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;
First described by Laurin in 1964 and subsequently by Sandrow in 1970.
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
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
There are no known specific
implications with this condition.
Kantaputra PN: Laurin-Sandrow syndrome with additional associated
manifestations. Am J Med Genet
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
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.