APLASIA-CUTIS-CONGENITA WITH ASSOCIATED EPIDERMOLYSIS-BULLOSA AND GASTRIC OUTFLOW OBSTRUCTION - A REPORT OF 3 SEVERE CASES WITH A LETHAL OUTCOME

Objective: To record three cases with an association between aplasia c utis congenita, epidermolysis bullosa and gastric outflow obstruction. Design: Case reports. Setting: The neonatal department at the North W est Armed Forces Hospital, Tabuk, Saudi Arabia. Results: Three neonate s with aplasia cutis congenita associated with epidermis bullosa and g astric outflow obstruction presented. A lethal outcome was recorded in all three infants in the neonatal period. Skin denudation ranged from 45 to 63%. Gastric outflow obstruction was diagnosed in all cases wit hin 72 hours of birth. With two of the indexed patients surgery was de ferred to allow time for total parental nutrition to improve the situa tion, but they succumbed within 4 hours and 10 days respectively. The third infant underwent surgery but died 2 days later due to sepsis. Co nclusion: Infants born with extensive aplasia cutis congenita associat ed with epidermis bullosa and gastric outflow obstruction invariably h ave a fatal outcome. Bart's syndrome is suggested to label this triad of clinical presentations.