Acquired pyloric obstruction in a patient with epidermolysis bullosa letalis

Abstract

The quantity of amniotic fluid was normal, being assured by the healthy co-twin who shared the same sack. This allowed natural fetal growth and may have prevented the te t ra& The tetrad characteristics have been reported absent in newborn infants with kidney agenesis and an alternative source of amniotic fluid, such as esophageal a tresia] myelomeningocele, iniencephaly, 3 or sirenomelia? Another demonstration of the importance of oligohydramhies has been seen in pregnancies with chronic leakage of amniotic fluid, 5 and in experimental amniocentesis in rats. 6 In these cases some tetrad features are present, although kidney agenesis is absent. Some authors disagree with Thomas and Smith and claim that the syndrome could derive from polygenic or autosomal-recessive inher i tanceY Because of the rarity of these conditions, they are difficult to demonstrate. Our case is a singular experimental model of nature, which tends to confirm Thomas and Smith's hypothesis on the basic role of oligohydramnios in causing the nonrenal features of Potter syndrome.