Retrospective Analysis of Whole-Body Magnetic Resonance Imaging of Bone Manifestations in Long-Term Treated Patients with Gaucher Disease Type 1.

PURPOSE: We retrospectively assessed bone and visceral manifestations in patients with Gaucher disease type 1 (GD1) with whole-body magnetic resonance imaging (WB-MRI) to determine the effects of different timing in initiating long-term enzyme replacement therapy. MATERIALS AND METHODS: In 17 patients with GD1, we performed 2 WB-MRI examinations at a median interval of 13 months. Patients had received enzyme replacement therapy with alglucerase/imiglucerase for a median of 13 years prior to the first examination. MRI results were retrospectively stratified based on treatment initiation into 2 groups: "early" (age ≤12 years, median 5 years) and "late" (during adulthood, median 32 years). We evaluated occurrence of irreversible avascular necroses (AVN) and applied several semi-quantitative scores, including the Bone-Marrow-Burden (BMB) score, the Düsseldorf-Gaucher score (DGS), the Vertebra-Disc-Ratio (VDR), and the Gaucher disease type 1 Severity Scoring System (GD-DS3). RESULTS: MRI assessments showed no AVN in the "early" group. AVN were observed in 2 patients of the "late" group; one also had a splenic Gaucheroma. The follow-up examinations showed slight improvements in the BMB-score, DGS, and VDR, with similar tendencies in both treatment groups. The GD-DS3 score only improved in "late" group. CONCLUSION: This retrospective study supported the ongoing clinical value of enzyme replacement therapy with alglucerase/imiglucerase, as WB-MRI-based scores stayed constant or slightly improved even after long-term treatment. Secondary complications were only observed in the late treatment group. Our results suggest that "early initiation" of enzyme replacement therapy may protect the bone.

Quantification of intramuscular fat in patients with late-onset Pompe disease by conventional magnetic resonance imaging for the long-term follow-up of enzyme replacement therapy.

OBJECTIVE: The objective of this study was to evaluate a quantitative method based on conventional T1-weighted magnetic resonance (MR) imaging to assess fatty muscular degeneration in patients with late-onset Pompe disease and to compare it with semi-quantitative visual evaluation (the Mercuri score). In addition, a long-term retrospective data analysis was performed to evaluate treatment response to enzyme replacement therapy with alglucosidase alfa. METHODS: MR images of the lumbar spine were acquired in 41 patients diagnosed with late-onset Pompe disease from 2006 through 2015. Two independent readers retrospectively evaluated fatty degeneration of the psoas and paraspinal muscles by applying the Mercuri score. Quantitative semi-automated muscle and fat tissue separation was performed, and inter-observer agreement and correlations with clinical parameters were assessed. Follow-up examinations were performed in 13 patients treated with alglucosidase alfa after a median of 39 months; in 7/13 patients, an additional follow-up examination was completed after a median of 63 months. RESULTS: Inter-observer agreement was high. Measurements derived from the quantitative method correlated well with Medical Research Council scores of muscle strength, with moderate correlations found for the 6-minute walk test, the 4-step stair climb test, and spirometry in the supine position. A significant increase in the MR-derived fat fraction of the psoas muscle was found between baseline and follow-up 1 (P = 0.016), as was a significant decrease in the performance on the 6-minute walk test (P = 0.006) and 4-step stair climb test (P = 0.034), as well as plasma creatine kinase (P = 0.016). No statistically significant difference in clinical or MR-derived parameters was found between follow-up 1 and follow-up 2. CONCLUSIONS: Quantification of fatty muscle degeneration using the semi-automated method can provide a more detailed overview of disease progression than semi-quantitative Mercuri scoring. MR-derived data correlated with clinical symptoms and patient exercise capacity. After an initial worsening, the fat fraction of the psoas muscle and performance on the 6-minute walk test stayed constant during long-term follow-up under enzyme replacement therapy.

Ultrasound Evaluation of Thyroid Gland Pathologies After Radiation Therapy and Chemotherapy to Treat Malignancy During Childhood.

PURPOSE: The purpose of this study was to evaluate correlations between treatment of malignancy by radiation therapy during childhood and the occurrence of thyroid gland pathologies detected by ultrasonography in follow-up examinations. METHODS AND MATERIALS: Reductions of thyroid gland volume below 2 standard deviations of the weight-specific mean value, occurrence of ultrasonographically detectable thyroid gland pathologies, and hypothyroidism were retrospectively assessed in 103 children and adolescents 7 months to 20 years of age (median: 7 years of age) at baseline (1997-2013) treated with chemoradiation therapy (with the thyroid gland dose assessable) or with chemotherapy alone and followed by ultrasonography and laboratory examinations through 2014 (median follow-up time: 48 months). RESULTS: A relevant reduction of thyroid gland volume was significantly correlated with thyroid gland dose in univariate (P<.001) and multivariate analyses for doses above 2 Gy. Odds ratios were 3.1 (95% confidence interval: 1.02-9.2; P=.046) for medium doses (2-25 Gy) and 14.8 (95% confidence interval: 1.4-160; P=.027) for high doses (>25 Gy). Thyroid gland dose was significantly higher in patients with thyroid gland pathologies during follow-up (P=.03). Univariate analysis revealed significant correlations between hypothyroidism and thyroid gland dose (P<.001). CONCLUSIONS: Ultrasonographically detectable changes, that is, volume reductions, pathologies, and hypothyroidism, after malignancy treatment during childhood are associated with thyroid gland dose. Both ultrasonography and laboratory follow-up examinations should be performed regularly after tumor therapy during childhood, especially if the treatment included radiation therapy.

Advantages and limits of 3-segment (paramedian) versus 2-segment (median) surgically assisted rapid maxillary expansion (SARME).

BACKGROUND: A novel maxillary 3-segment osteotomy (3SO) was evaluated versus the conventional 2-segment-osteotomy (2SO) for surgically assisted rapid maxillary expansion. 3SO was performed bilaterally between the lateral incisors and canines at the former junction of the lateral alveolar processes with the premaxilla. STUDY DESIGN: Of 98 included patients (n = 53 retrospectively and n = 45 prospectively; 68 complete datasets), 47 patients' preoperative and postexpansion cast models were evaluated for transverse maxillary dental and skeletal expansion, dental tipping, attachment loss, and front teeth angulation, and on photographs the pink esthetic score (PES) was assessed preoperatively versus postoperatively. RESULTS: Three-SO expanded to a larger extent with more symmetry than 2SO, at the price of more dental tipping and front teeth angulation (independent t test). The PES showed better esthetic outcomes for 3SO (Mann-Whitney U test). CONCLUSIONS: Disadvantages of 2SO, such as median diastema, midline shift, median papilla infringement, asymmetric expansion, septum and columella dislocation, high forces on the periodontal apparatus, and subsequent attachment loss were overcome with 3SO. Bilateral double osteotomies appeared to promote higher volumes of callus formation, thus preventing relapses.

Segmental stability of resorbable P(L/DL)LA-TMC osteosynthesis versus titanium miniplates in orthognatic surgery.

After two decades of the use of resorbable miniplates, new polymer compositions for resorbable osteosynthesis are still being developed to make the handling and outcome of operations even more predictable and give higher stability to the repositioned segments. This study investigates a new resorbable osteosynthesis system in orthognathic patients. 50 patients were treated with P(L/DL)LA-TMC resorbable osteosynthesis and compared to a group of 50 patients treated with titanium miniplates. Segmental stability and relapse were measured comparing preoperative, postoperative and follow-up lateral cephalograms. Throughout this study, resorbables appeared to be as stable as titanium miniplates except in maxillary elongation and mandibular setback. Here, the titanium miniplates showed significantly higher stability than resorbable plates. P(L/DL)LA-TMC osteosynthesis seem to have less strength against compressive forces after maxillary elongation and they are less resistant to the forces the tongue exerts, pressing against the mandible after setback. It can therefore be concluded that the resorbable osteosynthesis can be used in the same situations as titanium miniplates except in maxillary elongation and mandibular setback.

Patient satisfaction after prosthetic rehabilitation of bone-grafted alveolar clefts with nonsubmerged ITI Straumann dental implants loaded at three months.

OBJECTIVE: To assess clinical, aesthetic, and quality-of-life parameters of cleft lip and palate patients' implant-supported crowns with ITI Straumann nonsubmerged and loaded after 3 months implants. PATIENTS, MATERIAL, AND METHODS: In 17 bone-grafted cleft patients, 24 implants were placed nonsubmerged, loaded at 3 months to support crowns in the cleft area, and followed up at 40 months on average (± 22 months). The oral-health-related quality of life was assessed in all patients with the German Oral Health Impact Profile after implant therapy. Aesthetics were judged on photographs by dental professionals, nonprofessionals, and the patients themselves. The Implant Crown Aesthetic Index served as an objective tool. RESULTS: A total of 23 implants (95.8%) were loaded and in function. Clinical parameters were marginal bone loss of 1.15 ± 2.16 mm, peri-implant probing depth of 2.56 ± 0.66 mm, width of keratinized mucosa of 2.71 ± 1.1 mm, mucosal recession of 0.41 ± 0.8 mm, Periotest value of 0.18 ± 6.5, and German Oral Health Impact Profile summary score of ≤ 2. The best aesthetic outcome was assessed by the patients themselves. Soft tissues were rated worse than were implant-supported crowns. CONCLUSIONS: Implant-borne prosthetic rehabilitation of bone-grafted clefts with nonsubmerged ITI Straumann implants with 3 months' loading represents a reliable treatment option with high success rates in the long term. Functional aspects are comparable to those of noncleft patients. The oral-health-related quality of life of cleft patients is similar to that of noncleft patients. Aesthetics are restricted but satisfactory to patients and dental professionals. Aesthetics of peri-implant soft tissues should be improved in the future.

Assessment of surgically assisted rapid maxillary expansion regarding pterygomaxillary disjunction using thin volume-rendering technique: in variance analysis and in reliability, accuracy, and validity.

PURPOSE: To assess surgically assisted rapid maxillary expansion (SARME) with or without pterygomaxillary disjunction using a thin volume-rendering technique in variance analysis and in reliability, accuracy, and validity. MATERIALS AND METHODS: Thin volume-rendered images of 68 patients were evaluated preoperatively and 2.87 ± 1.59 months after expansion with respect to dentoskeletal effects. RESULTS: Variance analysis of SARME with pterygomaxillary disjunction showed an important decrease in transverse widening and segmental outward inclination and an increase in vestibular bone plate thickness (premolars) in patients younger than 20 years with bone-borne devices; the greatest increase in transverse widening was in patients with 3-segment osteotomy and tooth-borne devices. Analysis of SARME without pterygomaxillary disjunction showed an important decrease in transverse widening and segmental inward inclination in patients older than 20 years with bone-borne devices; the greatest pterygoid lateral bending was in patients with 2-segment osteotomy and bone-borne devices. CONCLUSION: The performance of pterygomaxillary disjunction should depend on patient age (ie, treatment with pterygomaxillary disjunction in those >20 years old; treatment without pterygomaxillary disjunction in those <20 years old). Patients with pterygomaxillary disjunction, 3-segment osteotomy, and tooth-borne devices tended to show an increase in transverse widening but at the price of greater attachment loss. Patients younger than 20 years with pterygomaxillary disjunction and bone-borne devices tended to show an increase in vestibular bone plate (premolars) but at the price of decrease in transverse widening.

Patients' preoperative expectations and postoperative satisfaction of dysgnathic patients operated on with resorbable osteosyntheses.

BACKGROUND: This study evaluated whether personal expectations and satisfaction throughout orthognathic surgery were fulfilled. In addition, patients were interrogated about their experience of resorbable osteosynthesis. METHODS: A total of 50 patients were interviewed 3 times each throughout the study by a mixed questionnaire of standard psychologic tests and a tailored itemized questionnaire regarding their expectations regarding resorbable osteofixation and their postoperative satisfaction. RESULTS: A postoperative increase in self-esteem and approach to life were evident. An examination of Oral Health-Related Quality of Life showed constant quality of life; an examination of Oral Health Impact Profile-Germany) showed no postoperative difficulties in dental hygiene and nutrition. No statistically significant change in any of the tests could be expressly determined. Avoidance of secondary surgery motivated 94% to choose resorbable osteofixations, although a mere 66% had heard of them before; 90% of patients were satisfied with the operation result. CONCLUSIONS: Orthognathic surgery cannot change preexistent depression or a problematic social background. Mastication and oral health improved, and postoperative happiness and confidence increased. When given the choice between resorbable fixation and titanium osteofixation, patients generally preferred resorbable fixations.

Long-term 3D cast model study: bone-borne vs. tooth-borne surgically assisted rapid maxillary expansion due to secondary variables.

OBJECTIVE: This study aims to compare bone-borne (BB) surgically assisted rapid maxillary expansion (SARME) to tooth-borne (TB) SARME with regard to dentoskeletal effects. PATIENTS AND METHODS: Measurements were performed on 3D scanned cast models of 34 patients preoperatively and 20.5 +/- 1.34 months post-expansion. Secondary variables were pterygomaxillary disjunction or not; bimaxillary osteotomy or not and patient age. RESULTS: BB SARME without pterygomaxillary disjunction on patients <20 years led to a symmetric increase in transverse widening; however, simultaneously, to more dental tipping (canines/second molars) and to the biggest overall attachment loss (frontal teeth/premolars), especially, after the performance of bimaxillary osteotomy. TB SARME led to an asymmetric decrease in transverse widening and to more dental tipping (premolars/first molars). CONCLUSION: In long-term effects, BB SARME led to a symmetric increase in transverse widening; however, at the price of frontal attachment loss, resulting from the initial asymmetric, but bigger, overall transverse widening, the initial major segmental rotation and the necessary orthodontics for tooth arch alignment before and after bimaxillary osteotomy.

Comparison of bipartite versus tripartite osteotomy for maxillary transversal expansion using 3-dimensional preoperative and postexpansion computed tomography data.

PURPOSE: To evaluate tripartite paramedian versus bipartite median osteotomy in surgically assisted rapid maxillary expansion. Tripartite osteotomy was performed between the lateral incisors and canines at the former premaxillary junction to avoid midline diastema, septal and columellar dislocation, and asymmetric expansion, minimizing high-distraction forces through bilateral doubled osteotomy on periodontia and thus reducing vestibular attachment loss and producing stable callus formation to avoid relapse. PATIENTS AND METHODS: The preoperative and postexpansion computed tomography data from 50 patients were analyzed in multiplanar viewing for bodily segment movement, vestibular bone loss, and transverse skeletal and dental widening with predefined landmarks. Of the 50 patients, 22 had undergone tripartite and 28 had undergone bipartite osteotomy. RESULTS: Using an independent t test, both osteotomies permitted adequate transverse skeletal expansion in the premolars, converging, however, in the molars. Bipartite osteotomy resulted in less symmetry in transverse skeletal widening, greater bodily segment movement in the first premolar/molar, and greater vestibular bone loss. Tripartite osteotomy resulted in greater overall expansion and less bone remodeling. On variance analysis, tripartite bone-borne distraction resulted in the greatest decrease of transverse expansion in patients older than 20 years. The tripartite osteotomy also provoked distractor- and age-independent outward segmental movement. Bipartite osteotomy resulted in distractor- and age-independent inward segmental movement. Bipartite osteotomy showed the greatest bone resorption in patients younger than 20 years old in the molars and tripartite osteotomy in patients older than 20 years in the premolars. CONCLUSIONS: Tripartite paramedian osteotomy allowed greater overall symmetric expansion compared with bipartite median osteotomy, with, however, a decline in transverse widening to the posterior. Bipartite osteotomy should be preferred whenever good periodontal status permits greater vestibular bone loss and a midline diastema and asymmetric expansion and a midline shift will be tolerated by the patient. Tripartite osteotomy should be chosen whenever a midline diastema and shift, septal and columellar dislocation, asymmetric expansion, and larger distraction forces on the paradontia, resulting in vestibular attachment loss, are to be avoided.

Evaluation of surgically assisted rapid maxillary expansion with or without pterygomaxillary disjunction based upon preoperative and post-expansion 3D computed tomography data.

UNLABELLED: PURPOSE, METHODS: This study (50 patients; Ø 25 years) compared surgically assisted rapid maxillary expansion (SARME) with (+/-PP) to SARME without pterygomaxillary (-PP) disjunction due to dentoskeletal effects in 3D CT preoperatively and Ø 11 weeks post-expansion. RESULTS: In t test, SARME-PP declined in transverse width from anterior to posterior but more symmetrically than SARME+PP. It produced more segmental inclination and vestibular bone resorption in the premolars. SARME+PP also declined in transverse width from anterior to posterior but more asymmetrically with an extreme convergence to the molars. It produced more segmental inclination and vestibular bone resorption (second molar) in the molars and a palatal bone plate thickness increase in the second premolar. With variance analysis, a further differentiation between the two independent groups due to secondary variables was made: SARME+PP produced the biggest decline in transverse width (patients <20 years) and the biggest segmental outward inclination from anterior to posterior in patients with bone-borne devices. SARME-PP in patients <20 years and SARME+PP in patients >20 years both produced the biggest lateral pterygoid bending. CONCLUSION: Pterygomaxillary disjunction should be based on patient age and individual requirements, i.e., in patients <20 years (SARME-PP) and >20 years (SARME+PP).

Comparison of tooth- and bone-borne devices in surgically assisted rapid maxillary expansion by three-dimensional computed tomography monitoring: transverse dental and skeletal maxillary expansion, segmental inclination, dental tipping, and vestibular bone resorption.

Comparison of bone-borne (BB) versus tooth-borne (TB) distraction in surgically assisted rapid maxillary expansion, secondary variables were bipartite (2S) or tripartite (3S) osteotomy, pterygomaxillary osteotomy (+PP) or not (jPP), and age (920 years old [920] and G20 years old [G20]). Fifty patients received three-dimensional computed tomography examination preoperatively and after expansion; 24 had BB, and 26 had TB distraction. Predefined preoperative landmarks versus postexpansion positions were assessed in multiplanar viewing. Bone-borne devices had bigger overall skeletal and dental maxillary expansion declining from anterior to posterior, more asymmetric expansion, less vestibular bone resorption, and less dental tipping; TB devices showed similar dorsal decrease in transverse skeletal and dental maxillary expansion from anterior to posterior, more vestibular bone resorption, particularly in the premolars, and more dental tipping. Bone-borne 3S +PP G20 had the biggest decrease in transverse skeletal widening and biggest segmental outward inclination; BB 3S +PP 920 had the biggest decline in transverse dental widening; TB 2S YPP G20 had the biggest segmental inward inclination; BB 2S YPP 920 had the biggest dental tipping; BB 2S G20 had the biggest vestibular bone resorption in molars; and TB 3S 920 and BB YPP had the biggest vestibular bone resorption in premolars. There was a significant correlation between transverse widening and segmental inclination; that is, a bigger amount of transverse widening provokes less segmental inclination.

Prospective changes to condylar position in symphyseal distraction osteogenesis.

OBJECTIVE: To evaluate whether symphyseal distraction for correction of mandibular transverse deficiencies and dental crowding provokes lateral shift of the condyles, because this may have secondary influence upon temporomandibular joint function. STUDY DESIGN: Nine patients' routine pre- and postoperative computerized tomography scans were evaluated for intercanine distance and condylar position before and 3 months after mandibular symphyseal distraction osteogenesis (MSDO). Identical bone-borne distraction was applied in all cases at 0.6 mm distraction per day, starting 5 days after surgery. RESULTS: Intercanine distance increased effectively by 3.8 +/- 1.8 mm (P = .004), condylar distance decreased by 1.2 +/- 0.8 mm (P = .02), condylar angulation decreased by 0.03 +/- 4.3 degrees (P = .7), and lateral, cranial, and inner condylar cortex to fossa distances changed by 0.4 +/- 0.5 mm (P = .8), 0.4 +/- 0.5 mm (P = .7), and 0.4 +/- 0.3 mm (P = .4), respectively. CONCLUSIONS: MSDO causes no lateral displacement and rather a decrease of condylar transversal distance whereas angulation, vertical medial, cranial, and lateral distance to the fossa remain unchanged. This finding asserts the clinical absence of temporo-mandibular dysfunction associated with MSDO as reported in the literature, even though soft tissue changes and long-term outcomes require further follow-up studies.

Bone harvesting at the anterior iliac crest using piezoosteotomy versus conventional open harvesting: a pilot study.

OBJECTIVE: The objective of this study was to evaluate piezoosteotomy for bone harvesting at the iliac crest in a pilot collective. STUDY DESIGN: Thirteen patients underwent anterior iliac crest bone graft harvesting using piezoosteotomy. These patients were compared to an age- and sex-matched retrospective cohort of 13 patients who underwent bone graft harvesting at the anterior iliac crest using conventional chisels and saws in an otherwise identical protocol. RESULTS: Harvested bone volumes and operation times were comparable; hospitalization time was briefer and pain levels at the first 2 postoperative days less in the piezoosteotomy group; pain medication requirement was comparable. Spearman's-rho correlation showed a strong tie between pain medication requirement and harvested bone volume in the piezoosteotomy group and between harvested bone volume and operation time in the conventional surgery collective. CONCLUSIONS: The correlation between required pain medication and harvested bone volume together with lower pain levels in the piezoosteotomy group indicate pain more exclusively related to local osseous damage than to correlated soft tissue lacerations. Conventional technique shows a correlation between operation time and harvested bone volume indicating a more time-effective procedure, although total operation time was comparable. Piezoosteotomy appears to have potential use in iliac crest bone harvesting.