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DOI: 10.1055/s-0040-1710329
Anesthetic Challenges of an Adolescent Patient with Epidermolysis Bullosa and Gitelman's Syndrome Undergoing Posterior Spinal Fusion Surgery
Abstract
Surgical correction for scoliosis is undertaken to avoid progression to cardiopulmonary compromise as well as improve the patient's overall quality of life. In this case report, we presented a case of a 14-year-old girl with epidermolysis bullosa simplex and Gitelman's syndrome who underwent posterior spinal fusion for scoliosis. The perioperative planning and intraoperative management of a patient with this unique combination of comorbidities undergoing a complex, high-risk surgical procedure were not previously chronicled in the literature. We detailed the steps undertaken to optimize the patient prior to surgery and the unique intraoperative surgical and anesthetic considerations that led to a successful completion of the surgery and recovery.
Consent
Written patient consent was obtained prior to the publication of this article.
Publication History
Received: 07 February 2020
Accepted: 23 March 2020
Article published online:
25 April 2020
© 2020. Thieme. All rights reserved.
Georg Thieme Verlag KG
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References
- 1 Weinstein SL, Dolan LA, Spratt KF, Peterson KK, Spoonamore MJ, Ponseti IV. Health and function of patients with untreated idiopathic scoliosis: a 50-year natural history study. JAMA 2003; 289 (05) 559-567
- 2 Lin AN, Lateef F, Kelly R, Rothaus KO, Carter DM. Anesthetic management in epidermolysis bullosa: review of 129 anesthetic episodes in 32 patients. J Am Acad Dermatol 1994; 30 (03) 412-416
- 3 Fabricant PD, Admoni S, Green DW, Ipp LS, Widmann RF. Return to athletic activity after posterior spinal fusion for adolescent idiopathic scoliosis: analysis of independent predictors. J Pediatr Orthop 2012; 32 (03) 259-265
- 4 Prodinger C, Diem A, Bauer JW, Laimer M. Mucosal manifestations of epidermolysis bullosa: clinical presentation and management [in German]. Hautarzt 2016; 67 (10) 806-815
- 5 Bissonnette B, Luginbuehl I, Engelhardt T. Epidermolysis bullosa. In: Syndromes: Rapid Recognition and Peri-operative Implications. New York: McGraw-Hill; 2006
- 6 Culpepper TL. Anesthetic implications in epidermolysis bullosa dystrophica. AANA J 2001; 69 (02) 114-118
- 7 Krane E. Guidelines for the anesthetic management of epidermolysis bullosa (EB). Pediatric Anesthesia and Pain Management. Accessed January 2020 at: http://med.stanford.edu/content/dam/sm/pedsanesthesia/documents/EBanesthesiaguidelines.pdf
- 8 Filippatos TD, Rizos CV, Tzavella E, Elisaf MS. Gitelman syndrome: an analysis of the underlying pathophysiologic mechanisms of acid-base and electrolyte abnormalities. Int Urol Nephrol 2018; 50 (01) 91-96
- 9 Seyberth HW, Weber S, Kömhoff M. Bartter's and Gitelman's syndrome. Curr Opin Pediatr 2017; 29 (02) 179-186
- 10 Blanchard A, Bockenhauer D, Bolignano D. et al. Gitelman syndrome: consensus and guidance from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int 2017; 91 (01) 24-33
- 11 Cruz DN, Shaer AJ, Bia MJ, Lifton RP, Simon DB. Yale Gitelman's and Bartter's Syndrome Collaborative Study Group. Gitelman's syndrome revisited: an evaluation of symptoms and health-related quality of life. Kidney Int 2001; 59 (02) 710-717