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The anesthetic management of an urgent cesarean section in a patient with Goldenhar syndrome: a case report
Abstract Number: T-71
Abstract Type: Case Report/Case Series
Goldenhar Syndrome (GS), also called oculoauriculovertebral spectrum or syndrome, is a relatively rare congenital condition (1). Exact etiology is not known but believed to be due to abnormal embryonic vascular supply to the first and second arch (2). Common characteristics include hemifacial microsomia, facial hypoplasia, lateral facial cleft, dental abnormalities, microtia, anotia and an unusually small mouth (3,4,5). Systemic manifestations may include heart, kidney, lung and spinal abnormalities (6,7). Fertility and longevity are not directly affected (8). Despite this, there are only two reported cases outlining the anesthetic management of pregnant patients with GS in the literature (9,10). We present a case outlining the anesthetic management of a 21-year-old pregnant women with GS presenting for an urgent cesarean section complicated by an intra-operative failed airway and a severe adverse reaction while managing postpartum hemorrhage. This case will not only add to the reported cases in the literature but will also outline the necessary steps required to safely manage patients with such a congenital disease. This is a unique patient population that should be managed at a tertiary care centre with high volume of high risk obstetrical patients with a standardized approach that include pre-operative multi-disciplinary planning and the involvement of multiple health care workers to ensure patient safety.
1)von Arlt F. Clinical presentation of diseases of the eye. 1st ed. Vienna: Braumuller publications; 1881.
2)Kulkarni V, Shah MD, Parikh A. Goldenhar Syndrome (a case report) J Postgrad Med. 1985;31:177–9.
3)Rollnick BR & Kaye CI. Oculo-auriculo-vertebral anomaly. On: Buyse M.D. Birth Defects Encyclopedia. Center for Birth Defects Information Services. Dover, MA: 1990; 1272-1274.
4)Tuna EB et al. Craniofacial and dental characteristics of Goldenhar Syndrome: A report of two cases. J Oral Sci. 2011;53:121-4.
5)Goldenhar M. Associated malformations of the eye and ear, particularly dermoid syndrome epibulbar-appendices, congenital auricular fistulas and its relations with Manibulofacial Dustosis. J Genet Hum 1952;1:243-82.
6)Goswami M et al. Goldenhar Syndrome: A Case Report with review. Int J Clin Pediatr Dent 2016;9(3):278-280.
7)Digilio MC et al. Congenital heart defects in patients with oculo-auriculo-vertebral spectrum (Goldenhar syndrome). Am J Med Genet Part A 146A:1815-1819.
8)Shokeir MH. The Goldenhar syndrome: a natural history. Birth Defects Orig Artic Ser. 1977;13(3C):67-83.
9)Hezelgrave NL, Srinivas K, Ahmad I, Mascarenhas L. Use of awake oral fibreoptic intubation (AFOI) for caesarian section in a woman with Goldenhar Syndrome: a case report. Eur J Obstet Gynecol Reprod Biol. 2011 Dec;159(2):479-80.
10)Lokhande C, Riad I, Tetzlaff J, Ayad S. The peri-operative management of a cesarean section in a patient with Goldenhar syndrome (GS): A case report. J Clin Anesth. 2016 Nov;34:448-51.