OBJECTIVE: To evaluate the effects of bone-anchored maxillary protraction (BAMP) treatment and longterm stability in growing cleft lip and palate and isolated cleft palate (CLP/CP) patients with mild maxillary hypoplasia and to compare maxillary growth patterns of BAMP-treated patients to matched control CLP/CP patients.
METHODS: Ten patients with CLP/CP were treated with BAMP; they were compared to the maxillary growth pattern of 10 age-matched cleft control patients with no maxillary protraction treatment, who later received surgical Le Fort I maxillary advancement after the growth period. The assessment of maxillary growth and the occlusion started at mean 8 years of age and continued until mean 18 years of age.
RESULTS: The use of BAMP orthopedic traction changed the growth pattern of mild hypoplastic maxilla toward a more anterior direction and advanced the face even above the level of Le Fort lll with only a minor effect on dentoalveolar units. The correction of occlusion and facial convexity were stable in the long term.
CONCLUSIONS: The using BAMP may improve the position of the maxilla relative to the anterior cranial base for the correction of mild maxillary hypoplasia in adolescent patients with CLP/CP. The achieved results are rather stable in the long term.

OBJECTIVE: Mucopolysaccharidosis (MPS) VI is a rare disorder caused by an autosomal recessive mutation in the short arm of chromosome 5 (5q12-13) leading to an N-acetylgalactosamine-sulfatase lysosomal enzyme deficiency and numerous systemic clinical changes. The oral and maxillofacial complex may exhibit tooth eruption anomalies, macroglossia, gingival hypertrophy, mouth breathing, increased lower facial height, open bite, retrognathia, and progressive TMJ arthrosis. This report describes craniofacial growth changes in two MPS VI patients, sisters and daughters of outbred parents, who were longitudinally monitored from 11 to 15 years of age.
METHODS: Skull lateral teleradiography and cephalometric tracings were performed. The measurements were assessed in the anteroposterior and vertical directions based on protocols by McNamara and Usp/Unicamp and compared to the normal reported ranges.
RESULTS: A similar skeletal class III malocclusion was observed in both patients. The jaw was retruded, the anterior skull base decreased, and the mandibular body was normal or larger than normal. The vertical growth direction differed between the patients; one was hyperdivergent, while the other was hypodivergent.
CONCLUSIONS: By understanding the craniofacial growth changes in MPS VI patients, new treatment options may be developed for affected patients.

BACKGROUND: Down syndrome presents dentists with several treatment problems due to hereditary olygodontia of permanent teeth, abnormal development of the midface causing small maxilla and class III occlusion, abnormal mineralization of teeth, reduced bone density and hypotony. The challenge of replacing missing permanent teeth was the trigger for the development of dental implants some 30 years ago. However, the abnormal development of jaw bones and the reverse occlusion caused concerns in the dental community regarding the possibility of using dental implants in Down syndrome patients. In 1999 the dental team of Barzilai Medical Center was the first to insert dental implants in a Down syndrome adolescent in order to replace 4 missing premolars. One implant was lost during the healing process, and the other 3 were followed for 15 years. One of the most important factors affecting implant success is gingival health. Periodontitis affects bone height and retention of the implant. Our patient was observed in the dental clinic every 3 months for the last 15 years for plaque and calculus control. The close follow-up and treatment kept the alveolar bone healthy and at the same height as at the beginning. This case report is unique since it is the first of its kind with a 15 year follow-up. Furthermore, it showed that frequent visits and control of gingival health keeps the bone healthy and at the same height as at the beginning.

BACKGROUND: This study aimed to investigate the dental and skeletal variables associated with disturbances of craniofacial development in oral-breathing (OB) individuals and the probability that these variables are related to this condition.
METHODS: This is an observational retrospective case-control study of 1596 patients divided into three groups of age n1 5-12, n2 13-18, and n3 19-57 years. Radiographic, clinical, and models data were analyzed. The control group was consisted of nasal breathing (NB) individuals. Statistical analyses of the qualitative data were performed with x (2) test to identify associations, and odds ratio (OR) tests were performed for the variables that the chi-square test (x (2)) identified an association.
RESULTS: In the descriptive analysis of the data, we observed that the class II malocclusion was the most frequent in the total sample, but when divided by age group and mode of breathing, there is a random division of these variables. In n1 group, class II, (OR = 2.02) short and retruded mandible (SM and RM) (OR = 1.65 and1.89) were associated with OB and it was considered a risk factor. In n2 group, class II (OR = 1.73), SM (OR = 1.87) and increased lower anterior height (ILAFH) (OR = 1.84) seemed to be associated and to be risk factors for OB. In the n1 group, decreased lower anterior facial height (DLAFH) and brachycephalic facial pattern (BP) seemed to be associated with NB and a protective factor against oral breathing.
CONCLUSIONS: This study showed that dental and skeletal factors are associated with OB in children, and it seems that it becomes more severe until adolescence. But adults showed no associations between OB and skeletal factors, only in dental variables, indicating that there is no cause-effect relationship between the dental and skeletal factors and OB. The treatment of nose breathing patient should be multidisciplinary, since OB remains even when dental and skeletal factors slow down.

BACKGROUND: To compare the lip closing force of patients with mandibular prognathism to that of patients without dentofacial anomalies.
METHODS: The subject group included 62 female patients of Class III relationship with mandibular prognathism. The control group been comprised of 71 patients of Class I relationships without skeletal deformities. Maximum lip closing force and average lip closing force were measured using a Y-meter. Student\'s t-test was carried out to analyse the differences between the groups. Correlation and stepwise multiple linear regression analyses were performed to analyse the relationship between lip closing force and craniofacial morphology.
RESULTS: The lower lip closing force of subjects with mandibular prognathism was significantly greater than that of patients in the control group (P < 0.001), while the upper lip closing force showed no difference (P > 0.05). The lower lip closing force of patients with mandibular prognathism was strongly correlated with IMPA (Lower Incisor - Mandibular Plane angle, P < 0.001) and FMA (Frankfort Plane-Mandibular Plane angle, P < 0.001). Multiple regression equations: (MaxLL) = 12.192 - 0.125 * (IMPA) + 0.082 (FMA); (AveLL) = 9.112 - 0.091 * (IMPA) + 0.054 (FMA).
CONCLUSIONS: The lower lip closing force was markedly increased in Class III patients with mandibular prognathism and was strongly correlated with lower incisor position and mandibular plane angle.

Orofacial myologists are frequently called upon to address retained oral habit concerns. During this process, current I.A.O.M. recommended treatment includes addressing tongue, lip, and jaw rest posture concerns. Following digit sucking remediation, we may also be called upon to address these rest posture issues, and tongue thrust more aggressively together. In this process, facial growth and development and jaw structure may coincidentally improve as a result of \'nature taking its course\' by addressing both swallow AND rest posture. In a select subset of clients, dramatic improvements may occur if the timing is right. This article discusses one such case that appears to have yielded a significant improvement in oral postures influencing improved facial and oral growth and development.

Fibrodysplasia ossificans progressiva (FOP) is a rare disease characterized by postnatal heterotopic ossification (HO). When HO affects the masticatory muscles, mouth opening becomes restricted. This paper presents the changes in facial morphology and occlusion of a patient with FOP who was followed from the age of 8 to age 21. At the initial examination, he had a severely protruded maxilla and Angle Class II Division 1 malocclusion. His mouth opening was restricted (5.0 mm). He had a large overjet and this enabled him to clean his teeth and to eat. Orthodontic correction was not planned, and his facial growth was closely followed with attention to his oral hygiene. The maxillary protrusion and a low mandibular plane angle became more prominent as the patient aged. His mandible rotated in a counterclockwise direction. His molars had delayed eruption or were impacted and seven were extracted. His mouth opening increased slightly and his oral hygiene improved to excellent.

BACKGROUND: Pierre-Robin sequence (PRS) is defined by micro- and/or retrognathia, glossoptosis and cleft soft palate, either caused by deformational defect or part of a malformation syndrome. Neurofibromatosis type 2 (NF2) is an autosomal dominant syndrome caused by mutations in the NF2 gene on chromosome 22q12.2. NF2 is characterized by bilateral vestibular schwannomas, spinal cord schwannomas, meningiomas and ependymomas, and juvenile cataracts. To date, NF2 and PRS have not been described together in the same patient.
METHODS: We report a female with PRS (micrognathia, cleft palate), microcephaly, ocular hypertelorism, mental retardation and bilateral hearing loss, who at age 15 was also diagnosed with severe NF2 (bilateral cerebellopontine schwannomas and multiple extramedullary/intradural spine tumors). This is the first published report of an individual with both diagnosed PRS and NF2. High resolution karyotype revealed 46, XX, del(22)(q12.1q12.3), FISH confirmed a deletion encompassing NF2, and chromosomal microarray identified a 3,693 kb deletion encompassing multiple genes including NF2 and MN1 (meningioma 1).Five additional patients with craniofacial dysmorphism and deletion in chromosome 22-adjacent-to or containing NF2 were identified in PubMed and the DECIPHER clinical chromosomal database. Their shared chromosomal deletion encompassed MN1, PITPNB and TTC28. MN1, initially cloned from a patient with meningioma, is an oncogene in murine hematopoiesis and participates as a fusion gene (TEL/MN1) in human myeloid leukemias. Interestingly, Mn1-haploinsufficient mice have abnormal skull development and secondary cleft palate. Additionally, Mn1 regulates maturation and function of calvarial osteoblasts and is an upstream regulator of Tbx22, a gene associated with murine and human cleft palate. This suggests that deletion of MN1 in the six patients we describe may be causally linked to their cleft palates and/or craniofacial abnormalities.
CONCLUSIONS: Thus, our report describes a NF2-adjacent chromosome 22q12.2 deletion syndrome and is the first to report association of MN1 deletion with abnormal craniofacial development and/or cleft palate in humans.

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OBJECTIVE: The children obstructive sleep apnea and hyponea syndromes has become a global research hot spot, but the research of craniofacial features in obstructive sleep apnea and hyponea syndromes (OSAHS) children was scarce. To evaluate the craniofacial features of obstructive sleep apnea and hyponea syndromes children.
METHODS: The subjects involved in this study fell into two groups: the patient group and the control one. The patients and controls were strictly matched by age and sex. Lateral head radiographs and cephalometric measurements were obtained and then compared between the two groups.
RESULTS: The findings demonstrated marked differences in terms of SNB, PG-NB, lower facial height, H-C3Me and A&T/P. The SNB angle (75.8±4.3) in the patient group was smaller than that in the control one (78.7±2.6) and the P value was 0.035; the PG/NB value in the patient group (1.3±0.8) mm was higher than that in the control one (0.6±0.6) mm and the P value was 0.02. The anterior face height was (65.1±5.9) mm in the patient group (P=0.04), while the anterior face height in the control group was (61.5±3.2) mm. The position of hyoid was lower in the patient group(5.3±3.7) mm, compared with the control one (2.6±2.6) mm, and the P value was 0.03. Furthermore, the patients of OSAHS had more swelled adenoids and tonsils than the controls.
CONCLUSIONS: The patient group differed from the control group in the length of mandible, lower facial height, position of hyoid and the chin, and the size of the adenoids and tonsils.