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Gulhane Med J. 2003; 45(4): 367-370

Anestgesia of Arthrogryposis Multiplex Kongenita : Four Cases

Dr. Pakize KIRDEMİR, Dr. Belgin AKAN, Dr. Anıl MARŞAN, Dr. Aykut URFALIOĞLU, Dr. Nermin GÖĞÜŞ.

Arthrogryposis multiplex kongenita (AMC) is a congenital defect characterized by multiple joint contractures. It is characterized by neuropathic and myopathic diseases or any reason that decreases fetal joint mobility. It is recognized at delivery with multiple and general symetric joint anomalies accompanying muscular and soft tissue hypoplasy. Those patients are frequently operated because of multiple joint contractures. Difficult intubation due to restricted mandibula mobility, torticollis, scoliosis, maligne hypertermia are the important problems for the anesthesiologist. Our aim is to publish our four experiences between 2002-2003. Case I: A 3.5 year-old male patient was operated for PEV. Thiopental + vecuronium was used for anesthesia induction and %1-2 isoflurane + %50 N2O/O2 mixture was used for the maintenance. Case II: A 7 year-old male was operated for the deformity at right hand. Thiopental + vecuronium was used for anesthesia induction and %1-2 isoflurane + %50 N2O/O2 mixture was used for the maintenance. Case III: A 3 year-old male was operated for congenital hip dislocation. Induction was performed by sevoflurane inhalation, %2-3 sevoflurane + %50 N2O/O2 mixture was used for the maintenance and 1 mcg.kg-1 fentanyl when needed. Case IV: A 9 year-old female was operated for pes PEV. For the induction 30 mg intramuscular ketamine and %50 N2O/O2 mixture by facial mask was the choice for the maintenance. AMC is a syndrome which carries potential risk for anesthesiologist as the patients need to be operated for several times.We would like to share our experiences of the anesthesia of the four AMC cases.

Key words: Arthrogryposis Multiplex Kongenita, Anesthesia of Arthrogryposis Multiplex Kongenita.

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