Lumboperitoneal shunt for treatment of dural ectasia in ankylosing spondylitis

doi:10.1016/j.jocn.2007.09.003

Journal of Clinical Neuroscience

Volume 15, Issue 10, October 2008, Pages 1179-1182

https://doi.org/10.1016/j.jocn.2007.09.003 Get rights and content

Abstract

Neurological complications of ankylosing spondylitis (AS) are reported in 2.1% of patients. Cauda equina syndrome (CES) is rare and occurs at the ankylosing stage. MRI and CT of the lumbar spine show a cauda equina deformation with dural ectasia and bony erosion. We report three patients with AS presenting with progressive CES. These patients underwent lumboperitoneal shunting (LPS) surgery. The motor deficit improved in all cases. We suggest that CES develops from arterial pulsation of the CSF on a dural sac with reduced elasticity and that LPS reduces these intradural pressure shock waves. A meta-analysis by Ahn et al. [Ahn NU, Ahn UM, Nallamshetty L, et al. Cauda equina syndrome in ankylosing spondylitis (the CES-AS syndrome): meta-analysis of outcomes after medical and surgical treatments. J Spinal Disord 2001;14:427–33] concludes that surgical treatment has a better outcome than conservative or no treatment. Adding our 3 patients to this analysis, it appears that LPS for CES in AS is more efficient than laminectomy. LPS is a routine procedure for a rare indication, which promises improvement or atleast a stabilization of this disabling evolution of the disease.

Introduction

Ankylosing spondylitis (AS) belongs to the group of seronegative spondylarthropathies. Our clinical diagnosis is based on negative serum markers and on the presence of lumbar inflammatory pain or synovitis and at least one of the following criteria (as recommended by the European Spondylarthropathy Study Group): buttock pain, sacroilitis, an enthesopathy, a family history, psoriasis, an inflammatory bowel disease or an arthritis followed within a month by urethritis, cervicitis or acute diarrhea. Neurological complications of AS are rare and occur in 2.1% of patients.¹ The cause is most often medullary compression by pseudarthrosis, vertebrae fracture or atlanto-axial subluxation. Additionally, cauda equina syndrome (CES) may occur, which is a described complication only at the ankylosing stage. It is rare and spontaneously evolving.[2], [3] MRI and CT show typically a cauda equina deformation with dural ectasia and bony erosions; the neural structures are often herniated in arachnoid diverticulae and compressed.[4], [5], [6] This article describes our experience with lumbo-peritoneal shunting (LPS) for treatment of this rare condition. We report three cases and compare outcomes with conservative and other surgical treatments reported in literature.

Section snippets

Materials and methods

Three patients presented with AS at the ankylosing stage. All patients had been followed in a rheumatology department over the last thirty years. They were referred to our institution as they were complaining of a slowly evolving CES. Patients were operated at the Department of Neurosurgery, Lariboisière’s Hospital (Paris, France). This is a retrospective analysis based on medical records, operative reports and outpatient clinics reports. The age, sex, duration since AS diagnosis, initial

Results

No post-operative complications occurred. One patient complained of transient superficial itching at the abdominal wound. Neurological progress after the surgical procedure with follow-up of at least eight months is shown in Table 2. The motor deficits improved in all patients. The sensory deficits evolved differently: patient 1, with hypoesthesia, developed buttock deafferentation pain; patient 2, initially without sensory deficit, developed transient sciatica after 8 months; patient 3

Discussion

Cauda equina syndrome in AS patients with dural ectasia develops slowly and has a spontaneous, progressive course. The radiological findings are typical with dural ectasia at several levels of the lumbar spine and with fluid collection on MRI of the same intensity as the cerebrospinal fluid (CSF); there are also erosions of the posterior walls of the vertebrae which is characteristic of a slowly evolving process (Fig. 2). Some degree of tethering of the spinal cord also occurs.

Several

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Cited by (16)

Surgical “Fat Patch” Improves Secondary Intracranial Hypotension Orthostatic Headache Associated with Lumbosacral Dural Ectasia
2017, World Neurosurgery
Citation Excerpt :
One case of spontaneous IH due to a thoracic extradural diverticulum causing invalidating orthostatic headache has been successfully treated by an open surgical ligation of the diverticulum.17 Another report describes treatment of lumbosacral dural ectasia with a lumboperitoneal shunt.18 Nguyen et al19 have described another case where an abundant injection of fibrin glue into the thecal sac distal to S2 and the rostral marsupialization of the dural ectasia effectively obliterated the distal thecal sac, reducing the size of the dural space.
Secondary intracranial hypotension is a clinical syndrome associated with reduction of cerebrospinal fluid volume and dural continuity violation. The main symptoms are orthostatic headache associated with nausea, vomiting, diplopia, dizziness, and tinnitus. The treatment is usually nonspecific.
A 37-year-old woman developed secondary intracranial hypotension caused by lumbosacral iatrogenic dural ectasia following detethering surgery. An orthostatic headache was the mainstay of her clinical picture, and it was confirmed by intracranial pressure monitoring. Conservative treatment including spinal blood patch improved symptoms for a limited amount of time (<1 month). Altered compliance of the dural spinal sac was suspected. Therefore thecal sac remodeling by placing autologous fat at the level of the dural ectasia was performed, improving the symptoms for 2 years.
Volumetric reduction of the epidural space may be considered as a valuable therapeutic option in case of intracranial hypotension that is unresponsive to medical treatments and spinal blood patch, as well as when an altered compliance of the dural sac is hypothesized.
Cauda equina syndrome associated with longstanding instrumented spinal Fusion
2013, World Neurosurgery
Citation Excerpt :
Some patients undergoing laminectomy might benefit from a postoperative dural leak with communication between the caudal sac and epidural space, which could secondarily reduce CSF pulse pressure (7). Lumboperitoneal shunt placement appears to be the preferred treatment for CES in AS, although for most patients the procedure halts progression of the neurologic signs and symptoms and only a minority improve (1, 4-6). Stabilization of the neurologic signs and symptoms may be all that can be expected from treatment of this condition given the duration and mechanism of the lumbosacral nerve root damage and the limited ability of these spinal nerves to regenerate (6, 7).
This study sought to describe a patient with a slowly progressive cauda equina syndrome (CES) who had imaging findings of the CES that occurs as a rare complication of ankylosing spondylitis (AS), but whose spinal fusion was due to thoracolumbosacral instrumentation, not spondyloarthropathy.
A 53-year-old woman presented with a 5-year history of slowly progressive CES affecting sensory and sphincter more than motor function many years after sequential instrumented fusions of her thoracic and then lumbosacral spine. Imaging studies showed enlargement of the caudal sac and dorsal arachnoid diverticula eroding into posterior elements of the lumbar spine similar to findings reported in patients with CES in longstanding AS. Despite extensive evaluation, no other cause for her CES was found. We postulate that our patient's CES is on the same pathophysiologic basis as that proposed for patients with longstanding AS (fibrosis of peridural tissues with increase in transmitted cerebrospinal fluid pulse pressure). Because of reports that patients with CES due to longstanding AS are helped by placement of a lumboperitoneal shunt, our patient underwent this procedure.
Seventeen months postoperatively, the patient's symptoms and neurologic examination are unchanged, and her previous slow progression has been halted.
This case strongly suggests that extensive surgical thoracolumbosacral fusion can, rarely and after many years, be associated with lumbosacral nerve root damage and a slowly progressive CES that is similar to that seen in patients with spinal fusion due to longstanding AS.
Cauda equina syndrome in the setting of longstanding instrumented spinal fusion
2013, World Neurosurgery
Anterior dural ectasia associated with cauda equine syndrome in ankylosing spondylitis: a case report
2020, Research Square
Dural Ectasia in Neurofibromatosis 1: Case Series, Management, and Review
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Cauda equina syndrome in ankylosing spondylitis: Challenges in diagnosis, management, and pathogenesis
2019, Journal of Rheumatology

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Case ReportLumboperitoneal shunt for treatment of dural ectasia in ankylosing spondylitis

Abstract

Introduction

Section snippets

Materials and methods

Results

Discussion

Clin Radiol

Nervous system involvement in ankylosing spondylitis

BMJ

Symptomatic spinal stenosis associated with ankylosing spondilitis

Neurosurgery

Ankylosing spondylitis and other B27 related pondylarthropathies

Baillieres Clin Rheumatol

Imaging findings in patients with ventral dural defects and herniation of neural tissue

Eur Radiol

Case Report
Lumboperitoneal shunt for treatment of dural ectasia in ankylosing spondylitis