Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
References
29
extension, urinary retention, impotence, and retrograde ejaculation. MRI showed an
intradural mass, well defined at L3 level isointense at T1-weighted imaging and
hyperintense at T2-weighted image with enhancement after gadolinium injection.
Laminectomy at L3, after duratomy, a pinkish mass was found attached to one of the
rootlets on the cauda equine and was ressected entirely. Histopathology showed
capillary hemangioma. Postoperative recovery was attained without evidence of
recurrence. The authors state vascular tumors account for 2–7 % of intraspinal
tumors. Capillary hemangiomas within the boundries of the dura are extremely rare.
They are usually distal in position, i.e., in the conus medullaris or in a root of cauda
equine presenting with myelopathy or radiculopathy. As regards to the pathogenesis
the capillary hemangioma arises from the vessels of nerve roots or from the subpial
vessels. For the pathogenesis there are two hypotheses:
• The lesion may evolve during the early somatic differentiation at the time of
angoblastic differentiation (days 21–24 of embryogenesis) due to the impaired
movement and differentiation of the primitive mesoderm for the embryonic
mesodermal plate.
• It may arise from the vascular structures within the epineurium of the nerve roots
during individual during ontogenesis.
As regards to the histopathology the tumor is multilobular with pseudo-capsule.
Its structure is made of capillaries lined with flattened endothelium. The lobules are
separated by collagen partitions. Mild lymphocytic infiltrations may be found.
Histochemical immune tests are positive against neuron-specific enolase and S-100
protein. The tumor cells stain strongly positive for endothelial markers CD31 and
CD34, which are compatible with the diagnosis of capillary hemangioma.
Reference
Miri SM, Habibi Z, Hashemi M, Meybodi AT, Tabatabai SA. Capillary hemangioma
of cauda equina: a case report. Cases J. 2009;2(1):80.
References
Choi BY, Chang KH, Choe G, et al. Spinal intradural extramedullary capillary hemangioma: MR
imaging findings. AJNR Am J Neuroradiol. 2001;22(4):799–802.
Holanda MM, Sarmento SA, Andrade RV, Nóbrega Ede S, Silva JA. Capillary hemangioma of the
spinal cord: case report. Arq Neuropsiquiatr. 2004;62(2A):357–9.
Kelleher T, Aquilina K, Keohane C, O’sullivan MG. Intramedullary capillary haemangioma. Br
J Neurosurg. 2005;19(4):345–8.
Shin JH, Lee HK, Jeon SR, Park SH. Spinal intradural capillary hemangioma: MR findings. AJNR
Am J Neuroradiol. 2000;21(5):954–6.
31
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_10
1 0
Spinal Intradural Cerebellar Ectopia
10.1 Definition
This is a condition in which cerebellar tissue is found in the spinal canal intradurally. It is independent from the Chiari I malformation, where the cerebellar tonsils
are found in the upper cervical canal and from Chiari II, where the vermis is herniated in the spinal canal.
10.2 Incidence
Chung et al., reported the fi rst case of true isolated cerebellar tissue in the lower
cervical spine.
10.3 Etiology
The exact cause is not known; however, the authors theorized the development of
this anomaly. They think that differentiation of totipotential cells within the dorsal
aspect of an endomesenchymal tract lead to the formation of a structure resembling
the cerebellar tissue.
10.4 Pathology
The mass was intradural dorsal to the spinal cord. It resembled cerebellar hemisphere with densely packed foliae, the dorsal and lateral wall were covered by thickened arachnoid. Microscopically it showed cerebellar cortex comprised of an outer
Abstracted from Chung et al. ( 1998 )
32
and inner molecular granular cells in layers. Deep to the cells were Purkinje cells.
An internal medullary layer was also present.
10.5 Clinical Picture
A boy 14 months old came with progressive scoliosis of 6 months duration. When
he walked the left foot was everted, mild hyperrefl exia of the lower limbs and positive right Babinski sign.
10.6 Diagnosis
Diagnosis is based on the following criteria:
• Radiography: midline segmental anomalies of T1–T3
• MRI: a mass in the spinal canal dorsal to the spinal cord at C6–C7 level. The
mass was isointense with spinal cord in both T1 and T2 weighted images but did
not enhance with gadolinium injection. Below the mass the spinal canal was
wider and a cystic abnormality was seen. T2 showed clefting
10.7 Management
The mass was approached after duratomy and was entirely removed. Twelve months
post-operative there was no recurrence and the neurological status was unchanged.
Reference
Chung CJ, Castillo M, Fordham L, Mukherji S, Boydston W, Hudgins R. Spinal intradural cerebel-
lar ectopia. AJNR Am J Neuroradiol. 1998;19(5):897–9.
10 Spinal Intradural Cerebellar Ectopia
33
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_11
1 1
Cystic Dilatation of the Conus Ventriculus Terminalis
11.1 Definition
The ventriculus terminalis (VT) of the conus medullaris referred to as the fi fth ventricle is the ependymal-lined space in the middle of the conus. It is present in childhood and its presence in adults is rare as dilatation to form a cyst is also rare. Cystic
dilation causes cauda equine syndrome.
11.2 Incidence
Ciappetta et al., reviewed the literature and found 22 cases and added two elderly
patients.
11.3 Etiology
The ventriculus terminalis is identifi able in the embryo between 43 and 48 days
after conception as part of the caudal neural tube and it regresses after birth although
it may be detected by MRI in 2.6 % of children under 5 years of age. For further
details of embryology see Ciappetta et al.; for anatomical details see Kernohan et al.
The cyst represents failure of regression. Possible etiologies according to Brisman
et al.:
1. Failure of fusion of the cranial and caudal neural tube due to congenital disease,
trauma, or ischemia
2. Association between spinal dysraphism and tethered cord
Abstracted from Ciappetta et al. ( 2008 )
34
3. A type of caudal syrinx or hydromyelia, which is not communicating with the
central canal
4. Ciappetta et al., state a possible involvement of the Reissner fi ber in the patho-
genesis of the VT should not be completely excluded. A Reissner fi ber lesion
could involve a localized dilatation of the CSF composition and dynamics caus-
ing a dilatation of the ependymal canal.
However the exact cause is unknown. Ciappetta et al., based on their study data
in surgery and histological characteristic of the lesion stating that the dilatation of
the ventriculus termanalis is distinct from other cystic dilatations of the conus
medullaris.
11.4 Pathology
The ventriculus terminalis, like a ventricular structure is lined by ciliated ependymal cells and is termed the “fi fth ventricle”. It is continuous with the central canal
of the anterior portion of the spinal cord (Ciappetta et al. 2008 ). In 11 cases reviewd
by Celli et al., one had a sacral lipoma and one had Chiari malformation type I. for
detailed histological and ultrastructural characteristics of the VT see Choi et al.
11.5 Clinical Picture
Cystic dilatation of the ventriculus terminalis (CDVT) may be asymptomatic and
may be discovered by an MRI done for other purposes. When symptoms develop
they may be mild or severe and in between. The course may be acute or chronic. The
symptoms are pain in the lower back, lumbar-sacral, sciatica-like. When the cauda
and the conus are involved the patient develops shooting pains in the legs, muscle
weakness, gait trouble, sphincter dysfunction, especially incontinence urinary and
rectal. Sensory impairment may be detected. The distal tendon refl exes are reduced.
The clinical picture is that of conus compression and/or the cauda equine.
11.6 Diagnosis
Diagonsis is based on:
1. History
2. Neurological signs
3. MRI: The cyst is well outlined and ahs signal intensity identical to the CSF on
both T1 and T2 weighted images. Usually there is no contrast enhancement. The
surrounding tissue is thinned out to about 2 mm.
4. Ultrasonography: can be done as preliminary test
11 Cystic Dilatation of the Conus Ventriculus Terminalis
35
11.7 Management
1. Asymptomatic cysts and these incidentally discovered should be treated conser-
vatively, with a follow up clinically and by MRI.
2. Symptomatic cases with neurological defi cit should be operated upon to avoid
further disabilities (cauda equine syndrome). Surgery is done through a laminec-
tomy, a midline myelotomy and cyst drainage may be enough.
3. Others use marsupialization or cystosubarachnoid tube drainage or cyst fenestra-
tion. (see Brisman et al. 2006 , for further details)
The results of surgery are satisfactory with reversal of most neurological defi cits,
although not all of them. Post-operative follow up is important to rule out recurrence and to monitor the result of surgery.
References
Brisman JL, Li M, Hamilton D, Mayberg MR, Newell DW. Cystic dilation of the conus ventriculus
terminalis presenting as an acute cauda equina syndrome relieved by decompression and cyst
drainage: case report. Neurosurgery. 2006;58(3):E585; discussion E585.
Ciappetta P, D’urso PI, Luzzi S, Ingravallo G, Cimmino A, Resta L. Cystic dilation of the ventricu-
lus terminalis in adults. J Neurosurg Spine. 2008;8(1):92–9.
References
37
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_12
1 2
Syringomyelia in Association with Dandy-Walker Syndrome
12.1 Definition
Dandy-Walker complex (DWC) is a condition in which there is atresia of foramena
of Magendie and Luschka causing congenital hydrocephalus. There is partial or
complete absence of the cerebellar vermis, posterior fossa cyst formation, continuous with the fourth ventricle. The cyst expands in the middle line causing the occipital bone to bulge and to dislodge the tentorium and torcula upward. There is an
obstruction of the foramen magnum with alteration of the CSF fl ow dynamics
resulting in cervicothoracic syrinx.
12.2 Incidence
Hammond et al., reviewed the literature and tabulated 17 cases and added their own
adult case of herniation of the DW cyst which blocked the foramen magnum resulting in cervicothoracic syrinx, which was reduced in size after cystoperitoneal shunting. In 2008, Kasliwal et al. (
2008 ), reported a pediatric case of Dandy-Walker
malformation associated with syringomyelia.
12.3 Etiology
The cause of DWC formation is atresia or occlusion of foramen of Magendie or
foramen of Luschka, resulting in congenital hydrocephalus with dilatation of the
fourth ventricle, the aqueduct, the third and lateral ventricles. A cystic structer representing the dilated fourth ventricle expands the midline and eventually herniated;
blocking the foramen magnum and the end result is a syrinx.
Abstracted from Hammond et al. ( 2002 )
38
12.4 Clinical Picture
The condition may be observed in neonates, some of whom may not survive due to
other abnormalities. They will show hydrocephalus, possible papilledema, and
occasional cranial palsy.
12.5 Diagnosis
Diagnosis is based on:
1. Clinical picture
2. X-ray and MRI: will show syrinx and DWC
12.6 Management
Cystoperitoneal shunting, sometimes ventriculopertioneal shunting in addition or
posterior fossa decompression.
References
Hammond CJ, Chitnavis B, Penny CC, Strong AJ. Dandy-Walker complex and syringomyelia in
an adult: case report and discussion. Neurosurgery. 2002;50(1):191–4.
Kasliwal MK, Suri A, Sharma BS. Dandy Walker malformation associated with syringomyelia.
Clin Neurol Neurosurg. 2008;110(3):317–9.
12 Syringomyelia in Association with Dandy-Walker Syndrome
39
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_13
1 3
Myelopathy Due to Duplication of the Laminae
13.1 Definition
This is a congenital condition in which the laminae are duplicated causing a mass
effect resulting in cord compression
13.2 Incidence
Two cases have been reported by Cushman.
13.3 Etiology
This is a congenital abnormality of which the cause is not clear
13.4 Pathology
There is a duplication of the laminae encroaching on the spinal canal causing stenosis and cord compression. The embryology of the laminae can be consulted in textbooks of embryology.
13.5 Clinical Picture
The condition is asymptomatic in childhood and teenage years. With development
of the bone and perhaps a minor trauma, symptoms appear. In the case reported a
23-year-old man developed weakness of the lower extremities and became
Abstracted from Cushman ( 1988 )
40
paraplegic in a few months with sphincteric dysfunctions and parasthesias of the
lower extremities. The refl exes were exaggerated with ankle clonus and positive
Babinski.
13.6 Diagnosis
Diagnosis is based on the following criteria:
1. Radiography of the spine: sharply marginated osseous density inside the poste-
rior elements
2. CT scan: severe spinal stenosis with cord compression due to a posterior wall
marginated osseous density representing the duplication of the laminae.
3. Myelography: complete block at T10–T11
No electrophysiological studies were performed.
13.7 Management
Cord compression was diagnosed and surgical decompression was done. After
exposure of the posterior elements, the laminae were removed. Incomplete recovery
was attained postoperatively.
Reference
Cushman JD. Duplication of cord laminae resulting in cord compression. AJNR Am J Neuroradiol.
1988;9(2):408–9.
13 Myelopathy Due to Duplication of the Laminae