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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
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segmental dorsal arteries during fetal development. This may account for the
common metameric origin of the pathological vessels that create the cutaneomeningospinal angiomas.
130.4 Pathology
The disease is usually thoracolumbar in location. It consists of cutaneous hemangiomas associated with vertebral, paravertebral, and intraspinal angimatosis, usually fed by branches from the intercostal vessels. There may be other visceral
hemangiomas. Myelopathy is caused by compression due to the bulk of the vascular
mass. The cord may show some degree of atrophy. The symptoms may be caused
also by venous hypertension and/or blood steal phenomena. However, the vascular
pathology varies from case to case; a combination of hemangioma and arterial
venous fi stula (AVF) is also encountered, in which case subarachnoid hemorrhage
is a possibility. Occasionally, spina bifi da and cord tethering have been recorded by
Brant et al. .
130.5 Clinical Picture
The disease may present itself at any age, usually in late childhood. The onset may
be slow or sudden, and the course progressive or rapid. The syndrome is congenital
manifested by cutaneous nevus on the back in the thoracolumbar region and crossing the middle line. Cord compression is manifested by spastic paraparesis, tetraparesis, or monoparesis. Headache, pains, pyrexia, menigism, and gluteal or limb
hypertrophy have been reported.
130.6 Diagnosis
Diagnosis is based on the following criteria:
1. History of birth nevus
2. Clinical exam: the skin angioma corresponds within 1–2 segments to the intraspinal angioma. The cutaneous nevus varies in size. There may be visceral angiomas as well.
3. Spinal angiography and aortogram
4. MRI: in case reported by Soeda, et al., T1 weighted showed low and high signal
intensity.
5. X-rays can reveal widening of the spinal canal
6. CT scans also were used diagnostically
130 Myelopathy in Cobb Syndrome
447
130.7 Management
Management especially, in infants is rather diffi cult. Primary surgical excision is
associated with high risk.
Methods adopted are:
1. Systemic corticosteroids therapy
2. Devascularization of the feeding arteries identifi ed by angiography and excluding spinal cord supply. Usually the angioma feeders are distinct from those of the
cord
3. Endovascular embolization using n- butyl-2-cyanoacrylate (NBCA)
4. Combination of radiotherapy and surgery
5. Conservative treatment
If all above fails, careful excision of the residual mass should be performed.
There are various choices in the series reviewed by Clark et al. , with various opinions, combining one or more of the above mentioned modalities. The management
has to be multidisciplinary: neurology, neurosurgery, interventional radiology with
decisions based on the pros and cons.
References
Brant AJ, James HE, Tung H. Cutaneomeningospinal angiomatosis (Cobb syndrome) with teth-
ered cord. Pediatr Neurosurg. 1999;30(2):93–5.
Clark MT, Brooks EL, Chong W, Pappas C, Fahey M. Cobb syndrome: a case report and system-
atic review of the literature. Pediatr Neurol. 2008;39(6):423–5.
Soeda A, Sakai N, Iihara K, Nagata I. Cobb syndrome in an infant: treatment with endovascular
embolization and corticosteroid therapy: case report. Neurosurgery. 2003;52(3):711–5. discussion 714-5.
References
449
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_131
131
Eale’s Disease
131.1 Definition
According to Jablonski, a noninfl ammatory disorder of the peripheral retinal vessels
characterized by frequently bilateral periphlebitis, avascular peripheral areas,
microaneurysms, rope ladder-like capillary dilatations, tortuosity of adjacent vessels, and spontaneous chorioretinal scars. Neovascularization, hemorrhage, vascular
orbliteration, and vascular sheathing may be associated. Neurological complications may include vascular lesions of the brain and demyelination of the optic nerve.
Epistaxis and constipation may occur.
131.2 Incidence
Extraoccular manifestations of Eales’ disease have been reported in the nervous
system only in the brain and spinal cord. Until 1993 only 16 cases of Eale’s disease
myelopathy have been reported.
131.3 Etiology
The etiology so far is unknown. Dastur and Singhal ( 1976 ) have suggested the pos-
sibility of vasculopathy with demyelination. Retinomyelopathy occurs as a hypersensitive mechanism in response to an infl ammatory agent, either bacterial or viral.
This could also apply to the myelopathy of the disease.
Abstracted and reported by Sawhney et al. ( 1986 ).
450
131.4 Pathology
The condition is that of vasculitis, periphlebitis, peripheral ischemia, microaneurysms, rope-ladder like capillary dilation, and tortuosity of adjacent vessels.
Hemorrhages and vascular obliterations may be detected. These vascular changes
may apply to the brain, optic nerve, and spinal cord; leading to demyelinating
myelopathy. There is selective pyramidal demyelination without any selective level
in the cord. There may also be a brain lesion.
131.5 Clinical Picture
The disease occurs in males between 16–40 years old and is of insidious onset, but
sometimes acute or subacute. There is unilateral or bilateral blindness of various
degrees. It starts by progressive weakness and stiffness of lower extremities with
micturition dysfunction. Sensory changes of different degrees are noticed and
refl exes are hyperactive.
131.6 Diagnosis
Diagnosis based on:
1. Ocular fundoscopy
2. Neurological exam
3. Myelography is usually negative
4. MRI: shows multifocal white matter abnormalities
5. CSF: may show increased lymphocytes
Differential diagnosis: syphilitic myelitis, tuberculosis meningomyelitis, multi-
ple sclerosis (MS), and hereditary spastic paraplegia.
131.7 Management
1. Steroid therapy and Medical care
2. Rehabilitation
3. Opthalmological care
Partial or no improvement may be expected.
131 Eale’s Disease
451
References
Dastur DK, Singhal BS. Eales’ disease with neurological involvement. Part 2. Pathology and
pathogenesis. J Neurol Sci. 1976;27(3):323–45.
Sawhney IM, Chopra JS, Bansal SK, Gupta AK. Eales’ disease with myelopathy. Clin Neurol
Neurosurg. 1986;88(3):213–5.
References
453
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_132
132
Ectopic Choroidal Plexus Tissue
132.1 Definition
This is an unique condition where there is ectopic choroid tissue in the spinal cord
causing pressure myelopathy.
132.2 Incidence
This is a single case reported, whereas intracranial cysts containing ectopic choroid
plexus or choroid-like tissue has been rarely reported (5 cases).
132.3 Etiology
The choroid plexus develops from the telachoroidal and projects into the lateral
ventricle on its medial aspect, roof of the third ventricles and roof of the fourth ventricle. Such tissue or like it has been found in the epithelial linked non- communicating
cysts intracranially. The exact causative factor is still unknown. It is considered as a
developmental aberration with a theory that the same origin and that the ectopic
choroid is formed by metaplasia of the ependymal rest.
132.4 Pathology
The histopathology and the immunohistochemical exam showed a normal choroid
plexus consisting of a single layer of choroid epithelium arranged in microvillar
patter with a microvascular care. Immunohistochemical staining showed with glia
Abstracted and reported by Dwarakanath et al. ( 2005 )
454
fi brillary acid protein and epithelial membrane architecture suggestive of a normal
choroid plexus confi rming the ectopic choroid plexus nature.
132.5 Case Report
A 30-year-old man complained of progressive descending weakness from the upper
to the lower limbs giving the neurological picture of tetraparesis. There was disassociated sensory loss between C2-C4 and sacral sparing
132.6 Diagnosis
Diagnosis is based on the following criteria:
• MRI of the spine: Expansion of the spinal cord; C6-T2, forming intramedullary
cystic lesion: hypointense on T1 weighted images and hyperintense on T2
weighted images. More details show holocord syrinx.
132.7 Management
Since the patient had compression myelopathy microsurgery performed was through
laminectomy C6-T2, the dura was expanded, opened, the cord distended, paper thin,
myelotomy and syringostomy through the cyst, which was dissected and removed
with its choroid content. Laminoplasty with miniplates and screws was done with
uneventful post-operative course.
Reference
Dwarakanath S, Suri A, Mahapatra AK, Mehta VS, Sharma MC. Intramedullary ectopic choroid
plexus: report of a rare case. Neurosurgery. 2005;56:869.
132 Ectopic Choroidal Plexus Tissue
455
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_133
133
Myelopathy due to Fibrocartilaginous Spinal Cord Embolism
133.1 Definition
Fibrocartilaginous embolism (FCE) is a condition in which a fragment of the
nucleus pulposa migrating to block spinal cord arterial supply, resulting in ischemic
infarction.
133.2 Incidence
Initially, all cases of spinal FCE were diagnosed at autopsy studies. In 1961,
Naiman et al., reported the fi rst case of FCE in a young man who developed tetraparesis and respiratory failure after minor trauma while playing basketball. At
autopsy a diffused embolic material of the nucleus pulposus in the anterior spinal
cord and the basilar arterial systems. Since then nearly 40 autopsy proven cases
have been reported. In 1991, a case was reported from the Massachusets General
Hospital, who survived. Mateen et al. (
2011 ), reviewed all the spinal cord infarc-
tion cases admitted to the Mayo Clinic over a 12 year period (1997–2004). They
found out of 164 patients with acute spinal infarction 9 met the inclusion criteria
for high likelihood of FCE (6 men and 3 women); median age: 46 years old
(21–64); all patients were severely affected (Rankin Scale 4); mean time to evaluation 57 days. One patient also had cerebral infarct. All had no improvement
from steroids.
Abstracted and reported by Mateen et al. ( 2011 )
456
133.3 Etiology
Patients with FCE have exclusion of the following conditions:
• Aortic dissection
• Hypercoagulability state
• Intracardiac embolic source
• AVF
• Rhematalogic disorders
• Demyelinating disease
• Infections and metabolic conditions of the spinal cord (syphilis, HIV, vitamin
B12, or Cu defi ciencies)
• Systemic malignancy
Potential precipitating event were marked in 7/9 (78 %) of the cases:
• Motor vehicle accident without recognized back injury (n = 2)
• Heavy lifting (n = 3)
• Physical exertion (n = 1) (valsava’s maneuver)
• Bending over (n = 1)
The onset of the symptoms was 6 h after the antecedent. Back pain was a common symptom in six of the patients; with a radicular component in three. Four
patients had vascular risk factors.
133.4 Clinical Picture
The onset is sudden characterized by back pain associated with paresthesia of the
upper and/or lower extremities, resulting in paraparesis or tetraparesis with sphincter dysfunction. Patient’s history should be negative for trauma, anticoagulants,
blood dyscrasias, etc.
133.5 Diagnosis
Diagnosis is based on the following criteria:
1. Clinical history and fi ndings.
2. MRI studies in nine patients demonstrate the evolution of MRI fi ndings from
herniated disk material on initial imaging to longitudinally extensive infarction
on subsequent imaging.
3. CSF analysis was only remarkably showed elevated protein in six patients (53–
90 mg/dl)
133 Myelopathy due to Fibrocartilaginous Spinal Cord Embolism
457
4. Negative for oligoclonal banding, hyperglycorrhachia, xathochromia, or other
markers for infl ammatory or infectious diseases.
5. Platelet count, international normalized ratio (INR), activated partial thrombo-
plastin time (aPPT) were all normal.
Differntial Diagonsis include prolonged arterial hypotension, spinal disk prolapse or herniation, thrombo-occlusive aortic disease, vasculitis, systemic lupus
erythematous, antiphospholipid antibody syndrome, dissecting aortic aneurysm,
sickle cell anemia, infections, etc.
133.6 Management
There is no treatment that has an impact on the patient’s symptoms in the acute
period. Common treatments include IV steroids, IV heparin, and plasma exchange.
The current understanding of FCE is based on case reports, nearly all discovered at
autopsy; therefore the prognosis of FCE is overwhelmingly pessimistic.
Reference
Mateen FJ, Monrad PA, Hunderfund AN, et al. Clinically suspected fi brocartilaginous embolism:
clinical characteristics, treatments, and outcomes. Eur J Neurol. 2011;18(2):218–25.
Reference