A Case of Pai Syndrome

A fourth patient suspected of having Pai syndrome is described. The female baby had a median cleft of the upper lip, a polypoid skin mass in the right nostril, and a conjunctival lipoma in the right eye. A median alveolar cleft was in the oral cavity. The polypoid skin mass was attached to the nasal septum, and a histologic examination disclosed mature adipose tissue with normal skin and no cartilaginous tissue. A computed tomographic scan revealed a lipoma of the corpus callosum. However, the patient was neurologically normal, and her electroencephalogram was also normal. Her growth and development have been within normal limits, and no convulsions have occurred.