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///2013 Abstract Details
2013 Abstract Details2019-08-02T16:57:45-05:00

Management of a Parturient with Diastrophic Dysplasia

Abstract Number: S 48
Abstract Type: Case Report/Case Series

Jacqueline S. Lagoy MD1 ; Nathaniel D. Kofford MD, MSPH2; Benoit J. Gosselin MD3; Benjamin D. Morley MD4

A 27 year old primagravida with Diastrophic Dysplasia (DD) presented to our obstetrics clinic at 8 weeks gestational age. DD is an autosomal recessive disease characterized by limb shortening, spinal deformities, and large joint contractures, with a normal-sized skull and normal intelligence. Little is known about pregnancy in individuals with DD, with only two case reports known in the literature. This patient had a history of airway difficulty at age 13 when intubation had been impossible and surgery was aborted. She also had a history of spinal fusion with a posterior Harrington rod extending from level T1 to S1, and known difficulty with venous access. She was referred to anesthesiology clinic for evaluation early in her pregnancy. Pulmonary function testing and otolaryngology evaluation were obtained. A mediport was placed to enable venous access. She was followed closely and remained stable with the exception of the onset of mild orthopnea at 30 weeks. Elective cesarean section was planned for 35 weeks gestation. She did not tolerate awake fiberoptic nasal intubation, and with an otolaryngologist present a modified rapid sequence induction was performed and the airway was secured with videolaryngoscopy. Cesarean section was uncomplicated and both mother and infant had an unremarkable postoperative course. The parturient with DD presents multiple challenges to perinatal anesthetic management. Airway management may be complicated by cervical kyphosis, micrognathia, or laryngomalacia. Restrictive lung disease should be suspected due to often severe scoliosis, and with uterine growth may progress to respiratory failure. This patient had undergone spinal fusion with a Harrington rod, and even in the absence of such instrumentation spinal deformities may be severe, with curvature up to 180° reported. Access to the neuraxial space may be difficult, and appropriate dosing for such patients is not well defined.

SOAP 2013