Orthodontic space closure in a young female patient with solitary median maxillary central incisor syndrome.

A solitary median maxillary incisor can occur as a rare single dental anomaly or a symptom of the early-intrauterine developmental brain disorder of holoprosencephaly. The few published case reports about orthodontic treatment for this disorder have only described space opening for prosthodontic replacement of a central incisor. In contrast, the present patient was treated with extraction of the solitary median maxillary central incisor and orthodontic space closure with subsequent minimally invasive restorations in order to avoid looming esthetic or periodontal sequelae associated with any type of fixed bridgework or implant-borne crowns in the sensitive maxillary anterior area-which is especially indicated in young girls with a hyperdivergent growth pattern.

Atypical extraction treatment for failing replanted maxillary and mandibular incisors with space closure of 2 mandibular incisors in the same quadrant.

We report the successful orthodontic treatment of a 13-year-old girl who had been involved in an accident with avulsion of her maxillary right central incisor and both mandibular left incisors. Fifteen months after replantation of the teeth, all showed severe root resorption with apical inflammation and had to be extracted. After compensatory removal of the maxillary left central incisor, all 4 extraction sites were closed within 20 months of active orthodontic treatment to avoid implant-prosthodontic replacement. By sequential extraction of the 2 hopeless replanted equilateral mandibular left incisors and common-sense management of straightwire mechanics, it was possible to move the right central incisor across the mandibular midline and close the large space completely. To our knowledge, no case report about orthodontic closure of 2 adjacent extraction spaces in the same quadrant has been published.

[The Phenotypic Spectrum of Ophthalmic Changes in CEP290 Mutations]. / Das phänotypische Spektrum der Augenveränderungen bei CEP290-Sequenzvariationen.

PURPOSE: Inherited retinal diseases (IRDs) may be caused by variations in genes affecting the connecting cilium of photoreceptor cells and intraflagellar transport, manifested as ciliopathies. CEP290 is frequently mutated in non-syndromic, but also syndromic IRDs. In preparation for clinical treatment trials, detailed phenotypic work-up including longitudinal follow-up is mandatory. METHODS: We performed genotype-phenotype correlations in 30 patients with biallelic mutations in CEP290. The study was approved by the IRB of the medical faculty of the Justus-Liebig University Giessen. The patients received a comprehensive clinical examination, including spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF) recording, and electrophysiology whenever possible. RESULTS: Thirty patients aged 1 month to 84 years (median at first visit 0.6 y) were followed between 5 months and 25.7 years (median 4.8 y). Twenty-three of these patients carried the c.2991+1655A>G mutation (30/60 allele 2, 7 homozygous). The second most frequent mutation was p.K1575* (9/60 alleles). The full-field electroretinogram showed residual response in a few patients only. After progression, electrophysiological responses were below threshold in all patients. Severely reduced visual acuity persisted from birth. Eight patients had quantifiable best corrected visual acuity (BCVA, logMAR 2 - 0.3), in one case up to the age of 84 y. Absent fixation of targets was noted in 15 patients during the first months of life. Ten of these patients did not improve during follow-up past the second year of life. The other patients developed at least light perception (LP, n = 7) or hand movement (HM, n = 3). Better BCVA was not restricted to the c.2991+1655A>G mutation. Fundus photography documented degenerative changes throughout the retina with macular degeneration and circular increased fundus autofluorescence signals (9/30 patients) in the perimacular ring and in the rod ring, and spotty changes in the periphery. SD-OCT (6/30 patients) disclosed reduced photoreceptor layer (OPL to OS) thickness and preserved inner retinal thickness (RNFL to INL). Better BCVA did not correlate to genotype or central photoreceptor layer thickness. CONCLUSION: As reported earlier, CEP290 variations are one of the most frequent causes of IRDs with infancy onset. In our patient cohort of 30 patients, only 33% had no LP, 67% at least LP, and among these 26% logMAR 2 to 0.3. Together with preserved ganglion cell and nerve fibre cell layers, success with gene therapeutic approaches appears possible.

Esthetic evaluation of implants vs canine substitution in patients with congenitally missing maxillary lateral incisors: Are there any new insights?

INTRODUCTION: The aims of the study were to determine how a panel of orthodontists, dentists, and laypersons rated the esthetic appeal of dentitions after orthodontic space closure by canine substitution compared with space opening and replacement of missing maxillary lateral incisors by implant-borne crowns and to compare the outcome with the results of a study in the United States in 2005. METHODS: A series of 9 posttreatment intraoral frontal photographs was presented to 87 orthodontists, 100 general dentists, and 100 laypersons. The photographs represented dentitions with either single-tooth implants or canine substitutions for missing maxillary lateral incisors and dentitions with no missing teeth. Each photograph was rated independently by assigning a number between 1 (best) and 5 (worst) for a series of 7 bipolar adjectives. Two-way analysis of variance and Tukey post hoc tests were performed to assess differences in intragroup and intergroup preferences for the various treatment options. Subsequently, the mean scores were compared with the mean scores in the study from 2005. RESULTS: Highly significant improvements (P <0.0001) in the esthetic outcome for implants were found in all respondent groups when compared with the study from 2005. To date, orthodontists and dentists rank implants and canine substitution as equally pleasing, but laypersons prefer space closure. CONCLUSIONS: Perceptions of dental esthetics can vary between dental professionals and laypersons. Investigating each patient's esthetic expectations is thus important, but in the patient's best interest, esthetic and functional aspects should be carefully weighed during comprehensive treatment planning.