CONGENITA An Anaesthetic Challenge Presenter Dr Mridul Dua Fellow in Paediatric Anaesthesiology Coauthors Dr Sandhya Ghodke Dr Vineela Reddy Nandyala Department of Anaesthesiology Rainbow Childrens Hospital ID: 908633
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GETTING A GRIP ON ARTHROGRYPOSIS MULTIPLEX CONGENITA - An Anaesthetic ChallengePresenter: Dr. Mridul Dua (Fellow in Paediatric Anaesthesiology); Co-authors: Dr. Sandhya Ghodke, Dr. Vineela Reddy Nandyala Department of Anaesthesiology, Rainbow Children’s Hospital, Marthahalli, Bengaluru
INTRODUCTIONArthrogryposis Multiplex Congenita (AMC): spectrum syndrome of multiple, persistent limb contractures, accompanied by associated anomalies, warranting multiple corrective surgeries.
CASE REPORT2 year old female, K/C/O AMC. H/o repeated aspiration and respiratory infections. No H/o congenital heart disease or seizures. Examination: Thin and wasted with multiple contracturesScoliosis, torticollis, restricted neck movementsAnticipated difficult airway, tracheal deviation, mouth breathing, micrognathia, high arched palate, maxillary prognathismDifficult IV access
ANAESTHETIC MANAGEMENTFIRST PROCEDURE: Hip deformity correctionInduction and airway management: Difficult airway cart prepared. Total intravenous anaesthesia (TIVA) chosen and agents that may trigger malignant hyperthermia (MH) avoided. Induction with IV propofol and fentanyl. Laryngoscopy with Macintosh blade: CL Grade IV. Glottis not visualised with AMBU scope. Ventilation with AMBU LMA inadequate. Finally, intubating LMA used for maintaining the airway. Maintenance: Propofol and dexmedetomidine infusionsPain management: IV fentanyl, paracetamol and local anaesthetic infiltration. Regional anaesthesia was not feasible. Postoperative: Extubated once fully awake; shifted to PICU for observation. SECOND PROCEDURE: Child brought to ER with respiratory distress due to pneumonia, 1 month after the surgery. Intubated in the PICU using Airtraq optical laryngoscope. THIRD PROCEDURE: Bilateral adductor tendon release surgery, 6 months later. Torticollis and tracheal deviation had increased.Oral fibreoptic bronchoscopy (with maintenance of spontaneous ventilation) done but ET tube could not be passed. Laryngospasm occurred, managed with IV loxicard and propofol. We failed to intubate with Airtraq, or ventilate with intubating LMA. Finally, nasal fibreoptic bronchoscopy was done, and 3.5 sized uncuffed ET tube successfully passed. Leak sealed with throat pack. Vitals remained stable throughout the procedure.
DISCUSSIONIncidence of AMC: 1 in 3000 live births. The anaesthetic challenges include: Difficult airway and positioningDifficult IV access and difficult regional anaesthesiaCongenital heart disease, cardiovascular instabilityDecreased pulmonary reserve, desaturation, postoperative ventilationMH with volatile agents (not proved), hyperkalemia with succinylcholineMyopathy, prolonged duration of neuromuscular blockers
CONCLUSION
Multi-disciplinary approach, good planning and communication is crucial
for the successful anaesthetic management of AMC children. Airway becomes more challenging as the child grows. Front of neck access may be necessary, and life saving in some situations.
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