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"Pinhole" Airway in a 21 Year Old Parturient with Ectodermal Dysplasia
Abstract Number: 141
Abstract Type: Case Report/Case Series
Introduction: We report a case of a parturient with severe glottal stenosis in the setting of ectodermal dysplasia. Airway stenosis is a rare condition encountered by the OB anesthesiologist. Management of the pregnant patient with this condition can be challenging and the literature does not offer consensus regarding its evaluation and anesthetic management. Ultimately, early interdisciplinary planning for an elective tracheostomy helped assure patient safety during advancing pregnancy and delivery.
Case report: A 21 year old, G1P0 female at 6.5 weeks gestation was referred to the OB anesthesia team for evaluation and peripartum management recommendations. The patient had a history of congenital complete glottic web that required a tracheostomy placement at birth. At the age of 4, she underwent decannulation and successful closure of the defect. At age of 12, the patient was given the diagnosis of ectodermal dysplasia. After being lost to follow up for years, learning about her pregnancy prompted the patient to consult an otolaryngologist. Her symptoms at that time were mild increased shortness of breath with exertion and she was concerned that this might become worse throughout the course of her pregnancy. A fiberoptic examination suggested a dangerously narrow airway with fixed vocal cords and a pinhole glottal aperture sizing to a 2-3 mm opening. After an in-depth discussion between the patient, the otolaryngologist, the obstetric and the anesthesia team, the decision was made to proceed with elective tracheotomy under local anesthesia and to delay definitive airway reconstruction until after delivery. Second and third trimesters were uneventful until at 35.3 weeks gestation, when the patient was admitted to the labor and delivery floor for non-reassuring fetal heart rate tracing. She subsequently underwent an uneventful cesarean delivery under spinal anesthesia, resulting in a growth restricted female infant with Apgars of 7 and 8. The infant had ectrodactyly ectodermal dysplasia with one hand and both feet affected. She is now in the midst of airway reconstructive efforts to achieve eventual decannulation.
Discussion: Parturients can present with rare conditions that seem absolutely incompatible with successful pregnancies. An interdisciplinary approach was taken to manage anticipated worsening of airway obstruction in this patient. With input from the OB team, otolaryngologist and OB anesthesia a temporary tracheostomy was placed during the first trimester to help assure access to her airway, as airway patency was certainly likely to become critically compromised without intervention.