Dubousset J, Haddad F, Zeller R, Queneau P
Service de Chirurgie Orthopédique, Hôpital Saint-Vincent-de-Paul, Paris.
Rev Chir Orthop Reparatrice Appar Mot. 1994;80(7):610-9.
A previously unreported condition is defined, diagnostic features identified, and clinical course and treatment modalities presented.
This is a retrospective study of 11 patients with ischio-vertebral dysplasia (IVD), accomplished by record and radiographic review.
11 patients were included in the study group, age at presentation from 1 day to 33 yrs. Follow-up ranged from 5-30 yrs. Involvement of 3 successive generations (grandmother, mother and daughter) with similar findings was present in 1 family. Common features of IVD include: peculiar facies incomplete ossification of the ischial ramus, and a dysplasic scoliosis with a significant kyphotic element, constituting a rotatory dislocation. 2 patients followed from birth demonstrated the natural history of the condition, beginning without spinal deformity, and progressing to significant deformity. The spinal deformity was manageable surgically. The scoliosis ranged from 10 degrees to 235 degrees, and the kyphosis from 0 degrees to 200 degrees (the 10 and 0 being at day 1 of age). 6 patients incurred neurological sequelae, either spontaneously or associated with surgical treatment. 2 patients died, 1 due to cardiopulmonary failure at age 33; the 2nd was an infant with severe neurologic and cardiopulmonary complications due to the spinal deformity. Multiple surgical approaches to the problems were employed. Analysis of results permitted formulation of a logical and successful approach to the problem. Pre-op mean kyphosis = 112 degrees, post-op at maximum follow-up = 67 degrees. Deformity stabilization and neurologic normalcy was produced in every patient operated but 1.
The characteristics of the syndrome are clear, as is the progressive nature of the deformity. The very high risk of neurologic involvement is emphasized. Before age 10, circumferencial fusion prevents progression and neurologic deterioration. Older patients require gradual correction by skeletal traction, followed by anterior concave strut stabilization and posterior fusion (with or without instrumentation). Extreme care is necessary for protection of the particularly vulnerable neurologic structures. Early stabilization and correction is recommended for prevention of the deterioration of cardiopulmonary function.
