[MIM  135 500]

(Laband syndrome)

Rare. Still unknown genetic origin. A few cases of 3p14.3 anomalies have been described. The phenotype is highly variable.

Association of:

-        gingival fibromatosis

-        absence or hypoplasia of nails of the terminal phalanx of the fingers and toes

-        face with coarse features: thick lips; local cartilage malformation produce thick and soft ears and a bulbous nose

-        sometimes: hepatosplenomegaly, hypertrichosis, bad hearing, joint hyperlaxity, scoliosis.

-        sometimes: mental retardation

Anesthetic implications:

hyperplastic fibrous tissue present at the level of the gums can render intubation or the use of a supraglottic device  difficult; check liver function

References :  

-        Lin Z, Wang T, Sun G, Huang X. 
Report of a case of Zimmermann-Laband syndrome with new manifestations. 
Int J Oral Maxillofac Surg 2010 ; 39 : 936-40.

Updated: August 2018