Zimmermann–Laband syndrome with bilateral developmental cataract – a new association?

Abstract

An unusual case of Zimmermann-Laband syndrome in a young male child with an unreported association of bilateral developmental cataract is presented. The pathognomonic triad of gingival fibromatosis, aplastic or hypoplastic distal phalanges with absent nails, and enlargement of soft tissues of the face were obvious, besides the known moderate learning disability and mild hearing loss. The case is discussed in the light of relevant literature. To the best of our knowledge, this is the first report of early developmental cataracts in association with the Zimmermann-Laband syndrome. Besides detection and timely recognition of the syndrome to allow adequate dental care, ophthalmic screening at periodic intervals is merited to improve the overall quality of life for these patients.

Effect of visual impairment upon oral health care: a review

“…There may also be a need to adapt oral hygiene instruction methods with the use of models and other aids. Studies of relevance have shown that 41 Kawamoto et al ; 42 Koseki; 29 Shah et al ; 39 Kumar et al; 43 Kuru et al ; 44 Barthelemy et al ; 45 Scully 2001; 46 Scully et al ; 50 Dimitrakopoulos et al ; 47 Lilly et al ; 48 Toumba et al ; 49 Mass et al ; 35 Naussbaum et al ; 36 Thompson et al 37 individuals with a visual impairment are used to relying on verbal instruc tions and following them carefully and respond well to simple information which is adequately explained.…”

“…An associa tion has been highlighted between some congenital ocular defects and gingival fi bromatosis/enlargement. 39 Impaired vision may prevent detection of asympto matic oral mucosal disease, [19][20] eg malig nancy, yet without good data this cannot be proved.…”

Expanding the phenotype of gingival fibromatosis–mental retardation–hypertrichosis (Zimmermann–Laband) syndrome

“…Rutherfurd et al [1931] reported a patient with gingival fibromatosis and corneal dystrophy. Shah et al [2004] described a ZLS individual with bilateral cataract. Our patient presented corneal leucoma and cataract in the right eye.…”

Report of a case of Zimmermann–Laband syndrome with new manifestations

“…1 The condition results from autosomal dominant inheritance and involves a highly variable phenotype 1,9,16 . In this study, the authors report on a patient with ZimmermannLaband syndrome with new manifestations.…”