The Pierre Robin sequence: review of 125 cases and evolution of treatment modalities

Plast Reconstr Surg. 1994 Apr;93(5):934-42.


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)

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Abnormalities, Multiple
  • Airway Obstruction / etiology
  • Airway Obstruction / surgery
  • Airway Obstruction / therapy*
  • Child
  • Enteral Nutrition
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Infant, Premature
  • Intellectual Disability / complications
  • Intubation, Intratracheal
  • Male
  • Pierre Robin Syndrome / classification
  • Pierre Robin Syndrome / mortality
  • Pierre Robin Syndrome / therapy*
  • Regression Analysis
  • Severity of Illness Index
  • Survival Rate
  • Tracheostomy