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Labor Analgesia for Parturient with Arthrogryposis Multiplex Congenita
Abstract Number: S-42
Abstract Type: Case Report/Case Series
Introduction: Arthrogryposis Congenita Multiplex (ACM) is a syndrome characterized by rigid, contracted joints and malformed extremities with different neurogenic, myopathic and even environmental causes (1). Pregnancies in patients with ACM are rare given genitourinary abnormalities such vaginal and uterine agenesis. This case demonstrates the successful use of labor neuroaxial analgesia in a patient with ACM.
Case: 26 y.o. G1P0 at 35 wks. presented with rupture of membranes. Patient is wheelchair bound and suffers from ACM. On physical exam there was a Class I airway, marked scoliosis, a normal size thorax, but short and underdeveloped extremities. Cardiovascular and pulmonary examinations were unremarkable. Given the lack of hip joint abnormalities and the presence of an adequate pelvic inlet, the patient was deemed a candidate for a vaginal delivery.
An early CSE was performed with a 17G Touhey needle and a 26G Gertie Marx spinal needle in the right lateral decubitus position. The epidural space was easily identified with a single attempt, 8 cm from the skin. The spinal was dosed with 20mcg of Fentanyl and 100mcg of PF Morphine. After the catheter was secured at the skin and aspiration was confirmed as negative, a test dose of 3ml of 1.5% Lidocaine (45mg) with epinephrine was administered. No signs of intravascular or intrathecal injection were noted, but the blood pressure decreased, requiring correction with IV fluids and pressors. A continuous infusion of 0.0625% Bupivacaine with Fentanyl was started at 8ml/hr.
6 hours later, the patient requested a top-up. Aspiration was reconfirmed as negative and a similar dose was administered, 60mg of 2% Lidocaine (3ml). Once again, the patient became hypotensive with symptoms of lightheadedness and diaphoresis. 200mcg of Phenylephrine and 15mg of Ephedrine were needed to restore the blood pressure. Over the remaining course of her 24 hours of labor, the patient required 3 additional boluses. Bupivacaine 0.125% was utilized as not to interfere with her expulsive strength. None of these boluses were greater than 3ml and the patient reported excellent, bilateral analgesia. The patient delivered a male infant via an unassisted vaginal delivery.
Discussion: The identification of the epidural space in patients with ACM is challenging due to vertebral abnormalities such as scoliosis and narrow foramina. When the epidural space is identified there could be enhanced spread with subsequent marked hemodynamic fluctuations, as demonstrated in this case, or abnormal spread leading to one sided or patchy blocks (2). Continuous spinal catheters have been used in the past when epidural placement has proven difficult (3). Caution should be also exercised with the dosing of spinal catheters as there is known differences with the production and resorption of CSF in patients with ACM.
1. Spooner, L. IJOA 2000; 9:282-5.
2. Quance, D. Can J Anaesth 1988; 6:612-4.
3. Benonis, J. IJOA 2008; 17:53-6.