Tetraventricular noncommunicating hydrocephalus: Case report and literature review

Background: Tetraventricular hydrocephalus is a common presentation of communicating hydrocephalus. Conversely, cases with noncommunicating etiology impose a diagnostic challenge and are often neglected and underdiagnosed. Herein, we present a review of literature for clinical, diagnostic, and surgical aspects regarding noncommunicating tetrahydrocephalus caused by primary fourth ventricle outlet obstruction (FVOO), illustrating with a case from our service. Methods: We performed a research on PubMed database crossing the terms “FVOO,” “tetraventriculomegaly,” and “hydrocephalus” in English. Fifteen articles (a total of 34 cases of primary FVOO) matched our criteria and were, therefore, included in this study besides our own case. Results: Most cases presented in adulthood (47%), equally divided between male and female. Clinical presentation was unspecific, commonly including headache, nausea, and dizziness as symptoms (35.29%, 21.57%, and 9.80%, respectively), with ataxic gait (65%) and papilledema (40%) being the most frequent signs. MRI and CT were the imaging modalities of choice (11 patients each), often associated with CSF flow studies, such as cine MRI and CT ventriculogram. Endoscopic third ventriculostomy (ETV) was both the most popular and effective surgical approach (50.85% of cases, with 18.91% of recurrence) followed by ventricle-peritoneal shunt (16.95% of patients, 23.0% of recurrence). Conclusion: FVOO stands for a poorly understood etiology of noncommunicating tetrahydrocephalus. With the use of ETV, these cases, once hopeless, had its morbimortality and recurrence reduced greatly. Therefore, its suspicion and differentiation from other forms of tetrahydrocephalus can improve its natural course, reinforcing the importance of its acknowledgment.

The pathophysiology of chronic noncommunicating hydrocephalus: lessons from continuous intracranial pressure monitoring and ventricular infusion testing

OBJECTIVEThe pathophysiology of chronic noncommunicating hydrocephalus (ncHC) is poorly understood. This present study explored whether lessons about the pathophysiology of this clinical entity might be retrieved from results of overnight monitoring of pulsatile and static intracranial pressure (ICP) and ventricular infusion testing.METHODSThe study cohort included adult patients (> 20 years of age) with chronic ncHC due to aqueductal stenosis in whom symptoms had lasted a minimum of 6 months. A reference cohort consisted of age- and sex-matched patients managed for communicating HC (cHC). Information about symptoms and clinical improvement following surgery was retrieved from a quality register, and results of overnight ICP recordings and ventricular infusion testing were retrieved from the hospital ICP database.RESULTSThe cohort with ncHC consisted of 61 patients of whom 6 (10%) were managed conservatively, 34 (56%) by endoscopic third ventriculostomy (ETV), and 21 (34%) using ETV and subsequent shunt surgery. In patients responding to surgery, pulsatile ICP (mean ICP wave amplitude) was significantly increased to a similar magnitude in patients with ncHC and the reference cohort (cHC). Furthermore, intracranial compliance (ICC) was reduced in clinical responders. The results of ventricular infusion testing provided evidence that patients responding to ETV have impaired ventricular CSF absorption, while those requiring shunt placement after ETV present with impaired CSF absorption both in the intraventricular and extraventricular compartments.CONCLUSIONSThe study may provide some lessons about the pathophysiology of chronic ncHC. First, increased pulsatile ICP and impaired ICC characterize patients with chronic ncHC who respond clinically to CSF diversion surgery, even though static ICP is not increased. Second, in patients responding clinically to ETV, impaired ventricular CSF absorption may be a key factor. Patients requiring shunt placement for clinical response appear to have both intraventricular and extraventricular CSF absorption failure. A subgroup of patients with ncHC due to aqueductal stenosis has normal ventricular CSF absorption and normal ICC and may not be in need of surgical CSF diversion.

Flow diversion of a recurrent, iatrogenic basilar tip aneurysm in a pediatric patient: case report

Endoscopic third ventriculostomy (ETV) is a common treatment for noncommunicating hydrocephalus. Although rare, vascular injury and traumatic pseudoaneurysm development during ETV have been reported. The authors present the case of a 13-year-old boy who underwent repeat ETV (rETV) for shunt and ETV failure, and who suffered an intraoperative subarachnoid hemorrhage due to iatrogenic injury to the basilar tip, with subsequent development of a pseudoaneurysm. Despite initial primary coil embolization, the aneurysm recurred and was definitively treated with flow diversion. In this report, the authors review complication rates associated with ETV and rETV as well as the emerging use of flow diversion and its applications in vessel reconstruction within the pediatric population.

Reduced subarachnoid fluid diffusion in enlarged subarachnoid spaces of infancy

Background and purpose Enlargement of the subarachnoid spaces in infancy (ESSI) is a common cause of macrocephaly without proven explanation. We have observed subarachnoid diffusion to be decreased in these patients. We aim to quantify the diffusivity of ventricular and subarachnoid cerebrospinal fluid in ESSI patients, to determine if diffusion characteristics deviate from normocephalic infants, and to propose a unique mechanism for ESSI. Materials and methods 227 consecutive brain magnetic resonance exams from different macrocephalic children were retrospectively reviewed after institutional review board waiver. Patients with noncommunicating hydrocephalus, substantial ventriculomegaly, atrophy, structural bone and/parenchymal abnormalities, abnormal brain signal, hemorrhages, meningitis, and normal imaging were excluded. A total of 53 exams from macrocephalic patients and 21 normocephalic subjects were analyzed. Mean quantitative apparent diffusion coefficient (ADC) values were obtained from the ventricular frontal horn and frontal subarachnoid spaces. The subarachnoid:ventricular ADC ratios were compared using a Mann–Whitney U-test. Results The mean age was 13 +/−8 months (macrocephalic cohort) and 13 +/− 6 months (normocephalic cohort). The subarachnoid fluid mean ADC was 2.50+/−0.26 × 10−3 mm2/s in the macrocephalic group and 2.84+/−0.29 × 10−3 mm2/s in the normocephalic group. The ventricular fluid mean ADC was 2.97+/−0.37 × 10−3 mm2/s and 2.74 +/−0.32 × 10−6 mm2/s, respectively. The mean quantitative ADC ratios in the macrocephalic group were 0.85, significantly smaller than the normocephalic group (1) ( z = −6.3; p = 0). Conclusion Subarachnoid space fluid diffusivity is reduced in patients with enlarged subarachnoid spaces of infancy. We propose insufficient frontotemporal capillary protein resorption to be the initiating factor in ESSI, leading to unbalanced osmotic/hydrostatic pressures, and secondary congestion.

Arne Torkildsen and the ventriculocisternal shunt: the first clinically successful shunt for hydrocephalus

Arne Torkildsen was a pioneering Norwegian neurosurgeon who introduced the ventriculocisternal shunt, the first clinically successful shunt for CSF diversion in hydrocephalus. The procedure, usually referred to as ventriculocisternostomy (VCS), Torkildsen’s operation, orTorkildsen’s shunt, became internationally recognized as an efficient operation for the treatment of noncommunicating hydrocephalus. The operation gained widespread use in the 1940s and 1950s before the introduction of extracranial shunts. In this paper, the authors look more closely at Torkildsen’s development of the VCS and examine how this surgical approach differed from other procedures for treating hydrocephalus before World War II. Long-term results of the VCS are presented.