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Anesthesia management of a rare mitochondrial disorder in a high risk pregnancy: clinical skills and human factors
Abstract Number: SU-95
Abstract Type: Case Report/Case Series
INTRODUCTION: Mitochondrial diseases result from maternally-inherited nuclear and mitochondrial DNA mutations that disrupt the mitochondrial electron transport chain (ETC). MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and stroke-like symptoms) is one of the most common of these disorders where reduced ETC protein synthesis results in inadequate ATP production to meet metabolic demands.(1) We present a case of MELAS syndrome in pregnancy in which anesthetists played a key role in a multidisciplinary approach to a rare disorder.
CASE: A 36 year old G2P0 with MELAS syndrome presented at 22 weeks gestational age with preeclampsia on a background of Type I diabetes, short stature, exertional dyspnea, bilateral hearing loss and anxiety. At 24 weeks she was transferred to Labor & Delivery (L&D) for management of hypertension and metabolic derangement including hyponatremia, hyperkalemia, erratic blood glucose, lactic acidosis and oliguria. The obstetric team counselled towards induction of labor for absent fetal growth and progressive placental insufficiency and the anesthetic team took a leadership role providing acute high dependency level care. We observed the potential for overlap, conflicting clinical care and error with multiple contributing teams and proposed an allocation of responsibilities and clearer communication whilst developing a plan for labor analgesia and anesthesia. Later the same day the patient had a sudden neurological event with headache, hypertension, lip tingling, dysphasia and hypertonic hyperreflexic limb weakness. A differential diagnosis of eclampsia or stroke was managed with oxygen, hydralazine and magnesium and symptoms resolved over 20-30 minutes. There was no acute pathology seen on CT head but chronic cerebral and cerebellar atrophy was noted. Induction of labor began in ICU and after 2 days she delivered a stillborn fetus with IV fentanyl analgesia and a postpartum hemorrhage. Manual removal of the placenta was performed under spinal anesthesia to avoid a potentially difficult airway secondary to widespread edema. She required 2 units of red blood cells and returned to ICU postoperatively for an increasing FiO2 requirement secondary to pulmonary edema, effusions and atelectasis. Her postpartum course was complicated by slow to resolve acute kidney injury, brittle glucose control with an episode of severe hypoglycemia and loss of consciousness, and endometritis. She spent 25 days in hospital.
DISCUSSION: This is to our knowledge the first case report of anesthetic management of obstetric complications in MELAS syndrome with metabolic derangement similar to perioperative changes in a non-obstetric case series.(2) We demonstrated the role of anesthetists in L&D to manage rapidly changing physiology and utilize situational awareness and crisis management skills to identify and mitigate the risks of multidisciplinary care.
REFERENCES: 1) Mol Gen Metab 2015;116:4-12; 2) Can J Anesth 2011; 58:751-763.