Clinics and Practice 2012; volume 3:e1

Syringomyelia regression after Case Report Correspondence: Athanasios K. Petridis, Wedau shunting of a trapped fourth Kliniken Duisburg, Germany, Zu den Rehwiesen ventricle A 28-year-old female was admitted in our 9, 47055 Duisburg, Germany. Tel. +49.208.7330. department because of a large right-sided E-mail: [email protected] 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 antibiotic treatment, the meningitis was cured potential conflict of interests. 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 only 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 touse C7. The fossa posterior or CSF-over drainage are asso- myelia resolved within 6 months and the associ- complete replacement of the dysfunctional ated neurological deficits improved significant- shunt system was immediately performed. ciated with tonsil herniation leading to a sec- 6 ly. An insufficiency of cerebrospinal fluid drain- Postoperatively, the syringomyelia resolved ondary syringomyelia is well established. 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 observedNon-commercial as a compli- cation after intraventricular hemorrhage, infec- tion/meningitis or as a result of chronic over drainage after hydrocephalic shunting.1-4 A 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.5 An 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 trapped fourth ventricle leads to syringomyelia. of the 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.7 The 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- 11,12 in not required for the formation of a syrinx. and arachnoids dissection. 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- only with shunting. space the cranio-cervical region causing an 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 1994;80:3-15. the central canal creating a syrinx.8 References 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. fourth ventriculo-peritoneal shunt, endoscop- Trapped fourth ventricle phenomenon fol- 10. Schulz M, Goelz L, Spors B, et al. ic aqueductoplasty and interventriculostomy lowing aneurysm rupture of the posterior Endoscopic treatment of isolated fourth to open fenestration via suboccipital cranioto- circulation: case reports. Neurosurgery ventricle:only clinical and radiological out- my.9,10 We 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. use 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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