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Open Reduction And Internal Fixation of Left Radius in Autosomal Recessive Osteogenesis Imperfecta Type VII Michael Pence, DO1, Caroline Wright MD2 1Department of Anesthesiology, Walter Reed National Military Medical Center, Bethesda, MD 2Department of Anesthesiology, Children’s National Medical Center, Washington DC The Patient 6 year old female with past medical history significant for osteogenesis imperfecta type VII with homozygous mutation of cartilage associated protein (CRTAP) gene, restrictive lung disease, developmental delay, GERD, scoliosis, and G-tube in place to supplement oral feeds. The patient had undergone multiple procedures previously for rod placement and hardware removal. She had a history of PICU admission with BiPAP support s/p 8 hour rod placement in the past. Post-Operative The patient was taken to the PACU after being extubated while deeply anesthetized to minimize the possibility of subsequent fracture in the setting of restraining the patient upon emergence. She maintained spontaneous ventilation and did not require additional airway support postoperatively as a oxygen saturation >93% was maintained. Her pain was well controlled post-operatively with one additional dose of 5mcg fentanyll in addition to oxycodone 1mg every 6 hours as needed and naproxen 50mg every 12 hours as needed. Pre-Operative The patient was to undergo an ORIF of the left radius after a fall with a subsequent radial fracture. She was anxious during initial evaluation. She was given a 6mg dose of midazolam (0.6mg/kg) in pre-op 15 minutes prior to transport to OR. Despite premedication, the patient was still anxious upon separation. Intra-Operative She was immediately connected to pulse-oximetry upon entering the operating room and an inhalational induction was performed with 8% sevoflurane, 70% nitrous oxide, and 30% oxygen. A blood pressure cuff was applied to the patient's right arm with neonatal settings to limit the maximum inflation pressure. The cuff was cycled every 15 minutes to minimize the risk of fracture after the patient was anesthetized. A 24g peripheral IV was placed without the use of a tourniquet in the patient's left foot. She was given 3 separate 10mg doses of propofol (1mg/kg) prior to laryngoscopy, which was performed gently with minimal cervical extension. Extra foam padding was applied underneath the patient during the procedure. Anesthesia was maintained with sevoflurane and the patient was given a total dose of 5mcg fentanyl throughout the case for pain control. Discussion This case illustrates the clinical challenges associated with perioperative care in a patient with osteogenesis imperfecta undergoing surgery. Extra precaution must be taken to prevent further fractures in the perioperative setting. This especially holds true for patients with severe forms of the disorder like the patient listed in this report. Short stature and cervical instability present additional issues that must be considered and can make airway management and intubation particularly difficult. Two classic associations with osteogenesis imperfecta type VII are coxa vara and rhizomelia which were both present in the above patient1. This genotype demonstrates a recessive inheritance pattern compared to types I-VI which demonstrate dominant inheritance and is only seen in 5-10% of cases2. In addition, pulmonary function must be taken into consideration based upon the degree of scoliosis present and chest wall deformities which can have a significant impact on perioperative management and ventilation strategies. Osteogenesis imperfecta was once considered a risk factor for malignant hyperthermia however; central core disease is the only musculoskeletal disorder that appears to have a true association with malignant hyperthermia. References Figure 1. (a) shortening of the humeri (rhizomelia). (b) bilateral coxa vara. Bowing deformity of the lower extremities is evident1. 1.Ward, L.m, F. Rauch, R. Travers, G. Chabot, E.m Azouz, L. Lalic, P.j Roughley, and F.h Glorieux. "Osteogenesis Imperfecta Type VII: An Autosomal Recessive Form of Brittle Bone Disease." Bone 31.1 (2002): 12-18. Web. 2.Osteogenesis Imperfecta Foundation." Osteogenesis Imperfecta Foundation | OIF.org. Osteogenesis Imperfecta Foundation, n.d. Web. 16 Jan. 2017. *Views expressed in this case report are the authors’ and do not reflect the official policy or position of the Department of the Army, Navy, Department of Defense, or the U.S Government.