These skin lipomas are benign, and are therefore more a cosmetic problem than a functional problem. The skin lipomas can develop on different parts of the face. The most common place is the nose. Other common places are the forehead, the conjunctivae and the frenulum linguae. The amount of skin lipomas is not related to the severity of the midline clefting.Patients with the Pai syndrome have a normal neuropsychological development. Until today there is no known cause for the Pai syndrome. The large variety in phenotypes make the Pai syndrome difficult to diagnose. Thus the incidence of Pai syndrome seems to be underestimated. Acromelic frontonasal dysplasia (AFND)
Acromelic frontonasal dysplasia is a rare subtype of FND. It has an autosomal dominant inheritance. Acromelic frontonasal dysplasia is associated with central nervous system malformations and limb defects including a clubfoot, an underdeveloped shin-bone, and preaxial polydactyly of the feet. Preaxial polydactyly is a condition in which there are too many toes on the side of the big toe. The phenotype of AFND is severe: a type Ia DeMyer and a Sedano type D. In contrast to the other subtypes of FND, AFND has a relatively high frequency of underlying malformations of the brain. Frontorhiny
Frontorhiny is another subtype of FND. It consists of multiple characteristics.
The other with hypotelorism (a decreased distance between the eyes), this includes holoprosencephaly (failure of development of the forebrain). In addition, a craniofacial cleft can be classified using the Tessier classification. Each of the clefts is numbered from 0 to 14. The 15 different types of clefts are then subdivided into 4 groups, based on their anatomical position in the face: midline clefts, paramedian clefts, orbital clefts and lateral clefts. FND is a midline cleft, classified as Tessier 0/14. Besides this, the additional anomalies seen in FND can be subdivided by region. None of these anomalies are specific for the syndrome of FND, but they do occur more often in patients with FND than in the population. The anomalies that may be present are:

Nasal: mild anomalies to nostrils that are far apart and a broad nasal root, a notch or cleft of the nose and accessory nasal tags. Ocular: narrowed eye slits, almond shaped eyes, epicanthal folds (extra eyelid tissue), epibulbar dermoids (benign tumors of the eye), upper eyelid colombas (full thickness upper eyelid defects), microphthalmos (one or two small eyes), congenital cataract and degeneration of the eye with retinal detachment. Facial: telecanthus (an increased distance between the corners of the eye), a median cleft of the upper lip and/or palatum, and a V-shaped hairline.