How Should Syringomyelia be Defined and Diagnosed?

Klekamp, Jörg

doi:10.1016/j.wneu.2017.12.156

Cited by 39 publications

(30 citation statements)

References 43 publications

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“…MRI flow studies are an integral part of the diagnostic work up of CM and SM in human patients ( 14 , 102 ) and for review of the clinical application for SM, arachnoid webs and the subarachnoid space see Li et al ( 104 ). Standard MRI sequences of the brain and spinal cord assume that the tissue is static with movement only occurring in blood vessels and CSF and the aim is to achieve higher spatial and contrast resolution typically at the expense of temporal resolution.…”

Section: Mri Protocols To Investigate CM and Smmentioning

confidence: 99%

“…The diagnosis of SM implies a fluid filled cavity related to disturbance of CSF flow, spinal cord tethering or intramedullary tumor; it is not an appropriate description for myelomalacia or cystic lesions ( 14 ). SM can be asymptomatic ( 34 ) and when interpreting MRI, an assessment should be made as to whether the location and severity of the syrinx would account for the signs.…”

Section: Diagnosis Of Symptomatic Smmentioning

confidence: 99%

“…CM is one of the most common causes of SM in the dog which is characterized by the development of cavities in the spinal cord containing a fluid similar to CSF ( 13 , 14 ) however SM can develop after any obstruction to CSF channels and has been reported in a variety of disorders ranging from acquired cerebellar herniation secondary to intracranial masses ( 15 – 18 ) to spinal arachnoid diverticulum ( 19 , 20 ) and spinal cord tethering ( 21 ). The terminology of SM is equally confused, with some veterinary papers referring to syringohydromyelia or hydrosyringomyelia.…”

Section: Introductionmentioning

confidence: 99%

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Clinical Application of Diagnostic Imaging of Chiari-Like Malformation and Syringomyelia

2018

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Chiari-like malformation (CM) and syringomyelia (SM) is a frequent diagnosis in predisposed brachycephalic toy breeds since increased availability of MRI. However, the relevance of that MRI diagnosis has been questioned as CM, defined as identification of a cerebellar herniation, is ubiquitous in some breeds and SM can be asymptomatic. This article reviews the current knowledge of neuroanatomical changes in symptomatic CM and SM and diagnostic imaging modalities used for the clinical diagnosis of CM-pain or myelopathy related to SM. Although often compared to Chiari type I malformation in humans, canine CM-pain and SM is more comparable to complex craniosynostosis syndromes (i.e., premature fusion of multiple skull sutures) characterized by a short skull (cranial) base, rostrotentorial crowding with rostral forebrain flattening, small, and ventrally orientated olfactory bulbs, displacement of the neural tissue to give increased height of the cranium and further reduction of the functional caudotentorial space with hindbrain herniation. MRI may further reveal changes suggesting raised intracranial pressure such as loss of sulci definition in conjunction with ventriculomegaly. In addition to these brachycephalic changes, dogs with SM are more likely to have craniocervical junction abnormalities including rostral displacement of the axis and atlas with increased odontoid angulation causing craniospinal junction deformation and medulla oblongata elevation. Symptomatic SM is diagnosed on the basis of signs of myelopathy and presence of a large syrinx that is consistent with the neuro-localization. The imaging protocol should establish the longitudinal and transverse extent of the spinal cord involvement by the syrinx. Phantom scratching and cervicotorticollis are associated with large mid-cervical syringes that extend to the superficial dorsal horn. If the cause of CSF channel disruption and syringomyelia is not revealed by anatomical MRI then other imaging modalities may be appropriate with radiography or CT for any associated vertebral abnormalities.

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Section: Mri Protocols To Investigate CM and Smmentioning

confidence: 99%

Section: Diagnosis Of Symptomatic Smmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical Application of Diagnostic Imaging of Chiari-Like Malformation and Syringomyelia

2018

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“…The pathophysiology behind the development of these lesions is still unknown and may be in part due to CSF dysregulation. To date, the best treatment, regardless of presentation, remains complete tumor resection for symptomatic cysts, which has been prevalent throughout the literature for solitary cysts [ 19 - 20 ]. However, our case demonstrates a rare scenario where complete surgical resection initially failed and thus resulted in more severe loculated pan neuraxis caterpillar-like cysts for the patient.…”

Section: Discussionmentioning

confidence: 99%

Recurrent Caterpillar-like Arachnoid Cysts Following Initial Resection: A Unique Presentation of a Disorder Where the Limits of Modern Medicine Are Reached

Kaloostian

Vartanian

Ordookhanian

et al. 2019

Cureus

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Of the many emergent neurological cases presenting to the emergency department (ED) and operating room (OR) for resection, arachnoid cysts are amongst one of the rarer pathologies. The proper resection of arachnoid cysts has substantially decreased the risk of recurrence. Current medicinal and surgical approaches have been refined over the years and prove to be successful for many patients. Where current practices begin to fail is during the treatment of complex and rare cases, such as the one presented in this manuscript. The commonly accepted surgical practices that were utilized to aid in the management of our patient, who initially presented with a simple arachnoid cyst, unexpectedly resulted in the further development of additional arachnoid cysts, a very rare occurrence, and a complication that should be discussed amongst all specialists in the hope of identifying more focused, novel, and less-invasive approaches to cyst removal and recurrence prevention.

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“…4 In children with VUR refractory to conservative measures, management includes surgery with ureteral implantation, bladder augmentation, or a combination of treatment methods. 3 Urinary incontinence and bladder dysfunction are rarely described as the first manifestation of syringomyelia, 5 6 a fluid-filled, gliosis-lined cavity within the spinal cord 7 prevalent in 8.4 cases per 100,000 children. 8 Hydromyelia refers to a fluid-filled cavity within the spinal cord lined by ependymal, which likely results from a developmentally nonobliterated central canal.…”

Section: Introductionmentioning

confidence: 99%

An Unusual Case of Syringohydromyelia Presenting with Neurogenic Bladder

Geljić

Abdović

Štampalija

et al. 2019

European J Pediatr Surg Rep

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We report the case of a 4-year-old boy who first presented with acute pyelonephritis at the age of 6 months. Diagnostic workup revealed high-grade bilateral vesicourethral reflux (VUR). At the age of 18 months, a bulking agent was used to treat bilateral VUR. Since the VUR persisted, an open bilateral Lich-Gregoir procedure was done at the age of 3 years. Immediately after surgery, he developed acute urinary retention with hydronephrosis that resolved with the placement of dwelling urinary catheter. After removal of the catheter urinary retention relapsed so placement of suprapubic urinary catheter was indicated since he did not have sensory loss. He was started with tamsulosin (α − 1-blocker) and prophylactic antibiotics. Urodynamics were performed and suggested bladder outlet obstruction. On the basis of previous urethroscopy and the absence of neurological sequelae, the differential diagnosis of Hinman syndrome was made. After removal of the suprapubic catheter, clean intermittent catheterization was started and α-blocker continued. However, magnetic resonance imaging of the brain and the spinal cord revealed syringohydromyelia extending from thoracic spine (Th5) to conus medullaris with 6 to 7 mm in diameter. Electromyoneurogram was normal. After a follow-up of 3 years, the hydronephrosis has resolved. The patient is on clean intermittent catherization and has no urinary tract infections.

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How Should Syringomyelia be Defined and Diagnosed?

Cited by 39 publications

References 43 publications

Clinical Application of Diagnostic Imaging of Chiari-Like Malformation and Syringomyelia

Clinical Application of Diagnostic Imaging of Chiari-Like Malformation and Syringomyelia

Recurrent Caterpillar-like Arachnoid Cysts Following Initial Resection: A Unique Presentation of a Disorder Where the Limits of Modern Medicine Are Reached

An Unusual Case of Syringohydromyelia Presenting with Neurogenic Bladder

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