PERIPARTUM MANAGEMENT OF A PATIENT WITH ISAACS-SYNDROME

Purpose: To describe the peripartum management of a patient with Isaac s' syndrome with specific reference to the anaesthetic implications of the disease process. Associated medical problem included obesity, pre gnancy induced hypertension and a difficult airway. Clinical features: This 30-yr-old gravida V para O woman presented to the anaesthesia co nsultation clinic at 37-wk gestation to discuss pain relief options fo r labour and delivery. She had a history of Isaacs' syndrome (a periph eral motor neuron disorder), congenital heart disease (ASD and VSD), t reated Hashimotos thyroiditis, obesity and a family history of haemach romatosis. On the day of consultation, she was hypertensive and periph eral oedema was noted. Her urine showed trace protein, Four days later , she presented the labour suite and her cervix was 9 fm dilated. An e pidural anaesthetic was given without difficulty and she had an uneven tful labour and delivery course. There were no subsequent neurological complications. Conclusion: Isaacs' syndrome is an extremely rare peri pheral motor neuron disorder, This patient was successfully managed wi th epidural analgesia for labour and delivered a healthy child with no congenital anomalies.