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Inherited developmental disorder characterized by pits of the lower lip and cleft lip or cleft palate. It is the most common cleft lip/palate syndrome.

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Cleft Lip and/or Palate with Mucous Cysts of Lower Lip; Lip-Pit Syndrome; Lip-Pit-Cleft Syndrome.

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Congenital genetic disorder first studied by A. Van der Woude in 1954.

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1:70,000 live births.

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Autosomal dominant.

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Caused by mutations in the gene encoding interferon regulatory factor-6 located on 1q32-q41; allelic to popliteal pterygium syndrome. Clinical expression is highly variable from one individual to another.

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Clinically evocated in patients with cleft lip or palate and mucous cysts of lower lip. Lip-Pit Syndrome is the most common manifestation present in 90% of the affected. The cleft of lip and palate occur in 21%.

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Generally concerns only head with lower lip pits (corresponding to accessory salivary glands), cleft lip, cleft palate, cleft uvula, and hypodontia.

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Evaluate the airway (clinical, radiographs) and teeth mobility.

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Direct laryngoscopy and tracheal intubation can be difficult because of cleft syndrome. Teeth lesions can occur more frequently.

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Avoid muscle relaxants until confirmation that face-mask ventilation is possible or airway is secured.

Guner U, Celik N, Ozek C, et al: Van der Woude syndrome. Scand J Plast Reconstr Surg Hand Surg 36(2):103, 2002.
Ryns JP, Devriendt K: On the nosology of van der Woude syndrome and popliteal pterygium syndrome: Implications for genetic counseling. Genet Couns 11(1):59, 2000.
van der Woude A: Fistula labii inferioris congenita and its association with cleft lip and palate. Am J Hum Genet 6:244, 1954.

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