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64.8 Prognosis
Ascending cervical lesions may involve the medulla; otherwise, incomplete recovery occurs.
Reference
Planner AC, Pretorius PM, Graham A, Meagher TM. Subacute progressive ascending myelopathy
following spinal cord injury: MRI appearances and clinical presentation. Spinal Cord.
2008;46(2):140–4.
Reference
207
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_65
6 5
Idiopathic Spinal Cord Herniation
65.1 Definition
Idiopathic anterior spinal cord herniation (SCH) is a very rare condition causing
progressive thoracic myelopathy.
65.2 Incidence
Idiopathic anterior spinal cord herniation is an increasingly recognized syndrome,
causing progressive thoracic myelopathy in the middle aged. According to White
and Tsegaye, in (White and Tsegaye 2004 ), the world literature is comprised of 37
publications, recording 61 cases reports. The incidence of spinal cord herniation is
higher in females, affecting only 19 males. In (Sasani et al. 2009 ), Sesani et al.
reviewed the English literature and found 100 cases reported. This increase is due to
the use of MRI as a diagnostic tool, especially in patients presenting with BrownSéquard syndrome; 67 of which were females.
65.3 Etiology
The exact cause of anterior spinal cord herniation is not very clear. There are theories to its cause, namely:
• Congenital anterior dural defect; against the theory is the late onset of the symptoms and in few cases the absence of congenital spinal deformity and dural defect
incidental magnetic resonance imaging (MRI) before the onset of symptoms.
Abstracted from Sesani et al. ( 2009 )
208
• Congenital duplication of the dura mater causes cord herniation through a single
layer of the dura that was adherent to the posterior vertebral surface.
• Previous trauma, which may affect the dentate ligaments, helped by the anatomical curvature of the thoracic spine leading to the ventrally lying cord with subsequent erosion and prolapse.
• Thoracic disc herniation; this may precede cord adherence to the dura with disc
resorption and subsequent dural pocket formation and simultaneous cord
herniation.
• Intradural arachnoid cyst may cause pressure and erosion; against this theory is
the negative computed tomographic myelogram (CTM).
• Erosion of the dura by herniated and calcifi ed disc.
65.4 Pathophysiology
The herniation occurs in the thoracic region from T2–T10, most commonly. The
spinal cord is displaced ventrally or ventrolaterally or just laterally. It may be
sharply angulated within a few segments. That means the cord is tethered by the
herniation. The subarachnoid space ventral to the displaced cord is obliterated
due to the herniation itself or due to scarring. Initially there is involvement of the
spinalthalamic tract, being disturbed within the margins of the dural defect. As
the disease progresses more of the lateral funiculus herniates into the defect involving the corticospinal tract. The posterior columns are usually spared, which gives
a predominant picture of Brown-Séquard syndrome. The herniation will result in
cord lesion, vascular compromise and cord infarction, with subsequent atrophy.
65.5 Clinical Picture
The onset is slow, the course of the disease and its progression are over several years,
with a range of 1–12 years. The sensory modality (spinalthalamic tract) is usually
affected before the motor (corticospinal tract). Sphincter dysfunction may also occur.
The symptoms are those of Brown-Séquard syndrome. It starts by weakness or muscle
atrophy or spasticity or all, on the ipsilateral side. On the contralateral side sensory
defi cit is manifested by loss of temperature and pinprick sensation. The propioreception is preserved. There may be intermittent pain in the thoracic region, chest wall, or
the lower extremities. If the condition is not treated it may progress to paraplegia,
especially due to cord infarction. The refl exes on the weak extremity may be exaggerated and Babinski’s sign may be positive. The senses of position and vibration are
preserved. The condition should be differentiated from spinal cord injury or spinal
tumors, disc degeneration, multiple sclerosis, arachnoid cysts, vascular malformations, etc. Careful history and imagining can easily diagnose the condition.
65 Idiopathic Spinal Cord Herniation
209
65.6 Diagnosis
Plain x-rays may show disc herniation or bony defect within the vertebral body and
endplate at the disc level opposite the cord herniation; calcifi cation may be seen at
that level. MRI is diagnostic and may sow the following signs:
1. Anterior displacement of spinal cord
2. Enlargement of dorsal subarachnoid space
3. Atrophy of the spinal cord with decrease in spinal cord size and signal changes
4. The ventral subarachnoid space in front of the displaced cord is obliterated.
CTM is rarely needed, as it is invasive but may be used to rule out masses dorsal to
the displaced cord or other pathological lesions; it also confi rms the absence of subarachnoid space anterior to the cord. Wada et al. ( 2000 ), illustrates this in their article.
65.7 Prognosis
The condition is progressive if untreated. Surgical correction has a good outcome.
On neglected cases spinal cord herniation will lead to paraplegia. As recurrence is
possible, so are negative results after surgery.
65.8 Management
To avoid increase of the neurological defi cit the spinal cord hernia should be reduced
and the defect in the dura repaired. The approach is usually through a 3-level laminectomy centered over the hernia. The dura is opened in the midline to provide
suffi cient visualization of the herniation. Microsurgery is performed to release the
adhesions between the pia of the herniated spinal cord and the circumferential dural
defect; thus, letting the cord fall back into its normal position inside the dural sleeve.
The dural defect is closed by duroplasty, using DuraGen® (artifi cial dura), it is
gently slipped between the dural defect and the spinal cord and fi xed in place with
a couple of stitches; as described by Massicotte, et al. Other techniques are used by
neurosurgeons. The dentate ligament may have to be divided laterally to facilitate
reduction of the spinal cord to its normal position. If there is a bony defect in the
body of the vertebra it is packed with bone chips taken from the laminectomy and
then covered by the Neuropatch
®
graft (artifi cial dura); as described by White and
Tsegaye. Sasani et al. described the original technique used by Masazawe. To obliterate the defect by muscle fascial fl ap, fat, lympholized dura and Gortex membrane.
But they followed the technique of Makazawa, widening the defect to prevent cord
strangulation. It is most feasible technique since the space is too narrow to pass the
needle and risk cord damage, especially the anterior spinal artery.
65.8 Management
210
References
Sasani M, Ozer AF, Vural M, Sarioglu AC. Idiopathic spinal cord herniation: case report and
review of the literature. JSCM. 2009;32(1):86–94.
Wada E, et al. Idiopathic spinal cord herniation: report of three cases and review of the literature.
Spine. 2000;25(15):1984–8. Review.
White BD, Tsegaye M. Idiopathic anterior spinal cord hernia: under-recognized cause of thoracic
myelopathy. Br J Neurosurg. 2004;18(3):246–9. Review.
65 Idiopathic Spinal Cord Herniation
211
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_66
6 6
Spontaneous Spinal Epidural Hematoma
66.1 Definition
Spontaneous spinal epidural hematoma (SSEH) is a rare condition in which compression myelopathy is due to an epidural hematoma, the cause of which is
unidentifi ed.
66.2 Incidence
According to Kong and Mak ( 2003 ), 300 cases were found in the English literature
review. The condition may occur in children or adults and the aged. According to
Rosenburg et al. ( 2003 ), 25 % of the cases occur in children and they discovered 23
cases in 1994 and increased to 27 in 1998. In children above 2 years it is more common and in males. Male to female ratio is 1.5:1 and the commonest age is 50–80,
accounting for 50 % of the total cases.
66.3 Etiology
SSEH is considered primary and idiopathic. It is unlike the secondary type, which
is due to trauma, even minor epidural puncture, blood dyscrasias, use of anticoagulants, arteriovenous malformation (AVM), hemangiomas, tumors, leukemias, and
excessive garlic ingestion (Rose et al.). A theory is known amongst many authors:
The epidural plexus of veins is valveless and quite numerous. Any sudden
increase of the intravenous pressure such as by valsava’s maneuver as trumpet blowing, increased intrathoracic or intra-abdominal pressure with forceful coughing,
straining for micturation or defi cation, sexual activity, lifting heavy objects, and
Abstracted and reported by Kong and Mak ( 2003 )
212
similar stimulations, such as labor will increase the intraspinal venous pressure
leading to rupture and forming a hematoma. It has happened during sleep which
weakens the above theory. In pregnancy fi ve cases have been reported, possibly due
to increased hemodynamics and hormonal association (Jea et al. 2005 ).
66.4 Pathology
The hematoma occurs most commonly in the cervicothoracic region and generally involves few segments of the cord, most commonly posteriorly (99 %). In the
thoracic region the myelopathy is usually complete due to narrow space and
watershed area leading to ischemia, especially between T4–T8 segments. The
bleeding is almost always venous. Arterial bleeding is very rare. The hematoma,
if not evacuated soon, can cause cord compression leading to ischemia, which is
not recoverable. After decompression the hematoma may recur but very rarely if
hemostatsis is complete. Spontaneous resolution is rare and only for minor
hematomas.
66.5 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.
66.6 Diagnosis
Diagnosis is based on the following criteria:
• Clinical history and fi ndings.
• MRI of the spinal cord is superior to CT scan. It shows dorsal biconvex mass
compressing the spinal cord, its vertical extent, and edema of the cord, since
it has multiplanar capability. Sagittal MRI shows the biconvex dorsal epi-
dural hematoma with well-defi ned contours. In the axial images it appears as
convex or concave mass. The spinal intensity of the hematoma changes over
time and that is why the age of the hematoma may be clearly determined by
the MRI, blood degradation products have magnetic properties. In the acute
phase the hematoma appears isointense on T1-weighted images and hyperin-
tense on T2-weighted images, for more details see Kirazli et al. (Kirazli et al.
2004 ) .
66 Spontaneous Spinal Epidural Hematoma
213
66.7 Management
The greatest majority of cases require emergent spinal cord decompression in order
to reverse the neurological defi cits. This entails laminectomy and evacuation of the
hematoma and control of the source of bleeding. Chances of recovery are best when
decompression is accomplished within 24-h, especially when neurological defi cit is
incomplete. Conservative management is rarely indicated. It must be tried in incomplete cases, where regressing and the size of the hematoma is small (Wagner
et al. 1996 ). Surgery should be resorted to on time in absence of regression or in
cases of progression supported by MRI series. In case of reduction f hematoma size
by MRI conservative treatment may be adopted.
References
Jea A, Moza K, Levi AD, Vanni S. Spontaneous spinal epidural hematoma during pregnancy: case
report and literature review. Neurosurgery. 2005;56(5), E1156.
Kirazli Y, Akkoc Y, Kanyilmaz S. Spinal epidural hematoma associated with oral anticoagulation
therapy. Am J Phys Med Rehabil. 2004;83(3):220–3.
Kong JK, Mak KH. Spontaneous spinal epidural haematoma--an unusual cause of spinal cord
compression. Hong Kong Med J. 2003;9(1):55–7.
Rosenberg O, Itshayek E, Israel Z. Spontaneous spinal epidural hematoma in a 14-year-old girl.
Case report and review of the literature. Pediatr Neurosurg. 2003;38(4):216–8.
Wagner S, Forsting M, Hacke W. Spontaneous resolution of a large spinal epidural hematoma: case
report. Neurosurgery. 1996;38(4):816–8.
References
215
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_67
6 7
Tumoral Calcinosis of the Spine
67.1 Definition
Tumoral calcinosis (TC) is an uncommon pathological entity of obscure etiology,
characterized by dystrophic calcifi cation in periarticular soft tissue. The calcifi cation usually takes the form of calcium hydroxyapatitic crystals surrounded by a
foreign body giant cell histiocytic reaction. TC shows the following characteristics
without common pathogenesis:
• Large size
• Juxta-articular locations
• Progressive enlargement over time
• Tendency to recur after resection
• Tendency to encase neighboring structure
67.2 Incidence
Carlson et al. ( 2007 ), reported one case and reviewed 21 reported cases of tumoral
calcium pyrophosphate dihydrate deposition disease and 39 cases of TC. Kokubun
et al. ( 1996 ) reported a case in the cervical and mentioned one previously reported
case. Durant et al. ( 2001 ) reviewed 21 pathological specimens of tumoral calcinosis
of the spine and stated, “several had radiculopathy in the distribution of the nerve
roots that were being compressed. A few pateints presented with severe cord compression manifested by paraparesis, or sensory defi cits.”
Abstracted from Carlson et al. ( 2007 )
216
67.3 Pathogenesis
This is not known. There are three categories:
1. Familial, inheritable disease
2. Patients on chronic renal dialysis
3. Sporadic
According to Durant et al. ( 2007 ) the following medical conditions were
described as possible, contributing to the pathogenesis:
• Hypertension
• Osteoarthritis
• Rheumatoid arthritis
• Diabetes mellitus
• Trauma to the involved area
• Previous surgery on the spine
• Severely osteoporotic spine
• Scleroderma
In the familial group inherited, there is a frequent association of hyperphosphatemia in siblings.
67.4 Pathology
Tumoral calcinosis is characterized by small descrete calcifi ed nodules, which
coalesce into lobulated tumor. The calcium crystals are identical to hydroxyapatite.
Scanning electron microscopy shows calcium pyrophosphate dehydrogenase
(CPPD). Amongst the crystals collagen fi bers are identifi ed as they form septa,
which are allahed peripherally to fi brous capsule. Microanalysis shows Ca and P1,
the atomic ratio of CalP from 0.91 to 1.08. Around the tumor there is hystiocytes
and foreign body giant cells. In the tumor may be some infl ammatory cells and
capillary vascularity.
67.5 Clinical Picture
Demographically, CPPD is more common in Japan, possibly due to higher incidence of spinal canal stenosis and older women are more predisposed. However, TC
cases with history of minor local trauma, genetic infl uence (sibling), and metabolic
disturbances are common. In Kukabin et al. the mass can be felt in the occipital
region. There was pain and restriction of neck movement.
67 Tumoral Calcinosis of the Spine