A rare disorder characterized by ulnar and fibula dimelia associated with polysyndactyly of the hands and feet in a mirror fashion and nasal anomalies.
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.
Fewer than 20 patients have been described.
Uncertain. Suggested mechanisms are mutations on MIPOLI: mirror-image Polydactyly gene located on chromosome 14 (14q13). Mutations of the LMBR1 gene on 7q36.3 are also possible.
Clinical, at birth. The association of polydactyly, absent tibia and radius, and nasal defect (hypoplastic nasal alae, grooved columella) confirm the diagnosis. The presence of mirror-image polydactyly is characteristic.
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. Life expectancy appears to be normal.
Precautions before anesthesia
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. Anterior nasal stenosis with respiratory failure has been reported making face-mask ventilation potentially difficult.
There are no known specific implications with this condition.
PN: Laurin-Sandrow syndrome with additional associated manifestations. Am J Med Genet
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.
et al: Laurin-Sandrow syndrome: Review and redefinition. Am J Med Genet Part A 146A: 2557, 2008.
M: Early surgical correction of the nasal deformity in Laurin-Sandrow syndrome. J Craniofac Surg
HH: Hereditary ulnar and fibular dimelia with peculiar facies: A case report. J Bone Joint Surg Am 52A:367, 1970.