VACTERL SYNDROME: DIFFERENT DENTAL FINDINGS

Öz

VACTERL syndrome is a congenital malformation that effects multisystem. The characteristic symptoms of this syndrome are vertebral anomalies (V), anal atresia (A), cardiac defects (C), tracheoesophageal fistula together with esophageal atresia (TE), radial dysplasia, renal anomalies (R), and limb defects (L). The prevalence of VACTERL syndrome is 1/10,000-1/40,000. Clinical diagnosis can be made with at least three out of the symptoms. Oral manifestation of VACTERL syndrome has not been reported in the literature. Oral findings are the most noticeable point of his syndrome. So, we aimed to draw attention to oral findings in the syndrome in this study. This is the first case report that investigated oral/dental findings which accompany supernumerary teeth and early tooth eruption as well as medical findings.

Anahtar Kelimeler

• Supernumerary teeth
• oral hygiene
• VACTERL syndrome

Kaynakça

1. 1.Quan L, Smith DW. The VATER association. Vertebral defects, anal atresia, T-E fistula with esophageal atresia, radial and renal dysplasia: a spectrum of associated defects. J Pediatr 1973;82:104-107.
2. 2.Temtamy SA, Miller JD. Extending the scope of the VATER association: definition of the VATER syndrome. J Pediatr 1974;85:345-349.
3. 3.Addis M, Meloni C, Congiu R, Santaniello S, Emma F, Zuffardi O, et al. A novel interstitial deletion in Xq25, identified by array-CGH in a patient with Lowe syndrome. Eur J Med Genet 2007;50:79-84.
4. 4.Solomon BD, Baker LA, Bear KA, Cunningham BK, Giampietro PF, Hadigan C, et al. An approach to the identification of anomalies and etiologies in neonates with identified or suspected VACTERL (vertebral defects, anal atresia, tracheo-esophageal fistula with esophageal atresia, cardiac anomalies, renal anomalies, and limb anomalies) association. J Pediatr 2014;164:451-457.
5. 5.Reutter H, Hilger AC, Hildebrandt F, Ludwig M. Underlying genetic factors of the VATER/VACTERL association with special emphasis on the "Renal" phenotype. Pediatr Nephrol 2016;31:2025-2033.
6. 6.Velazquez D, Pereira E, Havranek T. Neonate with VACTERL Association and a Branchial Arch Anomaly without Hydrocephalus. AJP Rep 2016;6:74-76.
7. 7.Chen Y, Liu Z, Chen J, Zuo Y, Liu S, Chen W, et al. The genetic landscape and clinical implications of vertebral anomalies in VACTERL association. J Med Genet 2016;53:431-437.
8. 8.Topper DC, Zallen RD, Kluender RL. A dental and facial anomaly not previously reported with VACTERL association: report of case. ASDC J Dent Child 1990;57:216-219.

9. 9.van den Hondel D, Wijers CH, van Bever Y, de Klein A, Marcelis CL, de Blaauw I, et al. Patients with anorectal malformation and upper limb anomalies: genetic evaluation is warranted. Eur J Pediatr 2016;175:489-497.
10. 10.Franklyn JA, Boelaert K. Thyrotoxicosis. Lancet 2012;379:1155-1166.