Turner-Kieser Syndrome or Iliac Horn Syndrome, a Challenge for the Anesthesiologist: A Case Report and a Literature Review

Abstract

Turner-Kieser syndrome is a rare genetic disorder, autosomal dominant, which is related to variable gene expression and high penetrance, due to mutations in the LMX1B gene that affects connective tissue. The clinic has characteristics with alterations in nails, knees, elbows, and presence of iliac horns that makes the anesthetic difficult. However, data in the literature needs more research on this area. The present report is a cesarean section under general intravenous anesthesia performed on a 32-week pregnant woman due to the maternal risk of full-term evolution and aims to contribute to the anesthetic management of patients with this syndrome as there are few descriptions in the literature regarding the anesthetic management in Turner-Kieser syndrome. Even being a case report, the syndrome is rare and it is important to be reported to all anesthesiologists becoming aware of its management and to choose the best technique and anticipate possible complications. During general anesthesia, the technique chosen for the case reported here, we emphasize the risks of possible difficulty in intubating and positioning the patient, as well as renal impairment caused by changes in the cardiovascular autonomic response due to the choice of some drugs. Therefore, in the present report, our option was intravenous general anesthesia because of maternal complications, where fast acting drugs without renal metabolism or excretion were selected, resulting in an uneventful anesthetic procedure.