To the Editor:
Osteogenesis imperfecta (OI) is a disease of connective tissue caused by mutations in one of the two genes encoding collagen type 1 (1). Its clinical features include susceptibility to bone fractures and retarded growth, as well as variable involvement of other connective tissues (1–3). Patients with OI often undergo surgery, most frequently orthopedic. There are several reports of hyperthermia during general anesthesia in these patients, although a direct relationship of OI to malignant hyperthermia is not substantiated (2). Difficult endotracheal intubation is common in patients with OI. The literature refers to the successful use of the laryngeal mask airway (LMA) and intubating LMA in these patients (3–5).
In this letter we report a case of a 12-yr-old, 33-kg boy who was admitted for surgery because of right olecranon epiphysiolysis. He had OI, and he had experienced several previous fractures requiring surgical repair. Dantrolene sodium, sodium bicarbonate and cold IV solutions were available before anesthetic procedure. After we premedicated the patient with midazolam (0.06 mg/kg) and administered oxygen to him for 5 min, we induced anesthesia with propofol (2.5 mg/kg), atropine (0.1 mg/kg), and remifentanil (1 μg/kg). We administered rocuronium (0.6 mg/kg) for neuromuscular block. We maintained anesthesia with sevoflurane at 1%–2% end-tidal concentration and a remifentanil infusion at 0.2 μg/·kg−1·min−1. We inserted a size 3 ProSeal LMA with his head in neutral position. After emptying the patient's stomach, we inserted a thermistor temperature probe into the esophagus through the ProSeal LMA drain tube and located it in the lower quarter of the esophagus (the optimal location described by Mitchell et al.) (6).
We monitored noninvasive arterial blood pressure, electrocardiogram, pulse oximeter, Fio2, ETco2, and esophageal core temperature. We did not find an increase of temperature, nor did we note any significant hemodynamic changes. We reversed the neuromuscular block with atropine (0.1 mg/kg) and neostigmine (0.3 mg/kg). Recovery was uneventful.
We conclude that anesthesia with propofol, sevoflurane, and remifentanil provided hemodynamic stability and that the ProSeal™ LMA was a useful and easy method for securing the patient's airway and monitoring his core temperature continuously.
M. Luisa Santos, MD
Cristóbal Añez, PhD
Ana Fuentes, MD
Bárbara Méndez, MD
Rocío Periñán, MD
María Rull, PhD
Department of Anaesthesiology
Hospital Universitari de Tarragona Joan XXIII
1. Prockop DJ, Kuivaniemi H, Tromp G. Enfermedades hereditarias del tejido conectivo. In: Fauci AS, Braunwald E, eds. Harrison: Principios de Medicina Interna. New York: McGraw-Hill, 2000;2483–97.
2. Porsborg P, Astrup G, Bendixen D, et al. Osteogenesis imperfecta and malignant hyperthermia. Is there a relationship? Anaesthesia 1996;51:863–5.
3. Kostopanagiotou G, Coussi T, Tsaroucha N, Voros D. Anaesthesia using a laryngeal mask airway in a patient with osteogenesis imperfecta. Anaesthesia 2000;55:506.
4. Karabiyik L, Parpucu M, Kurtipek Ö. Total intravenous anesthesia and the use of an intubating laryngeal mask in a patient with osteogenesis imperfecta. Acta Anaesthesiol Scand 2002;46:618–9.
5. Karabiyik L, Çapan Z. Osteogenesis imperfecta: different anaesthetic approaches to two pediatric cases. Pediatr Anaesth 2004;14:524–32.
6. Mitchell S, Brimacombe J, Keller C. Feasibility, accuracy and optimal location for oesophageal core temperature measurements using the ProSeal Laryngeal Mask Airway drain tube. Anaesth Intensive Care 2003;31:282–5.