Elsevier

World Neurosurgery

Volume 110, February 2018, Pages e514-e519
World Neurosurgery

Original Article
Transtentorial Distortion Syndrome: Consistent Complication Following Lateral and Fourth Ventricular Shunting in Adults

https://doi.org/10.1016/j.wneu.2017.11.032Get rights and content

Background

Complex hydrocephalus affecting lateral and fourth ventricles separately is occasionally managed with cerebrospinal fluid diversion via supratentorial and infratentorial ventricular catheters. The optimal configuration to reduce complications is currently unknown in adults. We describe a consistently similar clinical presentation of patients with complex hydrocephalus and a fourth ventricle separately drained by infratentorial shunt insertion.

Methods

This was a retrospective single-center case series. Medical notes were reviewed for clinical presentation, brain imaging, and neurophysiologic tests results. All patients underwent intracranial pressure monitoring (ICPM). Outcomes were determined by ventricular appearance on brain imaging computed tomography and symptomatic improvements postoperatively.

Results

Five adult patients referred to the hydrocephalus service had separate infratentorial and supratentorial shunt systems. A common clinical presentation was observed, including lower motor neuron facial palsy (confirmed with electrophysiology), ophthalmoplegia, dysarthria, impaired gait headache, and nausea. We refer to this as transtentorial distortion syndrome. Twenty-four-hour ICPM demonstrated clear low pressures. All patients underwent shunt revision connecting the transtentorial shunts via a Y-connector and the addition of a distal valve. All subjects had improved ventricular appearance on computed tomography scans post revision, and normalization of ICPM was observed. In the follow-up period of 6 months, no patient required further shunt revision.

Conclusion

To prevent transtentorial distortion syndrome, supratentorial and infratentorial shunt constructs in adults with encysted fourth ventricles should be similar to the shunt systems widely known in the pediatric population with Dandy-Walker syndrome (i.e., joint output to a single valve distal to the connection of the 2 proximal drainage catheters).

Introduction

Treatment of hydrocephalus is predominantly by cerebrospinal fluid (CSF) diversion through ventriculoperitoneal (VP) shunt insertion. Rarely, patients develop hydrocephalus requiring separate CSF drainage of the lateral ventricles and the fourth ventricle. Such conditions requiring this unusual shunt configuration include Dandy-Walker syndrome and other posterior fossa cystic formations such as a trapped or isolated fourth ventricle (TFV), resulting from infection, hemorrhage, inflammation, or postoperatively.1, 2, 3

Many of these patients require both supratentorial and infratentorial ventricular shunts to manage these symptoms.

However, such complex shunt arrangements are fraught with their own complications, particularly cranial nerve palsies.4, 5, 6 Imbalanced CSF drainage between the supratentorial and infratentorial ventricles is a well-described complication after fourth ventricular shunting in the pediatric population.3, 5, 6, 7 Although rare, a proportion of adult patients with complex hydrocephalus may also require supratentorial and infratentorial shunt systems.8, 9, 10 There are little published data in the adult population on the most effective strategy to manage a transtentorial shunt arrangement and to prevent complications of resulting from differential compartment drainage.

This series reports 5 adult patients with both supratentorial and infratentorial shunts, presenting with a common clinical picture. We consider the anatomic and physiologic rationale for the complications observed and propose a strategy to prevent and manage this rare condition.

Section snippets

Materials and Method

A retrospective single-center case series of 5 patients referred with complex hydrocephalus with both supratentorial and infratentorial shunts in situ, covering an overall period of 10 years, from September 2005 to September 2015. Clinical and operative records were reviewed for demographic information, medical history, and interventions. Clinical examination findings were verified independently by a consultant neurologist.

All 5 patients underwent 24-hour ICPM using intraparenchymal

Results

Five patients (1 male, 4 female), aged 38.6 ± 10.6 years (mean ± standard deviation), with a range of pathologies and comorbidities, were referred due to deteriorating symptoms since the insertion of supratentorial and infratentorial shunt constructs (Table 1).

Discussion

We present an unusual case series of 5 adults suffering with complications resulting from an imbalance of cerebrospinal fluid drainage between supratentorial and infratentorial compartments. A consistent observation was the constellation of 1) nausea, 2) headache, 3) lower facial nerve weakness (bilateral), 4) abducens nerve palsy (often bilateral), 5) dysarthria, 6) lower-limb weakness with impaired gait, and 7) sparing of the vestibulocochlear nerve but occasional involvement of trochelar,

Conclusion

This study presents a common complication phenotype occurring due to differential drainage of supratentorial and infratentorial ventricles. We emphasize the importance of treating fourth ventricular expansion early and recognizing symptoms indicative of cerebellar distortion. Even with early recognition and management, it appears symptoms in adults can be more persistent that those previously reported in children. To avoid development of transtentorial distortion syndrome, supratentorial and

References (18)

  • E. Ferrer et al.

    Third ventriculostomy and fourth ventricle outlets obstruction

    World Neurosurg

    (2013)
  • T. Takeda et al.

    Interstitial fluid pressure in the facial nerve: relationship between facial nerve pressure and cerebrospinal fluid pressure

    Am J Otolaryngol

    (1989)
  • D.H. Harter

    Management strategies for treatment of the trapped fourth ventricle

    Childs Nerv Syst

    (2004)
  • J.C. Hawkins et al.

    Isolated fourth ventricle as a complication of ventricular shunting. Report of three cases

    J Neurosurg

    (1978)
  • O.F. Unal et al.

    Ascending transaqueductal cystoventriculoperitoneal shunting in Dandy-Walker malformation: technical note

    Pediatr Neurosurg

    (2012)
  • H.G. Eder et al.

    Complications after shunting isolated IV ventricles

    Childs Nerv Syst

    (1997)
  • D. Pang et al.

    Progressive cranial nerve palsy following shunt placement in an isolated fourth ventricle: case report

    J Neurosurg

    (2005)
  • P. Spennato et al.

    Bilateral abducent and facial nerve palsies following fourth ventricle shunting: two case reports

    Childs Nerv Syst

    (2005)
  • W.E. Dandy

    The diagnosis and treatment of hydrocephalus due to occlusion of the foramina of Magendie and Luschka

    Surg Gynecol Obstet

    (1921)
There are more references available in the full text version of this article.

Cited by (0)

Conflict of interest statement: L. D. Watkins has received honoraria from and served on advisory boards for Medtronic, Codman, and B Braun. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Portions of this work were presented in abstract form at the Hydrocephalus 2015 Conference of the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders, Banff, Canada, 6 September 2015.

View full text