Non-eruption of teeth-maxillary hypoplasia-genu valgum syndrome

MedGen UID:: 341331
•Concept ID:: C1848903
•: Disease or Syndrome

Synonyms:	Multiple none-erupting teeth, maxillo-zygomatical hypoplasia and other congenital defects; Teeth noneruption of with maxillary hypoplasia and genu valgum
Modes of inheritance:	No inheritance data available MedGen UID: 989178 •Concept ID: CN307043 • Finding Source: Orphanet No information is available in the scientific literature on heredity of the clinical entity. See: This record No inheritance data available (Orphanet)

Monarch Initiative:	MONDO:0010104
OMIM®:	273050
Orphanet:	ORPHA2972

Definition

Noneruption of teeth - maxillary hypoplasia - <i>genu valgum</i> is an extremely rare syndrome that is characterized by multiple unerupted permanent teeth, hypoplasia of the alveolar process and of the maxillo-zygomatic region, severe <i>genu valgum</i> and deformed ears. [from ORDO]

Clinical features

From HPO

Genu valgum

MedGen UID:: 154364
•Concept ID:: C0576093
•: Anatomical Abnormality

The legs angle inward, such that the knees are close together and the ankles far apart.

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Low-set ears

MedGen UID:: 65980
•Concept ID:: C0239234
•: Congenital Abnormality

Upper insertion of the ear to the scalp below an imaginary horizontal line drawn between the inner canthi of the eye and extending posteriorly to the ear.

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Posteriorly rotated ears

MedGen UID:: 96566
•Concept ID:: C0431478
•: Congenital Abnormality

A type of abnormal location of the ears in which the position of the ears is characterized by posterior rotation (the superior part of the ears is rotated towards the back of the head, and the inferior part of the ears towards the front).

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Maxillozygomatic hypoplasia