ARTICLE: Axenfeld-Rieger Syndrome (ARS): A review and case report

Waldron, Jennie M.; McNamara, Clare; Hewson, Antonia R.

doi:10.1111/j.1754-4505.2010.00153.x

Cited by 27 publications

(43 citation statements)

References 17 publications

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“…In the literature, reported cases of ARS often had skeletal Angle Class III malocclusion, relative mandibular prognathism and midface and/or maxillary hypoplasia. 6,7,23,24 Mandibular hypoplasia and Angle Class II malocclusion are rare findings for ARS. Although, mandibular hypoplasia was reported previously, Angle Class II malocclusion was not reported in the literature for this syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…12 A wide variety of ocular manifestations are associated with ARS. 6,10 The ocular defects of ARS include iris hypoplasia, iridocorneal adhesion, corectopia, posterior embriyotoxon, pupil abnormalities, blue sclera, conjunctival xerosis, and other less frequent features such as cataracts, retinal detachment and microcornea. [16][17][18] Glaucoma and visual loss develops in 50-60% of cases during early childhood or early adulthood.…”

Section: Discussionmentioning

confidence: 99%

“…6,7 No gender or racial predilection is indicated. 8 Inheritance is autosomal dominant in 70% of cases, with 30% sporadic.…”

Section: Introductionmentioning

confidence: 99%

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Nadir görülen Axenfeld-Rieger sendromu: olgu bildirimi

Peker¹,

Biçer²,

Bozkaya³

et al. 2014

Acta Odontol Turc

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INTRODUCTION: Axenfeld-Rieger syndrome (ARS) is a rare autosomal dominant disorder. It is characterized mainly by anterior segment abnormalities of the eye, and also comprises non-ocular defects including dental, craniofacial and systemic abnormalities. The purpose of this case report was to present clinic and radiographic findings and treatment approach in a patient with ARS. CASE REPORT: A 21-year-old woman referred to our clinic with complaints of chewing difficulties, esthetic and speech problems. The medical anamnesis revealed several ocular disorders including glaucoma, cataract and strabismus, and the patient had been using eye-glasses since she was six-months-old. The dental history revealed no tooth extraction. In the extraoral examination, hypertelorism, malar hypoplasia, broad nasal bridge, short philtrum and mandibular retrognathia were observed. Intraoral examination disclosed the presence of an anterior open-bite, an Angle Class II malocclusion and four primary and seventeen permanent teeth. Presence of root anomalies (dilaceration, taurodontism, short roots), caries and a partially-erupted mandibular third molar were observed in radiographic examination. Cephalometric measurements suggested the presence of a variety of craniofacial abnormalities and hypoplasia in the maxilla and the mandible. A multidisciplinary operative approach comprising surgical, endodontic, restorative and prosthetic means were implemented for the oral rehabilitation of the patient. CONCLUSION: The case reported here was a rare presentation of ARS with unique craniofacial features including mandibular hypoplasia and Angle Class II malocclusion. The oral rehabilitation of these patients necessitates a multidisciplinary clinical work.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Nadir görülen Axenfeld-Rieger sendromu: olgu bildirimi

Peker¹,

Biçer²,

Bozkaya³

et al. 2014

Acta Odontol Turc

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“…Many reports have described cases of ARS; however, dental treatment is reported in only one patient (Waldron et al, 2010). The Patient 1.…”

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confidence: 96%

Two Cases of Axenfeld-Rieger Syndrome, Report of the Complex Pathology and Treatment

Bender

Elswijk

Prahl

et al. 2014

The Cleft Palate Craniofacial Journal

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The purpose of this case report is to report the orthodontic surgical treatment and subsequent dental rehabilitation in two patients with Axenfeld-Rieger syndrome. Axenfeld-Rieger syndrome is a rare autosomal dominant condition characterized by ocular, dental, craniofacial, and periumbilical abnormalities. The treatment of two patients with various anomalies in Axenfeld-Rieger syndrome is described and discussed. Early recognition of the syndrome and referral during childhood to a specialized oral and maxillofacial surgery, special dental care, and orthodontic unit provide the optimal starting point for this complex treatment.

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“…3 Ocular abnormalities would be significant for Axenfeld-Rieger syndrome in combination with palatal hypoplasia. 4 In hemimaxillofacial dysplasia, isolated palatal involvement is uncommon, whereas auricular, mandibular, infraorbital rim, or zygomatic bone growth disturbances are frequent. 5,6 Regarding the less complex segmental odontomaxillary dysplasia, 45 cases have recently been reported, 7 and many reports on missing maxillary lateral incisors exist in the literature.…”

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confidence: 99%

Orofacial Rehabilitation in Maxillary Aplasia

Landes

Zahn²,

Uhse³

et al. 2013

Journal of Craniofacial Surgery

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Orofacial rehabilitation in maxillary aplasia but the premaxilla requires, beyond addressing the oronasal communication, facial functional-aesthetic and dental prosthetic reconstruction.The report of a now 42-year-old patient, born with maxillary aplasia, presenting for surgical rehabilitation in 2002 (at 33 years old), further elaborates this complex challenge.A staged approach that included velopharyngoplasty was performed on the patient in 2002; alternatively to a preformed fibula, 2 zygomatic fixtures were later inserted bilaterally to the malar bone, and premaxillary teeth were retained. Subapical mandibular alveolar osteotomy was performed to reposition the malaligned alveolar process and teeth. Augmentation from the iliac crest to the left congenitally hypoplastic mandible followed, its alveolar nerve running atypically lingual and outside the bone. The maxilla was rehabilitated by telescoped coverdenture on ceramic primary crowns, the mandible with all ceramic crowns on standard implants after orthodontic tooth-arch harmonization.A most satisfying result was obtained regarding chewing, speaking, and swallowing. The good phonation and pharyngeal control that was shown by the patient after velopharyngoplasty was retained. Visual analog scale showed enhanced patient satisfaction from 4 to 8, and oral health impact profile 14G decreased from 16 to 7 impairment scores. Zygomatic implants and ceramic galvano-telescoped coverdenture were shown to be reliable, eventually for similar cases, combined if necessary with orthodontics, orthognathic surgery, and standard implants.

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ARTICLE: Axenfeld-Rieger Syndrome (ARS): A review and case report

Cited by 27 publications

References 17 publications

Nadir görülen Axenfeld-Rieger sendromu: olgu bildirimi

Nadir görülen Axenfeld-Rieger sendromu: olgu bildirimi

Two Cases of Axenfeld-Rieger Syndrome, Report of the Complex Pathology and Treatment

Orofacial Rehabilitation in Maxillary Aplasia

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