Vacterl syndrome: Medical and stomatological considerations for comprehensive patient management

Author:

Do Amaral Cristhiane Olívia Ferreira1ORCID,Souza Ana Flávia Ferreira Barros De2,Carlucci Guilherme Peratelli2,Lima Leticia Da Silva2,Amaral Mariana Olívia Ferreira Do3,Straioto Fabiana Gouveia4

Affiliation:

1. Faculty of Dentistry University of Oeste Paulista ‐ Unoeste São Paulo Brazil

2. Dentistry ‐ University of Western Sao Paulo ‐ Unoeste São Paulo Brazil

3. Medical School University of West São Paulo São Paulo Brazil

4. Department of Prosthodontics and Geriatic Dentistry, Dental School University of Western Sao Paulo São Paulo Brazil

Abstract

AbstractAimThe VACTERL association or Syndrome consists of Vertebral, Anorectal, Cardiac, Tracheo–Esophageal, Renal, and Limb defects. The diagnosis relies on the presence of at least three of these structural abnormalities. This study reports a single case of a patient with VACTERL Syndrome (VS), aiming to assist dentists in recognizing the general aspects, systemic changes, and oral care related to this condition.Case reportA 14‐year‐old female patient medically diagnosed with VS. The study evaluated the presence of systemic alterations, medication use, behavioral deviations, cognitive development, and oral aspects. The patient exhibited cardiovascular alterations including a ventricular septal defect (C), anal atresia (A), polydactyly (considered limb alteration—L), and scoliosis (a possible indication of vertebral anomalies—V). In the intraoral examination, findings included tooth crowding, enamel hypomineralization in several teeth, a deep and atretic palate, generalized gingivitis, bleeding, gingival hyperplasia, Class III malocclusion, and a right unilateral crossbite.ConclusionPatients diagnosed with VACTERL syndrome experience significant systemic impairments. The research subject presented compromised oral health, challenges in dental management, delays in neuropsychomotor development, though these are not inherent to the syndrome. It is crucial to prevent oral diseases and provide early dental care for these patients to avoid the need for complex and invasive dental treatments due to systemic impairments.

Publisher

Wiley

Reference24 articles.

1. First Trimester Diagnosis of VACTERL Association

2. VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of the literature

3. Esophageal atresia in newborns: a wide spectrum from isolated forms to a full VACTERL phenotype?;La Placa SL;Ital J Pediatr,2013

4. Ventricular septal defect closure in a patient with VACTERL syndrome;Hatemi AC;Tex Heart Inst J,2008

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