Dental issues in lacrimo-auriculo-dento-digital syndrome: An autosomal dominant condition with clinical and genetic variability.

BACKGROUND AND OVERVIEW: Lacrimo-auriculo-dento-digital (LADD) syndrome is an autosomal dominant disorder with variable lacrimal and salivary gland hypoplasia and aplasia, auricular anomalies and hearing loss, dental defects and caries, and digital anomalies. CASE DESCRIPTION: The authors present the cases of 2 unrelated children with enamel defects and history of dry mouth leading to recurrent dental caries. The referring diagnoses were Sjögren disease and hypohidrotic ectodermal dysplasia, respectively. The geneticist suspected LADD syndrome, which was confirmed by means of molecular studies showing mutations of 2 genes: fibroblast growth factor receptor 2 and fibroblast growth factor 10, respectively. Similarly affected relatives indicated an autosomal dominant inheritance. These relatives needed multiple dental rehabilitations during childhood and dentures in adulthood. CONCLUSIONS AND PRACTICAL IMPLICATIONS: Dry mouth, multiple caries, enamel defects, and abnormal tooth morphology were the reasons for seeking care from dentists. However, clinical evaluation and diagnostic imaging studies helped identify anomalies of the lacrimal and salivary glands, ears, and digits, indicating involvement of different areas of the body, compatible with LADD syndrome. Accordingly, dentists should consider genetic disorders in patients with multiple anomalies. For instance, oculodentodigital syndrome, oral-facial-digital syndrome, and LADD syndrome (among others) may have dental issues as the major clinical manifestation. Accurate identification of a particular syndrome is now commonplace with the use of genetic testing. When a patient has multiple anomalies suggestive of a syndromic condition, appropriate genetic testing can help verify the clinical diagnosis. Keeping genetics in mind helps earlier identification of other affected family members with diagnostic genetic testing and appropriate treatment; the economic advantage is to shorten the diagnostic odyssey and possibly preserve dentition.

Sodium hypochlorite pulpotomies in primary teeth: a retrospective assessment.

PURPOSE: In 2007, the University of Iowa's advanced training program in pediatric dentistry replaced the traditional formocresol vital pulpotomy technique with a 5% sodium hypochlorite (NaOCl) technique. The purpose of this study was to evaluate the clinical/radiographic success over 21 months of 5% NaOCl as the medicament in primary molar pulpotomies compared to published data for formocresol and ferric sulfate pulpotomies. METHODS: A retrospective chart audit was performed to evaluate results for all primary molar pulpotomies completed during a 12-month period using NaOCl. Dental records were reviewed for clinical and radiographic findings subsequent to pulp therapy. Clinical and radiographic criteria used to determine pulpotomy success were based on scientific literature. RESULTS: One hundred ninety-two NaOCl primary molar pulpotomies were completed in 118 patients; 131 (68%) primary molars from 77 children were available for follow-up examination (mean time since pulpotomy=10.5 months). NaOCl pulpotomies had a 95% clinical and 82% overall radiographic success rate. External root resorption was the most common pathologic finding. Pulpotomy success diminished over time. CONCLUSIONS: Clinical and radiographic success rates in this study on NaOCl pulpotomies are comparable to formocresol and ferric sulfate pulpotomies reported in the literature. Further study with longer observation periods is warranted.