Anesthetic Management of Children with Larsen Syndrome

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.