Anesthesia for Cesarean Section in a Parturient with Arthrogryposis Multiplex Congenita
Abstract Number: F-50
Abstract Type: Case Report/Case Series
Introduction: Arthrogryposis Multiplex Congenita (AMC) is a term used to describe a heterogeneous group of disorders with multiple joint contractures since birth. Etiology of AMC is multifactorial. Maternal factors such as infection, trauma and drugs, or fetal factors, notably fetal akinesia prevents normal joint development leading to joint fixation. AMC occurs with a frequency of 1 in 3000 live births in the USA. Joint contractures that are relevant include joint rigidity and fusion affecting extremities, lordosis and kyphoscoliosis of spine, restricted jaw movement, facial asymmetry and trismus. Respiratory reserve may also be diminished. Despite normal intelligence, life span may be reduced with severe disease. There is no clear guidance in the literature regarding management of parturients with AMC and is limited to few case reports. We describe the successful management with spinal anaesthesia for cesarean section (CS) in a parturient with AMC.
Case report: 21-year-old primigravida with AMC was referred to high-risk anesthetic clinic. She has had multiple corrective surgery to her joints. The main joints affected were feet, knees, hips, wrists, elbows, and shoulders. She was wheelchair bound. Her Mallampatti score was 1 with unrestricted jaw and neck movements with moderate degree of scoliosis of lumbar spine. Elective CS was planned. Literature review did not reveal any contraindications to regional anesthesia. Routine monitoring and IV access was established. With patient in left lateral position, SAB was performed at L3/4 level with 2.4mls of 0.5% Bupivacaine (H) and 300mcg Diamorphine. Sensory block was achieved till T4 at 5 minutes. Surgery was uncomplicated with delivery of a live baby with Apgar score 9/9. She had 5 iu Syntocinon, Gentamicin and Metronidazole (penicillin allergy) after delivery of baby. EBL was 400 ml and was transfused with 2 liters of crystalloids. She had uncomplicated recovery and was discharged home on day 3.
Discussion: Clinical manifestation of AMC differs widely between patients. Multidisciplinary approach, pre-operative review and planning are essential, as the safest technique will vary on individual patient basis. History of airway difficulty should always be sought and plans for airway management made accordingly.
In our experience, spinal anaesthesia can be performed safely and successfully in parturient with AMC. If GA is required, modified RSI with Thiopentone-Rocuronium is preferable than Suxamethonium due to the potential risk of potassium release from disused muscles. Anesthesiologists should be wary of a self-limiting hypermetabolic reaction with raise in body temperature that may occur under GA in this group of patients.
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