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Epidural placement for cesarean delivery in a patient with congenital arthrogryposis
Abstract Number: SU-72
Abstract Type: Case Report/Case Series
Arthrogryposis multiplex congenita encompasses a spectrum of syndromes characterized by progressive joint contractures and motor neuron degeneration. Etiology, inheritance, and pathogenesis are largely idiopathic with most cases being sporadic with a difficult to predict prognosis. It is rarely seen in the pregnant population but its effects, specifically on anatomy and cardiopulmonary physiology, pose significant challenges when administering anesthesia for cesarean delivery. Cardiorespiratory complications include alveolar hypoventilation, atelectasis, restrictive respiratory pattern, dyspnea, V/Q mismatch, and hypoxemia leading to cor pulmonale. Neuraxial anesthesia is difficult because of scoliosis and contractures making positioning challenging, furthermore there is an increased incidence of spina bifida occulta and sacral agenesis which can negatively impact the spread of local anesthetic in the epidural space impeding adequate analgesia and anesthesia.
Our patient is a 30 year old G4P0212 female presenting for scheduled cesarean delivery at 37 weeks gestation. She is 145cm and 37.2kg with a Mallampati class one airway with small mouth opening and micrognathia. She has severe dextroscoliosis associated with restrictive lung disease requiring BiPAP while asleep. Her two previous deliveries included a spontaneous vaginal delivery at 27 weeks gestation and a classical cesarean delivery at 25 weeks gestation. Her cesarean delivery required general anesthesia after failed neuraxial anesthesia. Her postoperative course was complicated by carbon dioxide retention requiring prolonged hospitalization.
The anesthetic plan for this cesarean delivery is an ultrasound assisted combined spinal-epidural (CSE) with BiPAP for respiratory support. Ultrasound was used to map out the patient’s anatomy in order to find midline and sacrum given her severe dextroscoliosis. CSE was initially attempted at the L4-5 interspace however was unsuccessful. Another attempt was made at the L3-4 interspace and loss of resistance to saline occurred at a depth of 7cm. Placement of the Whitacre needle demonstrated free flowing CSF and a low dose of anesthetic was injected intrathecally. An epidural catheter was easily threaded and taped at 12cm at the skin. The patient achieved an adequate sensory level for surgery to commence. The epidural catheter was bolused intermittently throughout the case and was supplemented with intermittent IV boluses of ketamine. Postoperative pain control was managed via PCEA and non-opioid IV medication to prevent respiratory depression.
Gambling DR, Douglas MJ, and McKay RSF. Obstetric Anesthesia and Uncommon Disorders. Cambridge University Press. 2008.
Lapinsky SE, Tram C, et al. Restrictive lung disease in pregnancy. Chest. 2014;145(2):394-398.
Cabrini L, Nobile L, et al. Intraoperative prophylactic and therapeutic non-invasive ventilation: asystematic review. BJA. 2015;43(4):880-888.