Dentinogenic Ghost Cell Tumour in Childhood Involving the Temporomandibular Joint: Considerations and Therapy.

Background: Although the uncommon dentinogenic ghost cell tumour (DGCT) is a benign entity, it possesses the ability to cause widespread destruction of the jaws and to recur after bone-preserving therapy. Hence, clear margins should be achieved upon surgery, and reconstruction techniques must often be used to restore osseous defects. However, this can be challenging in cases with involvement of the temporomandibular joint (TMJ), and especially in children. Case report: We present a case of a DGCT in a 12-year-old boy with wide infiltration of the mandible and the TMJ. A two-staged reconstructive approach was performed. Upon primary surgery, tumour-free margins were obtained and mandibular anatomy was restored using an iliac crest graft and an alloplastic condyle implant for temporary TMJ reconstruction. In a second step 5 months later, having received a customized TMJ prosthesis consisting of a fossa and a condyle component, the TMJ was completely replaced for definitive reconstruction. Conclusion: A customized TMJ prosthesis could be a solution for reconstruction of the TMJ in children. However, the further course with respect to growth disturbances must be evaluated upon short-term follow-ups and might require additional corrective interventions.

Melanotic Neuroectodermal Tumour of Infancy: Clinical Courses and Therapeutic Options-A Review of Three Cases.

BACKGROUND: Melanotic neuroectodermal tumour of infancy (MNTI) is a rare benign neoplasm. MNTI appears most often during the first year of life, arises predominantly in the maxilla and tends to recur. We discuss possible therapeutic options given in the literature and within our experience in three cases. PATIENTS: In our recent case, we used an intraoral approach to perform resection of the right-sided maxilla. Despite tumour-positive margins, there was no recurrence over the course of one year. In a previous case of MNTI, two recurrences occurred and 6 months after last resection patient received a rib graft for maxillary reconstruction. However, at the age of 7 years, the infant displayed severe maxillary hypoplasia. In a third case of MNTI, the patient was followed up after initial therapy for two decades and underwent multiple reconstruction procedures to achieve successful rehabilitation. CONCLUSION: Surgical treatment of MNTI should respect vital anatomic structures to avoid gross mutilation. The need for extended and repetitive tumour resection in early childhood can lead to growth disturbances and to further multiple reconstruction procedures in adulthood. Because of the rarity of MNTI, an international database is warranted to evaluate therapies and clinical courses over decades.

Case report: chronic inflammatory ulcer and osteoradionecrosis of the skull following radiotherapy in early childhood.

INTRODUCTION: Chronic inflammatory diseases of the skin are the most common differential diagnosis of tumorous lesions of the craniofacial region. Detailed information about a patient's medical history is important for the clinical diagnosis of such cases. Previous radiotherapy should be taken into account, especially in cases of chronic dermatitis, since complications include osteoradionecrois of the adjacent bone strucutres with surrounding inflammation. CASE REPORT: We present the case of a 77-year-old femal patient who was admitted to our department with a slightly progressive ulcerating lesion of the frontotemporal skull. The patient had received radiotherapy in early childhood as primary therapy for hemangioma. Diagnostic imaging and biopsies revealed a diagnosis of chronic ulceration with underlying osteonecrosis and fibrotic osteomyelitis of the skull. A complex reconstruction of osseous structures and soft tissue was necessary to resolve her complaints. CONCLUSION: Chronic radiodermatitis and osteoradionecrosis in adults, occurring as late complications, are uncommon, but can be observed even nearly 80 years after radiation. Large defects of the skull require a complete reconstruction to avoid several complications.

Total mandibular reconstruction following diffuse sclerosing osteomyelitis.

BACKGROUND: Diffuse sclerosing osteomyelitis (DSO) is a non-purulent chronic recurrent inflammation and affects the mandible in many cases. Belonging to the group of autoinflammatory diseases, in children and in cases with various additional symptoms including synovitis, acne, pustulosis, hyerostosis, and osteitis (SAPHO syndrome), therapy usually consists of non-surgical treatment. Against this background, we present an unusual course of DSO in an adult female patient. CASE REPORT: A 50-year-old female suffering from DSO without SAPHO syndrome was pretreated for years with conservative drug regimens and local surgery. Previous therapy was not successful, and subsequently, multiple surgical procedures were carried out focused on recurrent acute exacerbations of DSO. Surgery resulted in a total resection and alloplastic and autoplastic reconstruction of the mandible including both temporomandibular joints. Prosthetic rehabilitation was possible after dental implant loading, and the final outcome was very satisfactory. CONCLUSION: In the event that non-surgical options are not successful in DSO, an extended surgical therapy becomes necessary. Even if surgery results in complete resection of the mandible, a satisfactory rehabilitation can be achieved after complex reconstruction.

Continuity defects of the mandible: Comparison of three techniques for osseous reconstruction and their impact on implant loading.

Computer-aided design/manufacturing (CAD/CAM) is now widely used, but whether it can help to overcome complications in mandibular reconstruction and accelerate dental implantation is still a matter for debate. Therefore, we aimed to evaluate the benefits of this technique using vascularized iliac crest or fibula flaps in mandibular reconstruction, with respect to the time between reconstruction and implantation, and the ratio of planned to inserted implants. We reviewed retrospectively the records of 54 patients who underwent mandibular reconstructions between 2012 and 2016, and included in our study the last 10 cases representing each of the following groups: iliac crest flap with CAD/CAM (Group 1); fibula flap with CAD/CAM (Group 2); and fibula flap without CAD/CAM (Group 3). Groups 1 (p = 0.045) and 2 (p = 0.034) showed significantly shorter delays when compared with Group 3. Significant differences in average counts of implants placed were also found between Group 1 and Groups 2 (p = 0.04) and 3 (p = 0.019). The ratio of planned to placed implants was highest in Group 1. The observed differences between Group 1 and Groups 2 (p = 0.04) and 3 (p = 0.019) were significant. Our results indicate an accelerating effect of CAD/CAM on graft consolidation and dental rehabilitation.

Current concepts in cleft care: A multicenter analysis.

The current surgical techniques used in cleft repair are well established, but different centers use different approaches. To determine the best treatment for patients, a multi-center comparative study is required. In this study, we surveyed all craniofacial departments registered with the German Society of Maxillofacial Surgery to determine which cleft repair techniques are currently in use. Our findings revealed much variation in cleft repair between different centers. Although most centers did use a two-stage approach, the operative techniques and timing of lip and palate closure were different in every center. This shows that a retrospective comparative analysis of patient outcome between the participating centers is not possible and illustrates the need for prospective comparative studies to establish the optimal technique for reconstructive cleft surgery.

Moving the mandible in orthognathic surgery - A multicenter analysis.

Orthognathic surgery has always been a classical focus of maxillofacial surgery. Since more than 100 years, various surgical techniques for mandibular repositioning have been developed and clinically tested. Since the establishment of plate and screw osteosynthesis, orthognathic surgery became more stable and safe. Nowadays, different surgical methods for mobilising the mandible are existing. This international multicenter analysis (n = 51 hospitals) is providing first evidence based data for the current use of different surgical methods. The dominating techniques were Obwegeser/dal Pont (61%) followed by Hunsuck/Epker (37%) and Perthes/Schlössmann (29%). The main osteosynthesis materials were plates (82%), bicortical screws (23.5%), or a combination of both (5.9%). 47% of all centers reported to use several surgical methods at the same time, depending on the anatomical problem and the surgeon's preference. This shows that different surgical methods seem to work as comparable, safe, and reliable procedures in everydays clinical practise. On this basis, further prospective studies could evaluate possible advantages for our patients.

Longtime undetected microcystic adnexal carcinoma of the scalp: considerations and implications.

BACKGROUND: Microcystic adnexal carcinomas (MACs) are slow-growing and often asymptomatic malignant skin tumours that usually develop on the facial skin and may reach considerable size. We present an uncommon case of MAC arising on the hair-bearing scalp and discuss our experiences and considerations. CASE REPORT: A 58-year-old female was admitted with a histopathologically secured diagnosis of MAC of the temporal and occipital regions that had expanded to 10 × 12 cm. Magnetic resonance imaging revealed an infiltration of the skin and the subcutaneous adipose tissue. No lymphatic or haematogenic metastases were detected. Therapy consisted of resecting the tumour and reconstructing the area by applying an anterolateral thigh (ALT) flap. Histopathological evaluation revealed clear, 1-cm margins and a tumour-free periosteum. One-year postoperative follow-ups showed no evidence of recurrence, while the outcome was aesthetically pleasing. CONCLUSION: When screening for skin cancer, careful attention must be paid to the scalp. Resection of MAC with clear margins is mandatory to minimize the risk of recurrence. In this case, applying an ALT perforator flap to a large defect of the hair-bearing scalp led to a very satisfying result; it should be considered in comparable cases.

Perceptions of Pre- to Postsurgical Profile Changes in Orthognathic Surgery Patients and Their Correlation with Photogrammetric Changes: A Panel Study.

PURPOSE: The objective was to compare the pre- and postsurgical profile changes after surgical correction of prognathism and maxillary hypoplasia, as perceived by panels of orthodontists, maxillofacial surgeons, laypersons and patients and to identify photogrammetric changes that might be related to preferred ratings. MATERIALS AND METHODS: Each panel consisted of six males and six females who rated sets of pre- and postsurgical lateral photographs of 20 female and 20 male patients using a five-point scale. Patients rated their own set of photographs. Pre- to postsurgical differences of photogrammetrically assessed landmarks were recorded as a surgical change. RESULTS: No significant differences in ratings between panels and patients could be detected. Significant correlation coefficients (r) were obtained between the ratings of all panel groups and between the ratings and changes in facial convexity (r = 0.351-0.542). Correlations with changes of the mentolabial angle were found to be significant for old orthodontists, male laypersons, and male patients (r = 0.332-0.609). Ratings of female and young laypersons were correlated with the horizontal changes in the lower face (r = 0.324-0.379). CONCLUSION: Information gathered from this study will support the cooperation of the medical staff and might assist in treatment planning.

Orbital lobe resection provides a definite diagnosis of lacrimal gland lesions: a report of three cases.

BACKGROUND: Entities of lacrimal gland lesions comprise almost inflammatory and lymphoproliferative processes as well as benign and malignant solid tumors and usually cannot be differentiated by magnetic resonance imaging (MRI) exclusively. Hence, representative tissue samples are needed to arrive at sufficient histopathological diagnosis for further treatment decisions. CASE REPORT: Three women aged between 18 and 67 years were admitted to our center with clinical signs of a space-occupying mass in the lacrimal fossa. MRI revealed a circumscribed lacrimal gland lesion in all three cases. The orbital lobe was resected through a lateral orbitotomy approach for histopathological evaluation. Findings confirmed the diagnoses of pleomorphic adenoma, dacryoadenitis, and low-grade B cell non-Hodgkin's lymphoma. Further surgery was not necessary. No recurrence or symptoms of "dry eye" were observed over the course of a 1-year follow-up. CONCLUSION: In cases of non-specific masses in the lacrimal gland on MRI, histopathological diagnoses are vital and can be sufficiently provided by resection of the orbital lobe. Symptoms of dry eye are uncommon, and secondary surgical intervention can be avoided in cases of the presented entities. Further studies with larger patient cohorts are warranted to confirm these findings.

Comparison of intraoperative time measurements between osseous reconstructions with free fibula flaps applying computer-aided designed/computer-aided manufactured and conventional techniques.

PURPOSE: We aimed to determine whether computer-aided designed/computer-aided manufactured (CAD/CAM) techniques could save intraoperative time compared with the conventional technique, by comparing flap harvesting and ischemia times, and subsequently impact flap survival. METHODS: Twenty patients underwent concurrent osteocutaneous fibula flaps, either with (n = 10) or without (n = 10) the CAD/CAM technique. Demographic data, clinical history, complications, number of osseous segments, and times for virtual planning, flap harvesting, flap ischemia, tourniquet inflation, and total reconstruction were recorded. RESULTS: There was no significant difference between CAD/CAM and conventional techniques with respect to age, number of osseous segments, complication rates, and tourniquet inflation time. Flap harvesting times were significantly shorter in the conventional group (112.1 vs. 142.2 min, p < 0.001), while flap ischemia and total ischemia times were significantly shorter in the CAD/CAM group (70.7 vs. 98.6 min, p < 0.001; 174.8 vs. 198.9 min, p = 0.002, respectively). However, while total reconstruction time did not differ between groups, overall operating time (including the amount of virtual planning time and surgical reconstruction time) was significantly longer in the CAD/CAM group (mean 256.0 vs. 210.7 min, p < 0.001). CONCLUSIONS: Despite the advantages of the CAD/CAM technique, including reduced ischemia time of osteocutaneous fibula flaps, there is no impact on total reconstruction time or flap survival.

Costs incurred by applying computer-aided design/computer-aided manufacturing techniques for the reconstruction of maxillofacial defects.

This study aims to evaluate the additional costs incurred by using a computer-aided design/computer-aided manufacturing (CAD/CAM) technique for reconstructing maxillofacial defects by analyzing typical cases. The medical charts of 11 consecutive patients who were subjected to the CAD/CAM technique were considered, and invoices from the companies providing the CAD/CAM devices were reviewed for every case. The number of devices used was significantly correlated with cost (r = 0.880; p < 0.001). Significant differences in mean costs were found between cases in which prebent reconstruction plates were used (€3346.00 ± €29.00) and cases in which they were not (€2534.22 ± €264.48; p < 0.001). Significant differences were also obtained between the costs of two, three and four devices, even when ignoring the cost of reconstruction plates. Additional fees provided by statutory health insurance covered a mean of 171.5% ± 25.6% of the cost of the CAD/CAM devices. Since the additional fees provide financial compensation, we believe that the CAD/CAM technique is suited for wide application and not restricted to complex cases. Where additional fees/funds are not available, the CAD/CAM technique might be unprofitable, so the decision whether or not to use it remains a case-to-case decision with respect to cost versus benefit.

Mucoepidermoid carcinoma of the palate in a 5-year-old girl: case report and literature review.

BACKGROUND: Minor salivary gland tumors in children are uncommon. However, despite the low overall incidence, there is a high incidence of malignancy in these young patients which is reported to be above 50 %, with mucoepidermoid carcinoma (MEC) predominating. We hope that this case report will contribute to the enlightenment of the behavior and prognosis of pediatric MEC of palatal minor salivary gland tumors. CASE REPORT: A 5-year-old girl was admitted to our center with a histologically confirmed MEC of the left hard palate. Primary incision for drainage of a supposed abscess and secondary biopsy had been performed elsewhere 14 days before. After further workup, we excised the tumor and the adjacent bone; clear margins of 10 mm were revealed. Adjuvant therapy was not necessary. The defect was allowed to undergo secondary wound healing. As of the 1-year follow-up, there had been no recurrences. CONCLUSION: For differential diagnosis, MEC should be considered in cases of soft, slow-growing, painless, pale bluish-purple lumps of the palate even in young patients. Especially for nonresponders to initial treatment, early biopsy is recommended for histological confirmation or exclusion of a minor salivary gland tumor.

Application of computer-aided designed/computer-aided manufactured techniques in reconstructing maxillofacial bony structures.

BACKGROUND: Today, virtually planned surgery and computer-aided designed/computer-aided manufactured (CAD/CAM) tools to reconstruct bony structures are being increasingly applied to maxillofacial surgery. However, the criteria for or against the usage of the CAD/CAM technique are disputable, since no evidence-based studies are available. Theoretically, the CAD/CAM technique should be applied to complex cases. In this case report, we present our experiences and discuss the criteria for application. CASE REPORT: Three cases are reported in which subjects received an osseous reconstruction using CAD/CAM techniques. In the first case, resection of the mandibular body and ramus was carried out, and reconstruction with a vascularised iliac bone transplant was performed. During surgery, a repositioning of the ipsilateral condyle was necessary. The second case comprised a wide mandibular reconstruction together with a repositioning of the condyles and the soft tissue chin using a two-segment osteomyocutaneous fibula flap. In the third case, a two-flap technique consisting of a double-barrelled osseous fibula flap and a radial forearm flap was applied to cover a wide palatine defect. CONCLUSION: Our experience suggests that the CAD/CAM technique provides an accurate and useful treatment not only in complex cases, but also in simpler ones, to achieve an anatomically correct shape of the bone transplant and to reposition adjacent structures.

Clinical course and implications of congenital nasal pyriform stenosis and solitary median maxillary central incisor in a newborn: a case report.

INTRODUCTION: Congenital nasal pyriform aperture stenosis and solitary median maxillary central incisor are uncommon anomalies and are associated with further malformations. Solitary median maxillary central incisor itself has initially no impact on a child's health, but congenital nasal pyriform aperture stenosis is a potentially life-threatening condition. CASE PRESENTATION: A Caucasian baby boy showed severe dyspnoea and was intubated orotracheally. Multiple anomalies were detected, including urogenital and craniofacial malformations. Computed tomography scans revealed congenital nasal pyriform aperture stenosis with a diameter of 4.9mm and a solitary median maxillary central incisor. A 3.0mm tube was inserted in his left nasal cavity, and the baby was able to breathe sufficiently and spontaneously. The nasal tube was removed after seven days, and the baby was discharged under application of decongestant drops. After seven months, the baby was readmitted with respiratory distress, and surgery was carried out using an intraoral sublabial approach. The stenotic area of the pyriform aperture was widened, and 3.0mm tubes were inserted in both nasal cavities for 10 days. Over a period of six months, no further respiratory distress has occurred. CONCLUSIONS: The decision to perform surgery was delayed since the baby's nasal breathing was adequate as a result of the insertion of a nasal tube. Since treatment depends on the severity of symptoms, it is appropriate in some cases to take a conservative approach at first, and to keep surgery as a last resort. Once a conservative approach has been selected for congenital nasal pyriform aperture stenosis, awareness of the life-threatening nature of the condition should be kept in mind, and a surgical approach must still be taken into account.

Osteonecrosis of the maxilla related to long-standing methamphetamine abuse: a possible new aspect in the etiology of osteonecrosis of the jaw.

BACKGROUND: Osteonecrosis of the jaw (ONJ) related to toxic effects of illicit drugs such as cocaine is not very common and might be overshadowed today by the incidence of bisphosphonate-related osteonecrosis of the jaw. However, we present a case which suggests a close relationship between abuse of the illicit drug methamphetamine (MA) and ONJ. CASE REPORT: A 44-year-old male with extended osteonecrosis of the maxilla admitted chronic abuse and synthesis of MA for at least the previous two decades. Furthermore, he confessed self-extracting teeth since he became addicted to MA. However at presentation, he had been successfully cured of his addiction to MA. A step-by-step surgical treatment was planned using computer-aided design/computer-aided manufacturing techniques. After resection of necrotic bone, a vascularized osteomyocutaneous fibular flap was applied secondarily. DISCUSSION: Two possible mechanisms, alone or in combination, could possibly lead to MA-related ONJ. Self-extraction of teeth as a psychopathologic behavior of self-destruction among MA abusers results in wounds that allow unhindered invasion of microorganisms causing osteomyelitis and ONJ, while on the other hand, the heating of white phosphor releases toxic phosphorous vapor, which could be inhaled and consequently cause ONJ of the maxilla. However, since the worldwide prevalence of MA abuse is remarkably high, a relationship between MA abuse and ONJ will offer a new aspect in the etiology of ONJ and might present a further therapeutic challenge.

Adenoid cystic carcinoma of the skull base mimicking temporomandibular disorder.

BACKGROUND: Temporomandibular disorder (TMD) involves problems of the temporomandibular joint and its adjacent muscular system. Because TMD prevalence is high among Western populations, complaints in these regions are generally attributed to TMD. However, in rare cases, TMD symptoms are mimicked by malignant tumors of the head. CASE REPORT: Upon first presentation, an 18-year-old female complained about typical symptoms of TMD. After an initial splint therapy and physiotherapy, painful symptoms increased significantly. Twelve weeks after initial diagnosis, further diagnostic imaging revealed a tumor formation at the skull base with infiltration of the infratemporal fossa. Histological evaluation confirmed the diagnosis of adenoid cystic carcinoma. Two years after resection of the tumor, lung metastases were detected with no option of curative treatment. CONCLUSION: TMD symptoms, which are refractory to treatment or exhibit significant worsening during therapy, should be regarded as warning signals and as an indication that early further diagnostic imaging is warranted.

Soft tissue response in orthognathic surgery patients treated by bimaxillary osteotomy: cephalometry compared with 2-D photogrammetry.

PURPOSE: Since improvement of facial aesthetics after orthognathic surgery moves increasingly into the focus of patients, prediction of soft tissue response to hard tissue movement becomes essential for planning. The aim of this study was to assess the facial soft tissue response in skeletal class II and III patients undergoing orthognathic surgery and to compare the potentials of cephalometry and two-dimensional (2-D) photogrammetry for predicting soft tissue changes. MATERIAL AND METHODS: Twenty-eight patients with class II relationship and 33 with class III underwent bimaxillary surgery. All subjects had available both a traced lateral cephalogram and a traced lateral photogram taken pre- and postsurgery in natural head position (median follow-up, 9.4 ± 0.6 months). RESULTS: Facial convexity and lower lip length were highly correlated with hard tissue movements cephalometrically in class III patients and 2-D photogrammetrically in both classes. In comparison, cephalometric correlations for class II patients were weak. Correlations of hard and soft tissue movements between pre- and postoperative corresponding landmarks in horizontal and vertical planes were significant for cephalometry and 2-D photogrammetry. No significant difference was found between cephalometry and 2-D photogrammetry with respect to soft to hard tissue movement ratios. CONCLUSIONS: This study revealed that cephalometry is still a feasible standard for evaluating and predicting outcomes in routine orthognathic surgery cases. Accuracy could be enhanced with 2-D photogrammetry, especially in class II patients.

Microcystic adnexal carcinoma of the upper lip misdiagnosed benign desmoplastic trichoepithelioma.

BACKGROUND: Desmoplastic trichoepithelioma (DT) is a benign appendageal tumour predominately localized on the facial skin. The histological diagnosis can be difficult in some cases. Partial malignant transformation of a DT is a rarity and a complete transformation has never been described in literature. CASE REPORT: A DT of the upper lip was diagnosed histologically by a small biopsy 4 years previously. At presentation, the tumour had enlarged and had partly infiltrated the left side of the upper lip and subnasal region. Histological evaluation confirmed a microcystic adnexal carcinoma but without evidence of malignant transformation of the DT. It appeared that a too-small initial biopsy had led to the incorrect histological diagnosis of a benign tumour. Thus, it was necessary to perform a tumour resection and reconstruction using a two-flap technique including a rotation flap and an Abbé flap. Functional and aesthetic outcomes were good after 6 months. There were no recurrences during a 12-month follow-up. CONCLUSION: A facial DT should be resected completely. Patients should be attended for follow-ups, keeping in mind the difficulty of making a proper histological diagnosis from small biopsies or excisions and the consequences of ablative facial surgery. However, in particular cases, subtotal defects of the upper lip region are amenable to reconstruction without gross functional or aesthetic deficits.

Quality of Life in orthognathic surgery patients: post-surgical improvements in aesthetics and self-confidence.

The objective of this prospective study was to assess changes of Quality of Life (QoL) in patients undergoing bimaxillary orthognathic surgery. Questionnaires were based on the Oral Health Impact Profile (OHIP, items OH-1-OH-14) and three additional questions (items AD-1-3), and were completed by patients (n=50; mean age 26.9±9.9 years) on average 9.1±2.4 months before surgery, and 12.1±1.4 months after surgery, using a scoring scale. Item scores describing functional limitation, physical pain, physical disability and chewing function did not change significantly, whereas item scores covering psychological discomfort and social disability domains revealed significant decreases following surgery. AD-2 "dissatisfying aesthetics" revealed the greatest difference between pre- and post-surgical scores (p<0.001). If there was a perception of aesthetic improvement of facial features post-surgery, the benefit in QoL was generally high. The significant correlation of the pre- to post-surgical changes of item OH-5 "self conscious" to nearly all other item changes suggested that OH-5 was the most sensitive indicator for post-surgical improvement of QoL. Psychological factors and aesthetics exerted a strong influence on the patients' QoL, and determined major changes more than functional aspects did.