Factors related to surgical outcome after posterior decompression and fusion for craniocervical junction lesions associated with osteogenesis imperfecta

Abstract

Treatment for craniocervical junction lesions associated with osteogenesis imperfecta (OI) has been described, but there are divergent views on operative procedures and preoperative and postoperative therapies due to the small number of cases. It has been suggested that a major procedure such as combined anterior and posterior surgery with concomitant ventriculoperitoneal (VP) shunting is required for OI associated with basilar impression (BI). However, here we report a case with a good outcome after posterior decompression fusion only. The patient was a 29-year-old woman with OI (Sillence type-IA) who had neurological symptoms of vertigo, nausea, and shaking during walking. Diagnostic imaging revealed hydrocephalus, severe BI, and Chiari type-II malformation. Preoperative Halo traction led to improvement in symptoms, and posterior decompression fusion from the occipital bone to C6 was subsequently performed. Lateral mass screws and Nesplon cables as sublaminar wiring for reinforcement for fusion were used in the operation. The patient wore a Halo vest for 4 weeks postoperatively. She experienced no symptoms postoperatively. Bone fusion and improved hydrocephalus were clear on images at 3 years after surgery, and the postoperative course has been good. In craniocervical junction lesions associated with OI, instability with compression of the nerve and bone fragility in multiple sites can become problematic. Anterior odontoid resection and posterior fusion are required for OI with BI to give ideal decompression on images. However, the results of this case suggest that a good postoperative outcome can be achieved by performing not the combination of anterior odontoid resection and VP shunting, but only with posterior decompression fusion, especially for OI cases of Sillence type-I.

Basilar impression in osteogenesis imperfecta treated with staged halo traction and posterior decompression with short-segment fusion

“…[ 8 ] Anterior decompression alone may lead to postoperative instability requiring fixation, therefore, supporting the need for fusion. [ 9 ]…”

“…in their patient (type IA, 29 years old). [ 9 ] Our patient was type III OI (12 years old). Despite treating the most severe survivable OI type, with current modern implants, we were able to obtain stable fusion with the shortest documented fusion length that we are aware of in the literature, stopping at C2.…”

“…Despite treating the most severe survivable OI type, with current modern implants, we were able to obtain stable fusion with the shortest documented fusion length that we are aware of in the literature, stopping at C2. Prior studies extended to C6[ 9 ] and T1. [ 10 ]…”

Osteogenesis Imperfecta and Basilar Invagination

“…1,22,23 Axial halo traction followed by posterior occipitocervical fixation may give good long-term results in patients with BI and symptomatic hydrocephalus complicating OI. 9 Posterior decompression fusion can be performed for cases with reducible BI; halo traction should precede the surgical decompression. 6 However, it is controversial in the literature if VP shunt placement should be performed initially or should follow realignment of the craniocervical junction.…”

“…14 As suggested by Sawin and Menezes, ventriculoperitoneal (VP) shunting should be performed in cases of BI associated with hydrocephalus. 9 Irreducible ventral pathology of the craniocervical region has historically been addressed with posterior and anterolateral approaches. 9 Posterior decompression fusion can be performed for cases with reducible BI; halo traction should precede the surgical decompression.…”