An Official publication of The Asian Congress of Neurological Surgeons (AsianCNS)

Search Article
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Advertise Subscribe Contacts Login  Facebook Tweeter
  Users Online: 65 Home Print this page Email this page Small font sizeDefault font sizeIncrease font size  

   Table of Contents      
CASE REPORT
Year : 2021  |  Volume : 16  |  Issue : 1  |  Page : 170-173

Endoscopic third ventriculostomy in the fourth ventricle outlet obstruction associated with chiari malformation Type I and syringomyelia: Case report


Department of Neurosurgery, National Hospital Center, Nouakchott, Mauritania

Date of Submission26-Feb-2020
Date of Decision05-May-2020
Date of Acceptance04-Aug-2020
Date of Web Publication23-Feb-2021

Correspondence Address:
Dr. Sidi-Mohamed Salihy
Department of Neurosurgery, National Hospital Center, BP 612, Nouakchott
Mauritania
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajns.AJNS_67_20

Rights and Permissions
  Abstract 

Hydrocephalus by the fourth ventricle outlet obstruction (FVOO) associated with a Chiari malformation type I and syringomyelia is a well-known entity but a rare situation in clinical practice. Although suboccipital craniectomy with the opening of the obstruction membrane appears to be the most physiological approach, by restoring the original pathway of cerebrospinal fluid flow, the endoscopic third ventriculostomy (ETV) represents an important minimally invasive alternative. We report the case of an adult patient with tetra ventricular hydrocephalus by FVOO associated with Chiari malformation and syringomyelia. The ETV alone completely resolved all symptoms, as well as neuroimaging abnormalities on the control magnetic resonance imaging. The ETV is a minimally invasive option for the treatment of hydrocephalus in patients with obstruction at the exit of the fourth ventricle, even in cases associated with Chiari malformation and syringomyelia.

Keywords: Chiari malformation type I, endoscopic third ventriculostomy, foramen of Magendie, fourth ventricle outlet obstruction, hydrocephalus


How to cite this article:
Salem-Memou S, Amal T, Salihy SM, Soumare O, Najat B. Endoscopic third ventriculostomy in the fourth ventricle outlet obstruction associated with chiari malformation Type I and syringomyelia: Case report. Asian J Neurosurg 2021;16:170-3

How to cite this URL:
Salem-Memou S, Amal T, Salihy SM, Soumare O, Najat B. Endoscopic third ventriculostomy in the fourth ventricle outlet obstruction associated with chiari malformation Type I and syringomyelia: Case report. Asian J Neurosurg [serial online] 2021 [cited 2021 Apr 15];16:170-3. Available from: https://www.asianjns.org/text.asp?2021/16/1/170/309652


  Introduction Top


Hydrocephalus by the fourth ventricle outlet obstruction (FVOO) associated with a Chiari malformation type I (CM-I) and syringomyelia is a well-known entity, but a rare situation in clinical practice.[1] FVOO can be congenital or acquired. In adults, the occlusion is acquired rather than congenital, related to infection, head injury, intraventricular hemorrhage, tumors, or Chiari malformation.[2] Conventionally, this association is treated by suboccipital craniectomy with the opening of the obstruction membrane.[3] However, since the advent of neuroendoscopic techniques, endoscopic third ventriculostomy (ETV) has been successfully used to treat hydrocephalus as well as CM-I and syringomyelia.[1],[3],[4] We report a case that illustrates the success of neuroendoscopic treatment in this combination.


  Case Report Top


A 23-year-old male student in the English department at the university presented with intense headaches, nausea, dizziness, and visual blur that had been gradually developing over the past 11 months. No specific pathological history was found during the interview. The patient reported worsening of these symptoms during the past 3 months, with a significant decrease in visual acuity, forcing him to drop out of school. On admission to the neurosurgery department, the neurological examination found a conscious cephalalgic patient, without a neurological sensory-motor deficit. A slight exaggeration of the osteotendinous reflexes in all four limbs was noted. The fundus objectified a bilateral papillary edema stage 2.

Cerebral computed tomography scan revealed hydrocephalus with significant dilatation of all ventricles, including the fourth ventricle, and obliteration of the retrocerebellar space of the cerebrospinal fluid (CSF) [Figure 1]a. Complementary craniocervical magnetic resonance imaging (MRI) confirmed tetraventricular hydrocephalus and showed CM-I and cervical syringomyelia extending to C3 [Figure 1]b and [Figure 1]c. The interpeduncular and prepontine cisterns were significantly compressed and reduced in volume by the very large dilatation of the fourth ventricle [Figure 1].
Figure 1: Sagittal (a) Computed tomography scan demonstrating dilation of all ventricles. Sagittal (b and d) T1-weighted, sagittal (c) T2-weighted magnetic resonance imaging showing dilation of all ventricles, Chiari malformation type I, obliteration of the retrocerebellar cerebrospinal fluid space, and syringomyelia

Click here to view


Faced with this radio-clinical symptomatology, an emergent endoscopic operation was planned. A right transventricular approach was used with an entry point located 1 cm anterior to the coronal suture and 3 cm off the midline. Using a 30°rigid endoscope, a ventriculocisternostomy of the floor of the third ventricle (ETV) was performed. Perforation was performed in the region of the tuber cinereum with a blunt perforator and then dilated with the Fogarty balloon. The endoscope was introduced into the prepontine cistern to check that the procedure was complete. Endoscopic observations of the lateral ventricle, the third ventricle, and the prepontine cistern did not reveal any abnormalities suggestive of previous infection or hemorrhage.

The postoperative course was favorable, with a spectacular improvement in clinical symptomatology and resumption of schooling from the 1st month. The control MRI at 8 months postoperatively showed a virtual disappearance of syringomyelia, the ascension of the cerebellar tonsils, and the resolution of hydrocephalus [Figure 2]. Seen as an outpatient at 11 months postoperatively, the patient was well and had no neurological disturbance.
Figure 2: Sagittal (a) T1-weighted and Sagittal (b) T2-weighted postoperative magnetic resonance images showing complete disappearance of Chiari malformation type I and Syringomyelia with a resolution of hydrocephalus

Click here to view



  Discussion Top


FVOO is a rare but well-established cause of obstructive tetraventricular hydrocephalus.[5] Although numerous studies on FVOO have been published, the pathogenic mechanism of this condition remains unclear. Tetraventricular hydrocephalus by FVOO is usually either acquired, caused by an inflammatory process, or congenital, caused by abnormalities of the posterior fossa, such as Dandy–Walker Syndrome, Chiari malformation, and tuberous sclerosis.[2],[6],[7] In adults, the occlusion is acquired rather than congenital, often related to infection, head trauma, intraventricular hemorrhage, tumors, or Chiari malformation.[2]

FVOO is a rare syndrome that is associated with hydrocephalus and syringomyelia, which are frequently found in patients with CM-I; such an obstruction is caused by the chronic tonsillar herniation.[8] The mechanisms precipitating the onset of hydrocephalus associated with CM-I in adults are unknown. Some authors have proposed that meningoencephalitis, infraclinical inflammation, or even connective tissue proliferation without inflammatory changes may cause obstruction of the exit of the fourth ventricle.[1]

In a combination of CM-I and hydrocephalus by FVOO, the most difficult question is whether CM-I is the cause or consequence of obstructive hydrocephalus. We know that hydrocephalus can cause pressure on the cerebellar tonsils causing them to descend into the foramen magnum. However, overt hydrocephalus is reported in only 7%–10% of patients with symptomatic CM-I.[4] It has been argued that CM-I is primarily a developmental abnormality of the posterior fossa with subsequent arachnoid adhesions. Therefore, any associated hydrocephalus may be due to an obstructive phenomenon at the exit of the fourth ventricle CSF pathway.[9] Some authors believe that in cases of pronounced dilatation of the fourth ventricle with an increase in the size of the lateral cavities and no history of inflammation or hemorrhage, the diagnosis of Luschka's and Magendie's foramina atresia is most likely.[2],[6],[10],[11]

Our case had no known medical history and the endoscopic appearance of the lateral ventricles and the third ventricle showed no evidence of previous meningitis or hemorrhage. In addition, the lack of intraoperative endoscopic or macroscopic exploration of the fourth ventricle exits in our case does not permit to confirm that the hydrocephalus was primary to the FVOO.

The onset of clinical symptoms in adulthood, as in our case, would be explained by a late noncompensation of a constitutional “partial” imperforation of the Magendie foramen which was semi-permeable, obstructing the exit pathways of the CSF. However, the reason for this evolution remains uncertain.[7] Longatti et al. reported ten cases of primary obstruction of the fourth ventricle outflow. These authors endoscopically revealed a type of primitive stenosis such as restriction of the Magendie's contour with a thick and opaque membrane, transparent spiderweb-like membrane, and dense membrane with fissure acting as a valve.[7]

Orakdogen et al. reported a series of four patients who all had CM-I, tetraventricular dilatation, and syringomyelia. All four patients had a membrane obstructing the Magendie foramen visible intraoperatively after posterior fossa cerebral decompression and exploration.[12]

The treatment of these malformations is still controversial. Different approaches have been described for the treatment of hydrocephalus due to FVOO, such as ventriculoperitoneal shunt, ETV, fourth endoscopic ventriculostomy, suboccipital craniectomy with a simple opening of the obstructing membrane or placement of a catheter to maintain its permeability to the cisterna magna or the subarachnoid space of the anterolateral superior cervical spinal cord.[5],[6]

It is now well established that ETV is the standard treatment for obstructive hydrocephalus. Although recent studies have suggested that ETV is a less invasive treatment and an effective strategy for treating hydrocephalus with FVOO.[7],[13],[14],[15],[16] However, in cases associated with CM-I and syringomyelia, some prefer other options that focus on decompression of the foramen magnum. Indeed, posterior fossa craniotomy and membrane excision are still advocated by some.[12],[17]

Orakdogen et al. suggest that suboccipital craniectomy with the opening of the obstructing membrane and placement of a catheter is the most physiological approach, restoring the original pathway of CSF circulation.[5],[12] However, this technique is far from being minimally invasive. In addition, the ventriculo-subarachnoid shunt is a foreign material that can be responsible for complications such as infection and catheter migration.[10]

The principle of ETV is essentially to allow the passage of CSF from the ventricular system to the basal cisterns without the need for the CSF to pass through the fourth ventricle. Therefore, ETV should be beneficial in the case of hydrocephalus due to obstruction at the CSF outlet and the foramen magnum region.[3] The good issue of our case following ETV suggested that primary hydrocephalus was the etiology of the CMI. It has been successfully used by several authors and has, as in our case, solved all pathologies such as CM-I, hydrocephalus, and syringomyelia.[1],[3],[4],[7],[13],[14] In fact, ETV has many advantages. It is a safe physiological procedure and a minimally invasive technique with a low risk of infection and a low recurrence rate.[13] However, some distortions can make it difficult and risky to perform. Among these distortions is an anteriorly dislodged brain stem making the suprasellar and prepontine cisterns very narrow and a basilar trunk very close to the floor of the third ventricle. However in our opinion, inexperienced hands, even under these conditions, ETV is most often achievable.

Endoscopic foraminoplasty by direct fenestration of the membranous obstruction at the exit of the fourth ventricle is another reported treatment option.[5],[7],[18]

Although there have been reports of good results with this more demanding technique, it should be considered only when ETV is difficult or ineffective and should be performed by experienced neuro-endoscopists.[16]


  Conclusion Top


In this case, a pathological association of hydrocephalus by FVOO, CM-I, and syringomyelia is described in an adult without a pathological history. The effectiveness obtained in our case suggests that ETV may be sufficient to treat hydrocephalus as well as CM-I and syringomyelia and could be considered as a first-line treatment in the management of this entity, pending completion of further randomized control trials.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Suehiro T, Inamura T, Natori Y, Sasaki M, Fukui M. Successful neuroendoscopic third ventriculostomy for hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. Case report. J Neurosurg 2000;93:326-9.  Back to cited text no. 1
    
2.
Rougier A, Ménégon P. MRI evidence of membranous occlusion of the foramen of Magendie. Acta Neurochir (Wien) 2009;151:693-4.  Back to cited text no. 2
    
3.
Mohanty A, Suman R, Shankar SR, Satish S, Praharaj SS. Endoscopic third ventriculostomy in the management of chiari I malformation and syringomyelia associated with hydrocephalus. Clin Neurol Neurosurg 2005;108:87-92.  Back to cited text no. 3
    
4.
Hayhurst C, Osman-Farah J, Das K, Mallucci C. Initial management of hydrocephalus associated with chiari malformation type I-syringomyelia complex via endoscopic third ventriculostomy: An outcome analysis. J Neurosurg 2008;108:1211-4.  Back to cited text no. 4
    
5.
Longatti P, Fiorindi A, Feletti A, Baratto V. Endoscopic opening of the foramen of magendie using transaqueductal navigation for membrane obstruction of the fourth ventricle outlets. Technical note. J Neurosurg 2006;105:924-7.  Back to cited text no. 5
    
6.
Giannetti AV, Malheiros JA, da Silva MC. Fourth ventriculostomy: An alternative treatment for hydrocephalus due to atresia of the Magendie and Luschka foramina. J Neurosurg Pediatr 2011;7:152-6.  Back to cited text no. 6
    
7.
Longatti P, Fiorindi A, Martinuzzi A, Feletti A. Primary obstruction of the fourth ventricle outlets: Neuroendoscopic approach and anatomic description. Neurosurgery 2009;65:1078-85.  Back to cited text no. 7
    
8.
Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, et al. Chiari I malformation redefined: Clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 1999;44:1005-17.  Back to cited text no. 8
    
9.
Banerji NK, Millar JH. Chiari malformation presenting in adult life. Its relationship to syringomyelia. Brain 1974;97:157-68.  Back to cited text no. 9
    
10.
Karachi C, Le Guérinel C, Brugières P, Melon E, Decq P. Hydrocephalus due to idiopathic stenosis of the foramina of Magendie and Luschka. Report of three cases. J Neurosurg 2003;98:897-902.  Back to cited text no. 10
    
11.
Carpentier A, Brunelle F, Philippon J, Clemenceau S. Obstruction of Magendie's and Luschka's foramina. Cine-MRI, aetiology and pathogenesis. Acta Neurochir (Wien) 2001;143:517-21.  Back to cited text no. 11
    
12.
Orakdogen M, Emon ST, Erdogan B, Somay H. Fourth ventriculostomy in occlusion of the foramen of magendie associated with chiari malformation and syringomyelia. NMC Case Rep J 2015;2:72-5.  Back to cited text no. 12
    
13.
Mohanty A, Anandh B, Kolluri VR, Praharaj SS. Neuroendoscopic third ventriculostomy in the management of fourth ventricular outlet obstruction. Minim Invasive Neurosurg 1999;42:18-21.  Back to cited text no. 13
    
14.
Oertel JM, Mondorf Y, Schroeder HW, Gaab MR. Endoscopic diagnosis and treatment of far distal obstructive hydrocephalus. Acta Neurochir (Wien) 2010;152:229-40.  Back to cited text no. 14
    
15.
Ferrer E, de Notaris M. Third ventriculostomy and fourth ventricle outlets obstruction. World Neurosurg 2013;79:S20.e9-13.  Back to cited text no. 15
    
16.
Ishi Y, Asaoka K, Kobayashi H, Motegi H, Sugiyama T, Yokoyama Y, et al. Idiopathic fourth ventricle outlet obstruction successfully treated by endoscopic third ventriculostomy: A case report. Springerplus 2015;4:565.  Back to cited text no. 16
    
17.
Huang YC, Chang CN, Chuang HL, Scott RM. Membranous obstruction of the fourth ventricle outlet. A case report. Pediatr Neurosurg 2001;35:43-7.  Back to cited text no. 17
    
18.
Torres-Corzo J, Sánchez-Rodríguez J, Cervantes D, Rodríguez-Della Vecchia R, Muruato-Araiza F, Hwang SW, et al. Endoscopic transventricular transaqueductal Magendie and Luschka foraminoplasty for hydrocephalus. Neurosurgery 2014;74:426-35.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2]



 

Top
 
 
  Search
 
<
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)  

 
  In this article
   Abstract
  Introduction
  Case Report
  Discussion
  Conclusion
   References
   Article Figures

 Article Access Statistics
    Viewed137    
    Printed15    
    Emailed0    
    PDF Downloaded25    
    Comments [Add]    

Recommend this journal