Anaesthetic management of a parturient with severe muscular dystrophy,lumbar lordosis and a difficult airway

Purpose

This case describes the management of a 19-yr-old wheelchair bound primigravida with severe muscular dystrophy who presented for Caesarean section after spontaneous rupture of membranes. Anaesthesia was influenced by several features of her systemic disease which were impediments to both neuraxial and general anaesthesia.

Clinical features

Other than for a prenatal record and the history obtainable from the patient, little additional medical information was available. Physical examination showed diffuse muscular weakness and an anatomically abnormal airway. Examination of the spine showed slight 10–15° thoracolumbar scoliosis and > 45° lumbar lordosis. Fetal assessment was normal. Echocardiography revealed mildly decreased left ventricular function and was consistent with pulmonary hypertension. After discussion with the patient and her obstetrician, elective Caesarean delivery was deemed the best management. Neuroaxial anaesthesia was at an increased risk of failure due to the profound lumbar lordosis. A plan for awake intubation and general anaesthesia was described to the patient in case regional anaesthesia could not be initiated. A fibreoptic bronchoscope and difficult intubation kit were made available. General anaesthesia was expected to have increased risk of postoperative pulmonary complications, hence epidural anaesthesia was attempted. After difficult catheter insertion, a sensory block was titrated to a T₄ level. This was well tolerated by both mother and fetus. A healthy baby was delivered with Apgar scores of 9 and 9. Postoperatively the mother was transferred to the intensive care unit. After 72 hr, the patients respiratory status allowed transfer to the ward.

Conclusion

This case illustrates the use of epidural anaesthesia in the successful management of a severely compromised patient with limb-girdle muscular dystrophy undergoing elective Caesarean section.