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Figure 5.85. The same infant as in Figures 5.83 and 5.84 showing the congenital scalp defect in the occipital area. Note the strand of amnion attached to the defect.

5.87

Figure 5.86. The early amnion rupture spectrum (TEARS) in this infant shows the bizarre findings involving the face and head. There is a skin defect of the scalp with an encephalocele and gross malformation of the face. The ADAM complex is an example of TEARS. The complex includes Amniotic Deformities, Adhesions, and Mutilations. Findings in the ADAM complex include cleft lip, bizarre midfacial clefts, central nervous system abnormalities (hydrocephalus, microcephaly, asymmetric encephalocele), gastrointestinal abnormalities (omphalocele, gastroschisis), and ocular abnormalities (coloboma, anophthalmia, corneal opacity).

Figure 5.86. The early amnion rupture spectrum (TEARS) in this infant shows the bizarre findings involving the face and head. There is a skin defect of the scalp with an encephalocele and gross malformation of the face. The ADAM complex is an example of TEARS. The complex includes Amniotic Deformities, Adhesions, and Mutilations. Findings in the ADAM complex include cleft lip, bizarre midfacial clefts, central nervous system abnormalities (hydrocephalus, microcephaly, asymmetric encephalocele), gastrointestinal abnormalities (omphalocele, gastroschisis), and ocular abnormalities (coloboma, anophthalmia, corneal opacity).

Figure 5.87. This infant is another example of the early amnion rupture spectrum. Note the asymmetric encephalo-cele in addition to the bizarre anomalies of the face and eye. In general, encephaloceles are midline and an asymmetric encephalocele is always suggestive of a disruption.

Figure 5.88. This infant with TEARS has bizarre facial clefting, a central nervous system defect, an abdominal wall defect, and limb defects.

Figure 5.89. A close-up of this infant showing the bizarre facial clefting and a strand of amnion extending from the right eye to the scalp defect. Also note the severe defect of the mouth and palate. In general, bizarre facial clefting (oblique facial clefting) is noted in disruptions whereas lateral or transverse facial clefts occur in infants with syndromes (e.g., Goldenhar's syndrome).

Figure 5.89. A close-up of this infant showing the bizarre facial clefting and a strand of amnion extending from the right eye to the scalp defect. Also note the severe defect of the mouth and palate. In general, bizarre facial clefting (oblique facial clefting) is noted in disruptions whereas lateral or transverse facial clefts occur in infants with syndromes (e.g., Goldenhar's syndrome).

Figure 5.90. The same infant showing the abdominal wall defect (omphalocele) and strands of amnion extending from this to the central nervous system malformation.

Figure 5.90. The same infant showing the abdominal wall defect (omphalocele) and strands of amnion extending from this to the central nervous system malformation.

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