Definition:
Cenani Lenz syndactylism is a hereditary malformation syndrome that involves both the lower and upper extremity. It's an uncommon birth defect that's characterized by a variety of bone abnormalities. The syndrome was named after two medical geneticists; Turkish geneticist Asim Cenani, and German geneticist Widukind Lenz.
Diagnosis:
Diagnosis of whether the syndrome's deformity is due to joint/bone disease or neurologic disease is generally simple; an X-ray of the feet or hands can be useful several congenital and acromegaly disorders. Referral to neurologic or orthopedic specialist is typically specified if neurologic or bone involvement is likely.
Treatment:
Treatment options may include therapies and/or surgical procedures to correct the malformation.
Symptoms and Signs:
The syndrome is distinguished by almost symmetrical occurrence of spoon-like hand. There is also occasional mesomelic arm shortening, metacarpal and radio-ulnar synostosis, in addition to disorganized phalanges. The feet are mildly affected. Other symptoms include arm bones fusion, metacarpal fusion, brachymesomelia, numeric rays reduction, short radius, and short ulna.
Causes:
Cenani Lenz syndactylism shows autosomal recessive heredity as one cause. Some malformations of an individual's extremities originate from joint or neurologic diseases; however, there are several exceptions to the rule. Some of these causal diseases include vascular disease, inflammatory disease, neurologic disorders, degenerative diseases, intoxication, congenital disorders, autoimmune diseases, traumatic lesions, and endocrine disorders.
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