Mahmut Alp KARAHAN, Nuray ALTAY, Hasan Hüsnü YÜCE, Mehmet EROĞLU

Larsen Sendromlu Pediatrik Hastada Anestezi Yönetimi

Larsen sendromu; yüz dismorfizmi, konjenital eklem dislokasyonları ve kas-iskelet, solunum, kardiyovasküler ve nörolojik sistemleri içeren çoklu sistem tutulumu ile karakterize nadir bir kalıtsal bağ dokusu hastalığıdır. Ortopedik sorunlara bağlı olarak özellikle pediatrik hastalarda, var olan anomalileri nedeniyle, çeşitli cerrahi prosedürler geçirmek zorunda kalırlar. Bu nedenle bu hastalarda tekrarlayan anestezi ihtiyacı gerekebilmektedir. Anestezi yönetimi açısından hastaların majör problemleri genellikle perioperatif solunum fonksiyon bozuklukları, kardiyovasküler problemler ve zor havayolunu içerir. Laringomalazi, trakeomalazi, bronkomalazi ve subglottik stenoz, bu sendromun zor havayolu yönetimi açısından en büyük sorunları arasında yer alırlar. Bu olgu sunumunda Pes ekinovarus cerrahisi planlanan Larsen sendromlu çocuk hastada anestezi yönetimini literatür eşliğinde tartıştık

Anesthetic Management of a Pediatric Patient With Larsen Syndrome

Larsen syndrome is a rare, inherited, connective tissue disorder which is characterized by facial dysmorphisms, congenital joint dislocations and multi-system involvement including musculoskeletal, respiratory, cardiovascular, and neurological systems. Children with Larsen syndrome are prone to undergo a wide variety of surgical treatments for their orthopedic abnormalities. For this reason these patients may require repeated anesthetic care during surgical procedures. The major concerns about these patients regarding anesthesia include perioperative respiratory dysfunction, cardiovascular problems and difficult airway management. Laryngomalacia, tracheomalacia, bronchomalacia and subglottic stenosis are the main causes for difficult airway management in this syndrome. In this case report we discussed, in light of literature, the anesthetic management of a pediatric patient with Larsen syndrome who underwent a pes equinovarus surgery

Keywords:

larsen syndrome, general anesthesia, child, orthopedic surgery,

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