Journal of Pediatric Orthopaedics · 1989 · 25 citations · 0 references
MedicineNeurologyPediatricsPediatric NeurologyPediatric OrthopaedicsPediatric PathologyAtlantoaxial InjuryCervical DisordersCervical PathologySpinal DisordersSpinal StabilitySpine DisordersAtlanto-axial InjuryAtlantoaxial Rotatory SubluxationAtlantoaxial SubluxationC1-c2 InstabilityCervical Spine AnatomyCervical Spine DisordersCervical Spine InstabilityRoutine Screening
We report a 15-year-old boy with mucopolysaccharidosis (MPS) Type VII (Sly disease) who was found to have atlantoaxial instability with quadriparesis. Subluxation of C1 on C2 has also been documented in patients with Type I and Type IV MPS. Routine screening of the cervical spine is recommended in patients with "MPS Type VII" so that delayed diagnosis of instability, which may lead to neurologic compromise, is avoided.