Median Cleft Upper Lip Cutaneous Polyps

Overview

Type of disease: Rare conditions

Also called Pai syndrome (PS), median cleft upper lip – cutaneous polyps is a combination of symptoms, or a syndrome, that is present at birth. Median cleft lip is the incomplete formation of the lip, creating a gap in the middle of the upper lip. Cutaneous polyps are abnormal growths on the skin, in this case in the nose and on the face. Many children born with the syndrome have widely spaced eyes and in extreme cases a split nose. Intellectual development is almost always normal. The cause is unknown, and the incidence of PS is likely underestimated. Children with PS should be monitored for infections of the lungs and speech impediments. Eyesight and pressure within the eye should be monitored over time. If eyesight does diminish, it can usually be fixed by surgery. If risk of lung or mouth infection is detected at birth, immediate surgery is necessary. Most people born with PS live to adulthood.

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