Objective measurement of oral function in adults with spinal muscular atrophy.

BACKGROUND: Impairment of bulbar function in adult individuals with spinal muscular atrophy (SMA) usually is not assessed by established motor scores. Measurements of oral function including quantitative muscle and endurance tests are able to detect subtle changes. The aim of this study was to systematically evaluate the measurement of maximum bite force and endurance, maximum tongue pressure and endurance, as well as maximum mouth opening in adult individuals with SMA types 2 and 3. METHODS: Data from oral function tests in 43 individuals were analyzed. Differences in oral function between individuals with different SMA types and numbers of SMN2 copies were tested. Spearman´s rho correlations among oral function measures themselves as well as with established clinical outcome scales were analyzed. RESULTS: The absolute maximum measures of oral function (maximum bite force, maximum tongue pressure, maximum mouth opening) were able to discriminate between individuals with different SMA types, individuals with a different number of SMN2 copies and with different walking abilities. The pairwise correlations of the absolute maximum measures of oral function were fair to moderate in size; the same was true for their correlations with the established motor scores. All correlations assessing endurance measures of oral function were weaker and statistically insignificant. CONCLUSIONS: Among the oral function tests maximum tongue pressure and maximum mouth opening are particulary promising as clinical and sensitive outcome measures for clinical trials. Oral function tests may supplement existing motor scores, in particular concerning specific questions about bulbar function or in severely affected non-ambulatory individuals where mild (treatment-related) changes would otherwise remain undetected. Trial registration DRKS, DRKS00015842. Registered 30 July 2019, https://drks.de/search/de/trial/DRKS00015842.

Quality of orthodontic care-A multicenter cohort study in Germany : Part 1: Evaluation of effectiveness of orthodontic treatments and predictive factors.

AIMS: Orthodontic care and its effectiveness have increasingly become the focus of political and public attention in the recent past. Therefore, this multicenter cohort study aimed to report about the effectiveness of orthodontic treatments in Germany and to identify potential influencing factors. METHODS: A total of 586 patients from seven German study centers were screened for this cohort study, of which 361 patients were recruited at the end of their orthodontic treatment. Of these, 26 patients had missing study models and/or missing treatment information. Thus, 335 participants were included. The severity of malocclusion was rated using the Peer Assessment Rating (PAR) Index at baseline (T0) retrospectively and-prospectively-after the retention period (T1). Practitioner-, treatment- and patient-related information were analyzed in order to detect potential predictive factors for treatment effectiveness. RESULTS: Study participants (202 female and 133 male) were on average 14.8 (standard deviation [SD] ± 6.1) years old at start of active treatment. Average PAR score at T0 was 25.96 (SD ± 10.75) and mean posttreatment PAR score was 3.67 (SD ± 2.98) at T1. An average decrease of total PAR score by 22.30 points (SD ± 10.73) or 83.54% (SD ± 14.58; p < 0.001) was detected. Furthermore, 164 treatments (49.1%) were categorized as 'greatly improved' but only 3 treatments (0.9%) as 'worse or no different'; 81.5% of all cases finished with a high-quality treatment outcome (≤5 PAR points at T1). Logistic regression analyses detected staff experience as a significant predictive factor for high-quality results (odds ratio 1.27, p = 0.001, 95% confidence interval 1.11-1.46). CONCLUSION: The improvement rate among this selected German cohort indicated an overall very good standard of orthodontic treatment. Staff experience proved to be a predictive factor for high-quality results.

Diagnosis and treatment of isolated snoring-open questions and areas for future research.

STUDY OBJECTIVES: Snoring is a common phenomenon which is generated by vibration of soft tissue of the upper airway during sleep. Due to the high incidence of isolated snoring and the substantial burden for the patient and the bed partner, a thorough examination and appropriate therapy are required. Many recommendations for the treatment of isolated snoring are either not evidence-based or are derived from recommendations for the management of obstructive sleep apnea. Therefore, the aim of this study is the identification and description of open questions in the diagnosis and treatment of isolated snoring and the illustration of areas for further research. METHODS: In the context of the development of the new version of the German guideline "Diagnosis and treatment of isolated snoring in adults," a multidisciplinary team of experts performed a systematic literature search on the relevant medical data and rated the current evidence regarding the key diagnostic and therapeutic measures for snoring. RESULTS: The systematic literature review identified 2293 articles. As a major inclusion criterion, only studies on primary snoring based on objective sleep medical assessment were selected. After screening and evaluation, 33 full-text articles remained for further analysis. Based on these articles, open questions and areas for future research were identified for this review. CONCLUSION: Several major gaps in the literature on the diagnosis and treatment of isolated snoring were identified. For the majority of diagnostic and therapeutic measures for snoring, high-level scientific evidence is still lacking.

Prenatal indices for mandibular retrognathia/micrognathia.

OBJECTIVE: Micro- and retrognathia of mandibular origin may lead to life-threatening respiratory problems in connection with glossoptosis immediately after birth. Prenatal screening for this malformation is therefore increasingly important. Today this is accomplished by predominantly subjective standards. Objective criteria have been proposed but have not become established. We therefore made an effort to develop indices that would identify major skeletal discrepancies or micrognathia in as straightforward a fashion as possible during routine prenatal sonography. MATERIALS AND METHODS: Series of fetal jaw sonograms (Toshiba Aplio MX®) were obtained in 313 women with normal pregnancies from weeks 19-29 of gestation. Upper- and lower-jaw landmarks were interactively located on screen and evaluated for reproducibility. Linear parameters representative of maxillary and mandibular length (SpA'-SpP' and Rami-SymMe) were measured and related to femur length and gestational age. Based on these data, indices for maxillary, and mandibular length were derived and analyzed. RESULTS: High correlations were identified for mandibular length both with gestational age (R = 0.845; R(2) = 0.713) and with femur length (correlation coefficients (R) = 0.839; coefficients of determination (R(2)) = 0.704). For maxillary length, the respective correlation coefficients were 0.691 (R(2) = 0.477) and 0.656 (R(2) = 0.430). Estimates of mandibular and maxillary length based on gestational age and femur length were obtained by regression analysis. The mean bimaxillary length ratio was 0.628 ± 0.043. CONCLUSION: Maxillary and mandibular growth can be objectively evaluated via indices. It is conceivable to develop this approach into a sensitive and reliable method of prenatal jaw screening for major skeletal anomalies and congenital malformations.

Strong association of variants around FOXE1 and orofacial clefting.

Nonsyndromic orofacial clefting (nsOFC) is a common, complex congenital disorder. The most frequent forms are nonsyndromic cleft lip with or without cleft palate (nsCL/P) and nonsyndromic cleft palate only (nsCPO). Although they are generally considered distinct entities, a recent study has implicated a region around the FOXE1 gene in both nsCL/P and nsCPO. To investigate this hypothesis, we analyzed the 2 most strongly associated markers (rs3758249 and rs4460498) in 2 independent samples of differing ethnicities: Central European (949 nsCL/P cases, 155 nsCPO cases, 1163 controls) and Mayan Mesoamerican (156 nsCL/P cases, 10 nsCPO cases, 338 controls). While highly significant associations for both single-nucleotide polymorphisms were obtained in nsCL/P (rs4460498: p Europe = 6.50 × 10(-06), p Mayan = .0151; rs3758249: p Europe = 2.41 × 10(-05), p Mayan = .0299), no association was found in nsCPO (p > .05). Genotyping of rs4460498 in 472 independent European trios revealed significant associations for nsCL/P (p = .016) and nsCPO (p = .043). A meta-analysis of all data revealed a genomewide significant result for nsCL/P (p = 1.31 × 10(-08)), which became more significant when nsCPO cases were added (p nsOFC = 1.56 × 10(-09)). These results strongly support the FOXE1 locus as a risk factor for nsOFC. With the data of the initial study, there is now considerable evidence that this locus is the first conclusive risk factor shared between nsCL/P and nsCPO.

Maxillary reaction patterns identified by three-dimensional analysis of casts from infants with unilateral cleft lip and palate.

OBJECTIVES: In order to visualize and quantify the direction and extent of morphological upper-jaw changes in infants with unilateral cleft lip and palate (UCLP) during early orthodontic treatment, a three-dimensional method of cast analysis for routine application was developed. In the present investigation, this method was used to identify reaction patterns associated with specific cleft forms. MATERIALS AND METHODS: The study included a cast series reflecting the upper-jaw situations of 46 infants with complete (n=27) or incomplete (n=19) UCLP during week 1 and months 3, 6, and 12 of life. Three-dimensional datasets were acquired and visualized with scanning software (DigiModel®; OrthoProof, The Netherlands). Following interactive identification of landmarks on the digitized surface relief, a defined set of representative linear parameters were three-dimensionally measured. At the same time, the three-dimensional surfaces of one patient series were superimposed based on a defined reference plane. Morphometric differences were statistically analyzed. RESULTS: Thanks to the user-friendly software, all landmarks could be identified quickly and reproducibly, thus, allowing for simultaneous three-dimensional measurement of all defined parameters. The measured values revealed that significant morphometric differences were present in all three planes of space between the two patient groups. Patients with complete UCLP underwent significantly larger reductions in cleft width (p<0.001), and sagittal growth in the complete UCLP group exceeded sagittal growth in the incomplete UCLP group by almost 50% within the first year of life. CONCLUSION: Based on patients with incomplete versus complete UCLP, different reaction patterns were identified that depended not on apparent severities of malformation but on cleft forms.

[Sonographic evaluation of fetal clefts of the lip, alveolus and palate]. / Sonografische Diagnostik fetaler Spaltbildungen von Lippe, Kiefer und Gaumen.

Clefts of the lip, alveolus and palate are among the most common congenital malformations. Due to their frequent combination with other structural anomalies, chromosomal defects and genetic syndromes, the prenatal diagnosis of clefts plays an important role. Furthermore, the prenatal detection of clefts enhances the parents' psychological preparation and enables the planning of postnatal management. This article reviews the occurrence, appearance and pathophysiology of these malformations as well as the different sonographic techniques used to diagnose clefts and their extent. Previously reported 2D- and 3D-techniques as well as our own recent diagnostic approach focussing on the diagnosis of isolated cleft palate are described.

Primary respiratory support in preterm infants with cleft lip and palate.

OBJECTIVE: Preterm infants with respiratory distress are routinely treated by application of nasal mask continuous positive airway pressure. In preterm infants with cleft lip and palate, nasal mask attachment is not feasible due to air leakage through the cleft defect. Here, we describe a modified continuous positive airway pressure application method that overcomes this problem. DESIGN AND SUBJECTS: Observation study, university neonatal intensive care unit. The neonates (n = 4) were between 28 and 33 weeks' gestation and weighed 1160 to 1680 g at birth. Immediately after birth, infants with unilateral cleft lip and palate (n = 3) were respiratory stabilised by a Medijet generator using a nasal tube. To minimise the pressure cap, hydrocolloid bandages were adhered over the total cleft defect. Immediately after orthodontic passive palatal plates insertion (within 27 hours of life), the nasal tube was removed and continuous positive airway pressure was applied through a nasal mask covering the complete nose of the infant. RESULTS: The system proved suitable for patients with unilateral cleft lip and palate for whom the generated nasal mask continuous positive airway pressure remained constant between 5 to 7 cm of water but failed in the patient with bilateral cleft lip and palate. None of the patients had to be ventilated due to respiratory failure, and all survived to discharge. CONCLUSION: Preterm infants with unilateral, but not bilateral cleft lip and palate, can be successfully stabilised using the described nasal mask continuous positive airway pressure system, thereby avoiding primary intubation and its associated risk of complications.

Diagnosis and management of cervical teratomas.

We present three cases of cervical teratoma, two of which were detected prenatally by ultrasound. The two cases diagnosed prenatally showed rapid early growth, which indicated the aggressive nature of the tumour and assisted the parents' decision to terminate the pregnancy. The third case was undiagnosed prenatally and the child was born after a normal pregnancy and uncomplicated vaginal delivery. She recovered well after excision of the teratoma.

[Comparison of mechanical properties of orthodontic nickel-titanium wires]. / Vergleich mechanischer Eigenschaften orthodontischer Nickel-Titan-Drähte.

Two packages each, containing 10 wires per package, of different batches of 25 different types of orthodontic archwires made of super-elastic nickel-titanium alloys measuring 0.41 x 0.56 mm2, were investigated. The wires were characterized by obtaining the following measurements at an ambient temperature of 37 degrees: a three-point bending test with the supporting points spaced 10 mm apart, and determination of the torque/bending angle curves using a pure bending test. The force/deflection curves provided the parameters characterizing the super-elastic unloading plateau: average force, slope and endpoint. From the torque/bending angle curves, the parameters average torque, plateau endpoint and the elasticity parameters were determined. Average force (0.8-4.5 N), endpoint (0.2-0.9 mm) and the slope of the unloading plateau (0.2-2.1 N/mm) of the three-point bending test clearly differed for individual wires. Significant differences were also seen for average torque (1.5-11.5 Nmm), unloading plateau endpoint (2.7-20.0 degrees) and elasticity parameters epsilon 4, E4, E5 and E6 in the pure bending test. Individual batches showed only minor differences. The results permit the conclusion to be drawn that super-elasticity is applicable to only a small portion of the wires examined. Although other wires showed super-elastic behaviour, the unloading plateaus has a force level of up to 6 N, and cannot be recommended for orthodontic application. The super-elastic plateau is often of use only for deflections greater than 1.5 mm. The use of super-elastic archwires made of nickel-titanium alloys makes sense only when the elastic properties of the respective wires are known. This makes the provision by the manufacturer of relevant data on the elastic properties of wires a necessity.

Skeletal and dental effects of maxillary protraction in patients with angle class III malocclusion. A meta-analysis.

The aim of this study was a quantitative review of the published results concerning the treatment effects of maxillary protraction in patients with Angle Class III using the meta-analysis method. 85 articles published between 1966 and 1998 were reviewed under strict, pre-defined criteria. Of these, 12 studies presenting results of cephalometric measurements were selected for further analysis. The results of different cephalometric measurements were reviewed with Dstat 1.10 software in order to calculate a standardized treatment effect variable. The homogeneity of the variances of the different effect variables as well as a composite effect were calculated. For the latter, each individual effect variable was weighted by the reciprocal of its variance, with predefined study characteristics being used for categorical model calculations. Our results showed a significant composite effect of the therapy on the skeletal components (SNA: 1.4 degrees, composite effect = 1.11; SNB: -1.3 degrees, composite effect = -0.94; ANB: 2.6 degrees, composite effect = 1.73; lower anterior face height: 1.6 mm, composite effect = 0.85) as well as on the dental components of the face (inclination of upper incisors: 1.6 degrees, composite effect = 0.37; inclination of lower incisors: -3.7 degrees, composite effect = -0.87). However, several of the individual effect variables demonstrated a significant lack of homogeneity. Study characteristics which might possibly account for this variability were the patients' ages at the start of treatment and the combination of maxillary protraction with rapid maxillary expansion. In summary, maxillary protraction was shown to have a significant treatment effect. The meta-analysis method proved to be a valuable tool in judging the current status of a given clinical therapeutic concept.

Two- or three-dimensional cast analysis in patients with cleft lip and palate?

AIM: The aim of the present study was to evaluate different two- and three-dimensional cast analyses for the assessment of maxillary dimensions in patients with complete unilateral cleft lip and palate, using a set of representative cast series. PATIENTS AND METHOD: Consecutive casts were taken from ten patients at 1 week, and at 3, 6, and 12 months, respectively. First, cast surfaces were digitized two-dimensionally using a scanner. Subsequently, landmarks were identified on screen and the previously defined maxillary dimensions were determined automatically by computer. Additionally, three-dimensional measurements were carried out using a specialized coordinate measurement table. The differences between the respective measurements were analyzed for statistical significance. RESULTS: The present study shows that the precision of the cast analysis is influenced above all by the quality of the casts and less by the precision of the measuring system employed. Moreover, misinterpretation of the results can be attributed to using an inappropriate reference system or to considering mean values only. When assessing linear and angular measurements located in approximately the same plane, no significant amount of additional information could be obtained by including the third dimension. CONCLUSION: Thus, two-dimensional measurement procedures are appropriate in principle for quick, reliable cast analysis in patients with cleft lip and palate.

Tooth-borne distraction to widen the mandible. Technical note.

Based on reported animal studies, the case report presented demonstrates the use of tooth-borne distraction osteogenesis for mandibular widening of 1 cm in order to avoid extraoral distraction devices. Detrimental side effects on healthy anchoring teeth are not likely.

Tooth-borne distraction of the mandible. An experimental study.

Two mini-pigs and 6 micropigs were fitted with a dentally fixed device for osteodistraction of the mandible. Following bilateral osteotomy and after a latency period of 7 days, the apparatus was activated 1 mm per day. A 9 mm lengthening of the mandible was achieved. After a retention period of 6 weeks, the device was removed followed by a consolidation period of 6 weeks. During the experiment, the callus and bone formation and potential dental and periodontal reactions were radiologically examined. Polychrome sequential labelling was performed by injecting calcein green and xylenol orange for histological assessment of bone. The animals were killed at the 14th postoperative week and the area of distraction and the roots of the adjacent teeth were histologically studied. After 12 weeks, the osteogenesis in the distraction gap was complete, showing lamellar bone. No detrimental effects at the roots or in the periodontium of the adjacent teeth were seen. Thus, lengthening or widening of the mandible using a tooth-borne distraction device seems feasible in selected cases.

[3-D model analysis of the maxilla of infants with lip-jaw-palate clefts]. / 3-D-Modellanalyse des Oberkiefers von Säuglingen mit LKG-Spalten.

For the investigation of three-dimensional morphological changes in the maxilla of children with cleft lip and palate, the use of two-dimensional test analysis is inadequate. Since no standardised three-dimensional method has so far been available, a three-dimensional digital, computer-aided procedure was developed to visualize and metrically analyse the growth of the edentulous maxilla of infants with cleft lip and palate. Chronologically consecutive casts of the maxillas (obtained at the ages of one week, and three, six and twelve months) of five children with complete unilateral CLP were measured optically with the instrument Micromeasure 70. Following digitation, the casts were reconstructed in the computer, aligned and superimposed using the Orthosurf program. The distances between the surfaces were then measured; in addition, the surfaces were segmented perpendicular to the alveolar crest at reference points C1, C1', C2, C2' and I. The volumes of the resulting segments were determined and compare with one another. Specially designed software automated the following steps: 1. identification of reference points; 2. alignment of the cloud of points in a system of coordinates, and 3. identification of the alveolar crest. Our initial results show that (1) the new method enables visualization of the extent and direction of morphological changes of the mucosal surface, and (2) reproducible quantification of these changes via the determination of changes in the volume of defined alveolar segments. The three-dimensional analysis presented here permits a comprehensive three-dimensional measurement of the models of the edentulous maxilla of infants with cleft lip and palate.

3-dimensional analysis of cleft palate casts.

It is the aim of presurgical infant orthopedics (PSIO) to reduce the width of the cleft and to achieve optimal alignment of the cleft palate segments within the first few months of infancy prior to any surgical cleft closure. The question about the amount that PSIO can stimulate and steer the growth of the maxilla using a Hotz and Gnoinski passive appliance has not yet been satisfactorily answered. In this study, a 3-dimensional digital computer-aided procedure was developed to analyze metrically and to visualize the growth of the edentulous maxilla of cleft lip and palate in infants. In a pilot study, a series of digital scans of consecutive plaster casts was carried out. 5 infants with complete unilateral cleft lip and palate (UCLP), who had been treated with passive appliances were evaluated. Impressions were taken at the age of one week and after 3, 6 and 12 months. Following digitizing, the casts were computer-reconstructed and segmented perpendicular to the alveolar crest, the reference points being C1, C1', C2, C2' and I. The volume of the resulting segments was ascertained. Computer superimposition of reconstructed consecutive casts was employed to facilitate a visualization of the extent and direction of morphological changes. Our first results have shown that with our method it is possible to quantify the growth rate of defined segments of the maxilla. The 3-dimensional analysis presented here will be the basis for further studies to objectify PSIO.

[Dental assisted distraction osteogenesis of the mandible. Results of an animal experimental study and initial clinical application]. / Dental gestützte Distraktionsosteogenese des Unterkiefers. Ergebnisse einer tierexperimentellen Studie und erste klinische Anwendung.

In recent years lengthening of the human mandible by distraction osteogenesis has become an accepted treatment for correction of severe mandibular disto-basal discrepancies. Using extra-oral and intra-oral distraction devices the technique of osseous anchorage is usually preferred. To avoid the disadvantages of this method it is the aim of many research groups to develop a tooth-borne distraction. In the present animal study, six minipigs were treated with a new solely dentally fixed orthodontic device for mandibular distraction osteogenesis. Following bilateral osteotomy in the dentigerous area of the mandibular body and a latent period of 2-7 days, a mandibular lengthening of 9 mm was reached within a period of 9 days. This situation was retained using the distraction device for 6 weeks. After the removal of the apparatus, there followed a period of another 6 weeks of consolidation. The callus and bone formation and potential dental, periodontal and nerval reactions were radiologically examined and histological examination was performed at the end of the experiment. At 12 weeks after the mandibular lengthening, the osteogenesis in the distraction areas was complete. No destructive processes were recognizable, either at the roots or at the periodontium of the teeth near the osteotomy gap. The mandibular nerve at the osteotomy site remained intact. The results justified transferring this procedure to humans. The clinical application of the device is presented.

[Postoperative pain after interventions in the area of the mouth-jaw-face]. / Postoperative Schmerzen nach Eingriffen im Mund-Kiefer-Gesichts-Bereich.

In order to establish effective postoperative analgesia we studied the incidence and significance of pain following maxillofacial surgery. The trial included 102 patients undergoing one of six different surgical procedures. Postoperative pain was assessed using a visual analogue scale (VAS) and the short form of the McGill Pain Questionnaire (SF-MPQ) up to the third postoperative day. Postoperative pain intensity was significantly correlated to operating time, the frequency of analgesic demand and the type of surgery (orthognathic surgery > TMJ surgery > osteosynthetic surgery > osteotomy of impacted third molars > tumor resection > removal of osteosynthetic materials). Patient's age, sex and ethnic origin did not significantly affect the severity of postoperative pain.

[High condylectomy for control of pathological growth in condylar hyperplasia]. / Die hohe Kondylektomie zur Ausschaltung überschiessenden Wachstums bei der kondylären Hyperplasie.

With the aim of eliminating pathological growth during the active period of condylar hyperplasia, 17 patients were treated with a high condylectomy with a retroauricular incision. Postoperatively none of the patients showed signs of continuing growth activity neither clinically nor roentgenologically. Thus, the high condylectomy can be recommended as a reliable technique to stop pathological and untimely growth with a low risk of complications, before occlusion and skeletal asymmetry are corrected by orthodontic surgery.

Mandibular distraction osteogenesis. Preliminary results of an animal study with a dentally fixed distraction device.

Lengthening of the human mandible by distraction osteogenesis has become an accepted treatment for correction of severe mandibular disto-basal discrepancies in recent years. This usually entails the deployment of extra-orally located distraction devices. This type of device often causes conspicuous scarring. To avoid this scarring, an intra-oral device is to be developed. In the present animal study, 2 "Troll" mini-pigs and 2 "Yucatan" micro-pigs were fitted with a new solely dentally fixed orthodontic device for mandibular distraction osteogenesis. Following bilateral osteotomy in the dentigerous area of the mandibular body and a latent period of 2 to 7 days, the apparatus was activated 1 mm per day. Within a period of 9 days, a mandibular lengthening of 9 mm was reached. This situation was retained using the distraction device for 6 weeks. After removal of the apparatus, there followed another 6-week period of consolidation. During the experiment, the callus and bone formation and potential dental and periodontal reactions were radiologically examined and underwent histological examination at the end of the experiment. Twelve weeks after the mandibular lengthening, the osteogenesis in the distraction areas was complete. No destructive processes were recognizable, either at the roots or at the periodontium of the teeth near the osteotomy gap. Lengthening of the mandibular body using distraction osteogenesis with the help of a solely dentally fixed distraction device is thus feasible and can, in the future, become the appropriate treatment in the correction of severe mandibular deficiency.