New Classification Scheme of Proboscis Lateralis Based on a Review of 50 Cases

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Background:Among congenital nasal deformities, proboscis lateralis is one of the rarest. Boo-Chai classified proboscis lateralis into four groups. Recently, we encountered a new case of proboscis lateralis with median cleft lip. We noticed that this classification had not been considered according to convalescence and embryologics, and further refinement seemed to be needed.Methods:We reviewed all cases of proboscis lateralis reported in English through 2009 and classified them by intercanthal distance.Results:A total of 34 studies involving 50 cases were reviewed. Six cases were identified as having normal intercanthal distance. Three of them presented nose abnormalities and fit Boo-Chai group II category. The other three were consistent with group I. Hypertelorism was observed in 27 cases and was further divided into two groups based on the occurrence of a frontal encephalocele. Seventeen cases without a frontal encephalocele were compatible with Boo-Chai groups III and IV. The other 10 cases associated with a visible encephalocele had encephalopathy; most died at an early age, and long-term survival cases suffered developmental delay and mental retardation. Seventeen cases were defined as hypotelorism, and all cases also presented as holoprosencephaly.Conclusions:The redefined classification contains two new groups: group V as hypertelorism with encephalocele and group VI as hypotelorism. A new classification scheme is proposed as not only convenient for clinical application but also embryologically accurate.

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