Adjunctive orthodontic therapy for prosthetic rehabilitation in a growing child with Axenfeld‐Rieger syndrome: A case report

Abstract

The congenital oligodontia impeding the development of the alveolar process resulting in disproportionate jaw growth has been previously reported. This case report describes the interdisciplinary management of an 11-year-old girl with Axenfeld-Rieger syndrome exhibiting oligodontia and maxillary hypoplasia. Methods and results: An adjunctive orthodontic therapy was performed by taking advantage of bone-anchored maxillary protraction (BAMP) therapy using miniplates and 24-hour traction by intermaxillary Class III elastics. After 6 months of active treatment, the maxilla advanced by approximately 5 mm and upper lip relation improved by 3 mm without any significant changes in vertical relations. All the changes were maintained at 15th-month follow-up. A temporary removable prosthesis was given for immediate esthetics, and the definitive management is discussed. Conclusion:The BAMP therapy can be a befitting alternative in cases exhibiting complex presentation involving skeletal and dentoalveolar components. An appreciable profile improvement without any dentoalveolar side effects can be achieved with BAMP therapy.

Oligodontia in the Clinical Spectrum of Syndromes: A Systematic Review

“…However, many of the included studies in this systematic review did not perform molecular analysis. Only 23 studies [41,43,52,53,56,63,[66][67][68]70,73,74,76,78,79,[87][88][89][90]92,94,98,105] performed genetic analysis and reported the genetic variants associated with the syndromes. These genes can be grouped into two major groups: one with crucial roles at multiple stages of tooth development, also involving skin and sweat glands (AXIN2, CDH1, DSP, EDA, EDARADD, EVC2, FGFR2, LEF1, MSX1, PITX2, and WNT10A), which are involved in the signal pathway essential for ectodermal structure development [121,122]; the other with genes that intermediate cellular function and development (NPHP1, PCNT, PTCH1, IKBKG, SRCAP, and TBCE).…”

Axenfeld–Rieger syndrome: orthopedic and orthodontic management in a pediatric patient: a case report

“…Furthermore, improving the aesthetics of the smile as well as the support of the upper lip and the general appearance helps patients in their relationship life. In fact, delaying aesthetic dental rehabilitation can lead to discomfort in young patients with impaired social life at school age [19].…”

“…There are few reports concerning case presentations of patients with ARS and few papers [19][20][21][22] concerning orthopedic-orthodontic treatment relative to the patients with this syndrome. The aim of this study is to report the case of the orthopedic and orthodontic treatment in a patient affected by ARS and with facial dysmorphism and teeth anomalies associated to ocular anomalies.…”

Two families with Axenfeld-Rieger Syndrome, a case series describing clinical findings, treatment of the dental anomalies and dentofacial deformities and a review of the current clinical findings and genetic knowledge.

“…Relative to the dentofacial deformity, early treatment in childhood or early adolescence, in terms of adaptive growth treatment, eg. Hugo De Clerck type of treatment could possibly benefit the patients (17).…”