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The Pierre Robin sequence: review of 125 cases and evolution of treatment modalities
L Caouette-Laberge1, B Bayet, Y Larocque
1Division of Plastic Surgery, Hôpital Sainte-Justine, University of Montreal, Quebec, Canada.
Insights
Pierre Robin sequence severity impacts outcomes. More severe cases (Group III) had higher mortality rates, highlighting the need for timely interventions in respiratory distress and feeding difficulties.
Area of Science:
- Pediatric Surgery
- Craniofacial Anomalies
- Neonatology
Background:
- Pierre Robin sequence is a congenital condition characterized by micrognathia, glossoptosis, and airway obstruction.
- Severity of Pierre Robin sequence varies, necessitating classification for appropriate management and outcome prediction.
Purpose of the Study:
- To classify patients with Pierre Robin sequence based on symptom severity.
- To analyze the impact of associated anomalies, prematurity, and psychomotor impairment on outcomes.
- To evaluate the effectiveness of early therapeutic interventions in later cohorts.
Main Methods:
- Retrospective cohort study of children diagnosed with Pierre Robin sequence between 1964 and 1991.
- Classification into three groups (I, II, III) based on respiratory and feeding status.
- Data collection on associated anomalies, prematurity, psychomotor status, and surgical interventions.
Main Results:
- Mortality rates increased with severity: 1.8% in Group I, 10% in Group II, and 41.4% in Group III.
- Associated anomalies (22.8% mortality) and prematurity (60% mortality) significantly worsened outcomes.
- 23.1% of survivors exhibited psychomotor impairment.
Conclusions:
- Severity of Pierre Robin sequence is a critical determinant of patient outcomes.
- Early detection and intervention, particularly for respiratory distress and feeding issues, are crucial.
- Management strategies should address associated anomalies and potential psychomotor delays.
Abstract:
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)