83 巻 (1990) 7 号 p. 1043-1065
We have treated a congenital disease consisting of ankyloglossia and dislocation of the epiglottis and larynx. These infants developed dyspnea and skin and hair abnormalities. In addition, they had several other symptoms, such as feeding difficulties, crying hard, weak, cold and hypotonic extremities, snoring, slow or too fast development and difficulties with fixing the eye. Although they had these abnormalities, they had been considered to be healthy by their pediatricians.
Ankyloglossia consist of abnormalities of the frenulum and floor of the mouth. We divided states of the frenulum into four categories, 0-3, and that of the floor into three, 1-3. For the frenulum 24% were in category 0, 18% in category 1, 25% in category 2, and 33% in category 3. For the floor of the mouth, category 1 were in 16%, category 2 in 41% and category 3 in 43%. We considered to be ankyloglossia when the frenulum belong category 3 and/or the floor belong category 2 or 3. There were more anomalies of the floor of the mouth than of the frenulum alone.
The epiglottis leaned toward the base of the tongue in 55% and the larynx was elongated and curved toward the upper ventral position in 84%. Operations corrected 100% of the epiglottal and 78% of the laryngeal dislocations. Statistical analysis revealed a close relationship between ankyloglossia and dislocations of the epiglottis and larynx.
Oxygen saturation rate (SAO) were measured by a pulse oximeter while the infants were asleep, awake and feeding. The SAO was below 95 during sleep in 76%, while awake in 45% and during sucking in 53%.
The marmorated skin was in 63%, cyanosis was presented in 21% and in 18% the skin was normal. Piloerection was noted in 47°C. Scant hair in 37% and normal hair in 16%. There was a statistical significant correlation between skin changes and SAO.
After operation on the tongue these symptoms and the low SAO improved dramatically. The symptoms and the signs of this disease were very similar to those of Sudden infant death syndrome (SIDS). We concluded that this anomaly may be one cause of' SIDS.