Use of awake oral fibreoptic intubation (AFOI) for caesarian section in a woman with Goldenhar Syndrome: A case report

摘要

We recently managed a pregnancy in a patient with Goldenhar Syndrome, or oculoauriculovertebral dysplasia, a rare but well documented congenital disorder causing hemifacial microsomia and auricular abnormalities, as well as a wide spectrum of heterogenous medical problems linked to development of the face, vertebral column, reproductive, and genitourinary systems. Cases of Goldenhar syndrome in neonates have been well documented, however no cases of pregnancy have been described.A 37 year old primigravid woman with Goldenhar Syndrome became pregnant with a singleton pregnancy after 2 cycles of in vitro fertilisation. Diagnosed at age 13, she was born with severe left-sided facial underdevelopment of bones, muscles and tissues. She developed severe scoliosis and underwent multiple corrective procedures including extensive maxillofacial, orthopaedic and plastic surgery to increase the size of her jaw, reposition her ear and muscle and nerve transplants into her face. Aged 14, a spinal Harrison rod was inserted (T8-L4), leaving little residual back movement.