Cauda equina syndrome due to intradural lumbar disc herniation

Cauda equina syndrome due to intradural lumbar disc herniation

Intradural lumbar disc herniation is rare, with an incidence of 0.3%-1%, but has been well reported in the literature. Transdural migration of the disc penetrating both ventral and dorsal dura is extremely rare, and there is a dearth of literature in the pathophysiology and surgical management of transdural herniation. Lack of knowledge on this type of presentation can cause intraoperative surprises and inadvertent cauda equina root injuries and lead to prolonged operative time. 1).

Diagnosis

Intradural disc prolapse remains a diagnostic dilemma as it is very difficult to diagnose all the cases preoperatively. The presence of MRI findings of mass effect in the form of displacement of the traversing nerve roots due to large central disc with crumble disc sign were suggestive of early evidence of intradural disc herniation. Y sign in ventral dura due to splitting of ventral dura and arachnoid mater by disc material was a good diagnostic sign to suspect intradural extra-arachnoid disc. The presence of hypointense structure inside the dura with no continuity with the adjacent intervertebral disc on MRI was highly suggestive of an intradural disc, in patients having the large central disc on MRI, especially at L4-L5 levels, should raise suspicion of intradural herniation of disc. 2).


Ducati et al. described five cases of this pathology and review the literature as well as some considerations about the difficulties in the preoperative diagnostic issues and the surgical technique.

They concluded that for intradural disc herniations the diagnosis is mainly intraoperative, and the surgical technique has some special aspects 3).


A female patient with lumbosciatic pain who developed an incomplete cauda equina syndrome. An asymmetric discopathy of the L2-L3 space and a gas bubble with disc material within the spinal canal was noticed in the radiologic explorations. The literature and the authors’ experience are reviewed with the aim of confirming the frequency of intradural herniation in association with gas in the spinal canal.

A laminoarthrectomy of the involved space was performed followed by direct intradural examination, which revealed a disc fragment that was excised. An instrumented L2-L3 arthrodesis was performed. Postoperative evolution was satisfactory. To date, the authors have found this association in 2% of the patients with intraspinal gas.

The potential presence of an intradural disc herniation must always be considered when performing an open discectomy on a patient whose CT scan study shows the presence of epidural gas. This association is particularly striking given the relative rarity of intradural herniations and intraspinal gas. In the event that no clear disc herniation was found, an intradural examination may be indicated to justify clinical signs and symptoms or previous radiologic studies 4).

Treatment

Intradural exploration and/or transdural sequestrectomy avoids traction on already compromised nerve roots and is often safer than extradural sequestrectomy. The onset of bladder paralysis is a most important indication for immediate surgery. The cases presented show that there is a highly significant difference in the outcome of those cases operated on within 24h of bladder paralysis compared to those operated on after this period 5).

Case series

Sharma et al. presented a case series of six cases of intradural disc herniation at L4-L5 level diagnosed on the basis of intraoperative findings.

All the cases, on preoperative magnetic resonance imaging (MRI) were reported as having diffuse annular bulge with large posterocentral extrusion. The study comprised patients in age group of 30-60 years. Four cases out of six presented with cauda equina syndrome. In three cases, cauda equina was associated with sudden deterioration in the power of lower limb muscle groups.

They suspected that intradural herniation of disc was synchronous with cauda equina syndrome in these cases, which was very well documented in one of the cases. On retrospective analysis, MRI findings of mass effect in the form of displacement of the traversing nerve roots due to large central disc with crumble disc sign were suggestive of early evidence of intradural disc herniation. Y sign in ventral dura due to splitting of ventral dura and arachnoid mater by disc material was a good diagnostic sign to suspect intradural extra-arachnoid disc. The presence of hypointense structure inside the dura with no continuity with the adjacent intervertebral disc on MRI was highly suggestive of an intradural disc.

Intradural disc prolapse remains a diagnostic dilemma as it is very difficult to diagnose all the cases preoperatively. The presence of above-mentioned radiological signs on MRI in patients having the large central disc on MRI, especially at L4-L5 levels, should raise suspicion of intradural herniation of disc. 6).


Ducati et al. described five cases of this pathology and review the literature as well as some considerations about the difficulties in the preoperative diagnostic issues and the surgical technique.

They concluded that for intradural disc herniations the diagnosis is mainly intraoperative, and the surgical technique has some special aspects 7).

Case reports

Intradural lumbar disc herniation is rare, with an incidence of 0.3%-1%, but has been well reported in the literature. Transdural migration of the disc penetrating both ventral and dorsal dura is extremely rare, and there is a dearth of literature in the pathophysiology and surgical management of transdural herniation. Lack of knowledge on this type of presentation can cause intraoperative surprises and inadvertent cauda equina root injuries and lead to prolonged operative time. Pedaballe et al. reported 1 such case, described the surgical experience, and discussed the pathological mechanisms and signs.

A 30-year-old woman presented to outpatient clinic with chronic cauda equina syndrome due to massive L4-L5 disc herniation. L4-L5 decompression and transforaminal lumbar interbody fusion were planned. Unexpectedly, however, surgery revealed a transdural herniation, which was effectively managed with laminectomy, extension of durotomydiscectomy, repair of both dorsal and ventral dura, and interbody fusion, but at the expense of prolonged surgical time.

Transdural herniation of a lumbar disc is very rare presentation. It can be effectively managed with laminectomy, extension of durotomydiscectomy and repair of both dorsal and ventral dura. It can be diagnosed by magnetic resonance imaging preoperatively only if read with suspicion of such presentation. 8).


A 56-year-old man who developed cauda equina syndrome after several episodes of severe Valsalva maneuver.

The patient was found to have developed subacute urinary retention and leg weakness. Magnetic resonance imaging findings were concerning for an unusual-appearing lesion extending cranially at L2-3. Urgent decompression via an L2 laminectomy, exploration, and subsequent discectomy was performed. The patient recovered exceptionally well, regaining bladder function and ultimately being able to ambulate without assistance.

Cranially extending intrathecal disc herniations are a rare phenomenon and exceptionally uncommon above L3. The clinician should have a high level of suspicion for herniation when looking at the clinical and historical information consistent with such a diagnosis even in the presence of ambiguous imaging findings 9)


Nagaria et al. presented a case with intermittent symptoms and signs of cauda equina compression. They were unable to find in the literature, any previously described cases of intermittent cauda equina compression from a herniated intradural disc fragment leading to a “floppy disc syndrome” 10).


A 73-year-old male presented with a rare dorsally sequestrated lumbar disc herniation manifesting as severe radiating pain in both leg, progressively worsening weakness in both lower extremities, and urinary incontinence, suggesting cauda equina syndrome. Magnetic resonance imaging suggested the sequestrated disc fragment located in the extradural space at the L4-L5 level had surrounded and compressed the dural sac from the lateral to dorsal sides. A bilateral decompressive laminectomy was performed under an operating microscope. A large extruded disc was found to have migrated from the ventral aspect, around the thecal sac, and into the dorsal aspect, which compressed the sac to the right. After removal of the disc fragment, his sciatica was relieved and the patient felt strength of lower extremity improved 11).


Mailleux et al. described a case of anterior transdural L4-L5 disc herniation presenting as a partial cauda equina syndrome without related back pain or history of back pain. MRI allowed presurgical diagnosis showing an irregular intradural mass that did not enhance. That lack of enhancement could be related to the fact that the disc herniation was relatively recent 12).


A female patient with lumbosciatic pain who developed an incomplete cauda equina syndrome. An asymmetric discopathy of the L2-L3 space and a gas bubble with disc material within the spinal canal was noticed in the radiologic explorations. The literature and the authors’ experience are reviewed with the aim of confirming the frequency of intradural herniation in association with gas in the spinal canal.

A laminoarthrectomy of the involved space was performed followed by direct intradural examination, which revealed a disc fragment that was excised. An instrumented L2-L3 arthrodesis was performed. Postoperative evolution was satisfactory. To date, the authors have found this association in 2% of the patients with intraspinal gas.

The potential presence of an intradural disc herniation must always be considered when performing an open discectomy on a patient whose CT scan study shows the presence of epidural gas. This association is particularly striking given the relative rarity of intradural herniations and intraspinal gas. In the event that no clear disc herniation was found, an intradural examination may be indicated to justify clinical signs and symptoms or previous radiologic studies 13).


A 59 year-old man with ILDH. It was the only case of ILDH among 960 patients surgically treated, during the period 1989-1996. Clinically the patient demonstrated an acute cauda equina syndrome. The diagnosis was established by radiculograms, which showed a total block at the L3-L4 level. There was a 3 days time interval between the diagnosed syndrome itself and the operation. At surgery the L3-L4 level was intact, whereas dense adhesions were found between the L4-L5 disc and the dura. Root retraction to expose the nucleus pulposus mass was impossible. A laminectomy of the L4 was undertaken. An incision was made in the dura and arachnoid, revealing an extruded discal mass, lying between the roots of the cauda equina. It was carefully removed. The state of the patient at follow-up 1 year after surgery was unsatisfactory. The patient has moderate flaccid paraparesis, bladder dysfunction improved. The prognosis appeared to be linked to the preoperative duration of symptoms 14)


One case of intradural lumbar disc herniation at the L3-L4 disc level with cauda equina syndrome is reported. Myelo-CT demonstrated an intradural tumor-like lesion with complete block. An intradural fragment of sequestrated disc material was found intraoperatively. Accurate preoperative diagnosis of the intradural nature of the disease and prompt surgical treatment resulted in a smooth recovery 15).


A case of intradural disk herniation at L4-5 observed in a patient with longstanding low back pain and sciatica due to a herniated disk. After having undergone various surgical procedures for this disorder, the patient developed a multiradicular syndrome of the cauda equina 16)

References

1) , 8)

Pedaballe AR, Mallepally AR, Tandon V, Sharma A, Chhabra HS. An Unusual Case of Transdural Herniation of a Lumbar Intervertebral Disc: Diagnostic and Surgical Challenges. World Neurosurg. 2019 Aug;128:385-389. doi: 10.1016/j.wneu.2019.05.103. Epub 2019 May 20. PubMed PMID: 31121367.
2) , 6)

Sharma A, Singh V, Sangondimath G, Kamble P. Intradural Disc a Diagnostic Dilemma: Case Series and Review of Literature. Asian J Neurosurg. 2018 Oct-Dec;13(4):1033-1036. doi: 10.4103/ajns.AJNS_55_17. PubMed PMID: 30459862; PubMed Central PMCID: PMC6208249.
3) , 7)

Ducati LG, Silva MV, Brandão MM, Romero FR, Zanini MA. Intradural lumbar disc herniation: report of five cases with literature review. Eur Spine J. 2013 May;22 Suppl 3:S404-8. doi: 10.1007/s00586-012-2516-4. Epub 2012 Sep 27. Review. PubMed PMID: 23014741; PubMed Central PMCID: PMC3641279.
4) , 13)

Hidalgo-Ovejero AM, García-Mata S, Gozzi-Vallejo S, Izco-Cabezón T, Martínez-Morentín J, Martínez-Grande M. Intradural disc herniation and epidural gas: something more than a casual association? Spine (Phila Pa 1976). 2004 Oct 15;29(20):E463-7. Review. PubMed PMID: 15480124.
5)

Dinning TA, Schaeffer HR. Discogenic compression of the cauda equina: a surgical emergency. Aust N Z J Surg. 1993 Dec;63(12):927-34. PubMed PMID: 8285904.
9)

Tempel Z, Zhu X, McDowell MM, Agarwal N, Monaco EA 3rd. Severe Intradural Lumbar Disc Herniation with Cranially Oriented Free Fragment Migration. World Neurosurg. 2016 Aug;92:582.e1-582.e4. doi: 10.1016/j.wneu.2016.06.024. Epub 2016 Jun 16. PubMed PMID: 27318310.
10)

Nagaria J, Chan C, Kamel M, McEvoy L, Bolger C. Episodic cauda equina compression from an intradural lumbar herniated disc: a case of ‘floppy disc’. J Surg Case Rep. 2011 Sep 1;2011(9):6. doi: 10.1093/jscr/2011.9.6. PubMed PMID: 24950507; PubMed Central PMCID: PMC3649298.
11)

Kim JS, Lee SH, Arbatti NJ. Dorsal extradural lumbar disc herniation causing cauda equina syndrome : a case report and review of literature. J Korean Neurosurg Soc. 2010 Mar;47(3):217-20. doi: 10.3340/jkns.2010.47.3.217. Epub 2010 Mar 31. PubMed PMID: 20379476; PubMed Central PMCID: PMC2851086.
12)

Mailleux R, Redant C, Milbouw G. MR diagnosis of transdural disc herniation causing cauda equine syndrome. JBR-BTR. 2006 Nov-Dec;89(6):303-5. PubMed PMID: 17274584.
14)

Bayassi S. [Intradural lumbar disk herniation (ILDH). Case report and literature review]. Neurol Neurochir Pol. 1998 Sep-Oct;32(5):1295-301; discussion 1301-2. Polish. PubMed PMID: 10463243.
15)

Fang CM, Huang TJ, Chen WJ, Lee ST, Hsu RW. Intradural lumbar disc herniation–a case report. Changgeng Yi Xue Za Zhi. 1994 Sep;17(3):297-300. PubMed PMID: 7954013.
16)

Borgogno G, Fontanella C, La Camera V. [Herniated intradural lumbar disk: a clinical case]. Arch Putti Chir Organi Mov. 1991;39(1):87-91. Italian. PubMed PMID: 1842495.

Miyazaki syndrome

Miyazaki syndrome

Miyazaki syndrome is a cervical myelopathy or cervical radiculopathy caused by cervical epidural venous congestion, due to shunt overdrainage. The complex pathophysiology includes CSF pressure-changes consistent with the Monro-Kellie hypothesis and a non-functional Starling resistor, leading to spinal epidural venous plexus enlargement and dilation. This venous congestion may be significant enough to exert compression on the spinal cord or nerve roots. The typical clinical and imaging findings together with a history of ventricular CSF shunting may establish the diagnosis, proven by a successful treatment. The aim of treatment is the abrogation of CSF over-drainage. The eligible interventions may be the followings: the increase of the opening-pressure of the valve system by the insertion of a new programmable valve if necessary, closing or removing the shunt.


In 1997 Miyazaki et al. described a case of intracranial hypotension syndrome due to overdrainage of cerebrospinal fluid presented with hearing loss afterventriculoperitoneal shunting procedure. A 69-year-old man suffering from subarachnoid hemorrhage presented with an angiogram showing two aneurysms, one of the right internal carotid artery and one of the middle cerebral artery. Neck clipping was performed. One month later, he developed normal pressure hydrocephalus (NPH), which was treated by ventriculoperitoneal shunting procedure using low pressure Pudenz valve system. Trias of NPH were improved by insertion of shunt system. However, he complained of hearing loss which was worsened by upright position and improved by lying down. Such kinds of phenomenon were demonstrated by audiogram showing that the transitory decrease of hearing and electrocochleography showing the elongation of N1 latency at upright position. These data suggested that his hearing loss was caused by inner ear or auditory nerve lesion. After the shunt system was replaced into the antisiphon device, his hearing disturbance improved. Axial computed tomography of bone window at the level of orbitomeatal line demonstrated widely perilymphatic duct on both sides. This finding suggested that the fluctuation of intracranial pressure was easily transmitted into the cochlear through the widened perilymphatic duct, resulting in hearing disturbance 1).


Várallyay et al. want to call attention to this rare iatrogenic condition with potentially severe consequences.

They performed a systematic literature-review and presented ther five cases.

Once recognized in time, Miyazaki syndrome can be well taken care of.

Patients with chronic ventricular shunt need monitoring for CSF over-drainage to recognise potential complications such as cervical myelopathy or radiculopathy 2).


In 2018 a 33-year-old patient had undergone placement of a ventriculoperitoneal shunt with a pressure-adjustable valve for communicating hydrocephalus years before presenting to our department with the complaints of constant headache and unsteady gait. On the basis of the clinical picture and her history, plain and contrast-enhanced cranial and whole spine magnetic resonance imaging and magnetic resonance angiography examinations were performed, with the scans revealing signs indicative of cerebrospinal fluid hypotension typical of Miyazaki syndrome 3).


In 2015 Caruso et al. reported one case 4).

References

1)

Miyazaki Y, Tomii M, Sawauchi S, Ikeuchi S, Yuki K, Abe T. [A case of hearing loss caused by overdrainage of cerebrospinal fluid after ventriculo-peritoneal shunting procedure]. No Shinkei Geka. 1997 Apr;25(4):367-71. Japanese. PubMed PMID: 9125722.
2)

Várallyay P, Nagy Z, Szűcs A, Czigléczki G, Markia B, Nagy G, Osztie É, Vajda J, Vitanovics D. Miyazaki syndrome: Cervical myelo/radiculopathy caused by overshunting. A systematic review. Clin Neurol Neurosurg. 2019 Sep 24;186:105531. doi: 10.1016/j.clineuro.2019.105531. [Epub ahead of print] PubMed PMID: 31622897.
3)

Kovács A, Németh T, Csomor A, Novák T, Kövér F, Vörös E. Miyazaki Syndrome due to Ventriculoperitoneal Shunt Treatment. World Neurosurg. 2018 Aug;116:29-34. doi: 10.1016/j.wneu.2018.05.032. Epub 2018 May 31. PubMed PMID: 29775766.
4)

Caruso R, Wierzbicki V, Marrocco L, Pesce A, Piccione E. A Poorly Known Cerebrospinal Fluid Shunt Complication: Miyazaki Syndrome. World Neurosurg. 2015 Sep;84(3):834-8. doi: 10.1016/j.wneu.2015.04.030. Epub 2015 Apr 23. PubMed PMID: 25913430.

Morquio syndrome

Morquio syndrome

Morquio’s syndrome (referred to as mucopolysaccharidosis IV or Morquio’s) is an autosomal recessive mucopolysaccharide storage disease (see also lysosomal storage disorder), usually inherited.

It is a rare type of birth defect with serious consequences. When the body cannot process certain types of mucopolysaccharides, they build up or are eliminated, causing various symptoms. These involve accumulation of keratan sulfate.

Classification

This syndrome has two forms, A and B, referred to as Morquio A and Morquio B syndrome or MPS IVA and MPS IVB. The two forms are distinguished by the gene product involved; Type A involves a malfunction in the GALNS gene, while Type B involves a malfunction of the GLB1 gene.

Type A GALNS Missing enzyme: Galactosamine-6 sulfatase Chromosomal region: 16q24

Type B GLB1 Missing enzyme: Beta-galactosidase Chromosomal region: 3p22

Treatment

Complications

Atlantoaxial subluxation due to hypoplasia of the odontoid process and joint laxity resulting in myelopathy have been well described in the literature.

Spinal involvement in MPS-IV patients, with neurological impairment, other than atlantoaxial instability and thoracolumbar kyphosis, has been scarcely mentioned in the literature.

Neurological problems secondary to progressive kyphosis and stenosis at the cervicothoracic and upper thoracic spine are seen in children with Morquio syndrome. Early detection with a careful neurological assessment, whole spine MR imaging, and appropriate surgical treatment can prevent permanent neurological sequelae 1).

Case reports

A 17-year-old male patient with severe Morquio syndrome presented with cervical and upper thoracic spinal stenosis with spinal cord myelopathy, and progressive severe tracheal stenosis. Coordinated care among otolaryngology, orthopedic surgery, neurosurgery, anesthesiology, cardiovascular surgery, radiology, and pulmonology teams facilitated the successful planning and execution of two major surgical interventions in rapid succession. This is the first description of a successfully coordinated spine and airway repair in the literature 2).


The records of patients with syndromic Craniovertebral Junction Anomaly treated by Muthukumar during the period of 2012-2017 were retrospectively reviewed. Patients in whom intraoperative difficulties and complications were encountered were culled out from the database. Complications were divided into (1) technique related, (2) neural injury, (3) vascular injury, (4) instrumentation pull out/breakage, (5) screw misplacement and, (6) where postoperatively, the surgeon felt an alternate surgical technique could have yielded better results. Four patients with either unexpected intraoperative difficulties or complications or in whom the technique could have been refined were identified. There were 2 patients with proatlas segmentation anomalies and 2 with Morquio syndrome. The first patient had cage migration which necessitated a second procedure during craniovertebral realignment, the second had partial penetration of the screw into the transverse foramen, the third with bipartite atlas underwent a C1-2 fixation without a horizontal cross-connector and, the fourth had screw pull outs from the subaxial cervical spine intraoperatively during an attempted occipitocervical fusion. In children with syndromic CVJ anomalies, the surgeon should be aware of the high risk of intraoperative difficulties and complications. Potential pitfalls and the ways to avoid these complications are discussed 3).

References

1)

Baratela WA, Bober MB, Thacker MM, Belthur MV, Oto M, Rogers KJ, Mackenzie WG. Cervicothoracic myelopathy in children with morquio syndrome a: a report of 4 cases. J Pediatr Orthop. 2014 Mar;34(2):223-8. doi: 10.1097/BPO.0000000000000074. PubMed PMID: 24096444.
2)

Kiessling P, Stans AA, Dearani JA, Boesch RP, Fogelson JL, Matsumoto JM, Haile DT, Balakrishnan K. Coordinated approach to spinal and tracheal reconstruction in a patient with morquio syndrome. Int J Pediatr Otorhinolaryngol. 2019 Oct 11;128:109721. doi: 10.1016/j.ijporl.2019.109721. [Epub ahead of print] PubMed PMID: 31639621.
3)

Muthukumar N. Problems in Instrumentation of Syndromic Craniovertebral Junction Anomalies – Case Reports. Neurospine. 2019 Jun;16(2):277-285. doi: 10.14245/ns.1938176.088. Epub 2019 Jun 30. PubMed PMID: 31261467.
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