Spinal correction in patients with Fukuyama congenital muscular dystrophy

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusion: 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.