Anesthetic problems in Christ Siemens Touraine syndrome--a case report--
Kuriakose, R.; Balakrishnan, M.
Middle East Journal of Anaesthesiology 18(3): 647-650
2005
ISSN/ISBN: 0544-0440 PMID: 16381270 Document Number: 589159
Background: Autoimmune polyglandular syndromes (APS) are complex diseases with diverse clinical presentations resulting from involvement of multiple endocrine glands. Surgery under anesthetic in these patients is challenging. A case of Schmidt syndrome (Autoimune polyglandular syndrome type II) that developed adrenocortical insufficiency in the postoperative period is reported. Etiology, pathogenesis, types and anesthetic problems associated with these cases are discussed.Case report: A 41 yr old female patient, diagnosed to have APS (Schmidt syndrome) presented for uterine surgery. She had autoimmune glandular involvement of pituitary, thyroid, parathyroid, adrenals and melanocytes and was on hormone replacements for the deficiencies incurred, which were continued till the morning of surgery. Surgery was conducted under general anesthetic combined with epidural analgesia. In spite of supplementation of steroid in physiological doses prior to surgery, she developed hemodynamic instability in the early postoperative period, but could be successfully resuscitated with additional steroid dosage and fluids.Conclusion: This patient presented with multiglandular endocrine involvement necessitating timely, adequate hormone replacement and appropriate fluid management. These challenges require careful approach to anesthetic management.