Osteogenesis imperfecta (OI) which is also known as Vrolik’s disease is a connective tissue disorder [1]. OI tarda is an autosomal recessive disorder associated with abnormalities of type 1 collagen. The other form of the disease known as OI congenita is a dominant form in which the child usually dies in utero. This disorder associated with many potential problems; fragile bones and teeth, abnormal airway, megalocephaly, macroglossia, short neck, thoracic deformities, susceptibility to malignant hyperthermia, hyperthyroidism, congenital cardiac diseases, emphysema and bleeding disorders due to platelet dysfunction are the most prominent problems concerning anesthesia management [2]. Also, many potential problems are concerning operative management. It might be greatly increases the risk of a refracture due to the pathological induction of stress risers. Therefore, fracture treatment by unreamed elastic intramedullary nailing was combined with simultaneous correction osteotomy, resulting in anatomic alignment and uncomplicated fracture healing. The singlestage surgical stabilization performed allowed rapid mobilization along with a decreased likelihood of refracture [3].

We report the anesthetic and operative management of a patient in which osteotomy and intramedullary nailing were performed under general anesthesia with regard to recent anesthetic agents and techniques.

32 years old female patient who had a history of osteogenesis imperfecta and had undergone numerous surgical interventions presented with a history of five orthopedic surgery sessions under general anesthesia, usage of antidepressant medications (mitazapine) and a large head disproportionate to the body, short neck, thoracic abnormalities, severe kyphoscoliosis and growth retardation in lower extremities (Figure 1). Airway assessment revealed a short neck with sternomental distance of 5cm and hyomental distance of 4cm. Dentition was firm and airway was Mallampati class- III. The patient who was confined to bed was not able to use her lower extremities. Blood count, renal, liver and thyroid function tests and electrolyte levels were within normal limits. Bleeding time and clotting time were within normal limits. Chest radiography showed increased radiolucency in all bone structures, costal asymmetry and kyphoscoliosis while radiographs of the extremities revealed spontaneous fractures of both femur and tibia (Figures 2 and 3).

Figure 1: Multiple skeletal deformities.

Figure 2: Costal asymmetry and kyphoscoliosis.

Figure 3: Fractures of both femur and tibia.

The patient was brought to the operating theatre without any premedication and was placed on a medical air bed. Pads were placed over pressure points and under the blood pressure cuff. ECG, noninvasive arterial blood pressure, pulse oxymetry, body heat and endtidal CO₂ were monitored (Drager Infinity Delta Monitör TR Danvers, MA 01923, USA). Intravenous access was established without using a tourniquet. It was planned to secure the airway with endotracheal intubation following IV induction. Preparation was made for difficult airway management. A number 3 LMA classic, bougie, stylet and fiberoptic bronchscope were kept ready. Manupilations during the positioning of the head, placement of the ventilation mask, laringoscopy and intubation were carried out with great care.

She was preoxygenated and given injection midazolam 2mg (Dormicum, Roche, Istanbul, Turkey). Then, the patient was induced with propofol 200mg (Diprivan, Astra Zeneca, Istanbul, Turkey) and fentanyl 75μgr (Fentanyl Citrate, Abbott, Istanbul, Turkey) ability to mask ventilate was assessed before administering rocuronium bromide 30mg (Esmeron, Organon, Istanbul, Turkey). With gentle manipulation of the neck laryngoscopy was done using a Macintosh blade and a 6mm ID endotracheal tube was placed carefully in position with the help of a stylet. Desflurane 6% (Suprane, Eczacibasi, Istanbul, Turkey) (in 40% O₂ + 60% air mixture) and remifentanyl 10μg/h (Ultiva, GlaxoSmithKline, Istanbul, Turkey) was used in maintenance of general anesthesia. At the end of the procedure, after 65 minutes the patient was extubated when respiration was spontaneous, regular and adequate.

Multiple osteotomies and corrective surgery with intramedullary nailing were carried out by the orthopedic surgery team. 2 units of erythrocyte suspensions were given intraoperatively. Intravenous tramadol (10mg/h) (Contramal, Abdi Ibrahim, Istanbul, Turkey) PCA was used for postoperative analgesia. Thereafter, the postoperative period was uneventful and was discharged on the 7th postoperative day.

30 days later, osteolysis was diagnosed on one of the osteotomised bones and revision surgery was carried out for correction under general anesthesia. No bleeding diathesis or additional bone fractures were seen after these operations.

Osteogenesis imperfecta which was first reported as “Ivar the Boneless” of the 9th century, and first described in 1788 as “osteomalacia congenita” presents as two clinical distinct forms [1].

While osteogenesis imperfecta congenita is clinically evident in birth and fatal, osteogenesis imperfecta tarda is characterized by skeletal deformities and fractures later in life. Average life expectancy is undeterminate in osteogenesis imperfecta tarda and the oldest patient in literature is a 54 years old female patient who underwent total knee arthroplasty as reported by Nisihimura et al. [4,5]. Our patient who was diagnosed as osteogenesis imperfecta tarda is 32 years old and above average life expectancy in Turkey.

This patient had presented for an elective surgical procedure, a careful history revealed the details of her illness that guided us to do the minimal essential investigations that would suffice to exclude any existing problems and thereby provide safe anaesthesia.

Manipulations which may cause additional fractures such as the transportation of the patient, placement on the operation table and routine monitorization must be carried out in great care. Oliviero et al. [1] have reported a humerus fracture caused by arterial blood pressure measurement. Additionally practitioners must be prepared for a possible bleeding diathesis [6,7]. Agents which are known to trigger malignant hyperthermia must be avoided. Rampton et al. [8] have reported fatal intraoperative malignant hyperthermia in an osteogenesis imperfecta patient. We did not face any problem while maintaining anaesthesia with desflurane (in O₂+air mixture) and remifentanyl.

Karabiyik et al. [9] have recommended TIVA alongwith intubating LMA to manage elective cases but in our case made us to secure the airway with an endotracheal tube and maintain anaesthesia with inhalation agent. Malde et al. [10] has reported fracture of right shaft of femur in his patient, which occurred during transfer to the recovery room.

Computed tomography (CT) is the study of choice for diagnosis and follow-up because of its superior bony detail and accurate assessment of the extent of the lesion. In addition, CT can also assist by differentiating fibrous dysplasia from other osteodystrophies of the skull base, including Paget’s disease, osteogenesis imperfecta, otosclerosis, and osteopetrosis.

We have recommend recent anesthetic agents and techniques: desflurane (in O₂+air mixture) and remifentanyl for maintaining anaesthesia during successive anesthesia and orthopedic sessions for correction of multiple fractures in a case osteogenesis imperfecta.