A disorder associated with osseous syndactyly and
Cenani-Lenz Syndactyly; Cenani-Lenz
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
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
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