Spinal correction in patients with Fukuyama congenital muscular dystrophy.

Abstract

Fukuyama congenital muscular dystrophy (FCMD) is one of the most common congenital progressive muscular dystrophies in Japan. Some patients develop a severe spinal deformity that leads to an unstable sitting position or pain. Since 2008, we have treated FCMD using posterior spinal fusion. This study reports the short-term clinical and radiographic results of posterior spinal correction and fusion in FCMD. We retrospectively reviewed 11 consecutive FCMD patients, average age 13 years old, treated with posterior spinal instrumentation and fusion between 2008 and 2015. All patients were non-ambulatory and complained about difficulty sitting and/or buttock pain. Posterior spinal correction was performed to halt progression of spinal deformity and improve their sitting balance. Assessment was performed clinically and with radiological measurements at a mean follow up period of 34.5 months. To evaluate functional status of patients after surgery objectively, a Muscular Dystrophy Spine Questionnaire (MDSQ) was obtained at the final follow up. The mean height, weight and body mass index of the patients were 144.1 ± 11.8 cm, 26.5 ± 8.7 kg and 12.5 ± 2.6 kg/m². The average intensive care unit stay was 1.3 days. Five patients had complications related to surgery. The mean preoperative major Cobb angle and pelvic obliquity (PO) were 65.5 ± 41.7° and 31.4 ± 28.9°, respectively, were corrected to 34.4 ± 29.7° and 20.0 ± 18.7° just after the surgery, and were maintained at 35.1 ± 29.9° and 20.5 ± 21.1° at the final follow up. The average MDSQ score was 35.8 ± 13.2 at the final follow up. Posterior spinal correction and fusion in FCMD achieved good radiographic results and clinical improvement with acceptable perioperative complications. FCMD patients are mentally impaired and physically small, so post-operative observation and close attention to perioperative complications are critical.