Ellis Van Creveld

Polydactyly
Figure 2.43. Postaxial Polydactyly of the toes in an infant with Ellis-van Creveld syndrome. In diis syndrome, Polydactyly is noted in the fingers in 100% of cases but is present in the toes in only 10 to 20%.

2.44

Midline Defects Syndrome

Figure 2.44. In this infant with Ellis-van Creveld syndrome, note on the left the short upper lip widi midline defect due to fusion of the upper lip to the maxil-lary-gingival margin. On the right, note that the fusion of die upper lip to the maxillary-giiigival margin results in a lack of die mucobuccal fold or sulcus which normally is present anteriorly.

Figure 2.44. In this infant with Ellis-van Creveld syndrome, note on the left the short upper lip widi midline defect due to fusion of the upper lip to the maxil-lary-gingival margin. On the right, note that the fusion of die upper lip to the maxillary-giiigival margin results in a lack of die mucobuccal fold or sulcus which normally is present anteriorly.

2.45

Ellis Van Creveld Lip

Figure 2.45. This infant with Ellis-van Creveld syndrome demonstrates on the left the fusion of the labiogingival margins so there is no sulcus to the upper lip. Also note the hypoplastic neonatal teeth in the upper jaw. On the right, note the hypoplastic neonatal teeth in the lower jaw. Neonatal teeth are present in 30% of infants with Ellis-van Creveld syndrome.

Figure 2.45. This infant with Ellis-van Creveld syndrome demonstrates on the left the fusion of the labiogingival margins so there is no sulcus to the upper lip. Also note the hypoplastic neonatal teeth in the upper jaw. On the right, note the hypoplastic neonatal teeth in the lower jaw. Neonatal teeth are present in 30% of infants with Ellis-van Creveld syndrome.

Figure 2.46. A radiograph of die chest of an infant with Ellis-van Creveld syndrome. Note the long narrow chest and short ribs widi cardiac enlargement. Congenital heart disease is present in 50 to 60% of cases of Ellis-van Creveld syndrome. This infant had a large atrial septal defect, the most common lesion seen in Ellis-van Creveld syndrome.

Ellis Van Cleveld Syndrome

Figure 2.47. Radiograph showing die mesomelic shortening of the limbs and Polydactyly. The proximal end of the ulna and distal end of the radius are swollen and bulbous giving die appearance of two parallel drumsticks that point in opposite directions.

Ellis Van Creveld Syndrome Drumstick

Figure 2.48. Radiograph of the hand in an infant with Ellis-van Creveld syndrome. Note that the phalanges are short but that the proximal phalanges are relatively long compared to the others. Adults, therefore, cannot make a tight fist. Also note the fusion of the fifth and sixth metacarpals.

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