[Rieger syndrome as an expression of neural crest dysgenesis]

Malformations of the anterior segment of the eye can be associated with different systemic findings. The regular combination of aplasia of the permanent central and lateral incisors, typical ocular changes, and umbilical dysmorphology is known as the Rieger syndrome. Aplasia of the permanent incisors can serve as a diagnostic symptom. We report on three siblings who had characteristical symptoms of Rieger syndrome. The tissue involved derives from the neural crest cells. Mesenchymal dysgenesis is discussed as the etiology of this disorder.