Sandrow Syndrome

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.