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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
459
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_134
134
Intravascular Papillary Endothelial Hyperplasia
134.1 Definition
Also known as intravascular papillary endothelial hyperplasia (IPEH), Masson’s
vegetant hemangioendothelioma. Benign proliferation of endothelial cells, which
presents as slow growing, tender, violaceous cutaneous mass on the head, neck,
fi ngers, or trunk. Deep tumors occur as intramuscular hemangiomas, which may be
expansile and compressive. Involvement of CNS is rare, usually on top of hematoma from cavernous hemangioma, also may complicate intracerebral
hemorrhage.
134.2 Incidence
Only one case of spinal cord compression (T6) by Porter et al., in 1995 and one
other case of cauda equina compression reported by Taricco et al. in ( 1999 ). Ali
et al., in 1994, had one case of hemangioma infi ltrating the spinal meninges, causing
cord compression.
134.3 Etiology
Lesion is benign and considered to be a neoplasm by some authors and a benign
proliferation of endothelial cells by others. The mass occurs in certain pathological
conditions:
• On top of a thrombosis, which is organized
• May develop in normal vessels
Abstracted from Taricco et al. ( 1999 )
460
• Vascular lesions include cavernous angioma, hemangioma, pyogenic
granuloma
• On top of an organized hematoma
• Endothelial basic fi broblast growth factor secretion, which stimulates endothe-
lial cell proliferation.
134.4 Pathology
The lesion is exclusively intravascular. It may present in different morphologies:
• Vascular channels separated by fi brous septa with single endothelial cell layer
• Large blood spaces fi lled with papillary structures composed of a cord of con-
nective tissue and single layer of endothelial cells
• Fibrous connective tissue, which narrow vascular channels course and vascular
buds bearing a single layer of plump endothelium
Signs of malignancy are necrosis, mitosis, and nuclear pleomorphism are
lacking.
134.5 Immunohistochemistry
IPEH reacts to Factor VII, CD34 and CD32 corresponding to the endothelial cells
proliferative component
134.6 Clinical Picture
Although many intracranial IPEH have been described, only three cases involving
the spinal cord; in the case by Porter et al., at T6 and Taricco et al. ( 1999 ), at the
cauda equina. Neurological picture depends on the lever of compression, giving
sensory, motor, and sphincter dysfunction
134.7 Diagnosis
Diagnosis based on:
1. Clinical picture and physical exam
2. X-ray may not show tumor
3. CT scan with enhancement shows hyperdense tumor inside the spinal cord
134 Intravascular Papillary Endothelial Hyperplasia
461
4. MRI of the spinal cord: T1 weighted-images show an isointense nural paren-
chyma with strong homogenous gadolinium enhancement. T2 weighted-images
showed increased signal intensity
It should be differentiated from angiosarcoma, hemangioma, Kaposi’s sarcoma,
etc., seen in immunohistochemistry.
134.8 Management
The management of IPEH is complete surgical excision to prevent recurrence and
unnecessary radiotherapy. Verifi cation of pathology after surgery by histochemistry
is important to rule our more aggressive sarcomas. The outcome in the three reported
cases was favorable.
Reference
Taricco MA, Vieira JO, Machado AG, Ito FY. Intravascular papillary endothelial hyperplasia caus-
ing cauda equina compression: case report. Neurosurgery. 1999;45(6):1478–80.
Reference
463
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_135
135
Posterior Spinal Artery Syndrome
135.1 Definition
This syndrome is a result of spinal cord infarction involving the posterior section of
the cord, viz: posterior arteries, bilateral posterior horns, and posterior segments of
bilateral lateral columns.
135.2 Incidence
Only a few cases have been reported, which the authors reviewed in the discussion.
So far 30 cases have been reported.
135.3 Etiology
The incidence of spinal cord infarction is much less than the cerebral infarction due
to the fact the posterior spinal artery has many autonomic branches and is less liable
to atherosclerosis than the cerebral vessels. There are four to ten anterior radicular
arteries and ten to twenty-three posterior radicular arteries. Whereas cerebral infarction occurs in the elderly, the spinal category occurs in the young and has better
prognosis than the cerebral type. Until 1950s, meningovascular syphilis was the
main cause of the syndrome. Other causes are thromboembolism, arteriosclerosis,
dissecting aortic aneurysm, and minor indirect trauma to the posterior spinal arteries, intrathecal therapeutic phenol injection. In many cases no occlusion was
detected in the posterior spinal artery (PSA). With MRA some acute cases are
diagnosable.
Abstracted from Matsubayashi et al. ( 2013 )
464
135.4 Pathology
The upper thoracic and cervical spinal cord was much swollen and soft. The gray
and white matter were indistinguishable. The lower thoracic limbs and sacral cord
were normal. There was demyelination of the posterior columns bilaterally and in
all spinal cord segments. The axons were in the affected segments. There was also
mild demyelination and lymphocytic infi ltration in the lateral columns. There was
marked granulation tissue formation. There was mild glial nuclear atypia. There
were no changes to the anterior columns or anterior horns. There were no thrombosis or cartilage emboli in posterior or anterior spinal arteries. There was no ateriovenous malformation (AVM) or mass lesions or aortic aneurysm, no hematoma, or
neoplasia in the rest of the body.
135.5 Clinical Picture
An 84-year-old Japanese woman presented after a fall with a bruise of the head and
neck. Neck pain increased and she developed weakness and sensory loss of the
lower limbs. Five days after the fall she developed severe weakness of all extremities and rectovesical dysfunction. Deep tendon refl exes were very weak. No superfi cial sensory loss. MRI showed on T1-weighted images marked swelling of the
spinal cord and on T2-weighted images elongation of the cervicothoracic spinal
cord. Cerebral spinal fl uid showed high protein content 813 mg/100 ml and glucose
66 mg/100 ml. No atypical cells.
135.6 Management
Intravenous methylprednisolone 1000 mg/day; her neurological status worsened
and she developed respiratory failure and died 6 weeks after the onset.
Reference
Matsubayashi J, Tsuchiya K, Shimizu S, et al. Posterior spinal artery syndrome showing marked
swelling of the spinal cord: a clinico-pathological study. J Spinal Cord Med.
2013;36(1):31–5.
135 Posterior Spinal Artery Syndrome
465
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_136
136
Myelopathy due to Spinal Cord Vasculitis
136.1 Definition
Spinal cord vasculitis is a vascular pathology in the spinal cord leading to myelopathy and it is a part of the central nervous system vasculitis.
136.2 Incidence
According to Salvarani et al., primary CNS vasculitis (PCNSV) is uncomomon disease, which lesions are limited to the brain and spinal cord. The authors retrospectively identifi ed 101 consecutive patients with PCNSV in a series at the Mayo
Clinic. Spinal cord involvement was documented in fi ve cases. The spinal cord
symptoms developed before the cerebral symptoms in one, concurrently in two and
after the cerebral symptoms in two. Of the fi ve cases three were men; the median
age was 39. The interval before diagnosis was 1 month or less.
136.3 Etiology
The pathogenesis of the disease is not clear. It was presumed as an infl ammatory
condition, which responds to corticosteroids. Rare cases have been linked to nonvascular causes, e.g. Hodgkin’s lymphoma, Hashimoto thyroiditis, and druginduced allergenic reactions.
Abstracted from Salvarani et al. ( 2008 )
466
136.4 Pathology
From the case reported:
There is vascular wall infl ammation and destruction and granulomatous formation. Destruction of the vascular basement membrane was seen by periodic acidSchiff stain for vasculitis, necrotizing vasculitis, and granulomas were encountered.
Cordal or caudal infarcts were seen in two patients. Ropper et al., reported an
autopsy case. They described the cerebral pathology, which caused the mortality. In
the spinal cord small areas of cavitation, some with hemosiderin loaded with macrophages were seen. Foci of the myelomalacia in other areas and pallor are also
seen. Occlusion of the subpial vessels with fi brinous material. Thickened vascular
walls with subintimal eccentric proliferation of fi brinous tissue. Other fi nding
included endothelial swelling and disruption, and necrosis of the vessel wall. The
infi ltrate was composed of CD3 and less of CD8 and hemorrhagic infarcts. Viral
tests were all negative.
136.5 Clinical Picture
The condition is subacute. It starts with numbness of the feet, weakness of the lower
extremities, which creeps upwards, stumbling, inability to walk, sensory loss, and
incontinence. The condition progressed to paraparesis in a few weeks. It may be
complicated by cerebral vasculitis. Neurological exam shows weakness or paralysis, spasticity, increased tendon refl exes, and sensory loss. The thoracic cord is most
commonly involved; the cauda may be involved as well. There is no history of
infection or other know vascular causes
136.6 Diagnosis
Diagnosis is based on:
1. Clinical fi ndings
2. MRI of the spinal cord shows leptomeningeal enhancement
3. MRA shows vasculitis, may be with occlusion
4. Laboratory tests are negative for bacterial and viral diseases, CSF chemistry,
cytology, microbiology, or virology
Vasculitis needs to be differentiated form sarcoidosis, connective tissue diseases,
etc.
136 Myelopathy due to Spinal Cord Vasculitis
467
136.7 Management
Medcial treatment consists of:
1. Steroids include methylprednisolone, dexamethasone pulse therapy, oral pred-
nisolone for followup
2. Immunosuppression medications
(a) Cyclophosphamide
(b) Azathioprine
(c) Mycophenolate Mofetil
Plasma exchange, immunoglobulin, surgery are rarely indicated.
Reference
Salvarani C, Brown RD, Calamia KT, et al. Primary CNS vasculitis with spinal cord involvement.
Neurology. 2008;70(24 Pt 2):2394–400.
Reference
469
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_137
137
Epidural Varix
137.1 Definition
Varices are abnormal persistently dilated, elongated, and tortuous spinal epidural
veins. These varices may be asymptomatic, but in rare cases may present as radiculopathy or myelopathy.
137.2 Incidence
Spinal epidural varix causing neurologic compression is a rare entity. Gümbel et al.
( 1969 ), reported an incidence of 0.5 % of isolated nerve root compression caused by
varices among 1091 cases of sciatica. Wong et al. ( 2003 ), reported 2 cases. Pekindril
and Yaniz reported a case and reviewed the literature.
As regards to the myelopathy, the condition is extremely rare: Bapat and Metkar
( 2006 ), reported the fi rst case of acute tetraplegia. Dickman et al. ( 1988 ), reported a
case of myelopathy due to epidural varix.
137.3 Etiology
Epidural varicosities are of two categories: primary and secondary.
Primary epidural varices: these are extremely rare and they develop in the
absence of a predisposing pathology (see Dickman et al. 1988 ) Hypothesis:
The epidural venous plexus is a network of veins in the epidural spaces of the
whole spine from the cranium down to the sacrum. It is a valveless system which
allows effective venous return by allowing reversal of the blood fl ow during pressure fl uctuation with respiration, increased abdominal pressure as effort with
Abstracted from Bapat and Metkar ( 2006 )
470
coughing, sneezing, valsalva maneuver, and also increased jugular vein pressure
during neck movements and actions. The epidural pressure rises during these efforts
to come back down when they are terminated. If these factors persist they may lead
to primary varices.
Secondary varices are caused by:
• Congenital spinal anomalies: diastomyelia, AVF, dysplasia
• Spinal trauma leading to scaring
• Spinal stenosis and foramenal stenosis, deformity, stenosis
• Disk prolapse
• Vena cava obstruction or occlusion inferior vena cava (IVC) or superior vena
cava (SVC) The details of IVC occlusion have been thoroughly reported by
Paksoy and Gormus
2004 .
• Abdominal masses compressing the IVC (see Paksoy and Gormus 2004 ): portal
hypertension
• Pregnancy: obesity and epidural lipomatosis
• Jugular vein thrombosis or compression
• Secondary to intracranial hypotension
• Marfan’s syndrome
137.4 Pathophysiology
Myelopathy or radiculopathy are caused by:
• Direct pressure by the ectatic varix as a space occupying lesion. The same varix
on the root will cause radiculopathy.
• Venous congestion, especially in cases of IVC occlusion causes radicular pain.
• Hemorrhage from varix causes hematoma leading to pressure
• Intramural thrombosis
Acute irreversible neurologic defi cit may occur as a result of variceal bleeding or
an acute intramural thrombosis.
137.5 Clinical Picture
137.5.1 Myelopathy
This is a very rare condition. The typical case described by Bapat and Metkar:
Twenty-one year old man with inability to move the four extremities after an
uneventful auto-rickshaw ride. He had no neck injury, no neck pain. He came in
spinal shock, no cranial injury. Radiography of the spine was normal. MRI showed
space occupying epidural lesion over the dura from C7-D1 causing cord
137 Epidural Varix