RESUMEN
Aarskog-Scott syndrome (AAS) is characterized by different facial, skeletal and genital anomalies and may have oral manifestations. A 7-year-old boy was referred to the University General Hospital for treatment of speech difficulties and frequent regurgitation. Characteristics such as a triangle-shaped face, hypertelorism, low-set ears, flattened nose, shawl scrotum and partial syndactylia on hands and feet were observed. Based on these clinical features, the child was diagnosed with AAS. Upon intraoral examination, maxillary atresia and an incomplete cleft palate were observed. The mixed dentition was characterized by extensive coronary destruction of primary teeth and caries lesions on permanent teeth. Here, the case of a 9-year follow-up of this child with uncommon AAS associated with cleft palate is reported. The child was referred to a multidisciplinary team for planning and carrying out the treatment. In the follow-up visit after 9 years from the beginning of the treatment, the child showed greater sociability, with significant improvement in spontaneous speech and pronunciation of phonemes. However, the patient continues until now with articulation and spontaneous speech training. The correction of class II malocclusion, better dental alignment and canine extrusion were achieved. At the moment, the patient uses a nighttime extraoral device, and the treatment continues for dental alignment and prevention of tooth decay. The presence of cleft palate could be coincidental with AAS and may aggravate the prognosis, requiring careful patient monitoring by a multiprofessional team. Key words:Aarskog-Scott syndrome, cleft lip, cleft palate, multidisciplinary treatment.
RESUMEN
Purpose: The purpose of this study was to evaluate periodontal disease and oral biofilm in children and adolescents with cleft lip and/or palate (CLP). Methods: A total of 118 individuals aged six to 18 years, with CLP (n equals 60) and without CLP (n equals 58), were evaluated according to plaque index (PI), gingival bleeding index (BI), clinical attachment level (CAL), and probing pocket depth (PPD). Gingivitis and periodontitis were also diagnosed. Samples of subgingival oral biofilm were collected, and genomic DNA was extracted for oral microbial analysis. Aggregatibacter actinomycetemcomitans, Tannerela forsythia, Porphyromonas gingivalis, and Streptococcus oralis were identified and quantified by qPCR using Taqman primers and probes. Results: PI, BI, CAL, and PPD were statistically higher in the CLP group. Gingivitis was observed in 52 percent and 29 percent (P<0.001) and mild periodontitis was observed in 48 percent and 22 percent (P<0.001) of the CLP and control groups, respectively. When the permanent dentition group was evaluated, a greater occurrence of mild periodontitis was observed in the CLP group (P<0.001). The level of A. actinomycetemcomitans (P<0.001), P. gingivalis (P<0.001), and T. forsythia (P<0.001) was statistically higher in the subgingival biofilm of the CLP group. Conclusions: The presence of cleft lip and/or palate negatively affects oral hygiene and levels of periodontopathogens in oral biofilm. In addition, clinical and microbiological results highlight the importance of early assessment of young people with cleft lip and/or palate and the permanent dentition to prevent periodontal disease.