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1148 PART IV Obstetric and Fetal Sonography

A

B

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D

FIG. 33.17 Abnormal Ears. (A) Week 28 fetal sonography showing low-set ears and skin tag (arrows) on two-dimensional (B) and threedimensional

(C) images. (D) A different 27-week fetus with agnathia, microstomia, and low-set, abnormally turned ears.

EAR ABNORMALITIES

Abnormalities of the ears can be very diicult to diagnose on

fetal sonography, but low-set ears are associated with multiple

syndromes, including Noonan syndrome and certain trisomies.

Low-set ears are described as the helix joining the cranium at a

level below a horizontal plane through the inner canthi of the

eyes. Ear anomalies may be more easily detected on 3-D sonography

than on 2-D studies 44,45 (Fig. 33.17).

Microtia, or small ears, is a rare anomaly with an incidence

of approximately 1 per 10,000 live births 46 and is oten associated

with syndromes and aneuploidy. Of 96 aneuploid fetuses, Yeo

and colleagues 47 reported that 66% had small (<10th percentile)

ears on sonography.

Otocephaly is a condition with union of the ears on the front

of the neck and is caused by failure of ascent of the auricles

during embryologic development. his is usually a fatal anomaly,

oten associated with agnathia or micrognathia, as well as

holoprosencephaly. he most severe form of otocephaly may be

associated with absence of the eyes, forebrain, and mouth.

MIDFACE ABNORMALITIES

Hypoplasia

he midface is the area between the upper lip and forehead.

Midface hypoplasia can arise from a variety of causes, including

syndromes such as Apert, Crouzon, Treacher Collins, 48 Wolf-

Hirschhorn, 49 Pfeifer (see Fig. 33.13), Turner, and trisomy 21.

Midface hypoplasia may also result from facial clets, craniosynostosis,

and skeletal dysplasias. Midface hypoplasia is best

demonstrated on sagittal midline views of the face, where one

can see abnormal concavity of the midface, between the lower

margin of the orbits and the upper jaw (Figs. 33.18 and 33.19).

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