Aim: The 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 Report: A 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.
Conclusion: Patients 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.
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http://dx.doi.org/10.1111/scd.13006 | DOI Listing |
Childs Nerv Syst
December 2024
NJ Craniofacial Center, Morristown, NJ, 07960, USA.
Background: Goldenhar syndrome is a clinically heterogeneous disorder defined by a rare combination of congenital anomalies-an eye abnormality, in addition to two of the following three: ear anomalies, mandibular malformations, and vertebral defects. Notably, children with Goldenhar syndrome present with a high incidence of cervical spine malformations.
Clinical Case: In this report, we present an unusual case of a 15-year-old child with Goldenhar syndrome, who additionally presents with some clinical features of VACTERL syndrome.
Isr Med Assoc J
December 2024
Department of Neonatal Intensive Care, Schneider Children's Medical Center, Petah Tikva, Israel, Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
J Pediatr Adolesc Gynecol
November 2024
Medical Faculty of Ivane Javakhishvili Tbilisi State University, Tbilisi, Georgia.
Introduction: The co-occurrence of a unicornuate uterus with a noncommunicating, functioning rudimentary horn and VACTERL association represents an extremely rare condition, with only 3 similar cases reported in the literature.
Case: The patient, aged 12 years and 9 months, presented with pelvic pain and severe dysmenorrhea, which started shortly after her menarche at 12 years and 4 months. At birth, she exhibited 3 characteristic components of VACTERL association: bronchoesophageal fistula/esophageal atresia, anal atresia, and polydactyly.
Orthod Craniofac Res
November 2024
Department of Paediatric Respiratory and Sleep Medicine, Queensland Children's Hospital, South Brisbane, Australia.
Cureus
September 2024
Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, IND.
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