BEUDAMED
    FDA Adverse Event Injury Summary report: N

    UNKNOWN STRATA VALVE/SHUNT

    MDR report key: 7451895 · Received April 23, 2018

    Report

    Report Number
    2021898-2018-00213
    Event Type
    Injury
    Date Received
    April 23, 2018
    Report Date
    April 23, 2018
    Manufacturer
    MEDTRONIC NEUROSURGERY
    Product Code
    JXG
    Adverse Event
    Yes
    Product Problem
    Yes
    Report Source
    Manufacturer report
    Reporter Location
    UK
    Reporter Occupation
    PHYSICIAN

    Narratives

    Additional Manufacturer Narrative · 1

    IF INFORMATION IS PROVIDED IN THE FUTURE, A SUPPLEMENTAL REPORT WILL BE ISSUED.

    Description of Event or Problem · 1

    CLAUDIA L. CRAVEN, IRENE BAUDRACCO, SIMON D. THOMPSON, LEWIS THORNE, LAURENCE D. WATKINS, AHMED K. TOMA. TRANSTENTORIAL DISTORTION SYNDROME: CONSISTENT COMPLICATION FOLLOWING LATERAL AND FOURTH VENTRICULAR SHUNTING IN ADULTS. WORLD NEUROSURGERY 110 (2018). DOI: 10 .1016/J.WNEU.2017.11.032 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 NEURONFACIAL 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). REPORTED EVENTS. A 30 YEAR OLD MALE WITH A HISTORY OF CHRONIC PACHYMENINGITIS CAUSING HYDROCEPHALUS WITH A CERVICAL SYRINX HAD UNDER GONE 4 SHUNT REVISIONS BEFORE REFERRAL. THE PATIENT WAS REFERRED TO THE SPECIALIZED HYDROCEPHALUS TEAM FOR INVESTIGATIONS AND OPTIMIZATION OF THEIR SHUNT CONSTRUCT. SYMPTOMS COMMON TO THE PATIENT INCLUDED LOW PRESSURE HEADACHE, NAUSEA, DIPLOPIA, AND VERTIGO. CLINICAL EXAMINATION DEMONSTRATED BILATERAL 6TH CRANIAL NERVE PALSY, REDUCED RIGHT CORNEAL REFLEX, BILATERAL LOWER 7TH CRANIAL NERVE PALSY, WHEELCHAIR BOUND, POSTURAL AKINETIC TREMOR, BROAD-BASED GAIT, REDUCED LOWER LIMB POWER , AND HEEL-SHIN ATAXIA. RADIOLOGIC COMPARTMENT IMBALANCE WAS EVIDENT IN THE PATIENT PREOPERATIVELY. THE PATIENT WAS INITIALLY MANAGED CONSERVATIVELY WITH CAREFUL ADJUSTMENT TO VALVE SETTINGS. THE ADJUSTMENTS DIDN'T IMPROVE THE CLINICAL SYMPTOMS AND AS A RESULT, THE PATIENT UNDERWENT SURGICAL SHUNT REVISION. POSTOPERATIVELY, THE PATIENT REPORTED IMPROVEMENT OR RESOLUTION OF THEIR HEADACHES, NAUSEA, AND VERTIGO. THE PATIENT WAS ALSO ABLE TO STAND FROM A WHEELCHAIR. HOWEVER, WHEN MOBILE, THEY CONTINUED TO HAVE A BROAD-BASED GAIT. HE ALSO DEMONSTRATED AN INCREASED LOWER LIMB POWER AND THEIR CEREBELLAR SIGNS IMPROVED. THE BILATERAL FACIAL WEAKNESS AND OPHTHALMOPLEGIA WERE PERSISTENT AT 6 MONTHS¿ FOLLOW-UP. THE PATIENT UNDERWENT FURTHER ICPM POST REVISION, DEMONSTRATING A NORMALIZATION IN ICP (FROM 5.00 MM HG TO 0.03 MM HG), AND HE HAD A MODIFIED VENTRICULAR APPEARANCE ON MAGNETIC RESONANCE SCANS POST REVISION, WHICH CORRELATED WITH IMPROVED SYMPTOMS. THE FOURTH VENTRICLE SIZE HAD DECREASED POST INTERVENTION. IN THE FOLLOW-UP PERIOD OF 6 MONTHS, THE PATIENT DIDN'T REQUIRE FURTHER SHUNT REVISION. A 33 YEAR OLD FEMALE WITH A HISTORY OF HYDROCEPHALUS SECONDARY TO HEMORRHAGE COMPLICATED BY MULTIPLE REVISIONS HAD UNDERGONE 30 SHUNT REVISIONS BEFORE REFERRAL. THE PATIENT WAS REFERRED TO THE SPECIALIZED HYDROCEPHALUS TEAM FOR INVESTIGATIONS AND OPTIMIZATION OF THEIR SHUNT CONSTRUCT. SYMPTOMS COMMON TO THE PATIENT INCLUDED LOW PRESSURE HEADACHE, NAUSEA, DIPLOPIA, DYSPHAGIA, AND DYSARTHRIA. CLINICAL EXAMINATION DEMONSTRATED VERTICAL UPGAZE PALSY, BILATERAL 6TH CRANIAL NERVE PALSY, BILATERAL LOWER 7TH CRANIAL NERVE PALSY, WHEELCHAIR BOUND, DYSTONIC POSTURING, SPASTICITY IN ALL LIMBS, AND REDUCED LIMB POWER. RADIOLOGIC COMPARTMENT IMBALANCE WAS EVIDENT IN THE PATIENT PREOPERATIVELY. MR IMAGING IN THE PATIENT DEMONSTRATED THE CHALLENGE OF BALANCING SUPRA- AND INFRA-TENTORIAL CSF DRAINAGE. PREVALVE ADJUSTMENT, THE PATIENT HAD UNDERDRAINAGE OF FOURTH VENTRICLE WITH OVERDRAINAGE OF THE LATERAL VENTRICLES. POST-ADJUSTMENT THE ISSUE WAS VICE VERSA. THE PATIENT WAS INITIALLY MANAGED CONSERVATIVELY WITH CAREFUL ADJUSTMENT TO VALVE SETTINGS. THE ADJUSTMENTS DIDN'T IMPROVE THE CLINICAL SYMPTOMS AND AS A RESULT, THE PATIENT UNDERWENT SURGICAL SHUNT REVISION. POSTOPERATIVELY, THE PATIENT REPORTED IMPROVEMENT OR RESOLUTION OF THEIR HEADACHES AND NAUSEA. THE PATIENT HAD PERSISTENT DIFFICULTY WITH SPEECH; HOWEVER, THIS FLUCTUATED AND PREDATED THE FOURTH VENTRICULAR SHUNT. SHE SHOWED AN IMPROVEMENT IN DYSPHAGIA AND WAS ABLE TO SWALLOW SOFT FOOD SAFELY AT SPEECH AND LANGUAGE ASSESSMENT. THE PATIENT WAS ALSO ABLE TO STAND FROM A WHEELCHAIR. SHE ALSO DEMONSTRATED AN INCREASED LOWER LIMB POWER AND THEIR CEREBELLAR SIGNS IMPROVED. HOWEVER, DYSTONIC POSTURING, SPASTICITY IN ALL LIMBS, AND REDUCED LIMB POWER WERE STILL PERSISTENT. THE BILATERAL FACIAL WEAKNESS AND OPHTHALMOPLEGIA WERE PERSISTENT AT 6 MONTHS¿ FOLLOW-UP. THE FOURTH VENTRICLE SIZE HAD DECREASED POST INTERVENTION. IN THE FOLLOW-UP PERIOD OF 6 MONTHS, THE PATIENT DIDN'T REQUIRE FURTHER SHUNT REVISION.

    Devices

    Seq Brand Generic Product Code Manufacturer Model Lot UDI-DI
    295486 UNKNOWN STRATA VALVE/SHUNT SHUNT, CENTRAL NERVOUS SYSTEM AND COMPONENTS JXG MEDTRONIC NEUROSURGERY UNKNOWN-S UNKNOWN

    Patients

    Seq Age Sex Outcome Treatment
    1 Hospitalization| R