Werdnig-Hoffmann disease

Abstract

Introduction: Werdnig-Hoffmann disease or spinal atrophy type I is part of the spinal muscular atrophies and the most serious of the three clinical forms in existence. It is an autosomal recessive hereditary condition, with no treatment, progressive in nature and usually culminates with the death of the patient between the first and second year of life.
Objective: To describe the behavior of the anatomically difficult airway identified in a patient with Werdnig-Hoffmann disease operated for right renal lithiasis.
Clinical case: Male patient at age 39, higher education level, with a diagnosis of obstructive lithiasis in the right kidney, proposed to be performed a percutaneous nephrolithotomy. The exams in the anesthesia consultation provided diagnosis of an anatomically difficult airway. Despite having the cooperation of the patient, trained personnel, necessary equipment and proceeding according to the algorithms recommended in the literature, a surgical approach was needed to perform the operation. The proposed surgical procedure was carried out without complications and the patient left the operating room awake and conscious.
Conclusion: In case that another surgical intervention is required, it would be necessary to initiate intubation by optical fibroscopy in order to avoid edema of the respiratory tract. In case a safe airway is not obtained by this method, the patient would need a surgical airway.