[Teleradiographic analysis of velopharyngeal insufficiency in CATCH 22 association].
The CATCH 22 association (Cardiac defect, Abnormal facies, Thymic hypoplasia, Cleft palate, Hypocalcemia) combines certain signs common to various syndromes (Di-George syndrome, velocardiofacial syndrome, etc.) in combination with a 22q11 chromosomal microdeletion. Velopharyngeal insufficiency can be a complementary sign, or even a presenting sign of this chromosomal anomaly. After clinical examination of these patients, velopharyngeal insufficiency appeared to be due to a deep nasopharynx rather than to a short soft palate. We therefore reviewed 11 patients with teleradiography. We studied the length of the soft palate, the depth of the nasopharynx, the ratio of the depth of the nasopharynx over the length of the soft palate, and we compared our results with those observed in reference populations. The results demonstrate nasopharyngeal disproportion with a normal length of the soft palate and increased depth of the nasopharynx. This anatomical finding therefore encourages us to propose sphincteroplasty rather than velopharyngoplasty.
Houze de L'Aulnoit
S
S
Service de Chirurgie Plastique, Hôpital Roger Salengro, Lille, France.
Martinot
V
V
Breviere
G M
GM
Kulik
J F
JF
Pellerin
P
P
D000015:Abnormalities, Multiple; D000293:Adolescent; D000367:Age Factors; D002508:Cephalometry / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D002892:Chromosomes, Human, Pair 22; D002972:Cleft Palate / Q000150:complications / Q000235:genetics*; D005145:Face / Q000002:abnormalities*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000235:genetics*; D006801:Humans; D006996:Hypocalcemia / Q000150:complications*; D008297:Male; D011859:Radiography; D012886:Skull / Q000000981:diagnostic imaging*; D013950:Thymus Gland / Q000002:abnormalities*; D014681:Velopharyngeal Insufficiency / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*