Leri Pleonosteosis

Congenital disorder of the bone and joints with characteristic facial features. The presence of laryngeal stenosis may be a significant anesthetic consideration.

Only a few cases reported in the literature.

Autosomal dominant.

Etiology unknown. Primary pathology may be in the joint capsules rather than the bone itself. A characteristic feature is bony overgrowth of the cartilaginous skeleton, which produces increased width rather than length of the bones, together with enlargement of the bone ends.

Based on clinical features. Radiologic features include evidence of delayed bone age, marked broadening of the phalanges with evidence of flexion deformity, increase in width of the vertebrae, and bizarre enlargement of the posterior neural arches of the cervical vertebrae.

Short stature; mongoloid facies; brachydactyly; genu recurvatum; short, spade-like hands; broad thumbs in valgus position; thick palmar and forearm fasciae; shuffling, short-stepped gait; decreased joint mobility; and laryngeal stenosis.

Assess airway and neck mobility.

Difficult tracheal intubation because of limited neck movement and laryngeal stenosis.

No known specific pharmacological implications; however, several patients will be on chronic corticosteroid therapy and will require intraoperative supplementation.

Acromicric Dysplasia: Mild facial abnormalities, short hands and feet, short stature with delayed bone maturation (may be the same disease as Moore-Federman syndrome).

Léri-Weill Syndrome: Dominant pseudoautosomal skeletal dysplasia with mesomelic short stature as a result of the loss of one copy of the short stature homeobox gene (SHOX) located on the short arm of the X or Y chromosome.

Moore-Federman Syndrome: Short stature with disproportionately short legs, joint limitations, ocular anomalies, laryngeal stenosis. Autosomal dominant transmission.