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PYLORIC ATRESIA

PYLORIC ATRESIA

In 2 sibships, one with related parents, Bar-Maor et al. (1972) reported 5 cases of pyloric atresia. Others (e.g., Bronsther et al., 1971) have reported familial...

In 2 sibships, one with related parents, Bar-Maor et al. (1972) reported 5 cases of pyloric atresia. Others (e.g., Bronsther et al., 1971) have reported familial cases. The pylorus is reduced to a fibrous band or is obstructed by a diaphragm. Either may occur in the same family. Congenital pyloric atresia was observed by Tan and Murugasu (1973) in sibs, a male and a female infant. Both showed a thick membrane completely obstructing the pylorus. The parents, of German and English extraction, were nonconsanguineous and had a third normal child. Kadowaki et al. (1981) also reported familial occurrence.

Peled et al. (1992) observed the disorder in 2 sibs born to a Jewish-Iraqi couple related as first cousins. The first pregnancy terminated at 34 weeks of gestation due to preterm labor. The male neonate suffered from pyloric atresia and underwent emergency laparotomy in which gastroduodenostomy was performed. In a later pregnancy the uterus was found to be very distended and tender at 33 weeks of gestation. Ultrasound revealed severe polyhydramnios and a dilated stomach. To reduce the amount of amniotic fluid, amniocentesis was performed. Uterine contractions were arrested medically. During week 37 of gestation, active labor began and the medication (ritodrine) was stopped and a female infant was delivered. An operation for correction of pyloric atresia was performed on the second day of life.

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