Transverse Facial Cleft

Limb Reduction Defects

Figure 5.80. Another example of congenital amputation of the left leg and the toes of the right foot by amniotic bands. In primary limb reduction defects, the skin at the amputation is smooth and there is underlying subcutaneous tissue. In secondary limb reduction deformities, the skin shows ulceration or scarring and has no underlying subcutaneous tissue. Radiography shows the stump of the bone is smooth in primary limb reduction defects and the stump of the bone is jagged in secondary defects.

Figure 5.80. Another example of congenital amputation of the left leg and the toes of the right foot by amniotic bands. In primary limb reduction defects, the skin at the amputation is smooth and there is underlying subcutaneous tissue. In secondary limb reduction deformities, the skin shows ulceration or scarring and has no underlying subcutaneous tissue. Radiography shows the stump of the bone is smooth in primary limb reduction defects and the stump of the bone is jagged in secondary defects.

Figure 5.81. A radiograph of the same infant shown in Figure 5.80 showing the congenital amputation of the left leg at the site of the amniotic band.

Figure 5.82. Congenital amputation of the right hand. This is a primary limb reduction defect.

Figure 5.83. This infant presented with multiple anomalies including a bilateral cleft lip and palate, partial amputation of the right arm, and a congenital scalp defect.

Figure 5.82. Congenital amputation of the right hand. This is a primary limb reduction defect.

Congenital Constriction Bands

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Figure 5.84. In the same infant shown in Figure 5.83, note the amniotic band constriction down to the bone almost resulting in amputation of the right arm.

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Bizarre Facial Anomalies

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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.

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Transverse Facial CleftTransverse Facial Cleft

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.

Encephalocele
Transverse Facial Cleft
Figure 5.88. This infant with TEARS has bizarre facial clefting, a central nervous system defect, an abdominal wall defect, and limb defects.
Bizarre Insertion

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.

Omphalocele

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