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A patient with frontal metaphyseal dysplasia for caesarean section
Abstract Number: SU-54
Abstract Type: Case Report/Case Series
Frontal metaphyseal dysplasia FMD (Gorlin Cohen syndrome) is a rare x-linked disorder first described in 1969. Common features are prominent supraorbital ridges, progressive joint contractures and metaphyseal dysplasia. Other abnormalities include; hypertelorism, conductive/sensorineural hearing loss, high arched palate, dentition abnormalities and subglottic stenosis. Extra cranial features may include; vertebral fusion/abnormalities, muscle wasting, rib cage deformities, restrictive lung defects, primary pulmonary hypertension and mitral valve prolapse. Some cases may be associated with learning difficulties and genitourinary abnormalities.
Excluding paediatric cases, there is little literature available regarding the anaesthetic management of such patients as adults, particularly with reference to obstetric anaesthesia. Common problems encountered include difficult laryngoscopy and intubation, as well as positioning related to contractures.(1)
A 25 year old P1 with FMD was referred to our tertiary obstetric unit for caesarean section at 34 weeks gestation. Previously she had an SVD at her local hospital with entonox and diamorphine for analgesia. USS in this pregnancy demonstrated features in keeping with FMD in the foetus. A scan at 34 weeks showed a ruptured bladder in the foetus, requiring expedited delivery. The patient was not known to our obstetric anaesthesia service. She had had no previous anaesthetics and was not known to have a congenital heart defect. Height was 165cm, mass 61kg. Contractures of the upper limbs were present, with microagnathia and a high arched palate. Mallampati score was 3. There was thoracic scoliosis. ECG and blood results were unremarkable. Ultrasound scanning of the back showed no obvious abnormality in the lumbar region. A decision was made to proceed under spinal anaesthesia. Using a 25G pencilpoint spinal needle at L3/4 interspace, CSF was yielded on first pass. 2.5ml 0.5% hyperbaric l-bupivicaine and 0.3mg diamorphine was administered. There were no difficulties with block onset or height. Cardiovascular stability was maintained using a phenylephrine infusion. Careful attention was made to positioning, in view of the contractures. Spinal block regressed after 2 hours and the patient mobilised after 6 hours.
Endotracheal intubation was not required in this case, but a plan was made for this in case of failure of the spinal or prolonged surgery. FMD is a rare syndrome resulting in a spectrum of abnormalities. Our case report demonstrates that neuraxial blockade may be a feasible technique in such patients, reducing the need for general anaesthesia in the context of a complex airway. Bedside USS is a useful modality to assess the suitability for a neuraxial technique.
(1) Anaesthesia for genetic, metabolic and dysmorphic syndromes in childhood. Baum V and O'Flaherty J. Lippincott, Williams and Wilkins. 2007; p143.