Diagnostic accuracy of bone SPECT and SPECT/CT imaging in the diagnosis of unilateral condylar hyperplasia: A systematic review and meta-analysis.

Imaging with bone scans plays an important role in the diagnostic path of patients with unilateral condylar hyperactivity or unilateral condylar hyperplasia (UCH). The aim of this study is to perform a systematic review of the diagnostic performance of the bone SPECT and SPECT/CT scan for the diagnosis of UCH. PubMed, SCOPUS and EMBASE were searched electronically to identify diagnostic accuracy studies that assessed the diagnostic value of bone SPECT and SPECT/CT for the diagnosis of UCH, Meta-analyses were performed with Metadisc 1.4 and 2.0. A total of 14 studies, with a total number of 887 patients, were included in the qualitative analysis and 11 studies qualified for meta-analyses. The pooled sensitivity and specificity for the SPECT scan were 0.814 (95 % CI: 0.639-0.915) and 0.774 (95 % CI: 0.655-0.861), for the SPECT/CT scan these were 0.818 (95 % CI: 0.749-0.874) and 0.901 (95 % CI: 0.840-0.945). The summary receiver operating characteristics of the SPECT scan showed an area under the curve of 0.847 (95 % CI: 0.722-0.972) and that of the SPECT/CT scan was 0.928 (95 % CI: 0.876-0.980). CONCLUSION: Both bone SPECT scan and SPECT/CT scan provide a high diagnostic accuracy for UCH. The added value of the SPECT/CT scan is questionable and given the potential disadvantages of the SPECT/CT scan, including the increased radiation dose and costs, the diagnostic modality of first choice in patients with UCH should be a SPECT scan.

The learning curve of transoral condylectomy; a retrospective analysis of 100 consecutive cases of unilateral condylar hyperplasia.

In this study, 100 consecutive scheduled transoral condylectomies for unilateral condylar hyperplasia were included. The safety and surgical performances were assessed, using the operating time, conversion rate and complication rate. The conversion rate learning curve was evaluated with a learning curve cumulative summation (LC-CUSUM). The total conversion rate was 8.0%. The LC-CUSUM for conversion signaled at the 53th procedure, indicating sufficient evidence had accumulated that the surgeon had achieved competence. For procedures 54-100, the conversion rate was 4.0%. The operating time for the transoral condylectomy was 41.5 ± 15.3 min; when a conversion was necessary, the operating time was 101.4 ± 28.3 min (p < 0.05). The estimated operating time in the post-learning phase was 37 min, this was reached after approximately 47 procedures. There was 1 major complication of a permanent inferior alveolar nerve hypoesthesia. The complication rate was not significantly decreased after the learning curve. Within the limitations of the study, it seems that transoral condylectomy for UCH is a safe procedure with several advantages over the traditional preauricular approach. Surgeons starting this procedure should be aware of the potential complications and of the learning curve of approximately 53 procedures.

Diagnostic performance of SPECT-CT imaging in unilateral condylar hyperplasia.

Nuclear imaging plays an important role in the diagnostic path of patients with unilateral condylar hyperplasia (UCH). The purpose of this study was to determine the performance of single-photon emission computed tomography-computed tomography (SPECT-CT) in a large group of patients with suspected UCH. This study prospectively included 156 patients with a clinical presentation of progressive mandibular asymmetry. All patients underwent 99 mTc-HDP SPECT-CT and extensive baseline and follow-up documentation. The relative activity of the ipsilateral condyle in relation to the contralateral condyle was calculated for both the mean and maximum count, and the diagnostic accuracy of different cut-off values was determined. The area under the receiver operating characteristic curve of the SPECT-CT scan was 0.892 for the mean count and 0.873 for the maximum count. The optimal cut-off of> 8% (SPECT-CT mean count) resulted in a sensitivity of 87.0% and a specificity of 88.6%. SPECT-CT showed good diagnostic performance in UCH; however the benefit of the CT scan is questionable and the potential disadvantages have to be weighed against the benefits when compared to standard SPECT scanning. When using SPECT-CT in the diagnostic path in UCH, a mean value cut-off of>8% for the relative activity between the condyles is most accurate.

Molecular basis of unilateral condylar hyperplasia?

Unilateral condylar hyperplasia (UCH) causes progressive asymmetry of the mandible. The aetiology of this growth disorder is unknown. A two-centre prospective study was established, and 10 consecutive adult UCH patients scheduled for high condylectomy were included. The resected condylar tissue was divided into two parts, one for regular histopathology and one for DNA extraction. A panel of eight selected overgrowth genes (AKT1, AKT3, MTOR, PIK3CA, PIK3R2, PTEN, TSC1, TSC2) were sequenced using next-generation sequencing, with coverage of a minimum 500 times in order to be able to detect low-grade mosaicisms. Subsequently, untargeted whole exome sequencing (WES) was performed to detect variants in other genes present in three or more patients. No mutation was detected in any of the overgrowth genes, and untargeted exome sequencing failed to detect any definitively causative variant in any other gene. Ten genes had a rare variant in three or more patients, but these cannot be designated as causative without additional functional studies. The hypothesis that the cause in at least some patients with UCH is a somatic mutation in a gene that controls cell growth could not be confirmed in this study.

Unilateral condylar hyperplasia in hemifacial hyperplasia, is there genetic proof of overgrowth?

Hemifacial hyperplasia (HFH) is characterized by an increase in volume of all affected tissues of half of the face. It is present at birth, subsequently grows proportionally, and stops growing before adulthood. Unilateral condylar hyperplasia (UCH) consists of progressive asymmetric growth of the mandible and develops typically in early adulthood. Both disorders have an unknown aetiology. The overgrowth limited to one body part suggests somatic mosaicism, as this has been found in other similar localized overgrowth disorders. Often this includes a variant in a gene in the (PIK3CA)/PI3K/(PTEN)/AKT1/mTOR pathway. Here we report the case of an HFH patient with asymmetry present at birth, in whom a progressive growth pattern similar to UCH subsequently occurred, causing marked mandibular asymmetry. A condylectomy was successfully performed to stop the progressive growth. Somatic mosaicism for a mutation in PIK3CA was detected in the condylar tissue. This finding might indicate that both HFH and UCH can be caused by variants in genes in the (PIK3CA)/PI3K/(PTEN)/AKT1/mTOR pathway, similar to other disorders that result in asymmetrical bodily overgrowth.

Demographic features in Unilateral Condylar Hyperplasia: An overview of 309 asymmetric cases and presentation of an algorithm.

PURPOSE: Unilateral Condylar Hyperplasia (UCH) is an acquired deformity of the mandible, which can highly influence the symmetry of the face due to its progressive nature. It is caused by growth resembling pathology in one of the mandibular condyles. Definition as well as classification is subject to discussion. The aim of this study is to evaluate a large cohort of alleged UCH patients, and to describe the clinical characteristics, demographic features, classification and follow up. Secondly an algorithm is presented, in order to achieve uniformity in diagnosis and treatment. PATIENTS AND METHODS: From 1994 to 2014 a database of consecutive patients from 3 maxillofacial departments (Academic Medical Center, Amsterdam; VU Medical Center, Amsterdam and Spaarne Gasthuis, Haarlem) with suspected UCH was set up. Patients were referred by orthodontists, dentists, general practitioners or maxillofacial surgeons. Demographic features, bonescan outcomes, laterality, classification and follow-up were noted. Secondarily, all patients were retrospectively diagnosed by one surgeon (JWN), using available documentation. Missing data and follow-up were additionally retrieved from orthodontic offices. RESULTS: 394 asymmetric patients were evaluated. In 309 (78%) patients, the diagnosis UCH was justified and SPECT data were available. The mean age at presentation was 20.3 years (SD ± 7.7, range 9.0-54.5 years). In 48% of the patients, the bonescan was positive. 80% of these patients received surgical treatment, of which 62% were treated with a condylectomy only, 33% were treated with condylectomy plus additive corrective surgery, and 5% underwent corrective surgery only. Of the patient group without positive bonescan 42% of the patients received surgical treatment: 34% condylectomy only, 15% condylectomy plus additive corrective surgery, and 51% corrective surgery only. In total (N = 309) 96 (31%) patients underwent condylectomy as only surgical treatment and 124 (40%) patients received no surgical treatment at all. Treatment could be finalized with orthodontic treatment without further surgery in 64% and 41% respectively. 96 patients were subject to comparison of the classification as noted by the clinician and the author (JWN). In only 72% of the cases, the secondary screening was in agreement with the initial classification. CONCLUSION: Based on this study not all (active) UCH patients require corrective (orthognathic) surgery. A (transoral) partial condylectomy for active patients is recommended, with a postoperative remodeling period of 6 months with or without orthodontic treatment. Second stage correcting surgery may be necessary upon evaluation, using general orthognathic diagnostic and planning procedures. It appears difficult to classify patients reliably using the available clinical and radiological documentation. Objectivity and quantification in the diagnostic process is necessary: uniformity in documentation and parameters. The attached documentation form and UCH treatment algorithm is recommended.

Fulminant presentation of oral mucosal leishmaniasis as severe stomatitis and periodontitis.

This case report shows an atypical presentation of mucosal leishmaniasis infantum in the oral cavity resulting in severe stomatitis and periodontitis. The patient was immunocompromised because of rheumatoid arthritis for which he used prednisone and methotrexate. He was treated with intravenous liposomal amphotericin B and recovered within four weeks.

Panoramic imaging is not suitable for quantitative evaluation, classification, and follow up in unilateral condylar hyperplasia.

Patients with suspected unilateral condylar hyperplasia are often screened radiologically with a panoramic radiograph, but this is not sufficient for routine diagnosis and follow up. We have therefore made a quantitative analysis and evaluation of panoramic radiographs in a large group of patients with the condition. During the period 1994-2011, 132 patients with 113 panoramic radiographs were analysed using a validated method. There was good reproducibility between observers, but the condylar neck and head were the regions reported with least reliability. Although in most patients asymmetry of the condylar head, neck, and ramus was confirmed, the kappa coefficient as an indicator of agreement between two observers was poor (-0.040 to 0.504). Hardly any difference between sides was measured at the gonion angle, and the body appeared to be higher on the affected side in 80% of patients. Panoramic radiographs might be suitable for screening, but are not suitable for the quantitative evaluation, classification, and follow up of patients with unilateral condylar hyperplasia.

Microcomputed tomographic analysis of human condyles in unilateral condylar hyperplasia: increased cortical porosity and trabecular bone volume fraction with reduced mineralisation.

Unilateral condylar hyperplasia or hyperactivity is a disorder of growth that affects the mandible, and our aim was to visualise the 3-dimensional bony microstructure of resected mandibular condyles of affected patients. We prospectively studied 17 patients with a clinical presentation of progressive mandibular asymmetry and an abnormal single-photon emission computed tomographic (SPECT) scan. All patients were treated by condylectomy to arrest progression. The resected condyles were scanned with micro-CT (18 µm resolution). Rectangular volumes of interest were selected in 4 quadrants (lateromedial and superoinferior) of the trabecular bone of each condyle. Variables of bone architecture (volume fraction, trabecular number, thickness, and separation, degree of mineralisation, and degree of structural anisotrophy) were calculated with routine morphometric software. Eight of the 17 resected condyles showed clear destruction of the subchondral layer of cortical bone. There was a significant superoinferior gradient for all trabecular variables. Mean (SD) bone volume fraction (25.1 (6) %), trabecular number (1.69 (0.26) mm(-1)), trabecular thickness (0.17 (0.03) mm), and degree of mineralisation (695.39 (39.83) mg HA/cm(3)) were higher in the superior region. Trabecular separation (0.6 (0.16) mm) and structural anisotropy (1.84 (0.28)) were higher in the inferior region. The micro-CT analysis showed increased cortical porosity in many of the condyles studied. It also showed a higher bone volume fraction, greater trabecular thickness and trabecular separation, greater trabecular number, and less mineralisation in the condyles of the 17 patients compared with the known architecture of unaffected mandibular condyles.

[Unilateral condylar hyperactivity]. / Unilaterale condylaire hyperactiviteit.

Unilateral condylar hyperactivity is a growth disorder which is characterised by a progressive asymmetry in the mandibula and in some cases also secondarily in the maxilla. Various forms are hemimandibular hyperplasia, hemimandibular elongation and a hybridform. In deciding on a plan of treatment, it is important to determine whether there is a question of continuous and/or excessive condylar activity, possibly with the help of a skeletal scintigraphy.

[A gingival hyperplasia in a patient suffering from neurofibromatosis]. / Een gingivahyperplasie op basis van neurofibromatose.

A 30-year-old woman suffering from neurofibromatosis type 1 was referred by her dentist to the department of oral and maxillofacial surgery of a university medical centre for excision of a gingival hyperplasia in the mandibular frontal region. The hyperplasia was a neurofibroma, which was surgically removed, as were 2 neurofibromas of the tongue, a postauricular neurofibroma and 2 neurofibromas of the feet.

[Surprising white lesions]. / Verrassende witte laesies.

A 46-year-old man appeared with white lesions of the oral cavity. A previously taken biopsy revealed no classifying diagnosis and treatment with mouth rinse produced no improvement. A new biopsy was taken, on which the pathologist performed additional tests. This resulted in the diagnosis 'syphilis'. The patient was treated with benzylpenicillin and the oral white lesions disappeared. Although nowadays syphilis is rare, special attention is required when noticing these kinds of lesions of the oral cavity.