Occipitocervical fusion in skeletal dysplasia: a new surgical technique.
METHODS
Retrospective cohort study.


OBJECTIVE
This study describes clinical and radiological results of a new cable technique for occipitocervical fusion (OCF) in children with skeletal dysplasia (SD).


BACKGROUND
Anatomical variability and poor bone quality make upper cervical surgery technically challenging in patients with SD. We present a new cable technique for OCF in children with SD when the posterior elements are not of a size or quality for other types of instrumentation.


METHODS
Retrospective review of 24 patients with SD (8 boys, 16 girls) who underwent OCF between 2001 and 2011. In this technique, cables provide compression across a bone graft that is prevented from entering the canal and the graft resists excessive lordosis. Demographic and radiographical data are presented. All patients were followed for initial outcomes of surgery, and 20 patients (83%) were followed for 2 years or more for mid- and long-term outcomes.


RESULTS
Mean age at surgery was 6.5 years and mean follow-up was 4.1 ± 2.4 years. This technique was used as a primary procedure in 20 and a revision procedure in 4 patients. Diagnoses included Morquio syndrome (6), spondyloepiphyseal dysplasia (9), spondyloepimetaphyseal dysplasia (5), metatropic dysplasia (3), and Kniest syndrome (1). Ten patients had upper cervical instability and features of cervical myelopathy, and the remaining 14 patients had instability and signal changes on magnetic resonance image. Fusion extended from occiput to C2 in 71% patients, and upper cervical decompression was needed in 92% patients. Postoperatively, all patients were immobilized in a halo vest for mean duration of 12 weeks. Fusion was achieved in all patients. Complications included halo pin-tract infections (7), junctional instability (2), and extension of fusion (4).


CONCLUSIONS
This new cable technique is a good alternative for OCF in patients with SD who have altered anatomy at the craniocervical junction not amenable to rigid internal fixation.


METHODS
4.
From the Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE.
Prakash
P
Sitoula
William G
WG
Mackenzie
Suken A
SA
Shah
Mihir
M
Thacker
Colleen
C
Ditro
Laurens
L
Holmes
Jeffrey W
JW
Campbell
Kenneth J
KJ
Rogers
D000293:Adolescent; D001848:Bone Diseases, Developmental / Q000601:surgery*; D016025:Bone Transplantation / Q000379:methods; D002574:Cervical Vertebrae / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D002972:Cleft Palate / Q000601:surgery; D003095:Collagen Diseases / Q000601:surgery; D004392:Dwarfism / Q000601:surgery; D005145:Face / Q000002:abnormalities / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006819:Hyaline Membrane Disease / Q000601:surgery; D007223:Infant; D008297:Male; D009085:Mucopolysaccharidosis IV / Q000601:surgery; D009777:Occipital Bone / Q000601:surgery*; D010009:Osteochondrodysplasias / Q000601:surgery; D012189:Retrospective Studies; D013123:Spinal Fusion / Q000379:methods*; D016896:Treatment Outcome