Anesthetic management of a child with both Marfan syndrome and Turner syndrome.

ABSTRACT

Marfan syndrome is an autosomal dominant heritable disorder of the connective tissue that involves primarily the skeletal, ocular, and cardiovascular systems. Turner syndrome is a genetic disorder resulting from partial or complete X chromosome monosomy. We report the anesthetic management of a case of Marfan-Turner syndrome, which is the first such case to appear in the literature to our knowledge. A 3 year old ASA III girl was scheduled to undergo minor plastic surgery. She had a short webbed neck, prognathism, micrognathia, low-set ears, and a high palate. Her anterior and posterior facial heights were long. She had growth retardation, pectus excavatum, and joint laxity. She also had high-degree mitral insufficiency, mitral valve prolapse, and an atrial septal defect. After sevoflurane induction, the airway was secured using a size 2 LMA without any difficulty in the spontaneously breathing patient. Her blood pressure was within normal limits, no arrthymia occurred, and anesthesia was uneventful. Special care should be given to syndromic patients. Prior medical evaluations and any prior anesthetic history can help to focus preoperative evaluations and planning. Preoperatively targeting relevant organ systems, any anatomic or laboratory abnormalities that can be optimized, and perioperative airway management are all key to a successful outcome.