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Uploaded: 2021-11-14
Duration: 1 min 54 s
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Arslan   M,Ahmed   R,Mehmood   K,Mohamed   D     TUH Dublin

Background : Tropomyosin (TPM) 2  myopathy  is a rare genetic disorder 2:100000 live births [ 1 ]) ,It  presents  crucial challenges for  anaesthetic  management due to potential difficult airway, along with decreased  cardiorespiratory  reserves leading to hemodynamic instability at induction, Decreased muscle tone/respiratory muscle weakness is potential risk for airway compromise post-op. In addition it is risk factor for malignant hyperthermia. Patients with TPM2 requires (almost 1/3rd of dose of muscle relaxant to as compared to control.

Anesthetic Implications in TPM2  Myopathy

Discussion :.  TPM2 is a rare muscular disorder. Such patients should be managed with optimum pre-op assessment and preparation. To the best of our knowledge, there is no case reported on management of  anaesthesia  in patient with TMP2 disease. We followed step wise approach to best avoid risk factors for malignant hyperthermia and post-op complications by continuous monitoring, appropriate weaning and decreased use of  anaesthetic  drugs.

Case Report : A 45 year old female known patient of TPM2 presented with Abdominal pain to the  ED of Tertiary hospital of Ireland for emergency appendectomy. Her pre-operative systemic assessment revealed potential difficult airway, limited functional class (Mets<4), along with history, of use of  BiPaP  at home. In addition she had, scoliosis,  atrial   dysarrhythmias ,  hypotonias  and  left lower limb  dysesthesia .

Anaesthesia  induction was done following  preoxygenation  for 5  mins .  Propofol ,  opoids  and  rocuronium  were used in decreased  dosesat  induction. Her  bagmask  ventilation was easy .However, 1 st  attempt with McGrath was failed so  Fiberoptic  intubation was done by consultant  anaesthetist  using ETT 6.5mmID. Post induction invasive lines (CVP/A-line) were inserted for hemodynamic monitoring. Due to risk of malignant hyperthermia we used  propofol -TIVA  along with continuous core Temp monitoring and active warming. Following laparoscopic appendectomy, patient was transferred to PACU  intubated  and ventilated.  Patient was  extubated  in presence of consultant  anaesthetist  after 24 hours monitoring and  stabilisation  of her vitals.

TPM2  myopathy  includes mutations in gene TPM2,was first reported almost 14 years ago as  Nemaline   myopthy,Cap  disease, Congenital fiber type  disproportion,and  core rod  myopathy.About  7years ago,93 families in Europe were diagnosed with TPM2/TPM3  myopathies.These  usually affect proximal limbs more than distal limbs. . A recessive mutation causing  nemaline   myopathy  among the Old Order Amish has recently been identified in the gene for slow skeletal muscle  troponin  T.[2] References : 1 .Sanoudou D,  Beggs  AH. Clinical and genetic heterogeneity in  nemaline   myopathy —a disease of skeletal muscle thin filaments. Trends Mol Med 2001;7:362–8. . 2 .   Mutations in the β- tropomyosin  ( TPM2 ) gene – a rare cause of  nemaline   myopathy l KatiDonner ab MiinaOllikainen ab MaaretRidanpää ab Hans-JürgenChristen c Hans  HGoebel d Mariannede   Visser e KatarinaPelin ab CarinaWallgren-Pettersson ab

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 TPM2  myopathy  includes mutations in gene TPM2,was first reported almost 14 years ago as  Nemaline   myopthy,Cap  disease, Congenital fiber type  disproportion,and  core rod  myopathy.About  7years ago,93 families in Europe were diagnosed with TPM2/TPM3  myopathies.These  usually affect proximal limbs more than distal limbs. . A recessive mutation causing  nemaline   myopathy  among the Old Order Amish has recently been identified in the gene for slow skeletal muscle  troponin  T.[2] References : 1 .Sanoudou D,  Beggs  AH. Clinical and genetic heterogeneity in  nemaline   myopathy —a disease of skeletal muscle thin filaments. Trends Mol Med 2001;7:362–8. . 2 .   Mutations in the β- tropomyosin  ( TPM2 ) gene – a rare cause of  nemaline   myopathy l KatiDonner ab MiinaOllikainen ab MaaretRidanpää ab Hans-JürgenChristen c Hans  HGoebel d Mariannede   Visser e KatarinaPelin ab CarinaWallgren-Pettersson ab

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