Prognosis of airway obstruction and feeding difficulty in the Robin sequence

Mark C. Smith, Craig W Senders

Research output: Contribution to journalArticle

65 Citations (Scopus)

Abstract

Objective: To evaluate the course and prognosis of airway obstruction and feeding difficulty in the Pierre Robin sequence (PRS). Methods: Retrospective review of 60 patients with PRS between 1993 and 2002 at the University of California, Davis Medical Center. Patients were placed into diagnostic subgroups: (1) Isolated PRS; (2) Syndromic PRS (known syndrome with PRS); (3) Unique PRS (unique anomalies with PRS). Data regarding severity, duration, and management of airway obstruction and feeding difficulty were collected. Results: Airway obstruction requiring intervention beyond positional therapy was seen in 28% isolated, 42% syndromic, and 58% unique PRS. One-third of patients who failed positional therapy were temporarily stabilized with a nasopharyngeal airway or endotracheal intubation. The remaining two-thirds of patients, who failed positional therapy required a surgical airway procedure. Four patients underwent mandibular distraction osteogenesis, resulting in successful decannulation or avoidance of tracheostomy. Thirteen patients underwent tracheostomy; mean duration of tracheostomy-dependence was 17.0 months in Isolated PRS and 31.7 months in Unique PRS (p < 0.01). Successful decannulation by age of 3 years was confirmed in 85% of patients who underwent tracheostomy. Tube feeding was required in 53% Isolated, 67% Syndromic, and 83% Unique PRS. Forty-two percent of PRS patients with a successful positional airway still demonstrated feeding difficulty. Short-term (0-3 months) and intermediate (4-18 months) tube feeding was more commonly required in Isolated and Syndromic PRS, while long-term (beyond 18 months) gastrostomy tube feeding was more commonly required in Unique PRS (p < 0.01). By 3 years of age, a successful oral diet was seen in 91% Isolated, 92% Syndromic, and 78% Unique PRS. Conclusions: Diagnostic subgroups based on the presence of additional anomalies help families and physicians in understanding the severity and duration of feeding and airway difficulty in PRS. Two-thirds of PRS patients who fail positional therapy may ultimately require a surgical airway procedure. Feeding difficulty can be present in the absence of clinically significant airway obstruction. Families and physicians should be encouraged that by 3 years of age, most patients were successfully taking an oral diet without airway obstruction.

Original languageEnglish (US)
Pages (from-to)319-324
Number of pages6
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume70
Issue number2
DOIs
StatePublished - Feb 2006

Fingerprint

Pierre Robin Syndrome
Airway Obstruction
Tracheostomy
Enteral Nutrition
Family Physicians
Diet
Distraction Osteogenesis

Keywords

  • Feeding difficulty
  • Mandibular distraction osteogenesis
  • Pierre Robin sequence
  • Upper airway obstruction

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine
  • Surgery

Cite this

Prognosis of airway obstruction and feeding difficulty in the Robin sequence. / Smith, Mark C.; Senders, Craig W.

In: International Journal of Pediatric Otorhinolaryngology, Vol. 70, No. 2, 02.2006, p. 319-324.

Research output: Contribution to journalArticle

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abstract = "Objective: To evaluate the course and prognosis of airway obstruction and feeding difficulty in the Pierre Robin sequence (PRS). Methods: Retrospective review of 60 patients with PRS between 1993 and 2002 at the University of California, Davis Medical Center. Patients were placed into diagnostic subgroups: (1) Isolated PRS; (2) Syndromic PRS (known syndrome with PRS); (3) Unique PRS (unique anomalies with PRS). Data regarding severity, duration, and management of airway obstruction and feeding difficulty were collected. Results: Airway obstruction requiring intervention beyond positional therapy was seen in 28{\%} isolated, 42{\%} syndromic, and 58{\%} unique PRS. One-third of patients who failed positional therapy were temporarily stabilized with a nasopharyngeal airway or endotracheal intubation. The remaining two-thirds of patients, who failed positional therapy required a surgical airway procedure. Four patients underwent mandibular distraction osteogenesis, resulting in successful decannulation or avoidance of tracheostomy. Thirteen patients underwent tracheostomy; mean duration of tracheostomy-dependence was 17.0 months in Isolated PRS and 31.7 months in Unique PRS (p < 0.01). Successful decannulation by age of 3 years was confirmed in 85{\%} of patients who underwent tracheostomy. Tube feeding was required in 53{\%} Isolated, 67{\%} Syndromic, and 83{\%} Unique PRS. Forty-two percent of PRS patients with a successful positional airway still demonstrated feeding difficulty. Short-term (0-3 months) and intermediate (4-18 months) tube feeding was more commonly required in Isolated and Syndromic PRS, while long-term (beyond 18 months) gastrostomy tube feeding was more commonly required in Unique PRS (p < 0.01). By 3 years of age, a successful oral diet was seen in 91{\%} Isolated, 92{\%} Syndromic, and 78{\%} Unique PRS. Conclusions: Diagnostic subgroups based on the presence of additional anomalies help families and physicians in understanding the severity and duration of feeding and airway difficulty in PRS. Two-thirds of PRS patients who fail positional therapy may ultimately require a surgical airway procedure. Feeding difficulty can be present in the absence of clinically significant airway obstruction. Families and physicians should be encouraged that by 3 years of age, most patients were successfully taking an oral diet without airway obstruction.",
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