Infant Hemifacial Microsomia

Newborn Microsomia
Figure 1.158. Micrognathia in an infant with Goldenhar's syndrome (hemifacial microsomia). In addition, note the preauricular skin tags, unilateral macrostomia, and skin tags due to the extra branchial arch anomalies.

1.159

Thyroglossal Duct Newborn Photo

Figure 1.159. A congenital midline cervical cleft is a rare developmental anomaly. It represents failure of the branchial arches to fuse in the midline. It most commonly affects females, and presents at birth with a ventral midline defect of the skin of the neck. A reddened weeping strip of atrophic skin approximately 5 mm in width may occur at any level between the chin and sternal notch. Often there is a nipple-like projection at the upper end of the fissure and an associated sinus tract at the caudal end which may discharge mucoid material. This condition may be misinterpreted as a branchial cleft anomaly or thyroglossal duct cyst.

Midline Cervical Cleft

Figure 1.160. Another example of a congenital midline cervical cleft. Note the characteristic nipple-like projection, atrophic skin defect, and caudal fistulous tract. This may become a fibrous "cord" and result in a web-like contracture. This must be differentiated from a thyroglossal duct cyst/sinus which develops if the thy-roglossal duct fails to close after the descent of the thyroid gland into the lower neck. It can occur anywhere on a line connecting the sternal notch and the base of the tongue.

1.160

Figure 1.160. Another example of a congenital midline cervical cleft. Note the characteristic nipple-like projection, atrophic skin defect, and caudal fistulous tract. This may become a fibrous "cord" and result in a web-like contracture. This must be differentiated from a thyroglossal duct cyst/sinus which develops if the thy-roglossal duct fails to close after the descent of the thyroid gland into the lower neck. It can occur anywhere on a line connecting the sternal notch and the base of the tongue.

Newborn Cleft Cyst Neck

1.161

Figure 1.161. When delivery has involved excessive rotation or gross lateral rotation of the neck, a lump may appear in the stemomastoid muscle (stemomastoid tumor). This usually becomes apparent in the second week of life and commonly is situated in the lower half of the muscle. It may enlarge before resolving spontaneously, and may result in torticollis as a result of contraction of the sternomastoid muscle causing flexion of the head toward the side of the lesion. This condition must be differentiated from superior oblique palsy (IVth cranial nerve palsy) by an ophthalmologist.

Figure 1.162. Abnormal development of the branchial clefts and arches may result in remnants, fistulae or cysts. Defects are usually unilateral and the external opening lies at the anterior edge of the sternocleidomastoid muscle, usually at the lower third. Secondary bacterial infection and cyst formation may occur. In this infant there is a branchial cleft remnant.

Branchial Cleft Remnant

1.162

Branchial Sinus

Figure 1.163. The subtle finding of a branchial sinus may be missed if examination of the infant is not thorough.

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Responses

  • Peregrin Smallburrow
    What is goldenhar syndrome?
    6 years ago

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