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BackgroundCleft lip and/or palate is the most frequent congenital abnormality occurring in the craniofacial region and is often associated with numerous dental defects such as tooth agenesis, supernumerary teeth, microdontia, taurodontism, crown malformations, or delay in eruption. The prevalence of hypodontia in cleft-affected patients is much higher in comparison with a healthy population. The aim of this study was to evaluate the prevalence of second premolar hypodontia in patients with cleft lip and/or palate.Material/MethodsWe performed a retrospective, evaluation of panoramic radiographs and dental casts in the Department of Dentofacial Orthopeadics and Orthodontics, Wroclaw Medical University. Two independent observers evaluated the records of 469 patients with various types of clefts and analyzed dental casts and panoramic radiographs.Results202 individuals met inclusion criteria. The sample comprised 120 UCLP patients, 38 BCLP patients, 28 CP patients, and 17 CLA patients. Hypodontia in the premolar region was observed in 39 individuals (19.3%). A total number of 58 second premolars were missing, of which 35 were maxillary second premolars (U5) and 23 were mandibular second premolars (L5).ConclusionsEstimated hypodontia in the Polish CL/P sample was considerably higher than the hypodontia in permanent dentition reported for a European healthy population. The number of congenitally missing second premolars was higher in the maxillary arch than in the mandibular.

BackgroundCleft defects are one of the most frequent birth-deformities of the orofacial region and they are commonly associated with anomalies of the tooth structure, size, shape, formation, eruption, and tooth number. The aim of our study was to evaluate the prevalence, distribution, and potential association of combined hypodontia in cleft-affected patients with regard to all types of teeth in both jaws in the permanent dentition.Material/MethodsThis retrospective radiographic analysis included patients with various types of clefts treated orthodontically in the Department of Orofacial Orthopedics and Orthodontics at Heim Pàl Children’s Hospital, Budapest. There were 150 patients (84 males, 66 females) with non-syndromic unilateral (UCLP; n=120 patients) or bilateral (BCLP; n=30 patients) cleft formation (lip, alveolus and palate) who met the inclusion criteria. Statistical analysis was performed using the chi-square test and Fisher’s exact test (significance level p<0.05).ResultsHypodontia was significantly more frequent in patients with cleft-sided lateral incisor (104 patients, 69%), with a total of 235 missing teeth, followed by the second premolars of the upper and lower jaw. A significant correlation of congenital missing teeth was observed in left-sided clefts between the upper and lower second premolar in the cleft area.ConclusionsHypodontia inside and outside the cleft area was frequently observed. This should affect the therapy plans, especially if the cleft-sided premolar is also absent. Further comprehensive research including numerous random samples is necessary for better estimating other possible associations.

Alagille syndrome is a rare, autosomal, complex, dominant disorder associated with dysfunction of the liver, heart, skeleton, and eyes, as well as characteristic facial appearance. It is associated with the defect in component of the Notch signalling pathway. Here, we review the main features of Alagille syndrome with special focus on oro-facial manifestations like prominent forehead, moderate hypertelorism with deep-set eyes, a saddle or straight nose with a flattened, bulbous tip, and large ears. The article is based on the most recent and significant literature available from the Medline database.Contrary to healthy children, patients with Alagille syndrome have many problems, depending on several factors like the severity of cholestasis and scarring in the liver, heart or lung problems, presence of infections, or other problems related to poor nutrition that can manifest in their oral cavity in the dental and periodontal tissues, as well as oral mucosa. From the dentist’s view, the most important elements are careful observation, accurate diagnosis, and planned management of such patients, especially during the patient’s formative years, to prevent complications. Aggressive preventive oral care and consultations with medical specialists before any invasive procedure are obligatory. All this can improve quality of life in patients with Alagille syndrome.