Anesthetic Management of Children with Larsen Syndrome

Ayşe Çiğdem TÜTÜNCÜ,Pınar KENDİGELEN,Atasoy GÜRLEVİK,Gülçin KARACAN,Güner KAYA

Özet

Larsen sendromu, kollajen oluşumunda bozuklukla karakterize nadir görülen genetik geçişli bir hastalıktır. Yüz ve ekstremite ayrıca vertebra anomalileri ile karakterizedir genel anestezi altında bilateral inguinal herni operasyonu planlanan Larsen sendromu tanılı 7 aylık, 3750 gr. erkek hastada preoperatif muayenede diz eklemlerinde dislokasyon, clubfoot, sıradışı yüz anomalileri, pektus karinatum, şiddetli skolyoz vardı. Uzun QT sendromu tespit edilen hastaya preoperatif propranolol başlandı. Standart monitorizasyon sonrası, indüksiyon sevofluran/hava ile, entübasyon roküronyum (0,6 mg/kg) sonrası yapıldı ardından kaudal blok uygulandı. Peroperatif dönem hemodinamik açıdan sorunsuz geçti. Cerrahi sonrası nöromusküler blokaj geri dönüşümü sugammadex 2 mg/kg ile yapıldı. Tamamen uyanık, minimal substernal retraksiyon dışında solunum güçlüğü ve motor blok yoktu. Sonuç olarak Larsen sendromlu hastalar sahip oldukları kas-iskelet, solunum, kardiyolojik ve nörolojik problemler nedeniyle anestezi açısından preoperatif hazırlık iyi yapılmalı, per ve postoperatif dönemde yakın takip edilmelidir.

Abstract

Larsen Syndrome is the rare inherited disease of defect in collagen formation. It is characterized by facial and extremity abnormalities. Spine anomalies scoliosis, kyphosis, wedge vertebrae, and spondylosis also have been described in this syndrome. A 7 month old, male, 3750 gr. boy with a diagnosis of Larsen syndrome was scheduled for bilateral inguinal hernias operation under general anesthesia. The preoperative examination revealed knee joint dislocations, clubfoot and unusual face (flat faces, high palate). Long QT syndrome was detected and propranolol medication was started preoperatively. The patient had severe scoliosis, thorax deformities with pectus carinatus. Following standart monitors placement, anesthesia was induced sevoflurane/air, intubation facilitated with rocuronium (0.6 mg/kg) and then caudal analgesia was performed. The intraoperative course uneventful. The hemodynamic status and rhythm was stable during surgery and in the postoperative period. At the end of the surgery neuromuscular blockage was reversed with sugammadex. The child was totally awake without any respiratory difficulty except with minimal substernal retraction and there was no motor block. The patient was sent to the recovery room and he was discharged home following day without any complication. In conclusion, patients with Larsen syndrome have issues pertinent to anesthesiology relating to the musculoskeletal, respiratory, cardiac and neurological systems.