Interface between Robin sequence and ordinary cleft palate
To the Editor:
In 1999, I presented an editorial comment titled “Robin sequences and complexes: causal heterogeneity and pathogenetic/phenotypic variability” [Cohen, 1999]. Table I shows a slightly modified, updated version of my original Table II, in which I defined Robin sequence, using different criteria [Cohen, 1999]. Here, I have added definition V (non-Robin) that straddles the interface between Robin sequence and ordinary cleft palate. By this definition, the cleft palate is V-shaped and the mandible is normal or may be slightly small subjectively but impossible to be certain. By polysomnography, some respiratory compromise is evident. Thus, at the interface, it is not always possible to distinguish between Robin sequence and ordinary cleft palate.
| Definition | Mandibular deficiency | Cleft palatea | Upper airway obstruction |
|---|---|---|---|
| I | + | + (U) | + |
| II | + | + (U or V) | + |
| III | + | + (U) | − |
| IV | + | ± (U or V) | ± |
| V (non-Robin) | −b | +(V) | + |
- * Modified from Cohen [1999].
- a U, U-shaped cleft palate; V, V-shaped cleft palate.
- b Mandible is normal, but may be slightly small subjectively and impossible to be certain.
Respiratory compromise in Robin sequence, Robin complexes, and in some cases of non-Robin cleft palate is variable in degree. In some instances, upper airway obstruction may be delayed (24–51 days) [Wilson et al., 2000]. Polysomnography helps in evaluating such patients, and when not easily performed, continuous overnight pulse oximetry may be used for monitoring. Polysomnography is mandatory for patients with Robin sequence, Robin complexes, or non-Robin cleft palate who fail to thrive [Wilson et al., 2000].
