1Faculty Of Dentistry, Department of Pediatrıc Dentistry, Necmettin Erbakan University
Background: Adams-Oliver Syndrome has been described by Adams and Oliver in 1945. Original definition, along with aplasia cutis congenital syndrome and limb defects, has neurological and cardiological problems. In the first description, genetic defect passes variable autosomal dominant pattern. Afterwards the autosomal recessive and sporadic cases were published.
Case Report: 11-year old male patient complained of mobile teeth admitted to our clinic. He was noted to have characteristic view with distal-phalanx and nail hypoplasia of his hand and feet with occipital scalp defect. We consulted to genetics because of these findings and learned that he has the Adams-Oliver Syndrome. The patient has some orofacial manifestations including high-narrow palate, fissured tongue, crowding, dysmorphic facial features, facial asymmetry, deep-philtrum, delayed eruption, class III malocclusion. The extraction of mobile deciduous teeth, restoration of caries and also oral hygiene motivation was made. Then, the patient was referred to orthodontics.
Conclusions: Adams-Oliver syndrome represents are rare congenital alteration, insufficiently documented in scientific literature. This shows the need to document news cases.
- Adams FH, Oliver CP. Hereditary deformities in man due to arrested development. J Hered, 1945; 36:3–7.[Crossref]
- Lin AE, Westgate MN, Van der Velde ME, Lacro RV, Holmes LB. Adams-Oliver syndrome associated with cardiovascular malformations. Clin Dysmorphol, 1998; 7:235–241.[Crossref]
- Digilio MC, Marino B, Dallapiccola B. Autosomal dominant inheritance of aplasia cutis congenita and congenital heart defect: a possible link to the Adams-Oliver syndrome. Am J Med Genet A, 2008; 146A:2842–2844.[Web of Science]
- Snape KM, Ruddy D, Zenker M, Wuyts W, Whiteford M, Johnson D et al. The spectra of clinical phenotypes in aplasia cutis congenita and terminal transverse limb defects. Am J Med Genet, 2009; 149A:1860–1881.[Web of Science]
- Keymolen K, De Smet L, Bracke P, Fryns JP. The concurrence of ring constrictions in Adams-Oliver syndrome: additional evidence for vascular disruption as common pathogenetic mechanism. Genet Couns, 1999; 10:295–300.
- Whitley CB, Gorlin RJ. Adams-Oliver syndrome revisited. Am J Med Genet, 1991; 40:319–326.[Crossref]
- Luján JI, Ibarra AG, Méndez FV. Síndrome de Adams-Oliver. Reporte de un caso. Rev Hosp Jua Mex, 2004; 71:124–127.
- Girard M, Amiel J, Fabre M, Pariente D, Lyonnet S, Jacquemin E. Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism? Am J Med Genet A, 2005; 135:186–189.
- Zapata HH, Sletten LJ, Pierpont ME. Congenital cardiac malformations in Adams-Oliver syndrome. Clin Genet, 1995; 47:80–84.
- Roca OJL, Cendán MI, Alonso LF, Ferrero OME, Lantigua CA. Caracterización clínica del labio hendido con fisura palatina o sin ésta en Cuba. Rev Cubana Pediatr, 1998; 70: 43–47.
- Barragán MG, Vidrio GP, Aguirre AH, Nagano AY. Adams-Oliver syndrome. Case report. Rev Odonto Mex, 2011; 15:175–182.
Citation Information: Balkan Journal of Dental Medicine. Volume 21, Issue 1, Pages 60–64, ISSN (Online) 2335-0245, DOI: https://doi.org/10.1515/bjdm-2017-0009, March 2017