Infant Ear Syndrome

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Newborn Lupus
Figure 3.69. Posterior view of the same infant showing the short neck (congenital brevicollis) and the low hairline.
Abnormally Short Neck

Figure 3.70. A lateral view of the face, neck and chest of another infant with Klippel-Feil syndrome. Note the extremely short neck with low hairline and very abnormal ear.

3.70

Figure 3.70. A lateral view of the face, neck and chest of another infant with Klippel-Feil syndrome. Note the extremely short neck with low hairline and very abnormal ear.

Klippel Feil Infant

Figure 3.71. In this infant with the Klippel-Feil syndrome note the abnormal ear and short neck on the left. On the right, note that the abnormality of the ear results from pressure of the shoulder on the developing ear — a deformation.

3.71

Figure 3.71. In this infant with the Klippel-Feil syndrome note the abnormal ear and short neck on the left. On the right, note that the abnormality of the ear results from pressure of the shoulder on the developing ear — a deformation.

Figure 3.72. Anteroposterior radiograph of an infant with Klippel-Feil syndrome showing the numerous cervical spine vertebral body anomalies.

Klippel Feil Syndrome Infant

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Klippel Feil Syndrome And Newborn

Figure 3.73. Lateral radiograph of the same infant as in Figure 3.72 with Klippel-Feil syndrome again shows cervical vertebrael anomalies resulting from abnormal fusion of the cervical vertebrae. There may be other associated skeletal defects such as the Sprengel's deformity or thoracic hemivertebrae. If the brachial plexus is involved, it may result in deformities of the hand.

Langer Giedion Syndrome

Figure 3.74. Infants with the Langer-Giedion syndrome have a bulbous nose, tented alae nasi, and a prominent elongated philtrum. There is a thin upper lip with mild micrognathia and mild microcephaly. Scalp hair is sparse. The ears are hypertrophic with excessive folding and tissue mass. The skin is redundant and loose. This infant, in addition to the above findings, had cutis verticis gyrata.

Figure 3.73. Lateral radiograph of the same infant as in Figure 3.72 with Klippel-Feil syndrome again shows cervical vertebrael anomalies resulting from abnormal fusion of the cervical vertebrae. There may be other associated skeletal defects such as the Sprengel's deformity or thoracic hemivertebrae. If the brachial plexus is involved, it may result in deformities of the hand.

Figure 3.74. Infants with the Langer-Giedion syndrome have a bulbous nose, tented alae nasi, and a prominent elongated philtrum. There is a thin upper lip with mild micrognathia and mild microcephaly. Scalp hair is sparse. The ears are hypertrophic with excessive folding and tissue mass. The skin is redundant and loose. This infant, in addition to the above findings, had cutis verticis gyrata.

Cutis Verticis GyrataLanger Giedion Syndrome
Figure 3.75. In this figure of the same infant again note the bulbous nose, thin upper lip on the left and the cutis verticis gyrata on the right. The tri-chorhinophalangeal syndrome has many similarities to Langer-Giedion syndrome except that the redundant skin and microcephaly are not present.

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Vertical Creases Ear

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Figure 3.76. Close-up of the ears of the same infant showing the hypertrophy with excessive folding and tissue mass.

Langer Giedion Syndrome
Figure 3.77. The same infant had large vertical creases on both plantar surfaces anteriorly. These are strongly associated with trisomy 8. The Langer-Giedion syndrome recently has been associated with a deletion of the long arm of chromosome 8.

Figure 3.78. This infant with Larsen's syndrome demonstrates the prominent forehead, depressed nasal bridge, and dislocation of the elbows on the left. On the right there is dislocation of die hips, knees, and talipes equinovarus.

Figure 3.78. This infant with Larsen's syndrome demonstrates the prominent forehead, depressed nasal bridge, and dislocation of the elbows on the left. On the right there is dislocation of die hips, knees, and talipes equinovarus.

Larsen Syndrome

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