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Juvenile progressive scoliosis and congenital horizontal gaze palsy
1Department of Pediatric Orthopedics, Orthopedic Surgery Hospital, University of Heidelberg, Germany.
Insights
Progressive scoliosis and congenital gaze palsy in children may stem from brainstem or central nervous system issues. This combination often requires early surgical intervention despite other treatments.
Area of Science:
- Neurology
- Orthopedics
- Genetics
Background:
- Progressive scoliosis and congenital gaze palsy are distinct conditions.
- A rare association between these two conditions has been noted in medical literature.
- The underlying pathophysiology linking these disorders remains unclear.
Abstract:
Eleven articles reporting 39 patients with the combination of progressive scoliosis and familial congenital gaze palsy have now been published. This disorder appears to be caused by a malfunction of the normal equilibrial control mechanism related to the brainstem or the central nervous system. The scoliosis progresses when the children begin to walk. Even when they are treated with physiotherapy or brace, they often require early operation. We consider that progressive juvenile or infant scoliosis associated with familiar congenital horizontal gaze palsy constitutes a clinical entity. We report two sisters with congenital gaze palsy, in one of whom scoliosis of at least 70 degrees (Cobb) developed, and review and analyze all cases published until now.