Original ArticleTranstentorial Distortion Syndrome: Consistent Complication Following Lateral and Fourth Ventricular Shunting in Adults
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
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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.