Congenital onychodysplasia. Report of 11 cases

Abstract

We have seen 11 cases of congenital onychodysplasia over the past six years. Associated anomalies were present in six patients, most of whom had an anomaly of the hand. Roentgenograms of the affected fingers showed hypoplasia, narrowing at the distal third of the distal phalanx, and bifurcation of the distal phalanx. The etiology of this condition is unknown, but clinical study suggested that ischemia of the finger at a certain period of embryonic life might play an important part in its pathogenesis.

Diseases of the Nails in Children

“…Examples of these onycho-osteodystrophies are cartilage-hair hypoplasia (21) and congenital onychodysplasia of the index fingers (COIF) syndrome. The classic appearance of COIF is dystrophy of the index fingernails with loss of the radial aspect; bony abnormalities of the other digits may be associated (22). Possible mechanisms for these changes include digital ischemia or an abnormal grip in utero with pressure on the tip of the index finger from the thtimb (23).…”

Image Gallery: Congenital onychodysplasia of the toenails

“…Congenital onychodystrophy of the index fingers (COIF) identifies a clinical syndrome characterized by nail dysplasias of the index fingers associated with bone abnormalities. In our cases the terms congenital onychodysplasia or Iso and Kikuchi syndrome, rather than COIF, were preferred because the location could vary . Isolated congenital onychodystrophy of the toes with underlying bony abnormality has rarely been reported.…”

Congenital brachydactyly and nail hypoplasia: clue to bone-dependent nail formation

“…More often, however, anonychia and hyponychia form part of syndromes or are associated with skeletal abnormalities. Nail–patella syndrome (OMIM 161200) 1 and Iso–Kikuchi syndrome 2 may be the best known examples of this association. Other rare syndromes featuring bone and nail abnormalities include Coffin–Siris syndrome (OMIM 135900) 3 and brachydactylies.…”