Abstract
Turner Syndrome results from the absence of a sex chromosome, resulting in a female with the 45XO karyotype and major anatomic and physiological changes. Anatomically from head to toe, features may include a short and wide neck which may be webbed, hearing changes, limited neck mobility, high-arched palate, mandibular and maxillary hypoplasia, coarctation of the aorta, bicuspid aortic valve, a smaller than normal trachea, hypothyroidism, and gonadal dysgenesis. Patients have short stature and a higher rate of scoliosis. Physiologically, patients are at higher risk of cognitive impairment. Cardiac anatomic differences result in a higher risk of aortic dissection and intraoperative hypertension. Hypothyroidism often results in delayed gastric emptying and subsequent increased risk of aspiration. There is a higher risk of type 2 diabetes and insulin resistance, as well as liver dysfunction. Kidney abnormalities such as horseshoe kidney may be present; however, they rarely affect kidney function. There are no contraindications to anesthesia in patients with Turner Syndrome, but the phenotypic changes do demand attention to management.
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Thomas, DA., Missih, O., Zhu, R., Suchy, T., Vadivelu, N. (2021). Common Congenital Syndromes and Disease States Impacting Regional Anesthesiology Techniques. In: Narayan, D., Kapadia, S.E., Kodumudi, G., Vadivelu, N. (eds) Surgical and Perioperative Management of Patients with Anatomic Anomalies. Springer, Cham. https://doi.org/10.1007/978-3-030-55660-0_6
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