Airway Compromise Following Cleft Palate Repair in Robin Sequence

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.

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Respiratory Distress following Cleft Palate Repair in Children with Robin Sequence

The Cleft Palate-Craniofacial Journal ◽

10.1597/14-250 ◽

2016 ◽

Vol 53 (2) ◽

pp. 203-209 ◽

Cited By ~ 4

Author(s):

Manouk J.S. Van Lieshout ◽

Ilje E. Voshol ◽

Koen F.M. Joosten ◽

Marc P. Van Der Schroeff ◽

Irene M.J. Mathijssen ◽

...

Keyword(s):

Respiratory Distress ◽

Cleft Palate ◽

Robin Sequence ◽

Cleft Palate Repair ◽

Palate Repair

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Clinical Outcomes of the Furlow Z-Plasty for Primary Cleft Palate Repair

The Cleft Palate-Craniofacial Journal ◽

10.1597/07-063.1 ◽

2008 ◽

Vol 45 (5) ◽

pp. 501-510 ◽

Cited By ~ 42

Author(s):

Rohit K. Khosla ◽

Kelly Mabry ◽

Charles L. Castiglione

Keyword(s):

Cleft Palate ◽

Clinical Outcomes ◽

Velopharyngeal Insufficiency ◽

Fistula Formation ◽

Pierre Robin Sequence ◽

Robin Sequence ◽

Surgeon Experience ◽

Cleft Palate Repair ◽

Palate Repair ◽

Pierre Robin

Objective: To review the clinical outcomes following the Furlow Z-plasty for primary cleft palate repair. The primary objective was to determine if the presence of an associated sequence or syndrome (i.e., Pierre Robin sequence), age at palate repair, cleft type, or surgeon experience influenced speech outcomes after a Furlow Z-plasty. Design: The outcomes of 140 patients who underwent palate repair were analyzed retrospectively. Speech evaluations were performed to score the severity of hypernasality, nasal escape, articulation errors, and velopharyngeal insufficiency. Results: The mean age at latest evaluation was 4 years 9 months (age range 2 years old to 12 years old and 4 months). Of the 140 patients, 83% had no evidence of hypernasality, 91% had no presence of nasal escape, and 69% had no articulation errors. Overall, 84% of patients had no evidence of velopharyngeal insufficiency. Secondary posterior pharyngeal flap to correct velopharyngeal insufficiency was required in only 2.1% of patients. The formation of an oronasal fistula occurred in only 3.6% of patients. Nonsyndromic patients with Pierre Robin sequence and syndromic patients did just as well as purely nonsyndromic patients in terms of velopharyngeal insufficiency, hypernasality, and nasal escape. Syndromic patients were more likely to make mild-to-moderate articulation errors. In addition, age at palate repair, cleft type, and surgeon experience had no statistically significant effect on speech results. Conclusions: The Furlow Z-plasty yielded excellent speech results in our patient population with minimal and acceptable rates of fistula formation, velopharyngeal insufficiency, and the need for additional corrective surgery.

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