Fraser Syndrome

Figure 3.43. Another infant with the femoral hypoplasia-unusual facies syndrome. Note the small stature, predominantly the result of the small lower limbs.

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Clitoromegaly Newborns

Figure 3.44. Close-up of the lower extremities of the same infant showing the absence of the femora.

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Figure 3.45. Posterior view of the lower extremities of die same infant.

What Frazer Syndrome

Figure 3.46. Radiograph of the lower extremities of the same infant showing the absence of femora bilaterally, a hypoplastic fibula on the right and an absent fibula on the left. In these infants, the acetabula may be hypoplastic and there may be a constricted iliac base with a vertical ischial axis.

Figure 3.46. Radiograph of the lower extremities of the same infant showing the absence of femora bilaterally, a hypoplastic fibula on the right and an absent fibula on the left. In these infants, the acetabula may be hypoplastic and there may be a constricted iliac base with a vertical ischial axis.

Facies Parkinson

Figure 3.47. Another case of the femoral hypoplasia-unusual facies syndrome. Note the typical appearance of the face with the upslanting palpebral fissures, the short nose with hypoplastic alae nasi, long philtrum and micrognathia which was due to marked hypoplasia of the mandible. This infant also had a cleft palate which may be a part of this syndrome.

Figure 3.47. Another case of the femoral hypoplasia-unusual facies syndrome. Note the typical appearance of the face with the upslanting palpebral fissures, the short nose with hypoplastic alae nasi, long philtrum and micrognathia which was due to marked hypoplasia of the mandible. This infant also had a cleft palate which may be a part of this syndrome.

Hypoplastic PhiltrumFraser Syndrome

Figure 3.49. Radiograph of die same infant. The wings of the ilia are slenderized, the acetabular cavities are very shallow and ill formed, and there is bilateral dislocation of the hips. There are symmetric angular deformities in the midshafts of both femora which are shortened in length.

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Figure 3.49. Radiograph of die same infant. The wings of the ilia are slenderized, the acetabular cavities are very shallow and ill formed, and there is bilateral dislocation of the hips. There are symmetric angular deformities in the midshafts of both femora which are shortened in length.

Figure 3.50. This infant, born at 35 weeks gestation, had Fraser's syndrome (cryptophthalmos syndrome). Note the cryptophthalmos on the left and the microphthalmia on the right. The infant had a cleft lip on the left and a high arched palate. There was subglottic tracheal obstruction. In Fraser's syndrome there is cryp-tophthalmos usually with a defect of the eye, and hair growth on the lateral forehead extends to the lateral eyebrow. Cryptophthalmos is bilateral in 50% of cases. There may be hypoplastic, notched nares and a broad nose with a depressed bridge. There are ear anomalies, most commonly cupping. Other findings in the syndrome include laryngeal stenosis or atresia, renal agenesis, and incomplete development of the male or female genitalia. There may be partial cutaneous syndactyly.

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Robinow Syndrome Images
Figure 3.51. In this figure of the same infant, note the abnormal ears with marked cupping.

Figure 3.52. Cutaneous syndactyly (webbing) of the fingers is present in the same infant.

Fraser Syndrome With Webbed Feet

Figure 3.53. The same infant with Fraser's syndrome showing the webbing of the toes.

Figure 3.54. Clitoromegaly in the same infant with Fraser's syndrome. The uterus and ovaries were present on ultrasound and there was bilateral renal agenesis.

Figure 3.53. The same infant with Fraser's syndrome showing the webbing of the toes.

Cryptophthalmos With Other Malformations
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