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Hurler Sendromlu Bir Olguda CO2 Lazer Tedavisi

Kıroğlu F., Talas D., Özcan C., Görür K., Kıroğlu M.

Hurler sendromu (gargoylism) otozomal resesif geçişli, alfa-L-iduronidaz enzim eksikliği sonucu gelişen, mukopolisakkaridoz grubu bir hastalıktır. Semptomlar yumuşak dokularda ve kafatasında mukopolisakkarid birikimine bağlı olarak ortaya çıkar. Hastalarda erken yaşlarda hava yolu obstrüksiyonu bulguları görülür. Bu yazıda dil büyümesi, yemek yemede güçlük ve horlama şikayetleri olan dokuz yaşında bir erkek çocuk sunuldu. Klinik muayenesinde makroglossi, gelişme ve zeka geriliği saptandı. Larenjeal lezyonların evaporasyonunda ve dilin kısmi rezeksiyonunda CO2 lazer kullanıldı. Ameliyat sonrası dönemde hava yolu obstrüksiyonu nedeniyle trakeotomi yapıldı. Hastanın takiplerinde yemek yemede güçlük ve horlama şikayetleri yoktu. Hurler's syndrome (gargoylism) is an autosomal recessive mucopolysaccharide starage disease caused by alpha-L-iduronidase deficiency. Symploms are secondary to excessive deposition of mucopolysaccharide in skeletal and soft tissues. Children with Hurler's syndrome manifest upper airway obstruction signs early in life. A nine-years-old boy presented with complaints of tongue enlargement, snoring, and difficulty in oral feeding. Clinical examination revealed macroglossia, and mental and growth retardation. CO2 laser was used for the evaporation of the laryngeal lesion and partial resection of the tongue. In the postoperative period, tracheostomy was performed to relieve airway obstruction. He had no complaints of difficulty in oral feeding and snoring. Key Words: Airway obstruction/etiology/surgery; mucopolysaccharidosis I/pathology/complications; respiratory tract diseases/etiology; tracheal stenosis/etiology/surgery; Ira. cheostamy.

CO2 Laser Treatment In A Case With Hurler' s Syndrome CO2 Laser Treatment In A Case With Hurler' s Syndrome

Kıroğlu F., Talas D., Özcan C., Görür K., Kıroğlu M.

Hurler's syndrome (gargoylism) is an autosomal recessive mucopolysaccharide starage disease caused by alpha-L-iduronidase deficiency. Symploms are secondary to excessive deposition of mucopolysaccharide in skeletal and soft tissues. Children with Hurler's syndrome manifest upper airway obstruction signs early in life. A nine-years-old boy presented with complaints of tongue enlargement, snoring, and difficulty in oral feeding. Clinical examination revealed macroglossia, and mental and growth retardation. CO2 laser was used for the evaporation of the laryngeal lesion and partial resection of the tongue. In the postoperative period, tracheostomy was performed to relieve airway obstruction. He had no complaints of difficulty in oral feeding and snoring.

KBB İhtisas Dergisi C:8/S:1 Syf:62-65

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