Poland Syndrome

Poland Syndrome Pics
Figure 3.140. A close-up of the left hand showing the marked brachysyndactyly.

Figure 3.139. A close-up of the infant in Figure 3.138 showing the hand placed in the defect.

3.141

Poland SyndromeSyndrome Poland

Figure 3.141. In about 15% of cases there is an association of Poland's anomaly with Möbius' syndrome. In Poland's anomaly the hand anomalies are usually unilateral, whereas in Möbius' syndrome they are usually bilateral. This infant with Poland's anomaly and Möbius' syndrome shows the mask-like facies, ptosis of eyelids, drooping of the angles of the mouth, and the absence of the right pectoralis with a hypoplastic right forearm and hand.

Mask Like Facies

Figure 3.142. A close-up of the face of the same infant. Note the mask-like facies, eyelid ptosis, and drooping of the angles of the mouth. These infants have inefficient sucking and swallowing. In Möbius' syndrome there are usually bilateral cranial nerve palsies involving the sixth and seventh cranial nerves. Occasionally other cranial nerves (three, five, nine and twelve) may be involved.

Figure 3.142. A close-up of the face of the same infant. Note the mask-like facies, eyelid ptosis, and drooping of the angles of the mouth. These infants have inefficient sucking and swallowing. In Möbius' syndrome there are usually bilateral cranial nerve palsies involving the sixth and seventh cranial nerves. Occasionally other cranial nerves (three, five, nine and twelve) may be involved.

Figure 3.144. A chest radiograph in Poland's anomaly demonstrates the increased lucency of the right upper chest, especially of soft tissue, due to die absence of the pectoralis muscle. Compare the appearance of the scapulae on both sides. Note that the ribs are normal.

3.144

Microcephalic Newborns
Figure 3.145. The pathologic appearance of the chest in an infant who had congenital absence of the pectoralis muscle.

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