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
157
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_51
5 1
Malfunction of a Ventriculo-Peritoneal S h u n t
51.1 Definition
This is a condition of paraparesis secondary to a malfunction of ventriculo- peritoneal
shunt for hydrocephalus resulting in acute expansion of cervical syrinx.
51.2 Incidence
According to Lee et al., a retrospective study showed 4 cases of weakness of the
lower extremities amongst 70 cases of ventriculoperitoneal shunt malfunctioning.
51.3 Etiology
A ventriculoperitoneal malfunction or obstruction force reopening of the central
canal and propagates increasing pressure in it and causing expansion of the syrinx,
especially when the fourth ventricle foramina are blocked by previous meningitis.
This increased syrinx pressure causing mechanical pressure on the neural tracts as
well as vascular compromise resulting in paraparesis.
51.4 Clinical Picture
From the case described by Beswick et al.:
The 16-year-old female patient had the vetriculoperitoneal shunt for post-meningitis hydro-
cephalus. She had repeated malfunctions, which were corrected by reinsertion. She devel-
oped paraparesis associated with head, neck, and shoulder pains. She underwent posterior
Abstracted from Beswick et al. ( 2005 )
158
fossa decompression for Chiari I. Reinsertion of the shunt gave temporary relief. She devel-
oped spastic paraparesis. CT scan showed enlarged ventricles and MRI showed an exten-
sive syrinx from C1-T10. The syrinx acts as a fi fth ventricle for the obstructed shunt.
51.5 Management
Revision of the malfunctioning shunt as early as possible especially in the presence
of a syrinx. Although it has been suggested that prompt revision may result in good
outcome this does not appear to be universally so.
Reference
Beswick E, St George EJ, Solanki GA. Paraparesis secondary to expansion of cervical syrinx: an
unusual presentation of ventriculo-peritoneal shunt malfunction. Pediatr Neurosurg.
2005;41(6):334–6.
51 Malfunction of a Ventriculo-Peritoneal Shunt
159
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_52
5 2
Viper Antivenin Induced Brown-Séquard Syndrome
Description This is a unique case of myelopathy induced by antivenin injection.
Guillian-Barré Syndrome, which follows a viral infection or immunization, and is
an acute infl ammatory demyelinating neuropathy of acute onset affecting the
peripheral and cranial nerves, there is no similar affection of the spinal cord.
The case presented was a 40-year-old woman who received antivenin (polyvalent enzyme-refi ned globulin). She developed an immunological infl ammation.
The snake was a green pit viper and the antivenin was prepared in India. She manifested Brown-Séquard syndrome, confi rmed by computerized tomography. It was
manifested by right hemiplegia and absence of pain and temperature sensation on
the left half of her body below the 4th rib. There was loss of vibration sense and
joint position sense on the right side of the body. The neurological examination
indicated a defi cit at the level of C6. The follow up of the patient showed recovery
after 2 years.
Although, the pathology of the lesion was not determined this complication from
the antivenin may be important since there are many areas in the United States
where snakebites are not uncommon. There will be a possibility of such a complication of the antivenin serum; although, rarely. The venin itself may cause myelopathy. There is no defi nite Pathophysiology of the Brown Séquard syndrome; it might
be an immune reaction to the serum with an infl ammatory manifestation in the spinal cord itself. Since Guillian-Barré syndrome has developed as a reaction to immunization (fl u vaccine) this might be a weak supportive cause.
Abstracted from Biswas et al. ( 2004 )
160
Reference
Biswas R, et al. Anti-venom-induced myelopathy in semipoisonous snakebite. Int J Clin Pract.
2004;58(6):645–6.
52 Viper Antivenin Induced Brown-Séquard Syndrome
Part IV
Idiopathic
163
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_53
5 3
Baastrup’s Disease Causing Neurogenic Claudication
53.1 Definition
This is a condition where the adjacent spinous processes (kissing spines) come in
close contact due to hyperextension and increasing lumbar lordosis and when continued it leads to neoarthosis and osteoarthritis with synovial lining. Also called
Lumbar Interspinous Bursitis
53.2 Incidence
Bywaters and Evans conducted an autopsy study on 152 lumbar spines to fi nd out
the prevalence of Baastrup’s disease (BD). They noted pseudojoints, chondroid
metaplasia, endochondral ossifi cation and gross osteoarthritic bone erosion with
eburnation. They defi ned a “bursal index” to identify spines at risk of developing
this disease. The range was from 6.2 to 22.1 %. The spines with lower index were
more predisposed to the disease. Hagner suggested that there is higher prevalence in
professional divers mostly at L3-L4, L4-L5.
53.3 Etiology
Kissing spines occur in chronic hyperextension of the spine as in professional divers
adopting that position. It also develops in people with increasing lordosis. Associated
instable disc segments may contribute to the development of the disease. Diffused
fatty degeneration of the paraspinal muscles may predispose the disease due to
accompanying lordosis, obesity, and chronic limitations of hip fl exion.
Abstracted from Rajasekaran et al. ( 2003 )
164
53.4 Pathology
As mentioned above there are kissing spines processes with chondrifi cation, ossifi cation, degeneration, and bursa formation, associated lumbar degenerative
conditions.
53.5 Clinical Picture
Only one case of jumbo-bursa developed and penetrated in ligmentum fl avum epidurally and caused cauda equina symptoms as reported by Rajasekaran et al., and is
the main source for this subject and from which the clinical picture is summarized:
A 50-year-old female presenting with chronic lower back pain increasing with
activity and becoming progressive to neurologic claudication pain. Physical
exam showed palpable steps at L3-L4 and L4-L5, which were tender. Paraspinal
muscles were spastic on fl exion.
Radiography showed Grade I anterolisthesis of L3 over L4 and Grade II L4 over L5.
MRI T2-weighted images: neoarthrosis between the spinous processes with inter-
spinous bursitis, indicating Baastrup’s disease. A cyst was seen in posterior epi-
dural space at the same level with resultant spinal canal compromise mass. Minor
disc degeneration was seen; no pressure was noted.
53.6 Management
Surgery was indicated to resolve the spinous process artherosis, pain and to the
intraspinal cyst. At surgery neoarthrosis was confi rmed. The adjacent surfaces of the
spinous processes were facetted with well-formed pseudoarthotic cavity, of which
was formed by a thick fi brous wall and was found to have an hourglass communication intraspinally through midline cleft of the ligamentum fl avum. The intraspinal
cyst, thin walled, fi lled with fl uid and tense was causing epidural compression. The
spinous processes were removed, the cyst excised and vertebral stabilization done
by posterior Moss-Miami fi xation along with intertransverse fusion.
The result was relief of spinal pain and neuroclaudication. In other cases there
may be a failure due to associated degenerative diseases, such as disc prolapse,
osteoarthritis, spina bifi da occulta, spinal stenosis, etc.
Gato et al., reported a case of cervical myelopathy caused by osteophytic lipping and pseudoarthosis between the posterior tubercle of C1 and the spinous
process of C2
Updates
Abstracted from Signla et al. 2014
53 Baastrup’s Disease Causing Neurogenic Claudication
165
Case Report
A 67-year-old male presented with a gradually progressive low back pain of 2 years
duration. The patient had no history of trauma, radiation of pain, or any suggestion
of claudication, nor was there any comorbidity. The pain was aggravated by extension and relieved by fl exion. Swelling and neurological defi cit were not present;
however, muscle spasm was Radiographs of the spine revealed degenerative
changes in the lumbosacral spine, along with articulation of spinous processes at
lumbar spine at all levels, suggestive of Baastrup’s disease, commonly know as
kissing spine. The patient chose a conservative course, with week’s course of analgesics and muscle relaxants and then started spinal fl exion exercises. At 6 month
follow up he had signifi cant improvement.
Reference
Singla A, Shankar V, Mittal S, Agarwal A, Garg B. Baastrup’s disease: the kissing
spine. World J Clin Cases. 2014;2(2):45–7.
Reference
Rajasekaran S, Pithwa YK. Baastrup’s disease as a cause of neurogenic claudication: a case report.
Spine. 2003;28(14):E273–5.
Reference
167
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_54
5 4
Castleman’s Disease
54.1 Definition
Castleman’s disease (CD) is a rare lymphoproliterative disorder of unknown causes,
which involves the lymph nodes and less commonly the extranodal tissues.
Synonyms: angiofollicular hyperplasia, nodal hyperplasia, giant lymph node
hyperplasia, angiomatous lymphoid hamartoma, follicular lymphoreticuloma.
54.2 Incidence
CD is rare and its involvement of the central nervous system (CNS) is rarer. In the
spinal canal, only four cases have been reported: by Alper et al. ( 1996 ), from C6 to
T2; by Eisenstat et al. ( 2002 ), from T8 to T11 by Kachur et al. ( 2002 ), from T3 to
T5; and by Lee et al. ( 2002 ), from T2 to T3.
54.3 Etiology
The disease has no defi nite etiology. It is theorized to be:
• infl ammatory origin
• bacterial: although, no bacteria have been isolated
• hamartomatous process
• a response to an unknown stimulus with defective immunoregulation
168
54.4 Pathology
The sites affected by Castleman’s disease are:
• The nodal sites of CD involves most commonly the mediastinum, cervical, axil-
lary, less commonly it involves the mesenteric, retroperitoneal, and pelvic glands.
• The extranodal sites have been described in the larynx, thymus, lung, pericar-
dium, and vulva.
• In the CNS it presents as leptomeningeal mass, which may be well circumscribed
and may cause compression.
Microscopic fi ndings of CD are that it is a distinct lymphoproliferative disorder
characterized by lymphoid hyperplasia, with blood vessel proliferation. CD is
divided into two types: hyaline-vascular and plasma cell. The former is more common and is characterized by lymphoid follicles penetrating vessels and interfollicular vascular proliferation. This kind is usually asymptomatic. The plasma cell form
however, is associated with systemic symptoms. Large hyperplastic follicles with
intervening sheets of plasma cells create the plasma cell form of CD.
54.5 Clinical Picture
Although the disease is benign and frequently asymptomatic, the multicentric forms
may be aggressive; it is most commonly mediastinal. However, this presentation
focuses on compression myelopathy by CD. Involvement of the brain is far more
common than that the spinal cord. Four cases have been reported, which presented
as an epidural mass, causing spinal cord compression that produced constitutional
symptoms such as fever, headache, and lab abnormalities. The result is sensorymotor dysfunction at the level of the lesion and sphincteric dysfunction. The laboratory tests in CD show an elevated erythrocyte sedimentation rate (ESR), anemia,
hypergammaglobulinemia, luekocytosis, and thrombocytosis. A tissue biopsy will
show one or more typical pictures of CD better than imaging. Plain radiography
may be negative; magnetic resonance imaging (MRI) and computed tomography
(CT) myelography will show an intraspinal space-occupying lesion, similar to intraspinal tumors.
54.6 Diagnosis
Diagnosis is based on:
The disease should be differentiated from rheumatoid arthritis, Rosai-Dorfman
Disease (see sinus histiocytosis with massive lymphadenopathy entry), Sjogren syndrome, polyneuropathy, organomegaly, indocrinopathy, monoclonal gammopathy,
skin changes syndrome, autoimmune disease, lymphoma, plasma cell granuloma,
and Kaposi’s sarcoma. For further details see Kachur, et al.
54 Castleman’s Disease