OSTEOGENESIS IMPERFECTA (OI) : POINTS OF ANESTHETIC RELEVANCE 

🎲Bones and teeth are easy to break.The mandible is prone to fracture,but the facial bones are less so. Rib fractures have been reported. In the severest form, forced extension of the head during intubation carries a risk of vertebral fracture. Violent suxamethonium fasciculations can cause fractures.
🎲n the severe types of the disease,concern has been expressed that a blood pressure cuff may damage the humerus. Direct arterial monitoring has been suggested as an alternative
🎲Macrocephaly can be there. Airway problems may occur if the head is large, if there is macroglossia, or if the skeletal deformities are severe. If the head is large,a pillow placed under the chest may assist tracheal intubation.
🎲There is some evidence of hypermetabolism in this disease. Half of the patients have increased serum thyroxine levels. Hypermetabolic states, with hyperthermia, acidosis, sweating and cardiovascular instability, have been reported, but these are unrelated to Malignant Hyperthermia (MH).
🎲Surgery should be avoided in the pyrexial patient. Core temperature, oxygen saturation and ETCO2 should be monitored throughout surgery. Hyperthermia is reported to have responded to cooling alone.
🎲Platelet dysfunction may occur and produce a mild bleeding tendency, although the platelet count may be normal. But coagulopathies have been reported.
🎲Aortic and mitral valve insufficiency results from the defective connective tissue formation, but may be clinically inapparent. Sometimes cardiac surgery may be required
🎲Cranial developmental defects may cause brainstem compression, hydrocephalus, or vascular disruption. Softening of the basal portion of the occipital bone and upward movement of the cervical spine can combine to cause secondary basilar impression. Warning signs include cough, headache,vertigo, and trigeminal neuralgia. 
🎲Those patients with kyphoscoliosis may have restrictive pulmonary defects. Sixty per cent have significant chest wall deformities. A thoracic scoliosis of more than 60 degrees will have severe effects on lung function, with a reduction in vital capacity to below 50%
🎲Although skeletal deformities and deranged coagulation may make regional anaesthesia technically difficult, successful and safe epidural anaesthesia has been reported in patients with OI.
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#anaesthesia , #TheLayMedicalMan , #Orthopedics , #OsteogenesisImperfecta , #fracture

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