Congenital pits of the lower lip constitute a rare developmental malformation, transmitted by an autosomal dominant mode, with considerable heterogeneity as regards the expression of the disorder. They are present in van der Woude syndrome (VWS), in which clefts of the upper lip and/or palate are often observed. Literature related to the various parameters associated with and relevant to the disorder is extensive. The purpose of this review is to cover, synthesize and categorize the existing knowledge into distinct entities, in order to facilitate understanding of the aetiopathogenesis of the malformation, its clinical manifestations and histological features, the epidemiology of the syndromic situation and the fundamental approach to an integral differential diagnosis. Special emphasis is given to the rationale underlying the treatment modalities that have been suggested, and the necessity for appropriate genetic counselling, as the disorder shows a high affinity with clefts and a familial type of occurrence.
The clinicopathological findings on a 17-year-old female with the Möbius/Moebius syndrome are reported. The signs and symptoms of this neuromuscular condition include congenital bilateral or unilateral palsies of the facial and abducens cranial nerves and a broad scope of multisystem abnormalities. A case of unilateral deficiencies of cranial nerves VI and VII, congenital ectrodactyly of toes, and multiple congenitally missing primary and permanent teeth is reported. A review of the literature reveals various ideas regarding the diversity of symptoms and the etiology of the syndrome. The purpose of this article is to report oral manifestations, such as congenitally missing teeth, associated with Möbius syndrome.
The clinicopathological findings on a 17-year-old female with the Möbius/Moebius syndrome are reported. The signs and symptoms of this neuromus-cular condition include congenital bilateral or unilateral palsies of the facial and abducens cranial nerves and a broad scope of multisystem abnormalities. A case of unilateral deficiencies of cranial nerves VI and VII, congenital ectro-dactyly of toes, and multiple congenitally missing primary and permanent teeth is reported. A review of the literature reveals various ideas regarding the diversity of symptoms and the etiology of the syndrome. The purpose of this article is to report oral manifestations, such as congenitally missing teeth, associated with Möbius syndrome.
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