Author(s): Wohlgemuth, Walter A.
Author(s): Wohlgemuth, Walter A.
Infant with extensive, partly homogeneous, partly more reticular capillary malformation on all four extremities and on both sides of the trunk which was visible immediately at birth. Other conspicuous findings are only mild hemihyperplasia of the right leg, which is slightly larger than the left leg.
The same child at the age of 1.5 years. The extent of the capillary malformation is still identical in all four body quadrants. It has become slightly paler in the trunk region but is still clearly visible. The mild hemihyperplasia of the right leg, which is slightly larger than the left leg, has grown in exact proportion to the child.
The combination of extensive capillary malformation in all four quadrants of the body, often together with cutis marmorata telangiectatica congenita (CMTC), and mild congenital hemihyperplasia that is not progressive is strongly suggestive of DCMO syndrome. The prognosis in this case is very good.
Another example of an infant with extensive, partly homogeneous, partly more reticular capillary malformation on all extremities and on both sides of the trunk that is visible immediately at birth. In this case, however, it is clearly more pronounced on the left side. This case also presents mild hemihyperplasia, but this time affecting the left half of the body. Syndactyly of the 2nd and 3rd toes on both sides (not shown here).
The child is 3 years old in this picture. The capillary malformation has already faded well in all 4 body quadrants, the reticular pattern is hardly visible.
Furthermore the mild hemihyperplasia of the left half of the body is only discreetlysubtly visible.
The same child at the age of 3 years. The capillary malformation, patchy and extensive on both legs, is barely visible now. Syndactyly of the 2nd and 3rd toes on both sides.
The same child, aged 3 years. The capillary malformation is also much paler on the upper extremities. Rather reticular pattern in the sense of cutis marmorata telangiectatica congenita (CMTC) still visible on the right dorsum of the hand.
The left hand is slightly larger and more voluminous than the right hand (hemihyperplasia). All in all, a classic picture of a DCMO, thus a very good prognosis overall.
Two patient examples of diffuse capillary malformation with overgrowth (DCMO syndrome). An extensive capillary malformation, on all extremities and on the trunk on both sides, but often more pronounced on one side, is already conspicuous at birth. In addition, the capillary malformation often shows reticular components, similar to a cutis marmorata telangiectatica congenita (CMTC). These net-like skin changes become paler within a few years and quickly fade into the background.
Hemihyperplasia is also present at birth, which, in contrast to other syndromes with circumscribed overgrowth, is mild and grows only proportionally to the patient. Concomitant symptoms such as syndactyly or “sandal gap” are common. Children should have regular abdominal ultrasound until the age of 7 to exclude neoplasms of the upper abdomen. Otherwise, the prognosis is very good.
Published: 2020
All images © Wohlgemuth