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Between 1983 and 1995, 12 children with Pierre Robin sequence were treated with a subperiosteal release of the floor of the mouth (SRFM) to correct severe upper-airway obstruction (group 1: n = 10) and prolonged feeding difficulties (group 2: n = 2). Eight children had isolated Pierre Robin sequence, while four had associated anomalies. The age at surgery was 2 to 13 weeks (mean, 5.2 wk) in group 1, and 8.5 months to 5 years in group 2. Postoperative endotracheal intubation was maintained for 1 to 14 days, and nasopharyngeal intubation for 3 to 13 days. One child with a tracheostomy prior to SRFM was decanulated 11 weeks postoperatively. Postoperative course was uneventful and control polysomnograms (group 1) showed a marked improvement of the obstructive phenomenons and O2 saturation levels. Children were discharged 2 to 6 weeks postoperatively (mean, 4 wk) except for the child with a tracheostomy (19 wk). The only complication was a wound infection treated with systemic antibiotics. Oral feedings were initiated shortly after extubation: six children were discharged with oral feedings only, one child required supplemental gavages for 6 weeks at home, and three children had persistent inadequate oral intake. We have found SRFM to be an effective surgical procedure for the management of infants with Pierre Robin sequence.

Pharyngeal flaps are often used to correct velopharyngeal insufficiency. They produce a permanent partial obstruction of the velopharyngeal space. Respiratory obstruction and obstructive sleep apnea have been reported following this surgery. We undertook a study to find out the incidence of sleep apnea associated with pharyngeal flap surgery. Forty-one children (aged 2 to 22 years) admitted for a pharyngeal flap underwent a polysomnographic recording prior to their surgery. One child with Steinert's disease showed some episodes of obstructive apnea, and the surgery was canceled. Forty children underwent pharyngeal flap surgery, and polysomnography with continuous arterial saturation was repeated following surgery. Postoperative polysomnograms were normal in 26 patients (65 percent) and abnormal in 14 patients (35 percent). Among the 14 abnormal patients, we found 6 with obstructive apneas, 6 with central apneas, and 2 with both central and obstructive apneas. Ten of the 14 abnormal patients were restudied in the following months. Eight children had normal recordings, while 2 had central apneas. The 4 patients who declined a follow-up recording had no clinical symptoms of respiratory difficulty when sleeping. Of the 2 children with abnormal recordings on long-term follow-up, 1 is asymptomatic, while the second has persistent snoring, nocturnal awakening, sweating, and daytime lethargy. A section of his flap has been recommended. Independent analysis of arterial oxygen saturation revealed that the percentage of time with a saturation of less than 90 percent identifies patients with clinically significant apneas. Our data show that significant sleep apneas following pharyngeal flaps may not be as frequent or permanent as previously reported.(ABSTRACT TRUNCATED AT 250 WORDS)

All children admitted to our hospital between 1964 and 1991 with a diagnosis of Pierre Robin sequence were divided into three groups according 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 III: children with respiratory distress and endotracheal intubation and gavage. The presence of associated anomalies, prematurity, and psychomotor impairment was noted as well as the surgical interventions performed. We found 56 children (44.8 percent) in group I, 40 children (32 percent) in group II, and 29 children (23.2 percent) in group III. Seventeen children (13.6 percent) died: 1 of 56 in group I, 4 of 40 in group 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 Pierre 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 premature infants died (60 percent). Twenty-two children required at least one surgical procedure to relieve the upper airway obstruction. Among the 108 survivors in this study, 25 presented a psychomotor impairment (23.1 percent). The children admitted after 1986 were submitted to routine serial blood gases, oxygen saturation monitoring, and polysomnographic recordings. The therapeutic interventions were done earlier. Thirty-four children were followed after 1986: 14 in group I, 11 in group II, and 9 in group III.(ABSTRACT TRUNCATED AT 250 WORDS)

The Pierre Robin anomalad presenting with severe respiratory distress is a difficult situation still associated with a significant mortality rate. A surgical approach based on a new idea is described. The concept is that the musculature of the floor of the mouth is under increased tension and pushes the tongue upward and backward with secondary respiratory obstruction. The surgical approach consists of a subperiosteal release of the musculature of the floor of the mouth through a 2-cm submental incision. So far this procedure has been used in four patients with severe obstruction, and marked improvement has occurred postoperatively in all cases. This surgical technique is simple and is associated with little morbidity. It should lessen the need for tracheostomy, which carries a greater morbidity, especially in newborns. We think that this new surgical approach can significantly improve the outcome of the severe form of Pierre Robin anomalad.

It is possible to have a good velopharyngeal closure at 8 years of age and slowly deteriorate to present with velopharyngeal incompetence (VPI) at adolescence? Authors disagree on this subject so we undertook a study to find the answer in our cleft palate population. One hundred and seventy-three patients born between 1968 and 1972 with a cleft palate or cleft lip and palate were reviewed. The appreciation of their velopharyngeal closure at 8 years of age, as rated in their chart, was classified as good (group I, 125 patients), borderline (group II, 17 patients) or incompetent (group III, 35 patients). All children in group I had a good velopharyngeal closure before the age of 8 years, some never presented any symptoms of incompetency (VPI) while other corrected this VPI before the age of 8. The group II included children presenting inconstant or mild symptoms of VPI. All patients in group III received a pharyngeal flap to correct their VPI and were not included in this follow up study. Groups I and II were recalled and 52 presented for a late clinical evaluation of their speech. Group I: 47 examined, 42 still competent, 5 incompetent: group II: 5 examined, 3 competent and 2 incompetent. It is interesting to note that in group I, all five deteriorations of the V-P competency was seen in patients who had shown some symptoms of VPI in early childhood. They had become competent spontaneously or with speech therapy, before age 8.(ABSTRACT TRUNCATED AT 250 WORDS)