A Retrospective Longitudinal Treatment Review of Multidisciplinary Interventions in Nonsyndromic Robin Sequence With Cleft Palate.

OBJECTIVE: To document and analyze the overall longitudinal institutional treatment experience of children with nonsyndromic Robin sequence (RS) from infancy to early adulthood. DESIGN: Retrospective longitudinal treatment review. SETTING: A tertiary-care, referral, teaching hospital. PATIENTS: Children with nonsyndromic RS and cleft palate (N = 117) born between December, 1985, and January, 2012. INTERVENTIONS: Data regarding airway management, nutritional management, audiological interventions, orthodontic treatment, and surgical interventions were documented and analyzed in different growth/developmental stages. Comparative data from other international centers were collected from the literature. RESULTS: Airway management during infancy involved prone positioning (92%), nasopharyngeal airway (6%), tracheostomy (2%), and mandibular distraction osteogenesis (1%). Feeding with nasogastric, gastrostomy, and/or gastrojejunostomy tubes was used in 44%, Haberman feeders in 53%, and Mead Johnson feeders in 3%. Gastroesophageal reflux disease was documented in 6% of the sample. During childhood and early adolescent years, pharyngeal flap surgery was carried out in 22% of the children, while 11% had secondary palatal surgery. Audiological management included the use of tympanostomy tubes in 62%, with several children needing multiple tube replacements. At least 18% were diagnosed with obstructive sleep apnea. Adenoidectomy or adenotonsillectomy was undertaken in 4%. Analysis of data pertaining to middle childhood and adolescent years showed that orthodontic treatment was conducted for most children for crowding, tooth agenesis, and skeletal and/or dental dysplasia. Orthognathic surgery frequency (<18%) was low. CONCLUSIONS: Institutional treatment experience of children with nonsyndromic RS involves multidisciplinary care at different ages and stages of their development.

Prevalence and patterns of permanent tooth agenesis in individuals with Down syndrome: a meta-analysis.

The aims of the present study were to evaluate the prevalence and patterns of permanent tooth agenesis in individuals with Down syndrome, using meta-analysis methodology. Two independent investigators carried out a literature search to locate articles pertaining to permanent tooth agenesis in individuals with Down syndrome. The outcomes of interest were the prevalence and pattern of permanent tooth agenesis. Thirteen articles were selected for inclusion. The estimated overall prevalence of permanent tooth agenesis, excluding third molars, was 54.6% (95% CI: 44-66%). Considerable heterogeneity was present across the studies. Among subjects with tooth agenesis, approximately half had three or more missing teeth. The permanent teeth with the highest prevalence of agenesis were the maxillary lateral incisor (27%), mandibular second premolar (21%) and maxillary second premolar (18%). The single most commonly absent tooth, however, was the mandibular left second mandibular premolar (19.9%), followed by the maxillary left lateral incisor (19.4%). Little data describing specific tooth-agenesis patterns were available. In conclusion, individuals with Down syndrome display high prevalence rates and severity of agenesis of permanent teeth. Proper and timely diagnosis of missing permanent teeth is thus necessary, to allow a more comprehensive long-term treatment plan and a more favorable prognosis in these individuals.