Adams-Oliver Syndrome With Subcutaneous Scalp Venous Malformation: Three-Year Follow-Up Study. A Case Report

Yu-Tien Tsai ； Lei Wan ； Hui-Ju Lin ； Albert D. Yang ； Ming Chen ； Chao-Jen Lin

《The Changhua Journal of Medicine》 18卷1期 (2020/03) Pp. 18-22

https://doi.org/10.6501/CJM.202003_18(1).0004

摘要

Adams-Oliver syndrome (AOS) is a very rare congenital disorder characterized by a wide range of physical anomalies, including aplasia cutis congenita (ACC) and variable degrees of terminal limb defects. The pathogenesis remains unclear, but intrauterine vascular anomalies may be responsible for anomaly defects. We report the case of a 2-day-old female newborn with AOS, with ACC and subcutaneous scalp venous malformation, Cutis marmorata telangiectatica congenita and atrial septal defect, over 3 years of follow-up. The capillary malformation in the subcutaneous scalp disappeared and the central venous from the parietal to the occipital area became more engorged. AOS is a rare multisystem disorder. It has been suggested that infectious pressure during pregnancy is responsible for intrauterine vascular disruption, resulting in the development of variable structural defects. Three years of clinical observation may explain the natural evolution of the vascular malformation of the scalp during infancy in this case.

關鍵字

Adams-Oliver syndrome ； aplasia cutis congenita ； cutis marmorata ； telangiectatica congenita