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Micrognathia

Pierre Robin sequence is typically not inherited and involves underdevelopment of the jaw (mandible), cleft palate, and sometimes eye or ear defects. The initiating defect is a small jaw (micrognathia) which causes the tongue to be displaced backwards, preventing the roof of the mouth from fully closing during development and resulting in a cleft palate. Very rarely it can be inherited. Retardation of jaw development causes varying degrees of a small jaw and improper alignment of the teeth. No jaw development at all (agnathia) is associated with abnormally low placement of the external ears.

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28 views1 page

Micrognathia

Pierre Robin sequence is typically not inherited and involves underdevelopment of the jaw (mandible), cleft palate, and sometimes eye or ear defects. The initiating defect is a small jaw (micrognathia) which causes the tongue to be displaced backwards, preventing the roof of the mouth from fully closing during development and resulting in a cleft palate. Very rarely it can be inherited. Retardation of jaw development causes varying degrees of a small jaw and improper alignment of the teeth. No jaw development at all (agnathia) is associated with abnormally low placement of the external ears.

Uploaded by

Nabilla Faraliza
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as DOCX, PDF, TXT or read online on Scribd
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Pierre Robin sequence typically occurs de novo in most patients and is associated with

hypoplasia (under- development) of the mandible, cleft palate, and defects of the eyes and ears.
Rarely, it is inherited in an autosomal dominant pattern. In the Robin morphogenetic complex,
the initiating defect is a small mandible (micrognathia), which results in posterior displacement
of the tongue and obstruction to full closure of the palatal processes, result- ing in a bilateral cleft
palate (see Figs. 9-39 and 9-40).

(1)

Retardation of mandibular development gives rise to micrognathia of


varying degrees, with accompanying dental malocclusion. Total failure
of development of the mandible (agnathia) is associated with abnormal
ventral placement of the external ears (synotia) (see Fig. 3–21).
(2)

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