Successful Surgery for Scoliosis Supported by Pulmonary Rehabilitation in a Duchenne Muscular Dystrophy Patient With Forced Vital Capacity Below 10%
Herein, we report a 19-year-old male patient who suffered from gait disturbance since the age of 2 years when he often fell and had difficulty with climbing stairs. At the age of 7, he was diagnosed as DMD by muscle biopsy, and follow-up in the out-patient department of Gangnam Severance Hospital was stopped at his parents' will. He had not walked since 10 years of age and had been suffering from symptoms of hypercapnea, such as sleeping during the daytime and headache upon awakening from sleep. In July 2010, when he turned 18 years old, he was visited by the home visiting service of Gangnam Severance Hospital, and his partial pressure of end-tidal carbon dioxide (EtCO2) was above 50 mmHg. He was admitted to the Department of Physical Medicine and Rehabilitation at Gangnam Severance Hospital on August 7, 2010. In the arterial blood gas analysis, partial pressure of carbon dioxide (PaCO2) was 49.9 mmHg and partial pressure of oxygen (PaO2) was 91.8 mmHg. In the evaluation of pulmonary function, FVC in sitting position was 460 mL (8.6% of predictive value), FVC in supine position was 500 mL (9.4% of predictive value), unassisted peak cough flow (UPCF) was 80 L/min, and maximum insufflation capacity (MIC) was 1,390 mL. In the overnight transcutaneous monitoring of partial pressure of carbon dioxide (SenTec Digital Monitor System with V-Sign Sensor; SenTec AG, Therwil, Switzerland), retention of carbon dioxide was noticed, as the maximal PaCO2 was 76.6 mmHg and the mean PaCO2 was 64.6 mmHg. He received training for noninvasive intermittent positive pressure ventilation (NIPPV), which mode is assist-controlled ventilation (ACV). During application of NIPPV, the overnight monitoring showed a reduction in carbon dioxide retention, as the maximal PaCO2 was 46.1 mmHg and the mean PaCO2 was 40.6 mmHg. He was prescribed a home ventilator, and the ventilator settings were the mode of ACV, tidal volume of 1,150 mL, and respiration rate of 16 times per minute. Based on the daytime monitoring of PaCO2, after 6-hour discontinuance of home ventilator, hypercapnea had redeveloped; he was therefore educated to apply the home ventilator while sleeping and additionally in the daytime. Furthermore, he was educated to exercise air-stacking using a manual resuscitator bag and home ventilator, more than 3 times a day. After application of home ventilator, the symptoms of hypercapnea were resolved.\nAfter being discharged, the patient had no complaints related to respiratory failure, but he suffered from difficulty sitting even if he leaned back, due to severe scoliosis; Cobb's angle was 58.1°. He was considered high risk for scoliosis operation due to a very low FVC of less than 10% of predictive value. Although he was thought to be safe from respiratory complications after the scoliosis correction, he was trained on a home ventilator for 6 months to maintain proper ventilator states via NIPPV immediately after extubation. At the Department of Orthopedic Surgery, he underwent surgeries for anterior fixation from the fifth lumbar vertebra to the first sacral vertebra on February 10, 2011, and for posterior fixation from the second thoracic vertebra to the first sacral vertebra on February 22, 2011. During the operations, his vital signs were stable. From the time of admission, noninvasive PaCO2 monitoring was conducted during sleep. After serial surgeries, he was transferred to the intensive care unit with maintenance of the intubation tube; expectoration of sputum and air-stacking were implemented by mechanical insufflator-exsufflator (MIE). The first day after the operation, NIPPV via home ventilator was applied right after intubation tube removal. Upon confirmation that there were no complications and hypercapnea (the maximal and mean PaCO2 were 25 and 20.5 mmHg, respectively), he was transferred to the general ward.\nAt 8 days after the surgery, intravenous antibiotics were injected due to infection of the surgical wound. On April 9, 2011, 46 days after the operation, all stitches were removed. Cobb's angle was changed from 58.1° preoperatively to 48.5° postoperatively (). He still can sit only with a supporting back, but subjective comfort was improved by the reduction of the inclination in the sitting position. At postoperative day 51 and 300, pulmonary function was reevaluated. FVC, assisted peak cough flow (APCF), and MIC increased; MIC was particularly improved from 1,010 mL preoperatively to 1,880 mL postoperatively, although UPCF showed no significant change ().