Midline facial hypoplasia and holoprosencephaly: Its classification and surgical indications

Midline facial hypoplasia is usually accompanied with severe malformation of the brain, so that its management and treatment is difficult and the prognosis hardly encouraging. In this regard, DeMeyer has said,'the face predicts the brain', and classified holoprosencephaly into 5 groups that correlate with the severity of the facial anomalies. However, several patients with both midline facial hypoplasia and normal or near-normal brain development have been the subjects of recent reports, and in this paper, the authors discuss 4 cases of midline facial hypoplasia that they have encountered over the past three years for which plastic surgery was performed. DeMeyer's classification could not be applied to 2 of these 4 cases: one case presented a bilateral cleft lip with a very small prolabium, orbital hypotelorism, and a flat nose without a nasal septum but hypothalamic pituitary hypoplasia without holoprosencephaly, and the other case presented a median cleft lip columellar, orbital hypotelorism, a flat nose without a nasal septum and premaxilla but only a minor abnormality of the brain (an abortive holoprosencephaly) and no developmental retardation. Because DeMeyer's classification method was proposed for cases presenting brain anomalies, it thus appears that this classification method does not always suit each case, since anomalies of the face and brain do not always present a parallel relationship. Therefore, this requires the plastic surgeon to decide on the surgical indication after separately considering the severity of the brain anomalies and mental development from the severity of the facial anomalies. The most important consideration should be how best to control the severe functional limitations of such patients for the balance their lives. In contrast, however, patients who show signs of normal mental development and a good prognosis deserve to undergo surgery as soon as possible.