis in two. The response rate to the first two courses of chemotherapy was 71% for the tumor and 59% for the metastases. The pathological analysis of the residue after chemotherapy showed true medulloblastomas in seven cases, complete neuroglial maturation in three cases, and a mixture of both in nine cases. Three-year progression-free survival was 37% and was significantly better in children older than 5 years of age. There was one death related to the HDCT., Conclusions: Preoperative chemotherapy is feasible and safe in children with high-risk medulloblastomas provided that the hydrocephalus can be treated at diagnosis. A larger study is warranted to ensure that the high response rate to adjuvant chemotherapy can lead to better surgical results and survival advantage.

The authors report on the case of a diffuse pontine glioma in a 5-year-old boy in whom radiologically and cytologically occult leptomeningeal metastases led to the development of an atypical "external" hydrocephalus, associated with grossly elevated intracranial pressure (ICP). Initial neuroimaging demonstrated only mild communicating ventricular dilation associated with a noticeable enlargement of the subarachnoid space, particularly over the surface of the frontal lobes; these features are not usually associated with significantly elevated ICP. Possible pathophysiological mechanisms resulting in this unusual clinical presentation are discussed. Early recognition of the severity of the raised ICP despite the paucity of clinical and radiological signs may have averted the development of blindness due to optic atrophy.

Object: The failure of ventricles to enlarge after acute shunt malfunction in long-term shunt-dependent patients is generally attributed to the presence of periventricular rigidity resulting from gliosis. The aim of this study was to test the hypothesis that periventricular rigidity is present in these patients., Methods: Fifteen pediatric patients who presented with acute shunt malfunction were studied; slit ventricles were detected in all of these patients. Pressure measurements were recorded simultaneously in both the ventricle and the brain parenchyma during shunt revision and then repeated after bolus infusion of fluid into the ventricle. The mean intraventricular pressure (IVP) at presentation was 24.1 mm Hg (standard deviation 10 mm Hg). The mean baseline IVP after drainage of cerebrospinal fluid but prior to the infusion was 7.5 +/- 4.3 mm Hg. There was no significant difference in the increase in the IVP and the intraparenchymal pressure (IPP) after bolus infusion into the ventricle (3.6 +/- 2.4 mm Hg and 3.3 +/- 2.1 mm Hg, respectively; p = 0.39). The mean pressure volume index was 24.1 ml (standard error of the mean 4.6 ml)., Conclusions: The profile of the changes in IVP and IPP indicates that the periventricular wall does not restrict ventricular enlargement following shunt malfunction in long-term shunt-dependent patients.

Object: The aim of this study was to investigate the cause and outcome of intracranial hemorrhage (ICH) in children with cancer., Methods: The charts of 51 children who underwent treatment for both cancer and ICH between January 1985 and January 2003 were retrospectively reviewed. Assessment tools included the Karnofsky Performance Scale (KPS), Glasgow Coma Scale (GCS), and the Fisher exact and Student t-tests. Among the 51 cases, 30 involved brain tumors, 19 leukemia, and two lymphoma. The treatment group (Group 1) comprised 36 patients who suffered ICH during cancer treatment; the posttreatment group (Group 2) consisted of the 15 patients who suffered ICH after the completion of cancer treatment. The types of ICH included 22 cortical, four subcortical, 17 subdural, five brainstem, one subarachnoid, one epidural, and one ventricular. Thrombocytopenia was present in nine patients (25%) in Group 1. More patients in Group 2 (87%) than in Group 1 (44%) underwent cranial radiation treatment. Patients in Group 1 experienced a higher incidence of coagulopathy (37%) and ICH-related death (25%) than those in Group 2 (0 and 7%, respectively). Decrease in KPS and GCS scores of greater than 30 and greater than 3, respectively, at the time of ICH were indicators of increased mortality. Of the 17 children with subdural ICH, 13 suffered the hemorrhage following treatment for hydrocephalus and three patients suffered ICH associated with thrombocytopenia. In the 33 children alive at the 3-month follow-up examination after the ICH, no difference existed in the mean KPS scores pre- and post-ICH., Conclusions: Treatment for hydrocephalus, coagulopathy, thrombocytopenia, and hemorrhage into the tumor were the most probable causes of ICH among patients in Group 1. Radiation-induced vasculopathy was a possible cause of ICH in the patients in Group 2. Significant decline in the patient's neurological status at the time of ICH is a poor prognostic factor, but those patients who survive cancer and ICH are likely to regain neurological function.

The authors present a case of a child suffering from shunt-treated hydrocephalus and spastic quadriplegia who underwent surgery for placement of a baclofen pump. Magnetic resonance (MR) imaging performed prior to pump placement demonstrated no hindbrain herniation. Afterward, however, the patient exhibited symptoms of brainstem compression, and MR imaging revealed a significant Chiari I malformation along with a fully functioning ventriculoperitoneal shunt. Posterior fossa decompression was performed, and the patient's symptoms abated. The authors believe this to be the first report of an acquired Chiari I malformation in a patient with a baclofen pump. Clinicians should consider Chiari I malformation as a rare but severe complication of baclofen pump placement.

Object: In the preceding article, the authors described the Hydrocephalus Outcome Questionnaire (HOQ), a simple, reliable, and valid measure of health status in children with hydrocephalus. In the present study, they present their initial experience in using the HOQ to quantify the health status in a typical cohort of children with hydrocephalus., Methods: The mothers of children with hydrocephalus completed the HOQ and, with the child's attending surgeon, provided a global rating of their children's health. An exploratory analysis was performed using a multivariate analysis of variance (ANOVA) to determine which variables might be associated with worse health status. The mothers of 80 children, ranging in age from 5 to 17 years, participated in the study. The mean HOQ Overall Health score was 0.68, a value estimated to be equivalent to a mean health utility score of 0.77. The global health ratings provided by the mothers and the surgeons were moderately correlated with the HOQ scores (Pearson correlations 0.58 and 0.57, respectively). Results of the multivariate ANOVA indicated that the presence of epilepsy was strongly associated with a worse health status (p < 0.0001, F-test)., Conclusions: The health status of a typical sample of children with hydrocephalus was measured using the HOQ. The only consistently significant association with health status found was the presence of epilepsy.

The authors review the case of a patient treated by Dr. Walter Dandy. When the patient was a young child he underwent two right transtemporal third ventriculostomies during which he sustained an unrecognized contralateral subdural hygroma and a chronic subdural hematoma with a mild infantile hemiparesis. He was able to complete high school, albeit at a slower pace than usual. As an adult he held several limited employment positions, lived at home for several decades, and was later cared for at a nursing home for a short time. The patient died when he was 66 years of age.

This report documents clinical features in five children who developed transient reddening of the skin (epidermal flushing) in association with acute elevations in intracranial pressure (ICP). Four boys and one girl (ages 9-15 years) deteriorated acutely secondary to intracranial hypertension ranging from 30 to 80 mm Hg in the four documented cases. Two patients suffered from ventriculoperitoneal shunt malfunctions, one had diffuse cerebral edema secondary to traumatic brain injury, one was found to have pneumococcal meningitis and hydrocephalus, and one suffered an intraventricular hemorrhage and hydrocephalus intraoperatively. All patients were noted to have developed epidermal flushing involving either the upper chest, face, or arms during their period of neurological deterioration. The response was transient, typically lasting 5 to 15 minutes, and dissipated quickly. The flushing reaction is postulated to be a centrally mediated response to sudden elevations in ICP. Several potential mechanisms are discussed. Flushing has clinical importance because it may indicate significant elevations in ICP when it is associated with neurological deterioration. Because of its transient nature, the importance of epidermal flushing is often unrecognized; its presence confirms the need for urgent treatment.

Object: Because of political and economic pressures, primary care physicians are now charged with greater responsibility for the care of patients with disease processes definitively managed by neurosurgeons. The goal of this study was to establish the feasibility and efficacy of a neurosurgical curriculum designed to teach future primary care physicians about these diseases., Methods: A compact, seven-lecture curriculum was developed to teach 3rd-year medical students about degenerative spine disease, stroke, tumor- and hydrocephalus-related raised intracranial pressure, head and spine injury, and subarachnoid hemorrhage. This curriculum was given as part of a 6-week pilot course that included neurology, neurosurgery, ophthalmology, and rehabilitation medicine components. This course was administered to two groups of 18 medical students, and an examination was administered at the end of the pilot course. The same examination was administered to an additional 19 students immediately after their completion of the neurology course currently required. Students enrolled in the pilot neuroscience course performed significantly better (p<0.001) on the examination than those who had completed the standard neurology course. Striking improvements were noted in the recognition and management of head injury, hydrocephalus, and radiculopathy., Conclusions: Inclusion of a short neurosurgery-related curriculum in a combined neuroscience course significantly improved student performance on an examination focusing on the recognition and management of common neurosurgical disorders. Because primary care physicians are responsible for the initial recognition and management of these disorders, the knowledge gained may lead to improved patient care.

Object: There is still controversy regarding the optimum time to perform surgery for craniosynostosis. Some recommend surgery soon after birth and others delay until the age of 12 months. Intracranial pressure has been measured in an attempt to provide a scientific rationale, but many questions remain unanswered. To date, little attention has been given to intracranial volume and its changes during the first few years of life in children with craniosynostosis. The authors' goal was to focus on intracranial volume during this period and to compare measurements obtained in patients with craniosynostosis with measurements obtained in healthy individuals., Methods: Using the technique of segmentation, the intracranial volume of 84 children with various forms of craniosynostosis was measured on preoperative computerized tomography scans. The change in average volume that occurs with increasing age was calculated and compared with a model of normal intracranial volume growth. The age at presentation for children with craniosynostosis was 1 to 39 months; 76% of the patients were younger than 12 months. In eight patients in whom only one cranial expansion procedure was performed, postoperative intracranial volumes were measured as well. Several interesting observations emerged. 1) There was little difference in head growth between boys and girls with craniosynostosis during the first few months of life. After the age of 12 months, however, the difference in intracranial volume normally seen between the two genders was observed in the craniosynostosis group as well. 2) Excluding children with complex pansynostosis, who have smaller heads, children with all other types of craniosynostosis have similar head growth after the 1st year of life, with no difference between the number of and type of suture affected. Children with Apert's syndrome develop greater than normal intracranial volumes after the 1st year of life. 3) Although children with craniosynostosis are born with a smaller intracranial volume, by the age of 6 months volume has reached normal levels, and from that point on volume follows the pattern of normal head growth. 4) Children who presented after the age of 6 months and later developed recurrent craniosynostosis after initial successful treatment had a small intracranial volume at their initial presentation. 5) Of the patients whose postoperative intracranial volumes were measured, all but one had preoperative volumes at or above normal values, and their postoperative volumes were considerably higher than normal for their age. These children all followed a growth curve parallel to that of healthy children but at higher volume value. One patient with a smaller-than-normal initial intracranial volume was surgically treated at a very young age and, despite cranial expansion surgery, postoperative volume did not reach normal levels. It is postulated that this was due to the fact that the operation was performed at a time when craniosynostosis was still active., Conclusions: The results of this study indicate that the underlying mechanism leading to craniosynostosis and constriction of head volume "exhausts" its effect during the first few months of life. Measurement of intracranial volume in clinical practice could be used to "fine tune" the optimum time for surgery. In late-presenting children, this may be useful in predicting possible recurrence.

Object: The origin of chronic communicating hydrocephalus following subarachnoid hemorrhage (SAH) is not well understood. Fibrosis of the arachnoid villi has been suggested as the cause for obstruction of cerebrospinal fluid (CSF) flow, but this is not well supported in the literature. The goal of this study was to determine the relationship between blood, inflammation, and cellular proliferation in arachnoid villi after SAH., Methods: Arachnoid villi from 50 adult patients were sampled at autopsy. All specimens were subjected to a variety of histochemical and immunohistochemical stains. The 23 cases of SAH consisted of patients in whom an autopsy was performed 12 hours to 34 years post-SAH. Fifteen cases were identified as moderate-to-severe SAH, with varying degrees of hydrocephalus. In comparison with 27 age-matched non-SAH controls, the authors observed blood and inflammation within the arachnoid villi during the 1st week after SAH. Greater mitotic activity was also noted among arachnoid cap cells. The patient with chronic SAH presented with ventriculomegaly 2 months post-SAH and exhibited remarkable arachnoid cap cell accumulation., Conclusions: The authors postulate that proliferation of arachnoidal cells, triggered by the inflammatory reaction or blood clotting products, could result in obstruction of CSF flow through arachnoid villi into the venous sinuses. This does not exclude the possibility that SAH causes generalized fibrosis in the subarachnoid space.

Object: Cerebellar astrocytomas are benign tumors of childhood known to be associated with excellent long-term survival in patients in whom complete surgical resection is possible. However, the roles of other factors--clinical, radiological, histological, and therapeutic--in the survival of the patient, tumor recurrence, and long-term patient outcome remain imprecise. The goal of this study was to examine these factors and their relationships., Methods: To clarify these issues a retrospective review was conducted of 168 children who were surgically treated for a cerebellar astrocytoma at Hôpital Necker-Enfants Malades between 1955 and 1995. These patients' clinical files were examined, the histological characteristics of their tumors were reviewed, and their outcomes were assessed according to Bloom's scale and the Wechsler intelligence quotient test. Of the 168 patients in the study, 91 were male and 77 were female with a mean age of 6.9 years and a mean follow up lasting 7.7 years. Tumors were identified as being strictly located in the cerebellum in 76.2% of the patients and as involving the brainstem (referred to as the "transitional form") in 23.8% of the patients. Complete surgical excision was possible in 88.7% of cases. There was a total mortality rate of 4.2% and a tumor recurrence rate of 9.5%. Fifty-eight percent of the patients had no neurological sequelae at follow-up evaluation. Pejorative factors that were discovered by multivariate analysis to be important included: a long preoperative duration of symptoms and the transitional form of tumor with respect to survival; incomplete tumor excision with respect to an increased risk of recurrence; and a long preoperative duration of symptoms, an early epoch during which surgery was performed (1955-1974), severe ventricular dilation, and the transitional form of tumor with respect to a poorer long-term patient outcome., Conclusions: The presence of brainstem involvement (tumor in the transitional form) emerged as a significant negative prognostic factor and should be treated as a distinct nosological entity. The extent of surgical excision has a significant bearing on the risk of tumor recurrence.

Object: The incidence of epilepsy among children with hydrocephalus and its relation to shunts and their complications, raised intracranial pressure (ICP), and developmental outcome are explored in a retrospective study., Methods: The authors studied a series of 802 children with hydrocephalus due to varying causes, who were treated by ventriculoperitoneal shunt placement between 1980 and 1990, with a mean follow-up period of 8 years. Patients who had tumoral hydrocephalus and those whose files lacked significant data were excluded. Data extracted from medical records, including history of the hydrocephalus and history of seizures, if any, were analyzed. Thirty-two percent of the children had epilepsy, the onset of which frequently occurred at approximately the same time that the diagnosis of hydrocephalus was made. The majority of the affected children had severe uncontrolled epilepsy. The incidence of epilepsy was significantly affected by the original cause of the hydrocephalus. The presence of radiological abnormalities was also found to be a significant predictor of epilepsy. Similarly, shunt complications predisposed to epilepsy. Episodes of raised ICP related to hydrocephalus or in association with shunt malfunction may also predispose to epileptic seizures. Furthermore, the presence of a shunt by itself seems able to promote an epileptogenic focus. Finally, epilepsy appears to be an important predictor of poor intellectual outcome in hydrocephalic children with shunts., Conclusions: A prospective study is needed to identify clearly and confirm avoidable factors predisposing to seizures in these children so that we can strive to reduce the incidence of these seizures and, subsequently, improve these children's quality of life.

The authors examined whether measurement of resistance to outflow of cerebrospinal fluid (Rcsf) predicts outcome after shunting for patients with normal-pressure hydrocephalus (NPH). In four centers 101 patients (most of whom had idiopathic NPH) who fulfilled strict entry criteria underwent shunt placement irrespective of their level of Rcsf obtained by lumbar constant flow infusion. Gait disturbance and dementia were quantified by using an NPH scale and the patient's level of disability was assessed by using the modified Rankin scale (mRS). In addition the Modified Mini-Mental State Examination was performed. Patients were assessed prior to and 1, 3, 6, 9, and 12 months after surgery. Primary outcome measures were based on differences between the preoperative and last NPH scale scores and mRS grades. Improvement was defined as a change measuring at least 15% in the NPH scale score and at least one mRS grade. Intention-to-treat analysis of all patients at 1 year yielded improvement for 57% in NPH scale score and 59% in mRS grade. Efficacy analysis, excluding serious events and deaths that were unrelated to NPH, was performed for 95 patients. Improvement rose to 76% in NPH scale score and 69% in mRS grade. Six cut-off levels of Rcsf were related to improvement in NPH scale score using two-by-two tables. Positive predictive values were approximately 80% for an Rcsf of 10, 12, or 15 mm Hg/ml/minute, 92% for an Rcsf of 18 mm Hg/ml/minute, and 100% for an Rcsf of 24 mm Hg/ml/minute. Negative predictive values were low. More important was the highest likelihood ratio of 3.5 for an Rcsf of 18 mm Hg/ml/minute. Extensive comorbidity was a major prognostic factor. Measurement of Rcsf reliably predicts outcome if the limit for shunting is raised to 18 mm Hg/ml/minute. At lower Rcsf values the decision depends mainly on the extent to which clinical and computerized tomography findings are typical of NPH.

The authors report three adult patients who developed a symptomatic extraaxial collection of cerebrospinal fluid (CSF) after an intracranial hemorrhage. The fluid shifted from the extraaxial into the ventricular space as the patients' symptoms progressed. The symptoms resolved after placement of a ventriculoperitoneal shunt. External hydrocephalus, which is frequently observed in children, had not yet been described in adults. It is important to differentiate chronic subdural collections from external hydrocephalus, because ventricular CSF shunting increases the former while it is the treatment for the latter. The authors believe that symptomatic extraaxial fluid collections developed in these three adults during the early phase of posthemorrhagic hydrocephalus because the ventricles presented great resistance to distention at the onset of hydrocephalus. Animal experiments have led to the same result.

The authors present a case of secondary amenorrhea in a 32-year-old woman found to have noncommunicating hydrocephalus due to aqueductal stenosis. Although the presentation of hydrocephalus with amenorrhea has been previously reported, this association remains rare. After treatment via endoscopic third ventriculocisternostomy, the patient resumed normal menstruation and all hormonal abnormalities have resolved except hypothyroidism. A review of the literature on the etiology of endocrinological disturbances in patients with hydrocephalus is presented.

Diffuse villous hyperplasia of the choroid plexus, which is distinct from bilateral choroid plexus papillomas, is extremely rare and is often associated with hydrocephalus due to the overproduction of cerebrospinal fluid (CSF). The authors describe an infant with hydrocephalus, diagnosed by computerized tomography scanning, who developed ascites following placement of a ventriculoperitoneal shunt and, subsequently, demonstrated excessive CSF production when the shunt was externalized. The patient was later successfully treated by placement of a ventriculoatrial shunt. Magnetic resonance imaging demonstrated diffuse villous hyperplasia of the choroid plexus as the cause of the patient's hydrocephalus. The literature on diffuse villous hyperplasia of the choroid plexus is reviewed.

In a prospective open study, 15 patients with hydrocephalus secondary to cysticercosis who required insertion of a ventriculoperitoneal (VP) shunt were treated with 50 mg of prednisone given orally three times a week. Treatment began in the 1st postoperative week, with isoniazid and pyridoxine administered daily as antituberculous chemoprophylaxis. The drug regimen was continued with close follow up for 24 months. Clinical status, Karnofsky performance status (KPS) scores, and postoperative course in the prednisone-treated group were compared with 30 control patients with hydrocephalus due to cysticercosis. The control patients were matched by age and sex, underwent surgical shunting in the same period, and were followed routinely by the neurosurgery staff. Lumbar cerebrospinal fluid (CSF) was studied in 2, 16, and 32 weeks postoperatively in the prednisone group. At 24-month follow up two (13%) of 15 patients in the prednisone group and 19 (60%) of 30 patients in the control group required surgical shunt revisions for symptomatic shunt obstruction (p=0.002). Follow-up studies of CSF performed at 32 weeks in the prednisone group revealed improvement of abnormal values with statistically significant differences for glucose (p<0.02). Serial imaging studies in the prednisone group revealed persistence of cysticercal cysts with no change in size. Mean initial KPS scores were similar in both groups. At the end of the follow-up period, the mean KPS score was significantly higher in the prednisone group (p=0.003). Prednisone and chemoprophylactic drugs were well tolerated. These results suggest that in selected patients with hydrocephalus secondary to cysticercosis, intermittent long-term prednisone therapy after VP shunting may reduce shunt malfunction and improve the functional status of the patients.

The incidence of chronic tonsillar herniation (CTH) was evaluated with magnetic resonance imaging in 44 patients with Crouzon's syndrome and 51 with Apert's syndrome; the incidence was 72.7% in Crouzon's syndrome and 1.9% in Apert's syndrome. All the patients with Crouzon's syndrome and progressive hydrocephalus had CTH, but of 32 individuals with Crouzon's syndrome and CTH, only 15 had progressive hydrocephalus. Five patients with Apert's syndrome were treated for progressive hydrocephalus; none had CTH. The patterns of suture closure in these two groups of patients were studied, and significant differences in coronal, sagittal, and lambdoid sutures were found between patients with Crouzon's and Apert's syndromes. In Crouzon's syndrome, significant differences in the pattern of lambdoid suture closure were found between the groups with and without CTH; in the group with CTH, the lambdoid closure appeared earlier. The authors propose that the high incidence of individuals with CTH who have Crouzon's syndrome is related to the premature synostosis of the lambdoid suture in the first 24 months of age.

This report summarizes neuropathological, clinical, and general autopsy findings in 105 individuals with nonneoplastic syringomyelia. On the basis of detailed histological findings, three types of cavities were distinguished: 1) dilations of the central canal that communicated directly with the fourth ventricle (47 cases); 2) noncommunicating (isolated) dilations of the central canal that arose below a syrinx-free segment of spinal cord (23 cases); and 3) extracanalicular syrinxes that originated in the spinal cord parenchyma and did not communicate with the central canal (35 cases). The incidence of communicating syrinxes in this study reflects an autopsy bias of morbid conditions such as severe birth defects. Communicating central canal syrinxes were found in association with hydrocephalus. The cavities were lined wholly or partially by ependyma and their overall length was influenced by age-related stenosis of the central canal. Non-communicating central canal syrinxes arose at a variable distance below the fourth ventricle and were associated with disorders that presumably affect cerebrospinal fluid dynamics in the spinal subarachnoid space, such as the Chiari I malformation, basilar impression, and arachnoiditis. These cavities were usually defined rostrally and caudally by stenosis of the central canal and were much more likely than communicating syrinxes to dissect paracentrally into the parenchymal tissues. The paracentral dissections of the central canal syrinxes occurred preferentially into the posterolateral quadrant of the spinal cord. Extracanalicular (parenchymal) syrinxes were found typically in the watershed area of the spinal cord and were associated with conditions that injure spinal cord tissue (for example, trauma, infarction, and hemorrhage). A distinguishing feature of this type of cavitation was its frequent association with myelomalacia. Extracanalicular syrinxes and the paracentral dissections of central canal syrinxes were lined by glial or fibroglial tissue, ruptured frequently into the spinal subarachnoid space, and were characterized by the presence of central chromatolysis, neuronophagia, and Wallerian degeneration. Some lesions extended rostrally into the medulla or pons (syringobulbia). Although clinical information was incomplete, simple dilations of the central canal tended to produce nonspecific neurological findings such as spastic paraparesis, whereas deficits associated with extracanalicular syrinxes and the paracentral dissections of central canal syrinxes included segmental signs that were referable to affected nuclei and tracts. It is concluded that syringomyelia has several distinct cavitary patterns with different mechanisms of pathogenesis that probably determine the clinical features of the condition.

The case of a 50-year-old man with tetraventricular hypertensive hydrocephalus is presented, remarkable for fourth ventricle dilatation. This patient showed a significant sialorrhea as the main symptom, which is quite unusual. This condition was successfully treated by cerebrospinal fluid diversion. The uncommon features of this case are summarized.

Six patients with achondroplasia and symptoms suggestive of cervicomedullary junction compression are reviewed; these included three females and three males, with an average age of 8 years (range 7 months to 30 years). The mean duration of symptoms prior to intervention was 1.9 years. Symptoms included occipitocervical pain, ataxia, incontinence, apnea, and respiratory arrest. Radiological investigations consisted of plain films with flexion and extension views, pluridirectional tomography, thin-section computerized tomography, and magnetic resonance imaging. Typical findings included marked foramen magnum stenosis, ventrolateral cervicomedullary junction compression secondary to central and paramesial basilar invagination, and dorsal cervicomedullary junction compression secondary to ligamentous hypertrophy and invagination of the posterior atlantal arch. All patients underwent posterior fossa decompression and atlantal laminectomy. Surgery consistently revealed marked dorsal and paramesial overgrowth of the rim of the rim of the foramen magnum, with thickening and invagination of the atlantal posterior arch and a dense fibrotic epidural band resulting in dorsal cervicomedullary compression. Intraoperative ultrasonography was used to determine the extent of decompression required. Three patients required duraplasty. Three patients had concurrent hydrocephalus, two of whom had undergone ventriculoperitoneal shunting prior to surgical decompression of the posterior fossa. One patient developed a pseudomeningocele postoperatively, requiring serial lumbar punctures before it resolved. No patient developed craniovertebral instability following decompression. Improvement or resolution of symptoms was noted in all patients, with an average follow-up period of 4.8 years. Thus, cervicomedullary compression in patients with achondroplasia can be successfully treated with dorsal decompression of the craniovertebral junction. Dense epidural fibrotic bands are frequently noted in these cases and must be aggressively released to ensure satisfactory decompression.

A review of 51 cases referred for evaluation of fetal ventriculomegaly revealed adequate follow-up data in 40 patients. Three of the fetuses were electively aborted and 37 were delivered. There were no cases of death in utero. Of the 37 infants who were delivered, 26 (70%) were treated with shunt placement for neonatal hydrocephalus. The reasons for nontreatment included: inaccurate diagnosis, resolution of hydrocephalus by the time of delivery, neonatal death, and parental wishes. Survival time in the 26 shunt-treated patients ranged from 5 days to 14 years (average 4.25 years); five of these patients died within the first 2 weeks of life. Among the 26 treated patients, satisfactory cognitive ability was found in 10 (38%). Of the seven surviving nontreated infants, satisfactory cognitive ability was demonstrated in six (86%). Children with myelomeningocele displayed cognitive development similar to that in patients without myelomeningocele. The findings suggest that, of patients with in utero diagnosis of ventriculomegaly, approximately one-half survive (4-year average follow-up interval) and 38% of the survivors treated with shunt insertion have normal cognitive development. No subgroup likely to benefit from in utero treatment of ventriculomegaly was identified. Associated central nervous system or systemic malformations were identified in 26 (70%) of the 37 who came to delivery. This community-acquired series, not collected from a high-risk obstetrical-perinatal service, may reflect the general experience of the neurosurgeon consulting in such cases.

Given the widespread use of continuous external ventricular drainage in patients with aneurysmal subarachnoid hemorrhage (SAH), this investigation was undertaken to define the relationship of ventricular drainage to aneurysmal rebleeding. A historical cohort study of 128 patients with confirmed aneurysmal SAH was performed using a multivariate stepwise logistic regression analysis to examine the relationship between aneurysmal rerupture and ventricular drainage, while controlling for important clinical and radiological independent variables. The variables for ventricular drainage selected in the regression analysis were clinical grade, aneurysm size, and presence of hydrocephalus. The rate of rerupture was significantly higher in cases with ventricular drainage (odds ratio 5.31:1, p less than 0.05), poor clinical grade (odds ratio 4.90:1, p less than 0.02), and large aneurysm size (odds ratio 11.25:1, p less than 0.01). The significant effect of ventricular drainage was limited to patients with hydrocephalus. The increased risk of aneurysmal rebleeding in patients undergoing ventricular drainage may result from both: 1) a rise in aneurysmal transmural pressure, since intracranial pressure is lowered by ventricular drainage; and 2) an association between ventricular drainage and a more severely disrupted aneurysm which is more prone to rebleed as part of its natural history. This study found an increased risk of aneurysmal rebleeding among patients undergoing ventricular drainage, particularly in the presence of hydrocephalus.

The case of a 20-year-old man with obstructive hydrocephalus who suffered multiple shunt failures and shunt revisions is presented. The patient gradually developed a clinical syndrome of akinetic mutism. This behavioral syndrome failed to respond to shunt revisions, but did improve after the administration of a combination of bromocriptine and ephedrine.

The clinical features and pathophysiology of specific forms of hydromyelia are analyzed in this report together with the chronological changes of associated hydrocephalus. Nine patients were studied; all had hydromyelia with varying degrees of associated hydrocephalus. Clinically applicable classification systems were used to evaluate the progression of hydrocephalus (Stages I to IV) and to define the compartment isolated after shunting in the previously communicating cerebral ventricles (Types I to IV). Four patients had Stage IV disease (holoneural canal dilatation); one had Stage II and four had Stage I disease (both Stages I and II with supratentorial hydrocephalus). All patients were initially treated by ventriculoperitoneal shunting at an average age of 9.9 years. Five patients had progressive spinal symptoms before or after treatment of their hydrocephalus. Two patients had Type III isolation (an isolated rhombencephalic ventricle) with a functioning ventricular shunt; ventriculography confirmed a communication between the fourth ventricle and the hydromyelia, and both patients improved after placement of a shunt in the fourth ventricle. The remaining patients had Type IV isolation (isolated central canal dilatation) with a functioning ventricular shunt. This study indicates that in some cases the pathophysiology of hydromyelia is closely related to associated hydrocephalus. A new concept of the development of an isolated compartment after shunting is proposed to explain the progression of hydromyelia in these cases.

A 10-month-old child with achondroplasia with progressive head enlargement, ventriculomegaly, and wide subarachnoid spaces over the hemispheres was referred for evaluation. A steady-state lumbar infusion test revealed increased cerebrospinal fluid (CSF) outflow resistance (14 mm Hg/ml/min), and intra-arterial digital subtraction angiography (DSA) demonstrated bilateral venous outflow obstruction due to stenosis of the jugular foramen. Surgical decompression by opening the right jugular foramen relieved the clinical signs of intracranial hypertension. During the following year, the patient's head enlargement was moderate with relative normalization of size. Repeat DSA demonstrated improved venous runoff on the right side, and a steady-state lumbar infusion test demonstrated reduced CSF outflow resistance (10 mm Hg/ml/min). Venous decompression is causal therapy and may prove to be preferable to shunting in children with hydrocephalus and bilateral stenosis of the jugular foramen.

A 49-year-old woman presented with orthostatic hypotension. Vertebral angiography and ventriculography revealed a tumor of the left cerebellar hemisphere. Ventriculoperitoneal shunting was followed by complete disappearance of the orthostatic hypotension. The tumor was subsequently removed and microscopic study showed Lhermitte-Duclos disease. Orthostatic hypotension has been rarely reported in association with tumors of the posterior fossa except for those tumors destroying the medullary centers and interrupting the baroreceptor reflex arc. This case is of interest because the tumor was restricted to the cerebellum. The authors have found no previous case in which orthostatic hypotension was a presenting symptom of Lhermitte-Duclos disease.