A patient with osteogenesis imperfecta may present a number of challenges during administration of anesthesia. Careful planning and monitoring can help prevent perioperative complications.
(1) risk of fracture
(2) complications of previous fracture
(3) hypermetabolic hyperthermia (see previous section on hyperthermia)
(4) congenital malformations causing difficult intubation
(5) other congenital conditions affecting cardiorespiratory function
(6) qualitative platelet dysfunction resulting in increased bleeding
(7) choice of anesthetic agents
Risk of fracture:
(1) The patient should be positioned carefully to prevent fracture.
(2) Intubation should be done carefully to prevent dental and/or mandibular fracture. (The risk of dental fracture is seen in patients with dentinogenesis imperfecta.)
(3) Extension of the head should be done carefully to prevent fracture of the cervical spine.
(4) Use of a tourniquet or blood pressure cuff may cause fracture in the upper extremity.
Previous fractures may restrict neck and jaw mobility or may cause deformities that can make positioning difficult.
The position of the larynx may be distorted by:
(1) scoliosis of the cervical and upper thoracic spine
(2) pectus carinatum ("chicken", "pigeon", or "keel" breast)
Other factors affecting intubation:
(1) The tongue may be relatively large and may be associated with airway obstruction.
(2) The neck may be relatively short.
(3) The head may be relatively large, especially if hydrocephalus is present. Placing a blanket under the thorax can help in visualizing the glottis during intubation.
(4) Cleft palate may be present.
Cardiorespiratory function may be affected by:
(1) restrictive lung disease secondary to kyphoscoliosis
(2) aortic root dilatation with aortic valve insufficiency
(3) mitral valve prolapse
(4) premature atherosclerosis
(5) premature emphysema
Choice of anesthetic agents:
(1) Atropine and anticholinergic agents should be avoided since they may be associated with the hyperthermic reaction.
(2) Succinylcholine should not be used since muscle fasciculations may cause fractures. Other muscle relaxants (pancuronium bromide, atracurium) should be used.
(3) Ketamine hydrochloride can be a useful anesthetic agent if multiple procedures of short duration are required.
(4) Other anesthetic regimens include use of halothane, enflurane or neuroleptanesthesia (nitrous oxide, narcotics and tranquilizer)
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Specialty: Anesthesiology, Pedatrics, Genetics