MYASTHENIA GRAVIS 2010

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    Iskender Algithmi, MDRagab Shehata, MCs

    Cardiothoracic SurgeryUnit - KAUH

    2010

    1st experience with roboticThymectomy in KAUH

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    Introduction

    7 case underwent robotic Thymectomy in

    KAUH between January 2009 to march2010.

    Patients characteristics, preoperativepreparation, operative data, postoperativeresults will reviewed.

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    Preoperative Characteristics of Patients

    Gender Female 7 (100%)

    Mean age (range) 25 years (1745 years)Mean duration of symptoms 18 (6 64) (months)Osserman stage

    I ocular myasthenia 6 (85%) IIa mild weakness 2 (28%) IIb moderate weakness 3 (44%) III acute sever weakness 1 (14%) IV late sever weakness 1( 14%)

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    Preoperative medication therapy:

    Anticholinesterase 7 (100%) Steroid 7(100%) Azatioprine 2(28%) Cyclosporine 1(14%)

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    Complete labs:

    CBC, U&E, LFT, INR,PTT, TFT

    Pulmonary function test to asses

    respiratory function

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    Radiology

    Chest x ray PA & Lat. And CT chest.To evaluate any Mediastinal massesReveal enlarged thymus in 3 patient

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    Plasmapheresis

    For all patient 3 sessions to:

    - decrease Ab level- improve symptoms- decrease incidence of Myasthenic crises

    Anesthesia assessmentICU referral

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    Surgical technique

    Under general anesthesia

    double lumen endotracheal tube for selectivesingle lung ventilation during the time of operation

    One patient required bronchial blocker.

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    patient is positioned left side up at a 30-degree angle

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    Surgical technique

    A camera port for the three-dimensional 0-degree

    stereo endoscope is introduced through a 15 mmincision in the fifth intercostal space on themidaxillary lineTwo additional thoracic ports are inserted; one inthe third intercostal space on the midaxillaryregion and another in the fifth intercostal spaceon the midclavicular space

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    Surgical technique

    Two arms of the da V inci system are then

    attached to the two access points and another arm is attached to the port-inserted endoscope.During surgery the hemithorax was inflatedthrough the camera port with CO2 ranging inpressure from 6 to 10 mm Hg

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    Surgical technique

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    All anterior mediastinal tissue, including fat

    between the phrenic nerves, and from innominatetvein to diaphragm dissected and removed

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    Surgical technique

    After the hemostasis, a 28F drainage tube is

    inserted through the port of the fifth intercostalspace, the lung is reinflated, and the other wounds are closed.

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    Thymus specimen

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    At the end of procedure

    5 patient is extubated in the operating

    room and, after an adequate period of observation, returns to the floor of thesurgical thoracic ward.2 patient need ICU admission

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    Operative Data

    Robotic time:118 minutes

    (range 95 to 240 minutes)Total operative time: 258 minutes

    range (148 - 303 min)

    No major Intraopertaive complications

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    Post operative

    2 patients requires post op. ventilation for

    6, 48 hours and ICU stays 1, 4 days.

    Chest tube drainage: mean 240 mlPots op analgesia: tramadol 50mg po q6hHospital stay: 4 days (3-10)

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    Complications

    One patient developed post operative

    dyspnoa, wheeze and haemoptysis,Reintubated, ventilated , bronchoscopydone showing bronchial injury,

    ? Bronchial blocker frequent suction patient stabilized andextubated after 2 days

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    Histopathology

    Thymic hyperplasia: 3 patient

    Atrophic thymus: 2 patient

    Normal thymic tissue 2 patient

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    Follow up

    Follow up for 3 to 18 (mean 6)months shows:

    Significant improvement and decreasemedication in 3 patient .Mid to moderate improvement in 2 caseNo improvement in 2 cases

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    Brief review of Thymectomy in

    myasthenia gravis

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    MYASTHEN IA

    Autoimmune disease

    Affects neuromuscular junction receptorsCharacterized by:

    Localized or generalized weakness that improveswith rest

    Inability to sustain or repeat muscle contractions

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    CLASS IF ICAT IONS

    Osserman Group I ocular disease Group IIA mild, general symptoms Group IIB mod, general symptoms Group III acute, severe; lasts weeks-

    months; severe bulbar S. Group IV late; severe, marked bulbar S.

    and general severe weakness

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    MYASTHEN IA

    BULBAR WEAKNESS Oropharyngeal weakness, dysphagia Difficulty breathing Difficulty clearing secretions

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    MYASTHEN IA

    85% have antibodies to ACh receptors in skeletalmuscleAntibody binds close to receptor sitesdestruction of sitesThymus thought to be involved:

    30-50% pts with thymoma have MG After Thymectomy

    25% remission70-80% improve over weeks to months

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    DIAGNOS IS

    Clinical symptoms

    EMGImprovement after EdrophoniumBulbar symptoms = poor prognostic sign

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    TREATMENT

    GOAL:

    Improving neuromuscular function

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    TREATMENT (medical)

    Cholinesterase inhibitors Inhibit hydrolysis ACh increase its concentration Successful in mild disease Pyridostigmine (longer duration, less side effects)

    60 mg po Q6h

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    TREATMENT (medical)

    Corticosteroids Dec AChR antibodies

    80% remissionLimited by long term Side Effect

    G I bleed HTN, hyperglycemia Osteoporosis susceptibility to infection

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    TREATMENT (medical)

    Immunosuppressive Interferes with formation AChR antibodies Side effects

    Bone marrow suppressionSusceptibility to infectionsmalignancy

    Cyclophosphamide, azathioprine,cyclosporine

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    TREATMENT

    Plasmapheresis

    Short term improvement significant decease postop. complications

    IV Immunoglobulin Short term May be given pre op

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    TREATMENT (surgical)

    Thymectomy:Major source antibody productionArrests/reverses diseaseIndicated in:

    Adults with generalized disease Thymoma Thymic hyperplasia Drug resistant MG

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    Thymectomy (approach)

    The transsternal approach:w idespread surgical technique for Thymectomy.The main advantages are: an optimal exposition anddissection of the thymus and perithymic fat tissueLow er risks of vascular and nervous injuries.

    Disadvantages include invasiveness of the approachand a longer hospitalization.

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    Thymectomy

    The transcervical thymectomy:

    minimally invasive technique that is easily acceptedby young patients and neurologists.The advantages are a short hospitalization, fe w er complications and lo w er costs.

    Disadvantage: small space of access makingsurgical manoeuvres difficultImpossible to perform a thymectomy that extends tothe perithymic fat tissue.

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    Thymectomy

    V ATS thymectomy:

    minimally invasive techniquethrough the left- or right-sided approachgood visualization of the anterior mediastinum,achieving an extended thymectomy.

    The disadvantages are the 2-dimensional vie w of theoperative field and the limited manipulation of theendoscopic instruments.

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    Thymectomy

    The robotic Thymectomy:

    Combines the advantages of minimally invasivetechniques (fewer complications, minimalthoracic trauma, decreased postoperative pain,early improved pulmonary function, shorter recovery period and optimal cosmetic resultsthe specific advantages is 3-dimensional vision, ascale motion w ith tremor filtering and articulatedmovements.

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    Results

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    Conclusion

    In patients with MG, robot-assisted thymectomy canbe performed safely and efficiently.The improved visualization and instrument and itsadvanced technology may facilitate the minimallyinvasive approach to the thymus.We prefer to use the left-sided approach because it

    provides an enhanced visualization of the aorticwindow and it reduces the probability of phrenicnerves injury.A longer follow-up is necessary to verify long-term

    clinical results.

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    Thank you