Syringomyelia Regression after Shunting of a Trapped Fourth Ventricle.

0.08 MB·English

by  Morina, Dukagjin

Clinics and Practice 2012; volume 3:e1
Syringomyelia regression after
Case Report
shunting of a trapped fourth Correspondence: Athanasios K. Petridis, Wedau
Kliniken Duisburg, Germany, Zu den Rehwiesen
ventricle
A 28-year-old female was admitted in our 9, 47055 Duisburg, Germany. Tel. [phone].
E-mail: [email protected]
department because of a large right-sided
Dukagjin Morina, Athanasios K. Petridis, intracerebral hemorrhage as a result of a rup-
Key words: trapped fourth ventricle, syringomyelia.
Friederike S. Fritzsche, tured arteriovenous malformation. She under-
Georgios Ntoulias, Martin Scholz went neurosurgical and endovascular treat- Received for publication: 6 September 2012.
Department of Neurosurgery, ment. Due to a post hemorrhagic hydro- Revision received: 14 November 2012.
cephalus external ventricular drain was per- Accepted for publication: 6 December 2012.
Wedau Kliniken Duisburg, Germany
formed. During the intensive care treatment
she unfortunately developed meningitis. After Conflict of interests: the authors declare no
potential conflict of interests.
antibiotic treatment, the meningitis was cured
and a ventriculo peritoneal shunt was per-
Abstract This work is licensed under a Creative Commons
formed. However, the follow up imaging,
Attribution NonCommercial 3.0 License (CC BY-
revealed an isolated enlarged fourth ventricle NC 3.0).
We describe a case of progressive despite shunting of the lateral ventricles.
syringomyelia following post-infectious trapped Therefore a shunting of the fourth ventricle ©Copyright D. Morina et al., 2013
fourth ventricle (TFV), which resolved after has been done as well. Nearly 22 months later Licensee PAGEPress, Italy
shunting of the fourth ventricle. A 28-year-old the patient presented again with secondary Clinics and Practice 2013; 3:e1
doi:10.4081/cp.2013.e1
female who had previously undergone treat- walking and coordination impairment.
ment of intracerebral hemorrhage and menin- Neurological examination showed increasing
gitis developed a hydrocephalus with TFV. After ataxia and eye movement disorder beside the
3 years she developed disturbance of walking known left-sided hemiparesis. Cranial comput-
and coordination. Cranial-CT revealed an ed tomography (CT) demonstrated remarkable Discussion
enlargement of the shunted fourth ventricle as dilation of fourth ventricle indicating a shunt
a result of shunt dysfunction. Furthermore a dysfunction. Furthermore syringomyelia with-
The fact that acquired lesions in region of
cervical syringomyelia developed. The patient out tonsil herniation has occurred, which was
the foramen magnum like Chiari malforma-
underwent a revision of a failed fourth ventricu- identified through cervical magnetic resonance
tion, hydrocephalus, tumors of the posterior
lo-peritoneal shunt. Postoperatively, syringo- imaging (MRI) extending from C2 to C7. The
fossa posterior or CSF-over drainage are asso-
myelia resolved within 6 months and the associ- complete replacement of the dysfunctional
ciated with tonsil herniation leading to a sec-
ated neurological deficits improved significant- shunt system was immediately performed.
ly. An insufficiency of cerebrospinal fluid drain- Postoperatively, the syringomyelia resolved ondary syringomyelia is well established.6
ing among patients with TFV can be associated within 6 months and the associated neurologi- The pathophysiology of syrinx formation
with communicating syringomyelia. An early cal deficits improved as well. An entire resolu- still remains unclear. However, pressure differ-
detection and treatment seems important on tion of the trapped fourth ventricle was con- ence between the cranial cavity and the spinal
resolving syringomyelia and avoiding perma- firmed on CT scan (Figure 1). central canal causes CSF flow disturbance at
nent neurological deficits. Ventriculo-peritoneal
shunt in trapped fourth ventricles can resolve a
secondary syringomyelia.
Introduction
Trapped fourth ventricle is a rare and uncom-
mon entity which has been observed as a compli-
cation after intraventricular hemorrhage, infec-
tion/meningitis or as a result of chronic over
drainage after hydrocephalic shunting.1-4A post-
infectious occlusion of fourth ventricle outflow
(foramina of Luschka and Magendie) and sylvian
aqueduct is the second most common cause for
the development of trapped fourth ventricle.5An
increased cerebrospinal fluid (CSF) pressure
within the fourth ventricle can lead secondary to
the enlargement of the central canal in terms of
communicating secondary syringomyelia. The Figure 1. A) Trapped fourth
exact pathophysiological mechanism of develop- ventricle. B) The trapped
fourth ventricle lead to
ing syringomyelia generally is not well estab-
syringomyelia. C) Shunting
lished and remains yet controversial although the fourth ventricle with
several theories have been postulated. regression of the fourth
We report a case where a post-infectiously ventricle size and regression
of the syringomyelia.
trapped fourth ventricle leads to syringomyelia.
[Clinics and Practice 2013; 3:e1] [page 1]
Case Report
the cranio-cervical junction seems to play a proved as a simple, feasible and effective aqueductal stenosis as a cause of congen-
crucial role. Initially, it was hypothesized that treatment modality. ital hydrocephalus in the inbred rat
cardiac systolic pulsations are directly trans- In cases of syringomyelia caused by Chiari LEW/Jms. Childs Nerv Syst 1991;7:218-22.
mitted from the intracranial cavity to the cen- malformation the therapeutic strategy is dif- 6. Hagihara N, Sakata S. Disproportionately
tral canal inducing syrinx formation in terms ferent than that chosen in the present paper. large communicating fourth ventricle with
of a hydrodynamic water-hammer.7The use of In Chiari I malformations and syringomyelia in syringomyelia: case report. Neurol Med
diagnostic MRI disproved this theory since it general, Isu et al. recommended ventriculo- Chir (Tokyo) 2007;47:278-81.
has been shown that communication between peritoneal shunting, whereas Klekamp et al. 7. Gardner WJ. Hydrodynamic mechanism of
the fourth ventricle and a patent central canal suggest small craniectomy with dural opening syringomyelia: its relationship to myelo-
in not required for the formation of a syrinx. and arachnoids dissection.11,12 cele. J Neurol Neurourg Psychiatry 1965;
Another explanation is that acquired lesions In our case there was no compression of the
28:247-59.
at the cranio-cervical junction can occlude the fourth ventricle caused by stenosis in the fora-
8. Oldfield EH, Muraszko K, Shawker TH,
pulsatile flow of CSF along the subarachnoid men magnum, therefore we decided to treat
Patronas NJ. Pathophysiology of syringo-
space the cranio-cervical region causing an only with shunting.
myelia associated with Chiari I malforma-
increased CSF-pressure gradient from the per-
tion of the cerebellar tonsils. Implications
imedullary subarachnoid space to the spinal
for diagnosis and treatment. J Neurosurg
cord. This causes an increase of fluid within
References 1994;80:3-15.
the central canal creating a syrinx.8
9. Fritsch MJ, Kienke S, Manwaring KH,
In our case meningitis with basilar arach-
Mehdorn HM. Endoscopic aqueductoplasty
noiditis led to an occlusion of the fourth ven- 1. Eller TW, Pasternak JF. Isolated ventricles
and interventriculostomy for the treat-
tricular outflow. A Secondary progressive following intraventricular hemorrhage. J
ment of isolated fourth ventricle in chil-
syrinx developed. Neurosurg 1985;62:357-6.
dren. Neurosurgery 2004;55:372-7.
Treatment extends from placement of 2. Ferreira M, Nahed BV, Babu MA, et al.
10. Schulz M, Goelz L, Spors B, et al.
fourth ventriculo-peritoneal shunt, endoscop- Trapped fourth ventricle phenomenon fol-
Endoscopic treatment of isolated fourth
ic aqueductoplasty and interventriculostomy lowing aneurysm rupture of the posterior
ventricle: clinical and radiological out-
to open fenestration via suboccipital cranioto- circulation: case reports. Neurosurgery
my.9,10We consider that any of these options 2012;70:E253-8. come. Neurosurgery 2012;70:847-58; dis-
has their assets and drawbacks so the choice 3. Harrison HR, Reynolds AF. Trapped fourth cussion 858-9.
of treatment should be individually taken ventricle in coccidioidal meningitis. Surg 11. Isu T, Iwasaki Y, Akino M, Abe H. Surgical
depending on patient history and pathophysi- Neurol 1982;17:197-9. treatment of syringomyelia. Selection of
ology. The purpose of this report is to illus- 4. Martínez-Lage JF, Pérez-Espejo MA, surgical procedures. Neurol Med Chir
trate the possibility of syrinx resolution/ Almagro MJ, et al. Syndromes of over- (Tokyo) 1989;29:728-34.
treatment (caused by a trapped fourth ventri- drainage of ventricular shunting in child- 12. Klekamp J, Batzdorf U, Samii M, Bothe
cle) with the drainage of the fourth ventricle hood hydrocephalus. Neurocirugia (Astur) HW. The surgical treatment of Chiari I
via a ventriculo-peritoneal shunt. Implan- 2005;16:124-33. malformation. Acta Neurochir (Wien)
tation of a fourth ventriculo-peritoneal shunt 5. Yamada H, Oi SZ, Tamaki N, et al. Prenatal 1996;137:788-801.
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