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A disorder associated with osseous syndactyly and mesomelic brachymelia.

Cenani-Lenz Syndactyly; Cenani-Lenz Oligodactyly-Synostosis Syndrome.

Named after A. Cenani from Turkey and W. Lenz from Germany, two medical geneticists who described this disorder in 1967, although reports have described this syndrome as early as 1938.

Extremely rare (fewer than 10 patients have been described). Most often transmission is autosomal recessive, however, quasidominant inheritance has also been described. In most cases, parental consanguinity is present.

The main findings are short stature, syndactyly/synostosis with abnormal phalanges, and fusion of the metacarpals resembling the “spoon hand” deformity of Apert syndrome. The ulna and radius may be fused and shortened. The feet are usually less severely affected. Joint mobility may be restricted. Agenesis or hypoplasia of the kidneys, genital anomalies, cavernous hemangiomas, and supernumerary nipples have been observed occasionally. Facial features may include a high, wide, prominent forehead, hypertelorism, downslanting palpebral fissures, short nose with depressed nasal bridge, short but prominent philtrum, and malar hypoplasia.

Preoperative blood work should include a complete blood count (renal anemia) and levels of serum electrolytes, creatinine, and blood urea nitrogen. Airway anatomy should be assessed because airway management could potentially be difficult, depending on the degree of malar hypoplasia. Limb deformities and the site of surgery may limit (venous and arterial) vascular access sites. Careful intraoperative positioning and padding are required. Both fluid regimen and anesthetic drug doses should be adapted in the presence of renal dysfunction.

Cenani A, Lenz W: Totale Syndaktylie und totale radioulnare Synostose bei zwei Brüdern. Ein Beitrag zur Genetik der Syndaktylien. Zeitschr Kinderheilk Berl 101:181, 1967.  [PubMed: 4298043]
De Smet L, De Beere P, Fryns JP: Cenani-Lenz Syndrome in father and daughter. Genet Couns 7:153, 1996.
Temtamy SA, Ismail S, Nemat A: Mild facial dysmorphism and quasidominant inheritance in Cenani-Lenz syndrome. Clin Dysmorph 12:77, 2003.  [PubMed: 12868467]

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