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[Aplasia cutis congenita in 4 infants]
S J A Beekmans1, Th J Haumann, W P Vandertop
1Afd. Plastische en Reconstructieve Chirurgie, Vrije Universiteit Medisch Centrum, De Boelelaan 1117, Postbus 7057, 1007 MB Amsterdam.
Insights
Aplasia cutis congenita, a rare congenital skin defect, involves skull skin absence. Skin transplantation effectively treated four infants, demonstrating a viable surgical option for this condition.
Area of Science:
- Dermatology
- Pediatric Surgery
- Genetics
Background:
- Aplasia cutis congenita (ACC) is a rare congenital skin defect characterized by the absence of skin, typically on the scalp.
- Management strategies for ACC lack consensus, often leading to conservative approaches due to unfamiliarity.
Observation:
- Four infants (3 boys, 1 girl) diagnosed with ACC presented with skull skin defects.
- The defects varied in size, measuring a few to approximately 10 centimeters in diameter.
Findings:
- All four patients underwent successful skin transplantation.
- Over a year post-transplantation, all patients exhibited good skin coverage of the skull defect.
- For large ACC defects, early surgical intervention is recommended to prevent complications like hemorrhage and infection.
Implications:
- Skin transplantation, including rotation scalp flaps and split-skin grafts, is an effective treatment for ACC.
- In cases like Adams-Oliver syndrome with abnormal vascularity, split-skin grafting is the preferred method for achieving sufficient skin coverage.
Abstract:
In 4 infants, 3 boys and 1 girl, the congenital absence of skull skin was diagnosed: aplasia cutis congenita, a rare congenital skin defect, which usually presents as a solitary defect over the vertex of the skull measuring a few to approximately 10 centimetres. All four patients were treated with a skin transplantation and more than a year later the skin covered the skull well. There is no consensus concerning the treatment of aplasia cutis congenita. Due to unfamiliarity with this condition, a conservative approach is often adopted. The management strategy indicated should depend on the size of the defect and the child's physical condition. In the case of large defects, an early operative treatment is desirable to prevent serious complications such as haemorrhages and infections. Sufficient skin coverage can be obtained by using rotation scalp flaps, and if necessary additional split-skin grafts. In some patients large rotation flaps are not reliable due to the abnormal vascularity of the skin (Adams-Oliver syndrome). Split-skin grafting is the treatment of choice in these patients.