Clinical experience with the Pierre Robin sequence

The Pierre Robin sequence manifests itself in the neonatal period with symptoms of respiratory distress and feeding difficulties. We report our experience in the clinical management of this entity over 14 years and present an appraisal of risk factors for the surgical treatment of the cleft lip/palate and late outcome. Between 1986 and 1999, out of a series of 159 consecutive patients operated in our department for cleft palate closure, 30 had Pierre Robin sequence. Conservative pediatric management included positioning and cardiorespiratory monitoring. In the case of failure of the conservative treatment, endotracheal intubation and/or different surgical procedures were used. The cleft palate was closed according to Maleks technique. Associated malformations were found in 10 cases (33%) with cardiorespiratory immaturity in five of those patients. Acute respiratory distress was present in 12 newborns (40%). Among them, endotracheal intubation was necessary in seven cases and maintained for 1 to 4 days in three patients. Glossopexy or subperiosteal release of the floor of mouth musculature was done in four of these patients followed by tracheotomy in two. Nasogastric feeding was needed in 14 infants (47%) for a variable period with a mean of 57 days (range 1–210 days). Polysomnography studies, done in 24 patients, showed significant obstructive apnea episodes in 10 infants. Gastroesophageal reflux was found in 11 patients (37%). Hearing loss was found in 29 infants (96%) with a mean threshold of 40 dB. Sensorineural hearing abnormalities were revealed in 10 patients (33%). Palate repair was done at the average age of 5 months (range 3–12 months). Difficulties of intubation were encountered in all the cases with severe malformations (five infants). Myringotomy and/or ventilation tubes were used in 21 infants at the same time of palate closure. An early palatal plate was used before surgery in 18 cases (60%). Immediate postoperative complications included two local hemorrhages and in one of them a surgical exploration was needed to control the bleeding. Postoperative oronasal fistula occurred in eight patients (23%) and further surgery was needed for five of them (16%). Newborns who had associated malformation or severe respiratory distress presented more postoperative complications than those with minimal clinical problems (6/10 vs 2/20 patients respectively, p=0.007).