Severe Microcephaly

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Seckel Dwarfism

Figure 2.79. In Seckel's bird-headed dwarfism there is severe growth retardation with proportional dwarfism. This infant at 35 weeks gestation had a birth weight of 910 g, a length of 31.5 cm, and a head circumference of 23 cm, all less than the 10th percentile. There was severe microcephaly with premature fusion of all sutures, prominent eyes, a prominent beak-like nose, micrognathia, and malformed ears (low-set and lack of lobe). These infants have postnatal growth retardation and moderate to severe mental retardation.

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Microcephaly Pictures

Figure 2.80. A close-up of the face of die same infant showing die severe microcephaly, the prominent eyes, the beak-like nose, and micrognathia. Note the low-set ear with lack of ear lobe. On CT scan the ventricles were barely perceptible and small in size.

Figure 2.81. A less severe example of the Seckel's bird-headed dwarfism in which the microcephaly is striking but the other features are not as prominent.

Figure 2.82. Radiograph of die skull in die same infant with Seckel's birddieaded dwarfism. Note the narrow (but not closed) sutures and tooth bud mineralization (incisors and first molars) in this infant. The tooth mineralization indicates that this infant had a gestational age of 35 weeks.

Figure 2.83. This infant is a typical example of spondy-lothoracic dysplasia (Jarcho-Levin syndrome). She had marked shortness of the neck and posterior aspect of the chest, with an increased diameter of the thoracic cage. The limbs were long and thin with tapering digits. Note the broad forehead and wide nasal bridge with anteverted nares. These infants typically have multiple anomalies of the vertebrae and a short thorax with a diminished number of ribs.

Figure 2.82. Radiograph of die skull in die same infant with Seckel's birddieaded dwarfism. Note the narrow (but not closed) sutures and tooth bud mineralization (incisors and first molars) in this infant. The tooth mineralization indicates that this infant had a gestational age of 35 weeks.

Growths NostrilThanatophoric Dysplasia

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Figure 2.84. View of the back of the head and neck of the same infant showing the marked shortness of the neck and prominence of the occiput.

Figure 2.84. View of the back of the head and neck of the same infant showing the marked shortness of the neck and prominence of the occiput.

Microcephaly
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Responses

  • LAVINIA
    What percentile is considered microcephaly?
    3 years ago
  • reece
    What is the name of the growth on ear lobes?
    2 years ago

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