Maw. Wooldridge et al., APLASIA-CUTIS-CONGENITA WITH ASSOCIATED EPIDERMOLYSIS-BULLOSA AND GASTRIC OUTFLOW OBSTRUCTION - A REPORT OF 3 SEVERE CASES WITH A LETHAL OUTCOME, Saudi medical journal, 15(4), 1994, pp. 313-315
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.