ANESTHESIA IN A PARTURIENT WITH NOONANS-SYNDROME

Purpose: To report the management of labour analgesia and subsequent a naesthesia for postpartum bleeding in a 19-yr-old parturient with Noon an's syndrome. Clinical features: The patient presented in active labo ur at 36-wk gestation. She was known to have Noonan's syndrome and had been assessed regularly throughout pregnancy. Features of the syndrom e exhibited by the patient included typical facies, chest skeletal abn ormalities, pulmonary valve dysplasia, mental retardation and lymphoed ema. In addition, she had Factor XI deficiency (0.46 mg . L-1) and thr ombocytopenia (92 X 10(9) . L-1), previously unreported in a parturien t with this syndrome. Although epidural analgesia may have been consid ered the labour analgesic technique of choice, the risk of epidural ha ematoma caused by her bleeding diathesis made this unacceptable. This risk was balanced against the possibility of a potentially difficult i ntubation due to facial abnormalities, should emergency operative deli very become necessary. Labour analgesia was provided with intravenous patient controlled opioid analgesia (fentanyl 25 mu g bolus, five minu te lockout) despite her mental retardation. Dilatation and curettage r equired general anaesthesia after intubation with awake direct laryngo scopy using cautious sedation. Conclusion: Noonan's syndrome is charac terised by multi-system involvement, requiring thorough preoperative a ssessment of cardiovascular; skeletal, haematological and central nerv ous systems. Clotting and platelet defects considerably restrict the p ossible analgesic and anaesthetic options for labouring patients with this syndrome.