BEUDAMED
    FDA Adverse Event Injury Summary report: N

    UNKNOWN STRATA VALVE/SHUNT

    MDR report key: 8183335 · Received December 20, 2018

    Report

    Report Number
    2021898-2018-00572
    Event Type
    Injury
    Date Received
    December 20, 2018
    Report Date
    March 13, 2019
    Manufacturer
    MEDTRONIC NEUROSURGERY
    Product Code
    JXG
    Adverse Event
    Yes
    Product Problem
    Yes
    Report Source
    Manufacturer report
    Reporter Location
    MD, US
    Reporter Occupation
    PHYSICIAN

    Narratives

    Additional Manufacturer Narrative · 1

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

    Additional Manufacturer Narrative · 1

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

    Description of Event or Problem · 1

    ABDUL BADRAN, MATTHEW J. SHEPARD, ALEXANDER KSENDZOVSKY, ROGER MURAYI, CHRISTINA HAYES, DEEDEE SMART, PRASHANT CHITTIBOINA. HEMANGIO BLASTOMATOSIS-ASSOCIATED NEGATIVE-PRESSURE HYDROCEPHALUS MANAGED WITH IMPROVISED SHUNT. JOURNAL OF CLINICAL NEUROSCIENCE 58 (2018). DOI: 10.1016/J.JOCN.2018.08.028 ABSTRACT LOW-PRESSURE HYDROCEPHALUS (LPH) IS A RARE CLINICAL DIAGNOSIS, CHARACTERIZED BY NEUROLOGIC DECLINE AND VENTRICULOMEGALY THAT PERSISTS DESPITE NORMAL TO LOW INTRACRANIAL PRESSURE. LPH IS TYPICALLY MANAGED BY NEGATIVE-PRESSURE DRAINAGE VIA VENTRICULOSTOMY, FOLLOWED BY LOW-RESISTANCE SHUNT INSERTION. WE PRESENT THE CASE OF A MIDDLE-AGED MAN WITH A HISTORY OF HEMANGIOBLASTOMATOSIS WHO HAD SPONTANEOUS SUBARACHNOID HEMORRHAGE. HE WAS TREATED WITH A VENTRICULOPERITONEAL SHUNT AND THEN UNDERWENT RESECTION OF A MECKEL¿S CAVE HEMANGIOBLASTOMA AND WHOLE BRAIN IRRADIATION. ONE MONTH LATER, HE PRESENTED TO US WITH WORSENING SYMPTOMS AND HYDROCEPHALUS DESPITE SHUNT INTERROGATIONS AND REVISIONS REVEALING NO MALFUNCTION. VENTRICULOSTOMY DRAINAGE AT NEGATIVE-PRESSURE WAS REQUIRED FOR RESOLUTION OF SYMPTOMS AND VENTRICULOMEGALY, LEADING US TO A DIAGNOSIS OF LPH. THIS WAS SUCCESSFULLY TREATED USING AN IMPROVISED ULTRA-LOW PRESSURE VALVELESS VENTRICULOPERITONEAL SHUNT, WITH MAINTAINED RESOLUTION OF LPH FOR OVER ONE YEAR. THE SYSTEM WAS CREATED BY LIGATING THE DISTAL SLIT VALVE END OF A PERITONEAL CATHETER TO PREVENT REFLUX AND ALLOW SUB-ZERO PRESSURE DRAINAGE BY SIPHONING. REPORTED EVENTS. A (B)(6) YEAR-OLD MALE, WHO PREVIOUSLY UNDERWENT AN UNCOMPLICATED CEREBELLAR HEMANGIOBLASTOMA (HB) RESECTION SEVERAL YEARS AGO, PRESENTED WITH NUMBNESS OF THE LEFT SIDE OF HIS FACE, WORSENING HEADACHES, NAUSEA AND BLURRED VISION OVER THE PAST WEEK. FOLLOWING HIS PREVIOUS HB RESECTION, SURVEILLANCE NEURO-AXIS IMAGING REVEALED THE SPONTANEOUS DEVELOPMENT OF MULTIPLE HEMANGIOBLASTOMAS IN THE POSTERIOR FOSSA AND SPINAL CORD. HE HAD NO RELEVANT FAMILY HISTORY AND GENETIC TESTING EXCLUDED VON HIPPEL-LINDAU DISEASE. AT PRESENTATION, HIS NEUROLOGICAL EXAMINATION WAS REMARKABLE FOR LEFT FACIAL PARESTHESIA AND A LEFT ABDUCENS NERVE PALSY. COMPUTED TOMOGRAPHY (CT) OF THE HEAD REVEALED COMMUNICATING HYDROCEPHALUS. A LUMBAR PUNCTURE REVEALED AN OPENING PRESSURE OF 36 CM H2O, AND CSF ANALYSIS DEMONSTRATED ELEVATED PROTEIN AND XANTHOCHROMIA. GIVEN THE CLINICAL PRESENTATION, WE ATTRIBUTED THE RESULTS TO SPONTANEOUS HEMORRHAGE FROM MULTIPLE HEMANGIOBLASTOMAS. A RIGHT FRONTAL VENTRICULOPERITONEAL SHUNT WAS PLACED USING A FIXED MEDIUM-PRESSURE VALVE, RESULTING IN RESOLUTION OF VENTRICULOMEGALY. THE PATIENT THEN UNDERWENT RESECTION OF THE MECKEL¿S CAVE HEMANGIOBLASTOMA COMBINED WITH LEFT ANTERIOR PETROSECTOMY AND RETROSIGMOID APPROACHES. FOLLOWING INITIAL CONVALESCENCE, THE PATIENT UNDERWENT EXTERNAL BEAM IRRADIATION (4500 CGY IN 25 FRACTIONS) OF THE BRAIN AND CERVICAL SPINE WITH CONCURRENT VALPROIC ACID AS A RADIOSENSITIZER. ONE MONTH LATER, HE PRESENTED AGAIN WITH WORSENING HEADACHES. CT OF THE HEAD REVEALED VENTRICULOMEGALY WITH A TRAPPED LEFT LATERAL VENTRICLE. THE VENTRICULOPERITONEAL SHUNT WAS INTERROGATED IN THE OPERATING ROOM AND FOUND TO BE FUNCTIONAL. THE FIXED MEDIUM-PRESSURE VALVE WAS CHANGED TO A PROGRAMMABLE MEDTRONIC STRATA VALVE SET AT 0.5 (EQUIVALENT TO 3¿4 MMHG). DESPITE THIS, THE PATIENT FAILED TO SHOW RADIOGRAPHIC OR CLINICAL IMPROVEMENT EVEN AFTER THE VALVE WAS DIALED DOWN TO THE LOWEST SETTING (FIG. 2B). OVER THE NEXT MONTH, THE PATIENT BECAME INCREASINGLY CONFUSED WITH URINARY INCONTINENCE AND WORSENING VENTRICULOMEGALY. A SHUNT TAP SHOWED NO EVIDENCE OF PROXIMAL OR DISTAL OBSTRUCTION, BUT THERE WAS CLINICAL IMPROVEMENT AFTER ASPIRATION OF 30 ML OF CSF. THE VENTRICULOPERITONEAL SHUNT WAS REPLACED WITH AN EXTERNAL VENTRICULAR DRAIN, AND IT WAS DETERMINED AFTER SEVERAL FAILED SLOW-WEANING ATTEMPTS THAT DRAINING 15¿20 CC OF CSF PER HOUR WAS NECESSARY FOR CLINICAL AND RADIOGRAPHIC IMPROVEMENT. THIS WAS POSSIBLE WHEN THE VENTRICULOSTOMY WAS LOWERED TO 4 CM BELOW THE TRAGUS. ATTEMPTS TO RAISE THE VENTRICULOSTOMY TO THE LEVEL OF THE TRAGUS WAS ACCOMPANIED BY PROGRESSIVE VENTRICULOMEGALY AND SOMNOLENCE, CONSISTENT WITH LPH (FIG. 2D). FOR LONG-TERM MANAGEMENT, WE USED AN IMPROVISED RIGHT PARIETAL VALVELESS NEGATIVE-PRESSURE SHUNT SYSTEM. THIS WAS MADE UTILIZING A MEDTRONIC BARIUM IMPREGNATED VENTRICULAR CATHETER AND CONVERTIBLE RESERVOIR. USING THIS IMPROVISED LOW-PRESSURE SHUNT, CLINICAL AND RADIOLOGICAL IMPROVEMENT HAS BEEN MAINTAINED BEYOND ONE-YEAR FOLLOW-UP.

    Description of Event or Problem · 1

    ADDITIONAL INFORMATION RECEIVED REPORTED THAT IT WAS NOT A PRODUCT FAULT. THE PATIENT HAD LOW PRESSURE HYDROCEPHALUS NEEDING A REVISION, AND LATER A CUSTOMIZED SYSTEM TO SIPHON MORE CSF THAN USUAL.

    Devices

    Seq Brand Generic Product Code Manufacturer Model Lot UDI-DI
    1027347 UNKNOWN STRATA VALVE/SHUNT SHUNT, CENTRAL NERVOUS SYSTEM AND COMPONENTS JXG MEDTRONIC NEUROSURGERY UNKNOWN-S UNKNOWN

    Patients

    Seq Age Sex Outcome Treatment
    1 45 YR Hospitalization| R