Skip to Main Content

++

A rare disorder characterized by ulnar and fibula dimelia associated with polysyndactyly and peculiar facies.

++

Laurin-Sandrow Syndrome; Sandrow Sullivan Steel Syndrome; Duplication of Fibula and Ulna with Absence of Tibia and Radius; Mirror Hands and Feet with Nasal Defects; Tetramelic Mirror-Image Polydactyly; Mirror-Image Polydactyly.

++

First described by Laurin in 1964 and subsequently by Sandrow in 1970.

++

Autosomal dominant.

++

Causal mechanism seems to be a gene that may determine anterior-posterior pattern in early developing (MIPOLI: mirror-image Polydactyly gene). It has been located on chromosome 14 (14q13).

++

Clinical, at birth. Association of polydactyly, absent tibia and radius, nasal defect (hypoplastic nasal alae, grooved columella). The presence of mirror-image polydactyly usually confirms the diagnosis.

++

This polymalformative syndrome can be associated with other orthopedic abnormalities: tarsal fusion, wrist anomalies, joint dislocation, syndactyly of toes and fingers, talipes varus/valgus, restricted joint mobility, claw hand/camptodactyly of all fingers, genu valgum, genu recurvatum. Other clinical features are less frequent and can include capillary hemangioma ectopic testes.

++

Evaluate orthopedic functional status (clinical).

++

Intraoperative positioning should be achieved carefully and can be difficult because of deformation and restricted mobility. Blood pressure measurement and venous and arterial access can be difficult because of multiple limb deformations. Regional anesthesia is not contraindicated but can be difficult in presence of skeletal anomalies, especially with neuraxial blockade.

++

There are no known specific implications with this condition.

Kantaputra PN: Laurin-Sandrow syndrome with additional associated manifestations. Am J Med Genet 98:210, 2001.  [PubMed: 11169557]
Laurin CA, Favreau JC, Labelle P: Bilateral absence of the radius and tibia with bilateral reduplication of the ulna and fibula: A case report. J Bone Joint Surg Am 46A:137, 1964.
Sandrow RE, Sullivan PD, Steel HH: Hereditary ulnar and fibular dimelia with peculiar facies: A case report. J Bone Joint Surg Am 52A:367, 1970.

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.