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Results in Relation to 22q11 Microdeletion

Isabelle Rouillon, MD; Nicolas Leboulanger, MD; Gilles Roger, MD; Michel Maulet; Sandrine Marlin, MD, PhD; Natalie Loundon, MD; Marie France Portnoï, MD; Francoise Denoyelle, MD, PhD; Erea Noel Garabédian, MD

Arch Otolaryngol Head Neck Surg. 2009;135(7):652-656.

Objective  To evaluate the results of velopharyngoplasty for velopharyngeal insufficiency (VPI) in relation to 22q11 deletion or nonsyndromic VPI.

Design  Retrospective study.

Setting  Academic medical center.

Patients  Eleven of 45 patients with 22q11 microdeletion (group 1) and 9 patients without 22q11 microdeletion (group 2) with noncleft VPI (hypoplastic velum or hypodynamic velopharynx and deep pharynx) underwent velopharyngoplasty (midline pharyngeal flap with superior pedicle). Exclusion criteria included cleft palate, submucous cleft palate, all syndromic cases, and all associated malformations (except those related to 22q11 microdeletion in patients with DiGeorge syndrome).

Main Outcome Measures  Speech assessment before surgery using the Borel-Maisonny scale and at 9 months and 24 months after surgery. Velopharyngeal insufficiency was classified as normal, inconsistent, mild, moderate, and severe.

Results  Before surgery, in group 1, 3 patients had mild and 8 had severe VPI, and in group 2, 1 had mild and 8 had severe VPI. Postoperative outcomes at 9 months showed that in group 1, 2 patients had excellent results (normal and inconsistent) and 9 had mild VPI, while in group 2, 6 patients had excellent results and 3 had mild VPI (P = .03). Postoperative outcomes at 24 months showed that in group 1, 10 patients had excellent results and 1 had mild VPI, while in group 2, 8 patients had excellent results and 1 had mild VPI.

Conclusions  Surgical treatment of noncleft VPI by pharyngoplasty was efficient in 10 of the 11 patients (91%) in the 22q11 group and in 8 of the 9 patients (89%) in the nonsyndromic group. Postoperative remission took longer for patients with the 22q11 microdeletion than for the control group. However, long-term results following surgical treatment were equally good in the 2 groups.

Author Affiliations: Département d’Otorhinolaryngologie et de Chirurgie Cervico-Faciale, Hôpital d'Enfants Armand-Trousseau (Drs Rouillon, Leboulanger, Roger, Loundon, Denoyelle, and Garabédian and Mr Maulet), and Département de Génétique, Hôpital d’Enfants Armand-Trousseau (Drs Marlin and Portnoï), Assistance Publique–Hopitaux de Paris and Université Pierre et Marie Curie, Université Paris 6, Paris, France.

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