Adams-Oliver syndrome with widespread CMTC and fatal pulmonary vascular disease

Ophelia Entsir Dadzie, Lidia Tyszczuk, Susan E. Holder, Fernanda Teixeira, Aikaterina Charakida, Julia Scarisbrick, Anthony Chu

Hammersmith Hospital.
United Kingdom

Pediatric Dermatology
Pediatr Dermatol 2007; 24: 651-653
DOI: 10.1111/j.1525-1470.2007.00556.x

Abstract
We report a neonate with cutis marmorata telangiectatica congenita and clinical features of Adams-Oliver syndrome in association with severe pulmonary vascular disease. We provide an overview of cutis marmorata telangiectatica congenita, distinguishing it from cutis marmorata, a common and benign physiologic cutaneous disorder seen in neonates. We highlight the need for thorough medical evaluation in cutis marmorata telangiectatica congenita to exclude associated congenital anomalies.

Category
Stenosis or Obstruction of Normal Pulmonary Venous Connection
Genetic Factors Influencing the Onset, Severity or Outcome of Disease
Diagnostic Testing. Noninvasive
Diagnostic Testing. Invasive

Year of Publication: 2007

Age Focus: Pediatric

Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)

Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No