Cleft palate in diastrophic dysplasia. Morphology, results of treatment and complications

Forty-one of 95 Finnish patients (43%) with diastrophic dysplasia had open cleft palate (CP). Submucous CP or its microforms were observed in an additional 30 patients (32%). Even though most of the patients have micrognathia, the high frequency of submucous clefts speaks against the role of the interposed tongue in the pathogenesis of CP in the Pierre Robin sequence. There was no hypernasality of speech in 27 of the 30 patients with submucous CP or its microforms, and the spontaneous speech of the other three was good. This is in contrast with observations in isolated submucous CP in general. No operative treatment was indicated in any case. Results after treatment of open CP and speech problems were in good agreement with results from the patients with isolated CP from the same period of time. As newborns 11 of the patients (12%) had severe respiratory difficulties caused by glossoptosis, and a Douglas operation was performed in three patients. Spinal compression due to abnormalities in cervical vertebrae and to dorsiflexion necessitated by intubation and velopharyngeal surgery may be a dangerous operative complication of patients with diastrophic dysplasia.