Journal Article

Postoperative adrenal crisis in an adolescent with Loeys-Dietz syndrome and undiagnosed adrenoleukodystrophy

McCann, Mary Ellen, Kuisle, Ami M., Dauber, Andrew, Gauguet, Stefanie, Karlin, Lawrence I.

2011

Overview

Purpose We present an unusual case involving an adolescent who experienced cardiovascular collapse postoperatively. He had more than one rare life-threatening genetic disorder, and herein we review the anesthetic management of a patient with Loeys-Dietz syndrome (LDS) and adrenoleukodystrophy. Clinical features A 12-yr-old male (41.5 kg, American Society of Anesthesiologists’ physical status III) with LDS, a connective tissue disorder, underwent posterior spinal fusion for spondylolisthesis. This patient demonstrated many signs of LDS: bifid uvula, retrognathia, dilated aortic root, aortic arch aneurysm, inguinal hernias and vertebral subluxations, and multiple areas of tortuous intracranial vessels. A fibreoptic intubation was performed and a balanced anesthetic was administered, consisting of opioids and a low-dose volatile agent. The patient was stable throughout surgery, and at the end of the procedure, his trachea was extubated and he was admitted to the intensive care unit. During the postoperative period, the patient became acutely hemodynamically unstable. Initially, the intensive care team considered the differential diagnoses of postoperative hemorrhage, postoperative sepsis, and cardiac failure secondary to aortic dissection. Supportive care was instituted, and these diagnoses were systematically ruled out. The differential diagnosis was expanded, and the patient was treated with corticosteroids after baseline cortisol levels were drawn. Later he was found to have primary adrenal failure, and it was determined by biochemical and genetic blood analysis that he also had adrenoleukodystrophy. Conclusions Although Occam’s razor states that physicians should exercise diagnostic parsimony when treating patients, it is possible for a patient to have two or more life-threatening unrelated genetic disorders. Consequently, diagnosticians must always develop and test new hypotheses when treating patients.

• Publisher: Springer-Verlag,Springer,Springer Nature B.V
• Subject: Adrenal Insufficiency - etiology / Adrenoleukodystrophy - complications / Anesthesia / Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy / Anesthesiology / Biological and medical sciences / Blood vessels / Cardiology / Case Reports/Case Series / Child / Connective tissue / Critical Care Medicine / Drug dosages / General anesthesia / Hernias / Hormones / Hospitals / Humans / Intensive / Intensive care / Intubation / Loeys-Dietz Syndrome - complications / Male / Medical personnel / Medical sciences / Medicine / Medicine & Public Health / Pain Medicine / Patients / Pediatrics / Pneumology/Respiratory System / Postoperative Complications - etiology / Sepsis / Spinal Fusion / Spinal manipulation / Spondylolisthesis - surgery / Surgery