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Neurology, Neurosurgery, and Psychiatry 1995;58:649-654 649 Journal ofNeurology, Neurosurgery, and Psychiatry 1995;58:649-654 649 Journal of J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.6.649 on 1 June 1995. Downloaded from NEUROLOGY NEUROSURGERY & PSYCHIATRY Editorial The cystic spinal cord The spinal cord with cystic cavities and spaces within it is lum. There is a likelihood, however, that free fluid truly an intriguing sight. These cavities, when they are exchange occurs across the walls of the cystic cord in symptomatic, are an exclusively surgical problem.' spinal tumours. This is suggested by the speed with Modem surgery can transect the cord, make apertures in which intrathecal contrast material enters the cystic syrinxes, cut off the filum terminale, block up holes, open cavities as seen on postmyelographic CT. out subarachnoid spaces, and drain CSF from ventricles, subarachnoid spaces, or the syrinxes themselves to many other sites with adjustable control of pressure differen- tials. Surgery done in a timely way, and done well, may The cord: achieve enormous success in preventing crippling disor- cystic spinal classification according to associated conditions der and pain. Classification % Understanding of the nature of any phenomenon may Hindbrain related syringomyelia 72 be aided by classification. Because the Hindbrain herniation physical causation Idiopathic herniation (Chiari type 1) 32 of intracord cystic cavities is ill understood, the classifica- Secondary to birth injury 39 tion I propose is based on the Secondary to tumours 1-2 existence of associated Bony or meningeal tumours of the posterior fossa lesions. These associated anomalies are therefore not Tumours forming the hindbrain hernia causes the Intrinsic brain tumours above the lower fourth necessarily of cysts, but the associations may Secondary to bony abnormality suggest causes (table). Basilar invagination (idiopathic or birth injury) 10 Microscopy of the cavities mostly shows gliosis of the Sclerosteosis lining with the direction of fibres mostly annular. The Rickets Acro-osetolysis http://jnnp.bmj.com/ septations are not histologically do not Osteogenesis imperfecta intriguing. They Associated with hydrocephalus 10 always contain vessels in their edges, and there is no Intracranial arachnoid pouches 2 evidence that they contain fibres of the Dandy-Walker cysts intraspinally Early onset hydrocephalus (aqueduct stenosis) decussating spinothalamic tracts. Meningeal fibrosis of cisterna magna The fluid seems to Birth injury related 9 develop the cavities by cutting Postinflammatory through sites of structural weakness.23 Associated lesions Post-traumatic (postnatal) such as hindbrain herniation may be associated with the Infections Non-hindbrain related cases 22 presence of excess fluid inside the cervical cord before a Spinal tumours 1 on September 28, 2021 by guest. Protected copyright. clear cavity Intramedullary 1 develops. Cysts wholly or partly within the tumour The location and lining of the cavities has provoked Cysts outside the tumour extending in the cord discussion relating to whether or not the Extramedullary intradural tumours central canal is Extradural tumours, including disc disease involved. Nomenclature has varied and the suggestion Meningeal fibrosis has been made that Idiopathic "hydromyelia" might describe the Postinflammatory cavities when lined with ependyma. Human cases with Pyogenic meningitis intrauterine hydrocephalus and Epidural abscess 13 hydromyelia, usually Tuberculous meningitis associated with spina bifida, may have the cavities partly Myodil (Pantopaque) lined with ependyma but in most Post-traumatic human cases the cen- Secondary to spinal bony deformities 11 tral canal and the ependyma are irrelevant. The fluid Post-traumatic tracks readily either within or outside Tuberculous bone disease the canal. The term Idiopathic scoliosis "hydrosyringomyelia" has been suggested. Syringomyelia Dysraphic lesions has the advantage of familiarity, however, and authors With hydrocephalus 2 With hindbrain hernia 1 who prefer to use other terms should define them. With both the above 4 Tumours may be associated with syringomyelia cavi- With neither of the above 7 ties. These are usually intramedullary tumours and in Unknown cause (no associated conditions) some of them, notably haemangioblastomas, it seems Percentages are from a database of syringomyelia and related diseases. Diagnosis with no percentage given have an incidence of less than 1%. They likely that the cystic cord is due to secretion of proteinous do not add up to 100% because of intersections-for example, post-traumatic fluid such as may be thought responsible for similar cysts syringomyelia is associated with meningeal fibrosis and bony deformity. Some factors such as head injury are difficult to interpret. Others such as hydro- that are found with haemangioblastomas in the cerebel- cephalus, spina bifida occulta, or basilar impression are matters of degree. 650 Williams J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.58.6.649 on 1 June 1995. Downloaded from A further class of difficult cases occurs in association equilibrium at work in the established syringomyelia with with spina bifida.4 Hindbrain hemiation is not a part of some substances, certainly including water, moving freely the dysraphic process but consequent on it, and when it both ways across syrinx walls. is present the cases are usually variants of hindbrain One variety of syringomyelia that seems perhaps the related syringomyelia and treatment is straightforward, simplest of all is the syrinx found in association with a ventricular shunting for associated hydrocephalus being single web, usually dorsal to the cord, which forms an the most useful first step. Difficulties arise with spina almost complete transverse blockage across the subarach- bifida occulta, or sometimes spina bifida manque; that is noid space. These webs are usually idiopathic. to say, dysraphic lesions which lie between the occult and Commonly the morphology of the web shows the effect the full myelocele or myelomeningocele. Such of pressure surges in the CSF with a convexity, usually syringomyelia may be part of the dysraphic process-that directed upwards. Fluid which surges violently into the is, the failure of the cord to close and the place of concave side of the web tends to close off the surround- surgery is sometimes problematic. Intraspinal lipoma at ing spaces as the pouch like structure expands. They do the plaque site seems to be a cause of syringomyelia. The not show well on MRI directly, but they may be associ- place of "tethering" remains unclear in both the dys- ated with a displacement of the cord. Sometimes there raphic and posttraumatic varieties of syringomyelia. are several webs tending to retain fluid and the pathology How do these cystic cavities fill? There is somewhere may come to resemble a pouch in the subarachnoid within the human CNS, a group of neurons that derives space. Myelography shows them best but the associated satisfaction from a good explanation. Many people will syrinx and displacement of the cord are well appreciated not do things unless they have an explanation which pro- by MRI. Such webs may have a syrinx either above or vides them with reinforcement for their motivation. This below, sometimes both. The syrinx commonly does not human anfractuosity is the basis of religions, of interpre- reach up to, or apparently start from the level of the web tations of history, criticisms of the arts, and the livings but often starts 1 cm to 2 cm away from it (fig 1). gained by those who tell us exactly which were the moves The analogies with a cervical syrinx related to the that led Short to be defeated by Kasparov. In neuro- hindbrain that extends to within a few cm of the surgery there is no better illustration of the delights of impacted hindbrain are clear. In the normal human the proposing an explanation than the medical literature on upward surge of CSF produced by raised intraspinal the causation of syringomyelia. Consider the hindbrain pressure is vented via the cisterna magna to the intracra- related cases first. I recollect the satisfaction I gained nial compartment. This has a capacitance afforded to it from the "communicating hypothesis" in 1967.6 The fact mostly by compressibility of the venous system. If the that the proposal was wrong about the way in which the foramen magnum is plugged by tonsils, or by dense venous pressure impinges on the neuraxis and that subse- meningeal fibrosis then the situation may be similar to quent investigations have shown that the "communica- that illustrated by fig 1. Instead of the intracranial capaci- tion" from the fourth ventricle to the syrinx is not present tance acting as a damper the fluid is immediately in most cases, barely dims the recollection of the pleasure bounced back down the spine. The continuing arrival of at providing such a splendid, rational, and temporarily the pressure wave from the lower spine is likely to lead to convincing explanation. This same group of neurons chaotic disturbances of fluid below the site of blockage continues to give satisfaction to those who write new with consequences that may produce local conditions of explanations. Such are published often and commonly pressure such that fluid may be forced into the spinal are preceded by an attack on Gardner's views7 8 or on the cord. 25 year old hypothesis mentioned.7 One feature that The pressure inside the cord with a syrinx is almost http://jnnp.bmj.com/ many of these articles have
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