Treatment of velopharingeal insufficiency with turn over mucoperiosteal palatal flap in a patient with DiGeorge syndrome

Abstract

Introduction. DiGeorge syndrome (velocardiofacial syn-drome) occurs in about 1 in 4,000 people. It is characterized by incomplete gene penetration, due to which there is sig-nificant variability in the clinical picture in different patients. The paper describes the successful application of an invert-ed mucoperiosteal flap of the palate in a nine-year-old girl with DiGeorge syndrome who had pronounced velopha-ryngeal insufficiency (VPI). Case report. The girl was the first-born child from a normal pregnancy and a normal birth with a normal early psychomotor development. During breastfeeding, milk was occasionally returned to the nose, and later deviations from normal speech (incomprehensible and nasal speech) were noticed. At the age of 4, detailed ge-netic testing was performed and a microdeletion of 22q11 chromosome was found. Also, a submucosal cleft palate was established, and magnetic resonance angiography of the head and neck revealed an abnormal position of the left in-ternal carotid artery (ICA) that extended submucosally to the central axis of the posterior wall of the pharynx. Then, the submucosal cleft palate was surgically resolved in other clinical center, but without speech improvement. Pharyn-goplasty was not performed due to the risk of serious post-operative complications. It was explained to the parents that speech recovery will not be satisfactory without surgical treatment of VPI. At the age of 9, the girl was admitted to the Clinic for Plastic and Reconstructive Surgery and Burns of the Military Medical Academy in Belgrade for surgical treatment of VPI. Taking into account the potential risks of certain surgical methods, it was decided to perform intrave-lar veloplasty according to Furlow. Since it was intraopera-tively found that the soft palate is too short and that this procedure cannot provide its sufficient length, the mucoper-iosteal flap was lifted from the palate to the palatal aponeu-roses on the posterior edges of the palatine bones leaving their oral surfaces exposed. The mucoperiosteal flap raised in this way could not also provide the required length of the soft palate only by retroposition. However, its length is 160% of the soft palate axis length, which was enough to turn over the front of the flap towards the pharynx of the soft palate to reach its posterior wall. The raised mucoperi-osteal palatal flap has no muscles, so its motility was achieved by the fact that along the edges of the existing short and mobile palate, the flap was fixed to the existing palate and uvula. This provided the anatomical precondi-tions for speech recovery, shown during the one-year post-operative follow-up of the child. Exposed palatine bones and short palate was covered by mucosal tissue, without cystic formations. Conclusion. The mucoperiosteal palatal flap can be easily, successfully and maximally safely applied in the resolution of VPI in patients with DiGeorge syn-drome where there is an aberrant submucosal position of the ICA. This flap could be a 'flap of choice' for such pa-tients with atopic position of the ISA, too.