BEUDAMED
    FDA Adverse Event Injury Summary report: N

    ACTIVA

    MDR report key: 6415306 · Received March 17, 2017

    Report

    Report Number
    3007566237-2017-01026
    Event Type
    Injury
    Date Received
    March 17, 2017
    Date of Event
    October 1, 2012
    Report Date
    April 20, 2017
    Manufacturer
    MEDTRONIC NEUROMODULATION
    Product Code
    MHY
    PMA / PMN Number
    H020007
    Adverse Event
    Yes
    Report Source
    Manufacturer report
    Reporter Location
    UK
    Reporter Occupation
    HEALTH PROFESSIONAL

    Narratives

    Additional Manufacturer Narrative · 1

    THE DEVICE WAS USED FOR AN OFF LABEL INDICATION. A GOOD FAITH EFFORT WILL BE MADE TO OBTAIN THE APPLICABLE INFORMATION RELEVANT TO THE REPORT. 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

    A RESPONSE RECEIVED FROM THE CORRESPONDING AUTHOR STATED THAT THE OVERALL OUTCOME HAD BEEN SATISFACTORY, AND THAT THE ISSUES HAD BEEN MORE TO DO WITH THE COMPLEXITY OF CEREBRAL PALSY AND THE "CP GUT."

    Description of Event or Problem · 1

    LIN, JP., KAMINSKA, M., PERIDES, S., GIMENO, H., BAKER, L., LUMSDEN, DE., BRITZ, A., DRIVER, S., FITZGERALD-O¿CONNOR, A., SELWAY, R. BILATERAL GLOBUS PALLIDUS INTERNUS DEEP BRAIN STIMULATION FOR DYSKINETIC CEREBRAL PALSY SUPPORTS SUCCESS OF COCHLEAR IMPLANTATION IN A 5-YEAR OLD EX-24-WEEK PRETERM TWIN WITH ABSENT CEREBELLAR HEMISPHERES. EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY. 2017;21(1):202-213. DOI: 10.1016/J.EJPN.2016.11.017. SUMMARY: EARLY ONSET DYSTONIA (DYSKINESIA) AND DEAFNESS IN CHILDHOOD POSE SIGNIFICANT CHALLENGES FOR CHILDREN AND CARERS AND ARE THE CAUSE OF MULTIPLE DISABILITY. IT IS PARTICULARLY TRAGIC WHEN THE CHILD CANNOT MAKE USE OF EARLY COCHLEAR IMPLANTATION (CI) TECHNOLOGY TO RELIEVE DEAFNESS AND IMPROVE LANGUAGE AND COMMUNICATION, BECAUSE SEVERE CERVICAL ANDTRUNCAL DYSTONIA BRUSHES OFF THE MAGNETIC AMPLIFIER BEHIND THE EARS. BILATERAL GLOBUS PALLIDUS INTERNUS (GPI) DEEP BRAIN STIMULATION (DBS) NEUROMODULATION CAN REDUCE DYSKINESIA, THUS SUPPORTING CI NEUROMODULATION SUCCESS. METHODS: WE DESCRIBE THE IMPORTANCE OF THE ORDER OF DUAL NEUROMODULATION SURGERY FOR DYSTONIA AND DEAFNESS. FIRST WITH BILATERAL GPI DBS USING A RECHARGEABLE ACTIVA-RC NEUROSTIMULATOR FOLLOWED 5 MONTHS LATER BY UNILATERAL CI WITH A HARMONY (BTE) ADVANCED BIONICS HI RES 90 K COCHLEAR DEVICE. THIS DOUBLE NEUROMODULATION WAS PERFORMED IN SERIES IN A (B)(6) TWIN WITHOUT A CEREBELLUM. RESULTS: RELIEF OF DYSKINESIA ENABLED CONTINUOUS USE OF THE CI AMPLIFIER. LANGUAGE UNDERSTANDING AND COMMUNICATION IMPROVED. DYSTONIC STORMS ABATED. TOLERANCE OF SITTING INCREASED WITH EMERGENCE OF MANUAL FUNCTION. STATUS DYSTONICUS ENSUED 10 DAYS AFTER ACTIVA-RC REMOVAL FOR INFECTION-EROSION AT 3 YEARS AND 10 MONTHS. HE REQUIRED INTENSIVE CARE AND DBS RE-IMPLANTATION 3 WEEKS LATER TOGETHER WITH 8 MONTHS OF HOSPITAL CARE. TODAY HE IS VIRTUALLY BACK TO THE LEVEL OF FUNCTIONING BEFORE THE DBS REMOVAL IN 2012 AND BACKGROUND MEDICATION CONTINUES TO BE SLOWLY WEANED. CONCLUSION: THIS CASE ILLUSTRATES THAT EARLY NEUROMODULATION WITH DBS FOR DYSTONIC CEREBRAL PALSY FOLLOWED BY CI FOR DEAFNESS IS BENEFICIAL. BOTH SHOULD BE CONSIDERED EARLY I.E. UNDER THE AGE OF FIVE YEARS. THE DBS SHOULD PRECEDE THE CI TO MAXIMISE DYSTONIA REDUCTION AND THUS BENEFITS FROM CI. THIS REQUIRES CLOSE WORKING BETWEEN THE PAEDIATRIC DBS AND CI SERVICES. REPORTED EVENT: A (B)(6) BOY WITH BILATERAL GLOBUS PALLIDUS INTERNUS (GPI) DEEP BRAIN STIMULATION (DBS) FOR DYSTONIC CEREBRAL PALSY (DCP) EXPERIENCED SIGNIFICANTLY REDUCED TRUNCAL AND NECK DYSTONIA AFTER IMPLANT, HOWEVER 3 YEARS AND 10 MONTHS AFTER IMPLANT THEY DEVELOPED EROSION OF THE IMPLANTABLE NEUROSTIMULATOR (INS) THROUGH THE SKIN ASSOCIATED WITH INFECTION/LONG TERM USE OF ANTIBIOTICS. THE AUTHORS SUGGESTED THAT SEVERAL ATTEMPTS AT SURGICAL REPOSITIONING WERE MADE, BUT THESE ATTEMPTS FAILED TO FULLY RESOLVE THE ISSUE AND THE INS WAS ULTIMATELY COMPLETELY REMOVED. APPROX 10 DAYS AFTER REMOVAL THE PATIENT DEVELOPED STATUS DYSTONICUS ASSOCIATED WITH GUT ILEUS AND ACUTE GASTRIC BLEEDING REQUIRING VENTILATION, INTRAVENOUS SEDATION WITH BENZODIAZEPINE, MORPHINE AND PROPOFOL INFUSIONS AND TOTAL PARENTERAL NUTRITION. THE DBS SYSTEM WAS RE-IMPLANTED 3 WEEKS LATER BUT IT TOOK 230 DAYS AFTER THE RE-IMPLANTED DBS SYSTEM TOGETHER WITH MEDICATION TO RESTORE SLEEPING AT NIGHT AND SOME DAYTIME SITTING ALBEIT WITH A WEANING DOSE OF MORPHINE OVER THE FIRST THREE MONTHS FOLLOWED BY DISCHARGE HOME ALONG WITH HIGH DOSE TRANSDERMAL CLONIDINE (900 MG DAILY) AND REGULAR NITRAZEPAM. AS OF 7 YEARS POST-IMPLANT THE PATIENT WAS REPORTEDLY THOUGH TO BE ALMOST BACK TO THE LEVEL OF FUNCTION BEFORE THE EPISODE OF STATUS DYSTONICUS. IT WAS NOT POSSIBLE TO ASCERTAIN SPECIFIC DEVICE INFORMATION FROM THE ARTICLE OR TO MATCH THE REPORTED EVENT WITH ANY PREVIOUSLY REPORTED EVENT.

    Devices

    Seq Brand Generic Product Code Manufacturer Model Lot UDI-DI
    194607 ACTIVA STIMULATOR, ELECTRICAL, IMPLANTED, FOR PARKINSONIAN TREMOR MHY MEDTRONIC NEUROMODULATION 37612

    Patients

    Seq Age Sex Outcome Treatment
    1 5 YR Hospitalization| L| R