THE PIERRE-ROBIN-SEQUENCE - REVIEW OF 125 CASES AND EVOLUTION OF TREATMENT MODALITIES

All children admitted to our hospital between 1964 and 1991 with a dia gnosis of Pierre Robin sequence were divided into three groups accordi ng to the severity of their symptoms: group I: adequate respiration in prone position and bottle feeding; group II: adequate respiration in prone position but feeding difficulties requiring gavage; and group II I: children with respiratory distress and endotracheal intubation and gavage. The presence of associated anomalies, prematurity, and psychom otor impairment was noted as well as the surgical interventions perfor med. We found 56 children (44.8 percent) in group I, 40 children (32 p ercent) in group II, and 29 children (23.2 percent) in group III. Seve nteen children (13.6 percent) died: 1 of 56 in group 1, 4 of 40 in gro up II, and 12 of 29 in group III. Among the 125 patients, 57 presented at least one associated anomaly other than a cleft palate and the Pie rre Robin triad. Thirteen deaths were found in this group (13 of 57 = 22.8 percent). Ten children were premature (10 of 125), and 6 of the p remature infants died (60 percent). Twenty-two children required at le ast one surgical procedure to relieve the upper airway obstruction. Am ong the 108 survivors in this study, 25 presented a psychomotor impair ment (23.1 percent). The children admitted after 1986 were submitted t o routine serial blood gases, oxygen saturation monitoring, and polyso mnographic recordings. The therapeutic interventions were done earlier . Thirty-four children were followed after 1986: 14 in group I, II in group II, and 9 in group III. Only one death occurred (2.9 percent). I n conclusion, the Pierre Robin sequence has a mortality rate increasin g with the severity of the symptoms, the associated anomalies, and the prematurity. When all factors are taken into account simultaneously ( logistic regression), only the severity of the symptoms, and therefore the group, affects the mortality rate significantly. The incidence of psychomotor impairment is high. The use of a standardized investigati on and treatment for these children may improve the prognosis.