Secondary Chiari malformation due to enlarged spinal arachnoid villi–like structure: illustrative case

BACKGROUNDSecondary Chiari malformation can be caused by various disorders associated with cerebrospinal fluid (CSF) leakage at the spinal level. In this report, the authors describe a rare case of secondary Chiari malformation caused by excessive CSF absorption through the enlarged spinal arachnoid villi–like structure.OBSERVATIONSA 20-year-old woman presented with progressive severe headache and posterior neck pain. Magnetic resonance imaging showed tonsillar herniation and decreased subarachnoid space around the spinal cord. A hypointense signal area was observed in the ventral spinal canal on a T2-weighted image. An axial image revealed multiple small, arachnoid cyst–like structures at the right T1 nerve root sleeve. Direct surgery revealed that the cyst-like structures were continuous with the arachnoid membrane and protruded into the abnormally large epidural venous sinus. The cyst-like structures were resected, and the dural sleeve was repaired using fascia. The patient showed good improvement of symptoms after surgery.LESSONSExcessive CSF absorption through the enlarged spinal arachnoid villi–like structure can cause secondary Chiari malformation. Neurosurgeons should be aware of this unusual mechanism of CSF leakage. Simple posterior fossa decompression will be ineffective or even harmful.

Human spinal arachnoid villi revisited: immunohistological study and review of the literature

Object Few have described the relationship between arachnoid protrusions (villi) and adjacent spinal radicular veins, and the descriptions that do exist are conflicting. Some authors have even denied the presence of spinal arachnoid villi, suggesting that they play no role in cerebrospinal fluid (CSF) absorption. Methods To further elucidate these structures, laminectomies from C-2 inferiorly to S-2 were performed in 10 fresh human adult cadavers. Following removal of the laminae, the dural nerve sleeves were identified and the spinal nerves excised 1 cm lateral and medial to the intervertebral foramina. Samples were submitted for histological and immunohistological analysis. Results The authors identified arachnoid villi in all specimens. The length of these structures was approximately 50 to 170 μm. Regionally, these villi were more concentrated in the lumbar region, but they were not present at every vertebral level, with observed skip zones. Occasionally, more than one villus was identified per vertebral level. The majority of villi were intimately related to an adjacent radicular vein. There was a direct relationship between the size of the adjacent radicular vein, and the presence and number of arachnoid villi. Conclusions Findings in the present study have demonstrated that arachnoid villi exist and are morphologically associated with radicular veins. These data support the theory that CSF absorption occurs not only intracranially but also along the spinal axis. Further animal studies are necessary to prove that CSF traverses these villi and is absorbed into the spinal venous system.

Cerebrospinal Fluid Transport: a Lymphatic Perspective

The textbook view that projections of the arachnoid membrane into the cranial venous sinuses represent the primary cerebrospinal fluid (CSF) absorption sites seems incompatible with many clinical and experimental observations. On balance, there is more quantitative evidence suggesting a function for extracranial lymphatic vessels than exists to support a role for arachnoid villi and granulations in CSF transport.

Does neonatal cerebrospinal fluid absorption occur via arachnoid projections or extracranial lymphatics?

Arachnoid villi and granulations are thought to represent the primary sites where cerebrospinal fluid (CSF) is absorbed. However, these structures do not appear to exist in the fetus but begin to develop around the time of birth and increase in number with age. With the use of a constant pressure-perfusion system in 2- to 6-day-old lambs, we observed that global CSF transport (0.012 ± 0.003 ml · min−1 · cmH2O−1) and CSF outflow resistance (96.5 ± 17.8 cmH2O · ml−1 · min) were very similar to comparable measures in adult animals despite the relative paucity of arachnoid villi at this stage of development. In the neonate, the recovery patterns of a radioactive protein CSF tracer in various lymph nodes and tissues indicated that CSF transport occurred through multiple lymphatic pathways. An especially important route was transport through the cribriform plate into extracranial lymphatics located in the nasal submucosa. To investigate the importance of the cribriform route in cranial CSF clearance, the cranial CSF compartment was isolated surgically from its spinal counterpart. When the cribriform plate was sealed extracranially under these conditions, CSF transport was impaired significantly. These data demonstrate an essential function for lymphatics in neonatal CSF transport and imply that arachnoid projections may play a limited role earlier in development.

Intracranial pressure accommodation is impaired by blocking pathways leading to extracranial lymphatics

Tracer studies indicate that cerebrospinal fluid (CSF) transport can occur through the cribriform plate into the nasal submucosa, where it is absorbed by cervical lymphatics. We tested the hypothesis that sealing the cribriform plate extracranially would impair the ability of the CSF pressure-regulating systems to compensate for volume infusions. Sheep were challenged with constant flow or constant pressure infusions of artificial CSF into the CSF compartment before and after the nasal mucosal side of the cribriform plate was sealed. With both infusion protocols, the intracranial pressure (ICP) vs. flow rate relationships were shifted significantly to the left when the cribriform plate was blocked. This indicated that obstruction of the cribriform plate reduced CSF clearance. Sham surgical procedures had no significant effects. Estimates of the proportional flow through cribriform and noncribriform routes suggested that cranial CSF absorption occurred primarily through the cribriform plate at low ICPs. Additional drainage sites (arachnoid villi or other lymphatic pathways) appeared to be recruited only when intracranial pressures were elevated. These data challenge the conventional view that CSF is absorbed principally via arachnoid villi and provide further support for the existence of several anatomically distinct cranial CSF transport pathways.

Human arachnoid villi response to subarachnoid hemorrhage: possible relationship to chronic hydrocephalus

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.