Malocclusion and the need for orthodontic treatment in 8-year-old children with Down syndrome: a cross-sectional population-based study.

OBJECTIVES: To describe the arch morphology and the prevalence and severity of malocclusions in 8-year-old children with DS, and to evaluate their need for treatment using the Norwegian Need for Orthodontic Treatment Index (NOTI), and comparing the findings with a control group. MATERIALS AND METHODS: This population-based cross-sectional study was part of a national prospective study evaluating upper airway function, hearing, dental, and craniofacial characteristics in a cohort of children with DS in Norway. The cohort consisted of 32 children with DS and represented 57% of all children born with DS in Norway in 2002. Dental arch morphology and malocclusion were assessed on dental casts. Data were collected prospectively at the TAKO-centre, National Resource Center for Oral Health in Rare Medical Conditions. RESULTS: All the children with DS (100%) had objectively assessed needs for orthodontic treatment, compared with 30% of the normative comparison group. Among the children with DS, 68% were objectively categorized as being in "great need" (Category B) for treatment and the 75% were categorized as having "obvious need" (Category C). In comparison, only 20% of children without DS were in Category B, and 44% were in Category C. CONCLUSION: This study indicates significantly higher prevalence and severity of malocclusions in 8-year-old children with DS compared with control group.

Multidisciplinary Aspects of 104 Patients With Pierre Robin Sequence.

OBJECTIVE: To describe Pierre Robin sequence patients with a cleft palate from a multidisciplinary perspective. PATIENTS: A total of 104 individuals with Pierre Robin sequence and cleft palate, born between 1980 and 2010. METHOD: Data were collected retrospectively and compared with large control groups. RESULTS: Of 104 patients, 19 (18.3%) were treated with a nasopharyngeal or oropharyngeal tube, continuous positive airway pressure, and/or a tracheotomy. The mean weight percentile for newborns with Pierre Robin sequence was 30.9. It decreased to 29.9 at the time of cleft palate repair (mean age, 13.7 months) (P = .78). Of 87 patients, 30 (34.5%) developed normal speech after cleft palate repair. Of 93 nonsyndromic Pierre Robin sequence patients, 31 (33.3%) had or are having surgery for velopharyngeal insufficiency, a rate that is significantly higher when compared with a control group of cleft palate-only patients (19.4%; P = .004). Of 31 patients, 25 (80.6%) developed normal resonance after surgery for velopharyngeal insufficiency. There was no significant difference in the rate of syndromes between the Pierre Robin sequence patients and a control group of cleft palate patients without Pierre Robin sequence (P = .25). Seven of 39 boys (17.9%) with Pierre Robin sequence had a diagnosis of autism spectrum disorder. CONCLUSION: Even though the mean weight percentile for newborns with Pierre Robin sequence was low, the patients did not show a growth spurt during the first year of life. The high rate of velopharyngeal insufficiency after cleft palate repair in patients with Pierre Robin sequence needs further investigation. Also, the high rate of autism spectrum disorder among boys with Pierre Robin sequence prompts further investigation.

Bilateral Hypodontia in Adolescents With Pierre Robin Sequence.

OBJECTIVE: To describe the frequency of hypodontia and left-right symmetry of hypodontia in the permanent dentition of children with Pierre Robin sequence (PRS). PATIENTS AND METHOD: The sample consisted of 78 children born with PRS between 1980 and 2006 and referred to the Oslo Cleft Lip and Palate Team (OCLPT). Data were collected retrospectively from the archives of the OCLPT. Panoramic radiographs were evaluated to document hypodontia; third molars were excluded. RESULTS: Of the 78 children with PRS, hypodontia was found in 33 (42%). Of the 33 children with hypodontia, 27 (82%) showed hypodontia in the micrognathic lower arch, 24 (73%) were missing two or more permanent teeth, and 2 (6%) had oligodontia. The teeth most often missing were the mandibular second premolar (72%), followed by the maxillar second premolar (29%). Most cases of hypodontia 21 (64%) occurred bilaterally. Bilateral hypodontia of the mandibular second premolar occurred in 27 (73%) of the patients. CONCLUSION: Hypodontia was found in 33 (42%) of the children with PRS, which is seven times higher than the prevalence among children without PRS (6%). Unlike in the general population, bilateral hypodontia was more common than unilateral hypodontia in the micrognathic mandible of children with PRS.

Obstructive sleep apnea in younger school children with Down syndrome.

OBJECTIVE: We aimed to assess the prevalence of obstructive sleep apnea (OSA) in 8 year old school children with Down syndrome (DS). While the prevalence in otherwise healthy children is below 5%, the prevalence estimates in children with DS are uncertain (30-80%). OSA directly affects cognitive development and school performance. STUDY DESIGN: Population based cross sectional study in a limited geographical area. METHODS: Polysomnography (PSG) with video and audio recordings was performed in 8-year-old children with DS in a pediatric sleep unit according to the guidelines of American Academy of Sleep Medicine. Twenty-nine of all 32 children with DS within a restricted area comprising >50% of the Norwegian population and 54% of the children with DS born in Norway in 2002 were enrolled. RESULTS: This study reports an apnea hypopnea index AHI>1.5 in 28 of 29 children and an obstructive apnea index (OAI)>1 in 24 of 29 children. 19 children (66%) had an AHI>5 and 17 children (59%) had an OAI>5 which indicated moderate to severe OSA. No correlation was found between OSA and obesity or gender. CONCLUSION: The high prevalence of disease found in these previously undiagnosed 8-year-old children underlines the importance of performing OSA diagnostics in children with DS throughout childhood. These findings suggest that the prevalence of OSA remains high up to early school years. In contrast to earlier publications, this current study has the advantage of being population based, the study is performed on children of a narrow age band to estimate prevalence of disease and the diagnostic gold standard of PSG is applied.

Bilateral hypodontia is more common than unilateral hypodontia in children with Down syndrome: a prospective population-based study.

BACKGROUND: In individuals with simple hypodontia, congenital absence of teeth commonly affects just one tooth of a pair, not both. However, patterns of hypodontia have not been fully explored in children with Down syndrome (DS). OBJECTIVE: We describe the frequency and left-right symmetry of hypodontia in the permanent dentition of 8- to 9-year-old Norwegian children with DS. MATERIALS AND METHODS: This population-based cross-sectional study was part of a national prospective study evaluating upper airway function, hearing, dental, and craniofacial characteristics in a cohort of children with DS born in 2002. The cohort consisted of 29 children with DS and represented 57 per cent of all children born with DS in Norway in 2002. Hypodontia was assessed using panoramic and/or dental radiographs. Data were collected prospectively at TAKO-Centre, National Resource Centre for Oral Health in Rare Medical Conditions, Lovisenberg Diakonale Hospital, Oslo, Norway. RESULTS: Hypodontia of permanent teeth, excluding third molars, was found in 61.5 per cent of the 26 children included in the final sample. Among the 16 children with hypodontia, 75.0 per cent were missing two or more permanent teeth. Two children (7.7 per cent) had severe hypodontia (oligodontia). The teeth most often missing were the maxillary lateral incisors, followed by the mandibular second premolars and maxillary second premolars. Most (68.9 per cent) cases of hypodontia occurred bilaterally. CONCLUSIONS: The majority of the children with DS were missing one or more permanent teeth. Unlike in the general population, bilateral hypodontia was more common than unilateral hypodontia in this sample of children with DS.

Taurodontism: A minor diagnostic criterion in Laurence-Moon/Bardet-Biedl syndromes.

OBJECTIVE: The objective of the study was to present the prevalence of taurodontism in the permanent dentition in individuals with Laurence-Moon/Bardet-Biedl syndromes (LM/BBS). METHODS AND PARTICIPANTS: Thirty-nine individuals were studied, which comprises the whole population of known LM/BBS patients in Norway. Data were collected retrospectively. Panoramic radiographs (OPG) were evaluated to document taurodontism. RESULTS: Taurodontism was found in 82.9% of the individuals with LM/BBS. The second mandibular molars had the highest (72.3%) prevalence of taurodontism and the first mandibular molars the lowest (58.2%). CONCLUSION: This study suggests that taurodontism should be included as a minor diagnostic criterion for the Laurence-Moon/Bardet-Biedl syndromes (LM/BBS).

Pharyngoplasty after primary repair of clefts of the secondary palate.

Our aim was to identify factors associated with the incidence of pharyngoplasty in patients with clefts of the secondary palate (CP). We studied 351 children born between 1968 and 1999 with CP alone, who had their primary operation at the Department of Plastic Surgery, Rikshospitalet University Hospital, Oslo, Norway. Data were collected retrospectively from the archives of the Oslo Cleft Team. During the study period 1968-2007, 68 children (19%) had a pharyngoplasty to improve their speech. Mean follow up time from the primary operation was 16 years (range 2-37). The estimated incidence of pharyngoplasty at 5, 7, 10, and 15 years after the primary operation was 9%, 12%, 14%, and 15%, respectively. Thirty-one (46%) of the pharyngoplasties were done more than seven years after the primary palatal repair. Children with total clefts of the secondary palate had a pharyngoplasty more often than children with clefts of the soft palate alone (7 years incidence: 11 (42%) compared with 22 (10%), p<0.001). Sex, age at time of primary operation, and surgeon's experience were not significantly associated with the necessity for pharyngoplasty. Our study suggested that the necessity for pharyngoplasty is related to the severity of clefts, and duration of observation.

Clefts of the secondary palate referred to the Oslo Cleft Team: epidemiology and cleft severity in 994 individuals.

OBJECTIVE: To describe Norwegian individuals born with cleft of the secondary palate (CP) in relation to cleft severity, Pierre Robin sequence, associated anomalies, syndromes, and hypodontia and to present estimated longitudinal prevalences. PATIENTS: Nine hundred ninety-four individuals with CP born between 1960 and 2002 referred to the Oslo Cleft Team (OCT). METHOD: Data were collected retrospectively from the archives of the OCT. RESULTS: Among all CP, 7.6% had recognized syndromes, 30.5% had associated anomalies, and in 61.9%, CP was the only malformation. Pierre Robin sequence was recorded in 11.7%. Female predominance was observed both in general and in each cleft severity subgroup. Of 610 individuals with CP, only 25.1% were diagnosed with a submucous cleft palate, 45.9% had a cleft of the soft palate only, and in 29%, the cleft extended into the hard palate. Among 309 individuals over the age of 10 years with CP only, hypodontia was found in 28.5%, and in individuals with Pierre Robin sequence, hypodontia was found in 44.1%. During the period 1965 to 1999, the estimated prevalence of individuals with CP in the OCT uptake area was found to be 0.59 per 1000 live births. The estimated prevalence increased slightly over time, both overall and within the cleft severity subgroups. CONCLUSION: A large proportion of a Norwegian population with CP had syndrome or additional anomalies. The estimated prevalence of CP in this population increased slightly during the observation period.

Palatal fistulas after primary repair of clefts of the secondary palate.

Our aim was to assess whether severity of cleft, age at the time of repair, and the operating surgeon's experience contributed to the development of fistulas in patients with clefts of the secondary palate. We studied 814 children born between 1960 and 1999 with clefts of the secondary palate who had had their primary operation at the Department of Plastic Surgery, Rikshospitalet University Hospital, Oslo, Norway. Data were collected retrospectively from the archives of the Oslo Cleft Team. Palatal fistulas developed in 36 patients (4%), among whom 17 patients required correction (2% of the total). The incidence of fistulas was not related to sex. Patients with clefts of the hard and soft palate developed fistulas more often than patients with clefts of the soft palate only (8% compared with 1%, p<0.001). Patients with submucous cleft palates developed fistulas significantly more often than patients with clefts of the soft palate only (5% compared with 1%, p=0.02). Among patients with clefts of the hard and soft palate, the incidence of fistulas increased significantly with increasing age at the time of palatal closure (p=0.005). The incidence decreased significantly the more experienced the operating surgeon was for treating clefts of the hard and soft palate (p<0.001) but not for submucous clefts. Among patients with clefts of the hard and soft palate who had the palate closed at 14 months of age or later, the incidence of fistulas decreased from 21% when the operating surgeon had little experience to 0 when the surgeon had much experience. The incidence of fistulas was related to severity of cleft, age at palatal closure, and the operating surgeon's experience.

Feeding, artificial sucking habits, and malocclusions in 3-year-old girls in different regions of the world.

PURPOSE: The way babies and young children are reared is important to their health and development. Extensive breast-feeding has also been shown to reduce the development of artificial sucking habits like digit or pacifier-sucking. The aim of this study was to determine feeding methods, artificial sucking habits, and the presence of malocclusions in 3-year-old girls living in different regions of the world. METHODS: Children from the following countries were involved in the present study: (1) Brazil (Porto Alegre); (2) Japan (Niigata); (3) Mexico (Mexico City); (4) Norway (Oslo); (5) Sweden (Falköping); (6) Turkey (Istanbul); (7) and the United States (Iowa City, Iowa). During the interview and examination, the following variables were evaluated and registered: (1) breastfeeding and bottle-feeding; (2) duration and frequency; (3) sucking habits; (4) posterior and anterior crossbites; and (5) other malocclusions/normal occlusion. RESULTS: The prevalence of breast-feeding was very high in all groups, ranging between 78% and 98%. The prevalence of bottle-feeding in the different areas was also high. Except for Iowa City, the prevalence of digit-sucking was relatively low. Pacifier-sucking is fairly popular in most areas, with the exception of Niigata. The prevalence of normal occlusion in different cities ranged from 38% to 98%. CONCLUSIONS: There are considerable differences in feeding, as well as artificial sucking habits, in different areas of the world and at different periods.