Cervical duraplasty with tenting sutures via laminoplasty for cervical flexion myelopathy in patients with Hirayama disease: successful decompression of a "tight dural canal in flexion" without spinal fusion

Object: Hirayama disease, juvenile muscular atrophy of the distal upper extremity, is a rare type of cervical flexion segmental myelopathy and its etiology is still being debated. Two theories have been proposed: a "contact pressure" theory and "tight dural canal in flexion" theory. Previously reported treatments, including conservative neck collar therapy and surgical spinal fusion, used fixation of the cervical spine with the aim of avoiding contact pressure between the cord and anterior structures. On the other hand, treatment by duraplasty without spinal fusion has also been used, which aims at decompressing a tight dural canal in flexion by preventing abnormal forward displacement of the posterior dura mater without restricting cervical motion in young patients. The authors developed a new surgical approach for treating a tight dural canal in flexion in patients with Hirayama disease: cervical duraplasty with tenting sutures via laminoplasty without spinal fusion. With this treatment they aimed to both decompress the spinal cord and preserve as much cervical motion as possible. The purpose of this study was to assess the clinical outcomes of patients who underwent this new surgical procedure and to investigate the etiology of Hirayama disease.

Methods: Six male patients (age range 17-23 years) with Hirayama disease underwent surgery between 2006 and 2012. The pre- and postoperative anteroposterior diameters of the dural canal in the flexed neck position, grip strength of the bilateral upper extremities, cervical alignment (C2-7), and cervical local flexion range of motion were compared. The presence or absence of surgical complications was assessed. To investigate the comparison group of Hirayama disease treated with spinal decompression, the PubMed database was searched for all relevant English-language case reports and series published between 1990 and 2013.

Results: The postoperative anteroposterior diameters of the dural canal were significantly expanded in the flexed neck position (7.2 ± 2.2 mm preoperatively vs 9.8 ± 1.7 mm postoperatively, p = 0.001). Grip strength of the upper extremities significantly improved bilaterally (20 ± 14 kg preoperatively vs 26 ± 15 kg postoperatively, p = 0.001). No significant difference was observed between pre- and postoperative cervical alignment in the neutral neck position (7.7° ± 8.1° preoperatively vs 9.0° ± 7.7° postoperatively, p = 0.74) or the cervical local flexion angle in the flexed neck position at the corresponding level of laminoplasty (16.6° ± 5.1° preoperatively vs 15.0° ± 9.4° postoperatively, p = 0.8). No surgical complications were noted, except for transient CSF leakage, which was resolved after lumbar drainage. The systematic review identified 37 cases from 7 reports: 26 with spinal fusion only, 5 with duraplasty without fusion, and 6 with combined duraplasty and fusion. In the largest series, in which 12 cases were treated with anterior fusion, cervical alignment was maintained, but local flexion motion was significantly decreased as a result of fixation. Although significant improvements in or stabilization of grip strength occurred in all 7 reported studies regardless of decompression procedures, one major delayed surgical complication was noted in a patient treated with anterior fusion. The patient developed severe kyphotic changes, which required reconstruction surgeries.

Conclusions: Cervical duraplasty with tenting sutures via laminoplasty prevented abnormal forward displacement of the posterior dura mater while preserving normal anterior structures and flexion motion of the cervical spine without major surgical complications. The clinical improvements achieved by the authors' method support evidence that a tight dural canal in flexion largely contributes to segmental myelopathy in patients with Hirayama disease.