959 Clinical Anaesthesia File Afiaesthetic manage- ment of a parturient with severe muscular dystrophy, lumbar lordosis and a difficult airway Case presented by Purpose: This case describes the management of a 19-yr-old Michael P. Pash MD FRCPC wheelchair bound primigravida with severe muscular dystro- phy who presented for Caesarean section after spontaneous rupture of membranes. Anaesthesia was influenced by several Case discussed by features of her systemic disease which were impediments to John BalatOn MD FRCPC both neuraxial and general anaesthesia. Chris Eagle MD FRCPC FANZCA Clinical features: Other than for a prenatal record and the Department of Anaesthesia history obtainable from the patient, little additional medical Foothills Hospital information was available. Physical examination showed dif- University of Calgary fuse muscular weakness and an anatomically abnormal air- way. Examination of the spine showed slight 10-15 ~ thoraco- lumbar scoliosis and >45 ~ lumbar lordosis. Fetal assessment was normal. Echocardiography revealed mildly decreased left ventricular function and was consistent with pulmonary hyper- tension. After discussion with the patient and her obstetrician, elective Caesarean delivery was deemed the best management. Neuroaxial anaesthesia was at an increased risk offailure 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 bron- choscope and difficult intubation kit were made available. General anaesthesia was expected to have increased risk of postoperative pulmonary complications, hence epldural anaesthesia was attempted. After difficult catheter insertion, a sensory block was titrated to a T4 level. This was well tolerat- Key words ed by both mother and fetus. A healthy baby was delivered ANAESTHESIA" obstetrical; with Apgar scores of 9 and 9. Postoperatively the mother was ANAESTHETIC TECHNIQUES: epidural; transferred to the intensive care unit. After 72 hr, the patients ANALGESIA: postoperative, respiratory status allowed transfer to the ward. MUSCLE: limb-girdle muscular dysrophy. Conclusion: This case illustrates the use of epidural anaesthe- Address correspondence to: Dr. Chris Eagle, Department of sia in the successful management of a severely compromised Anaesthesia, Foothills Hospital, Calgary, Alberta, T2N 2T9. patient with limb-girdle muscular dystrophy undergoing elec- Accepted for publication 13th May, 1996. tive Caesarean section. CAN J ANAESTH 1996 / 43:9 / pp959--63 960 CANADIAN JOURNAL OF ANAESTHESIA Objectif: Ddcrire la prise en charge d'une primipare de 19 admissions to hospital during the past three three ans cloude ?t la chaise roulante par une dystrophie musculaire years and no regular follow-up by any medical spe- grave, hospitalis6e pour une cdsarienne aprks une rupture cialists. She had had one or two bouts of pneumonia spontande des membranes. L'anesthdsie a dtd influencde par each year for the last few years which required only plusieurs propres ~ cette maladie syst~mique g~nant ?t aussi ambulatory treatment. She had stopped smoking at bien l' anesthtsie rdgionale centrale que l'anesthdsie gdndrale. eight weeks gestation. She denied having fever, chills, Caract~ristiques cliniques: Peu de renseignements ~taient cough, sputum, or any dyspnoea. The patient did not disponibles ~ part le dossier prdnatal et l'histoire obtenue de take any medications and had no allergies. Fetal la patiente. L'examen physique r#vdlait une faiblesse muscu- movements were normal. laire diffuse et voies adriennes anormales. L' examen du rachis montrait une l~gbre scoliose thoracolombaire et une lordose ddpassant 45 ~ L'dvaluation foetale dtait normale. L'dlectro- This patient presents two issues of concern for her cardiographie indiquait une diminution ldgbre de la fonction anaesthetic management, muscular dystrophy and preg- ventriculaire gauche et dtait compatible avec un hypertension nancy. She gives a history of severe weakness and fre- pulmonaire. Aprks discussion avec le patiente et son ob- quent respiratory infection, suggesting either impair- stdtricien, un accouchement par cdsarienne programmde dtait ment of normal clearance mechanisms and/or weakness d~cid~. Une anesthdsie rdgionale centrale risquait d'dchouer fi involving the bulbar muscles with resulting recurrent cause de l'importance lordose lombaire. Pour pallier & un pulmonary aspiration. Duchenne muscular dystrophy is dchec de l' anesthdsie rdgionale, un plan comportant une intu- most commonly an 'X' linked recessive disorder but has bation vigile et une anesthdsie gdndrale dtait expliqud gt la been reported in female patients.~ In any event, the case patiente. Un bronchoscope gl fibres optiques et une trousse will need to be managed without knowledge of the exact pour intubation difficile dtaient maintenus en disponibilitd. type of muscular dystrophy. Comme l'anesthdsie gdndrale augmentait le risque de compli- The anaesthetic considerations for the muscular dys- cations pulmonaires, une anesthdsie dpidurale dtait entreprise. trophies are similar in many respects. All patients with Apr~s l'insertion laborieuse d'une cathdter dpidural, le niveau muscular dystrophy show sensitivity to medications de bloc sensitif dtait progressivement installd & T4, ce que la including opioids and sedatives and the non-depolariz- m~re comme le foetus ont bien toldrd. Un enfant en bonne ing muscle relaxants. ! Patients have been described as santd avec des scores d'Apgar de 9 et 9 ~tait mis au monde. existing in a state of "partial curarization. ''! The adverse Aprds l'intervention, la mdre dtait transfdrde aux soins inten- events reported following administration of succinyl- sirs. Aprds 72 heures, la condition respiratoire de la patiente choline vary considerably. For example, some patients permettait le transport gt sa chambre. with Duchenne muscular dystrophy have suffered Conclusion: Cette observation illustre l'utilisation de hyperkalaemic responses with rhabdomyolysis and car- l'anesthdsie dpidurale pour la prise en charge d'une patiente diac arrest. 2 The risk of malignant hyperthermia with the gravement atteinte de dystrophie musculaire des ceintures use of succinylcholine and inhalational anaesthetics is soumise gt une cdsarienne programmde. probably increased both in patients with Duchenne mus- cular dystrophy and limb-girdle muscular dystrophy but this relationship is difficult to quantify. Avoidance A 19-yr-old primigravida patient at 37 wk gestation of succinylcholine and the inhalational anaesthetics arrived by air ambulance after spontaneous rupture appears to be the most prudent approach) of membranes 12 hr previously. Her prenatal care Pregnancy presents a number of considerations for comprised four visits to her obstetrician. A history of anaesthetic management which are beyond the scope of "severe Duchenne Muscular Dystrophy" was record- this discussion. In this case, spontaneous rupture of ed on the antepartum record. She described a history membranes places the patient at an increased risk for of muscle weakness beginning at the age of three sepsis which would then be a relative contraindication years and progressing to confinement in a wheel- to regional anaesthesia. 4 chair by age 10 yr. The patient was now totally dependent on nursing care. A complete functional assessment was last done three years earlier in Physical examination revealed a gravid 59 kg woman another institution, but the results of these tests were near term in no acute distress. Her heart rate was not available. Past surgical history included ventric- 110 bpm, blood pressure 100/60 mmHg, respiratory ular sept.al defect repair at 18 mo and tonsillectomy rate 20 per minute. She was afebrile. Physical exami- at 13 yr. The associated anaesthetics were described nation confirmed the presence of profound muscular by the patient as uneventful. There had been no weakness, which was more severe proximally. Air- Pash.: CLINICAL ANAESTHESIA FILE 961 way examination revealed a small mouth, large ment and congestive he.art failure may occur. Typical tongue, reduced neck extension, and a thyromental ECG changes are sinus tachycardia and a right bundle distance of two fingers. The airway was assessed as branch block. Diaphragmatic muscle may also be Mallampatti Grade IH. Air entry was decreased involved. 1 Similarly, Duchennes .muscular dystrophy is symmetrically, but no adventitious sounds were characterized by frequent cardiac, and electrocardio- audible. Some use of the accessory muscles of respi- graphic abnormalities, respiratory weakness, and kypho- ration was apparent. Examination of the cardiovas- scoliosis. Although the exact type of muscular dystro- cular system revealed normal neck veins and mini- phy presented here was not initially clear, the important mal edema. A left parasternal lift was present with a consideration is recognition of the commonly shared loud, palpable P2. No murmurs or extra heart features of the muscular dystrophies. sounds were audible. The non-stress test showed a There is little or no information in the literature con- reactive fetal heart tracing with a baseline fetal heart cerning the effects of the muscular dystrophies on uter- rate of 130 bpm. ine muscle function. Potentially, uterine smooth muscle function could be affected, as are cardiac and gastric smooth muscle. This might lead to an abnormal pattern Physical examination revealed a high probability of dif- of labour and possibly increasing