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Robin sequence: obstructive sleep apnea following pharyngeal flap
D L Abramson1, E M Marrinan, J B Mulliken
1Department of Otolaryngology and Communication Disorders, Children's Hospital, Boston, Massachusetts, USA.
Insights
For Robin sequence patients, V-Y palate repair showed low velopharyngeal dysfunction (VPD). However, the von Langenbeck repair led to higher VPD rates and obstructive sleep apnea after pharyngeal flap surgery.
Area of Science:
- Craniofacial surgery
- Pediatric otolaryngology
- Speech pathology
Background:
- Robin sequence (RS) is a congenital condition affecting facial development, often involving a cleft palate.
- Velopharyngeal dysfunction (VPD) is a common complication following cleft palate repair, impacting speech intelligibility.
- Pharyngeal flap surgery is a common intervention for VPD, but carries potential risks.
Purpose of the Study:
- To evaluate the speech outcomes and velopharyngeal function after different cleft palate repair techniques in children with Robin sequence.
- To assess the incidence of obstructive sleep apnea (OSA) following pharyngeal flap surgery in this patient cohort.
Main Methods:
- Retrospective review of 24 children with Robin sequence who underwent cleft palate repair.
- Palatal closure performed between 9 and 14 months using either V-Y repair (n=16) or von Langenbeck repair (n=8).
- Speech and velopharyngeal function assessed in patients aged 5 years or older.
Main Results:
- Only 1 of 16 children (6.3%) undergoing V-Y repair experienced borderline velopharyngeal dysfunction (VPD).
- Six of eight children (75%) who had von Langenbeck repair developed VPD, with four requiring pharyngeal flap surgery.
- In nonsyndromic Robin sequence patients, 6 of 7 (85.7%) who had palatoplasty and pharyngeal flap developed obstructive sleep apnea (OSA), necessitating flap takedown.
Conclusions:
- Conventional pharyngeal flap surgery for VPD in nonsyndromic Robin sequence children is associated with a high incidence of obstructive sleep apnea.
- Alternative management strategies for VPD in these patients should be considered, including modified pharyngeal flaps, palatal lengthening techniques (V-Y or double-opposing Z-plasty), or speech bulbs.
Objective:
We reviewed 24 children with Robin sequence who underwent cleft palate repair.
Method:
All patients were 5 years of age or older at the time of review, allowing for accurate assessment of speech in relation to velopharyngeal function. All infants had palatal closure between 9 and 14 months of age, either V-Y repair (n = 16) or von Langenbeck repair (n = 8).
Results:
Only 1 of 16 children who had V-Y repair had borderline velopharyngeal dysfunction (VPD). For reasons that are unclear, in the von Langenbeck repair group, six of eight children had VPD, and four of six underwent pharyngeal flap. Three additional patients with nonsyndromic Robin sequence had palatoplasty and subsequent pharyngeal flap. Six of the combined total of seven children with nonsyndromic Robin sequence developed obstructive sleep apnea and require flap take-down.
Conclusion:
Since conventional pharyngeal flap for VPD in nonsyndromic Robin sequence children resulted in a high incidence of obstructive sleep apnea, alternative management should be considered: modification of the standard pharyngeal flap, palatal lengthening (V-Y or double-opposing Z-plasty), or construction of a speech bulb.