Total intravenous anaesthesia and the use of an intubating laryngeal mask in a patient with osteogenesis imperfecta

Abstract

Osteogenesis imperfecta is a genetically determined rare disease of the connective tissue, associated with abnormalities of type 1 collagen. The primary bone lesion is the lack of normal ossification of the endochondrial bone. Patients with osteogenesis imperfecta present several problems for anaesthetists. They have a tendency to develop malignant or non-malignant hyperthermia. During laryngoscopy and tracheal intubation, the mandible, teeth and cervical spine may be fractured or injured, and mucosal bruising or bleeding may occur. Renal or ureteral stones are common. The main problems are thus with airway control and intubation, and the risk of anaesthetic agents triggering malignant hyperthermia. We describe the successful anaesthetic management of a patient with osteogenesis imperfecta, undergoing nephrolithotomy and ureterolithotomy with total intravenous anaesthesia including propofol, remifentanil and cisatracurium, using an intubating laryngeal mask.

The Progression of Isoflurane-induced Malignant Hyperthermia and Its Attenuation by Cisatracurium in a Pre-clinical Porcine Model of Heart Transplant

“…While non-depolarizing skeletal muscle relaxants are vastly used during surgeries, their role as MH-triggers is not well understood [34][35][36]. In our studies, the addition of cisatracurium to the protocol attenuated and delayed the apparent multi-systemic involvement and progression of MH despite comparable durations of surgery.…”

Anesthetic Management of the Child with Osteogenesis İmperfecta: Seven General Anesthesia Experience in one patient

“…Ayrıca barbitüratlar, fentanil, pankuronyum ve nitröz oksit kullanılan bazı hastalarda da taşikardi, metabolik asidoz ve malign hipertermi gelişebildiği bildirilmiştir (13) . Farklı mekanizmalarla oluşabilen intraoperatif hipertermiyi önlemek için TİVA uygulamasını öneren ve başarılı bir şekilde kullanan çalışmalar mevcuttur (14) . TİVA'nın Oİ çocuklarda inhalasyon anestezisine göre daha güvenli bir yöntem olduğu varsayılır.…”

Anaesthesia in children with osteogenesis imperfecta — report of 14 general anaesthetics in three children

“…Kyphoscoliosis or limited mobility of the neck may result in a difficult airway [2]. Successful use of laryngeal and intubating laryngeal masks has been described in the literature [3][4][5]. Patients with concomitant dentinogenesis imperfecta are also at a greater risk of dental injury during the perioperative period than the general population [2].…”