Dural ectasia in a child with Larsen syndrome

We present a case of an incidental finding of dural ectasia in a child diagnosed with Larsen syndrome. Larsen syndrome is a rare inherited disorder of connective tissue characterized by facial dysmorphism, congenital joint dislocations of the hips, knees and elbows, and deformities of the hands and feet. Dural ectasia is as an abnormal expansion of the dural sac surrounding the spinal cord and may result in spinal morphologic changes, instability, and spontaneous dislocation. To the best of our knowledge, the presence of dural ectasia in Larsen syndrome has not previously been reported.

A 6-year-old boy diagnosed with Larsen syndrome presented with an upper thoracic curve measuring 74 degrees, a right thoracic curve measuring 65 degrees, and significant cervicothoracic kyphosis with 50% anterior subluxation of C6 on C7 and C7 on T1. Advanced imaging studies showed dural ectasia (evidenced by spinal canal and dural sac expansion), thinning of pedicles and lamina, and C4 and C6 pars defects with cervical foramen enlargement. The patient received growing rod instrumentation (attached to cervical spine fixation) by a combined anterior/posterior surgical approach using intraoperative halo. Complications included intraoperative medial breach (fully resolved), wound dehiscence, 2 instances of bilateral broken rods, and a broken cervical rod. Following 7 lengthening procedures, the patient underwent definitive fusion.

Surgeons should be aware of the potential for dural ectasia in patients with Larsen syndrome. Its presence will cause difficulties in the surgical intervention for spinal deformity. Multiple factors must be considered, and surgical approach and technique will require modification to avoid complications. Although dural ectasia confounds surgical intervention in these patients, surgery still appears to outweigh the risks associated with delayed intervention. The presence of dural ectasia should not preclude surgical decompression and stabilization. This report adds to the body of knowledge on the treatment of Larsen syndrome by demonstrating the potential existence of dural ectasia and highlights the importance of careful and thorough preoperative evaluation and diagnostic imaging.