Treatment of Cleft Palate Associated with Robin Sequence: Appraisal of Risk Factors

Abstract

Mandibular hypoplasia, airway obstruction, and a typical wide U-shaped cleft palate comprise the Robin sequence. Although much has been written regarding the treatment of these patients in the neonatal period, the literature reveals little information regarding later care of the cleft palate in these patients. The purpose of this study is to examine patients with the Robin sequence and evaluate the risk of postsurgical problems and outcome related to the neonatal period. Thirty-six patients with the Robin sequence presenting from 1972 through 1990 were reviewed. A majority of patients had feeding and respiratory difficulties, to varying degrees, following birth. These problems were treated successfully by maneuvers ranging from positioning to two infants who eventually required tracheostomy. Thirty-four patients had palate repair. Age at repair averaged 16.2 months, and one third of patients had associated anomalies. Infants who experienced problems following palatoplasty were those who had histories of severe difficulties and complications in the early months of life. In addition, patients with associated congenital anomalies had significantly more problems at the time of palate repair than those without anomalies. Those patients with the Robin sequence, who historically had minimal difficulty following birth, experienced few complications at the time of palate repair. Of the 34 patients with repaired palates, 23 demonstrated sufficient follow-up to allow for evaluation of speech outcome. Satisfactory or normal speech production was noted in 65.4%. This is not significantly different from that observed in all patients undergoing cleft palate repair during this same time period (74.9%). Secondary pharyngoplasty procedures were required in 17.4%. An overall complication rate of 29.4% was noted with palatal fistula occurring in 11.8%. Examination of an infant's immediate postnatal period, as well as for the presence of associated anomalies, will provide important predictive information on the potential difficulties following cleft palate repair. In addition, palatoplasty, as part of the overall team approach to the cleft patient, results in a satisfactory speech outcome in approximately two thirds of patients with the Robin sequence.

Subperiosteal Release of the Floor of the Mouth to Correct Airway Obstruction in Pierre Robin Sequence: Review of 31 Cases

“…Two patients underwent emergent reintubation after early accidental extubation without complications and 25 patients underwent palate repair and were intubated without any difficulty at 1 year of age. This contradicts the observation that endotracheal intubation remains difficult in children with PRS at the time of palate repair (Lehman et al, 1995) and suggests that SRFM improves airway access.…”

“…Nevertheless, dehiscence, infection, damage to Whartons' ducts, and scarring of the lip and tongue are known complications. In addition, the glossopexy procedure always requires a second surgery to release the tongue (Lehman et al, 1995; Kirschner et al, 2003; Denny et al, 2004). Some authors believe that glossopexy can improve the airway in the short term but that anomalies persist (Lehman et al, 1995) and necessitate later additional surgery (Denny et al, 2004).…”

Acute airway obstruction in an infant with Pierre Robin syndrome after palatoplasty

“…Patients with Pierre Robin sequence and other identified syndromes have been reported to have an increased risk of developing airway obstruction, respiratory distress, severe feeding difficulties, and even death after palatoplasty (2,3,5). Airway morbidity after Wardill-Kilner or von Langenbeck and recently Furlow repair, but not Perko-revised palatoplasty, has been described (6)(7)(8).…”

“…Post-operative airway complications after palatoplasty have been described in a few previous studies. All the patients had a history of pre-operative airway problems, the presence of a syndrome such as Pierre Robin sequence or other genetic disorders (3).…”

Neonatal Mandibular Distraction Osteogenesis

“…Unfortunately, patients with Robin sequence have a high incidence of obstructive sleep apnea 62 and airway complications after cleft palate repair (22% to 47%). [63][64][65][66] Many centers advocate delaying cleft palate repair in the Robin sequence population until 18 months of age to limit risk to postoperative airway complications. Our unit follows an airway-assessment protocol prior to cleft palate repair in which polysomnography is used to screen all patients with Robin sequence prior to cleft palate repair.…”