PMC4344985_01
PMC4344985_02
Unilateral hyperpigmention of the trunk, back and axilla.
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Hyperpigmented skin lesions exclusively on the left side of the body of variable sizes, extending over the nape of the neck (Figure 1), the left axilla with verrucal changes (Figure 2) and downward to the trunk and abdomen till the level of the umbilicus, anteriorly and posteriorly.
Congenital Cutis aplasia.
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Hyperpigmented skin lesions exclusively on the left side of the body of variable sizes, extending over the nape of the neck (Figure 1), the left axilla with verrucal changes (Figure 2) and downward to the trunk and abdomen till the level of the umbilicus, anteriorly and posteriorly.
right The limbal dermoid seen encroaching onto the cornea, left - at birth (provided by the parents).
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These were clinically diagnosed as Aplasia Cutis Congenita (ACC), although no biopsy was taken (Figure 3).
CT angiogram showing long segment coarctation of descending aorta above the origin of superior mesenteric artery (as indicated).
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Right eye examination showed congenital right-sided coloboma of the eyelid, strabismus with amblyopia and dermolipoma with a limbal dermoid at the superolateral limbus, extending over the cornea covering part of the visual axis (Figure 4).
MRI Brain (right) axial tbl2WI showing bright right temporal arachnoid cyst (arrow head), and (left) tbl1WI showing absent right and normal left lacrimal gland (arrow head).
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of the abdomen revealed a 3.5 cm long segment descending and pre-renal aortic coarctation, which was 6 mm in largest diameter (Figure 5). There was neither intra-orbital extension of the dermolipoma nor any intracranial lipomas; however, absence of the right lacrimal gland was noted (Figure 5).
MRI spine tbl1WI sagittal (left) and axial (right) showing bright intraspinal posterior intradural lipoma impressing the cord (arrows).
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revealed a right temporal pole extra-axial arachnoid cyst causing mass effect on the right temporal lobe (Figure 6). MRI of the spine revealed posterior intra-spinal and intra-dural lipomas extending along the cervico-dorso-lumbar levels the largest at the cervico-dorsal region, indenting the dorsal spinal cord (Figure 6).