To date, no epidemiological data are available, however, the prevalence can be estimated at around 1/50,000–100,000 live births in Western Europe. Am J Surg Pathol. Congenital tufting enteropathy (CTE), also known as epithelial dysplasia , is an autosomal recessive disorder causing severe diarrhoea in the first week of life together with various dysmorphic features including choanal atresia, oesophageal/rectal atresia in some of the affected infants [6, 7, 17]. Small intestine - Olmesartan enteropathy. Congenital tufting enteropathy (CTE), also named intestinal epithelial dysplasia, is a rare, autosomal recessive enteropathy with persistent and life-threatening intractable diarrhea early in life. Clinical features, gene mutations, and treatments of CTE in reported studies. The … Less frequently, superficial punctuated keratitis, anal atresia, cleft lip and palate, dermatological anomalies, and bone malformations could be observed.