Alloplastic Temporomandibular Joint Reconstruction in Congenital Craniofacial Deformities.

ABSTRACT: Temporomandibular joint (TMJ) reconstruction with alloplastic prosthesis is a commonly performed procedure, which can be used as a treatment for improving pathologic conditions affecting the TMJ. Depending of the severity of the condition, the patient's ability to eat can be impaired, which in turn can affect their quality of life. Several syndromes can affect the patient's facial features, and some of those also affect TMJ's. Use of alloplastic prosthesis in TMJ's affecting syndromes, by means of correcting TMJ conditions and mandibular deformities, is still rather uncommon. Purpose of our study is to review the use TMJ total prosthesis as a treatment modality for correcting TMJ conditions and the reconstruction of mandibular deformities in congenital syndromes affecting the facial features. This study consists of a retrospective cohort of patients treated with TMJ reconstruction with alloplastic prosthesis at the Helsinki University Hospital during the past decade. All subjects with congenital syndrome who underwent TMJ reconstruction were included the study. The cohort consisted of seven patients and ten joints treated. Temporomandibular joints affecting syndromes were Goldenhar syndrome, hemifacial macrosomia, Nager syndrome, and Treacher-Collins syndrome. The majority of the patients have had several previous operative treatments on the facial skeleton during their childhood, which in turn probably affected and compromised the TMJ surgery outcome. Mouth opening was sufficiently achieved as average maximal opening was 34 mm (range 24-42 mm) postoperatively. Temporomandibular joint reconstruction with alloplastic prosthesis can offer new tools and approaches for treatment of facial deformities in syndromes with craniofacial abnormalities. Impaired jaw functions and asymmetry can be addressed with the use of alloplastic prostheses, achieving sufficient mouth opening and restoring the symmetry of the lower facial skeleton.

Surgical Treatment of Dentofacial Deformities Caused by Juvenile Idiopathic Arthritis.

The purpose of our retrospective study was to evaluate the results of orthognathic treatment, distraction osteogenesis, and/or prosthetic reconstruction of the temporomandibular joints in patients with juvenile idiopathic arthritis (JIA).Twelve patients with severely affected temporomandibular joints (TMJs) and reduced ramus height were treated with mandibular advancement with orthognathic surgery (11) and additional bilateral or unilateral mandibular ramus distraction (3) or additional bilateral or unilateral prosthetic reconstruction of the TMJ (3). One patient was treated surgically with bilateral TMJ prosthetic reconstruction only. The patients were followed up clinically and radiologically with emphasis on healing, TMJ function, stability of the occlusion, skeletal stability, and facial appearance for an average of 2.3 years after the final surgery. The mean mandibular advancement was 10.1 mm. The mean relapse at pogonion was 2.1 mm, which represents 20.8% of the surgical advancement. The occlusion was stable in 11/12 patients. The TMJ function was good and the facial esthetics improved in all patients. Orthognathic treatment and mandibular ramus distraction osteogenesis provide beneficial lengthening of the mandibular body in JIA patients with asymptomatic and stabile condyles. In adult patients with relapse of the disease or postoperative condylar relapse prosthetic total joint replacement is a reliable and safe alternative.

CAD/CAM Engineered Patient-Specific Impants as a Reposition Device in Le Fort I and Modified Subcondylar Osteotomies: Case Report of Facial Deformity Correction in Acromegaly.

Acromegaly is a medical condition where elevated growth hormone or insulin-like growth factor I levels cause several changes in the craniofacial soft and hard features. We report the correction of facial deformity and posterior open bite with Le Fort I and modified subcondylar osteotomies in a patient affected by acromegaly. Computer-aided design and manufacturing generated saw and drill guides were used to perform osteotomies and segment removal. The placement of the patient-specific implants (PSIs) was guided by predesigned drill holes ensuring the required and planned movement of the jaws and position of the PSIs. After segment removal, the PSIs fitted the predesigned drill holes with high precision and were secured without problems. The planned amount of mandibular and maxillary movement was achieved. The occlusion and osteotomies remained stable for the follow-up of 22 months. The use of PSIs combined with guided surgery can be beneficial for selected cases with asymmetry or posterior open bite enabling new approaches and yielding good functional and aesthetic outcome. The modification of conventional ramus osteotomy combined with utilization of ramus segment removal and the use of PSI for reposition is an interesting and promising technique for rare conditions with ramus height asymmetry.

No differences in infections between patient-specific implants and conventional mini-plates in mandibular bilateral sagittal split osteotomy - Up to 3-year follow-up.

The use of individually designed osteotomies, combined with individually manufactured osteosynthesis material, is rapidly becoming a standard for more challenging maxillofacial surgery. The benefits of patient-specific implants (PSI) in orthognathic surgery are clear in complex cases. PSIs can enhance precision and ease up the surgical protocol. We previously reported on the benefits of PSIs as reposition and fixation systems during Le Fort I osteotomy. The aim of this study was to evaluate a cohort of 28 patients, treated with bilateral sagittal split osteotomy (BSSO) and PSIs for fixation, with regard to healing for up to 3 years. A retrospective cohort of 48 patients with conventional mini-plate repositioned mandibles was also collected for statistical analysis. No statistically significant differences were found with regard to infection, soft tissue problems, or reoperations between these two groups.

A New Yeast Peroxin, Pex36, a Functional Homolog of Mammalian PEX16, Functions in the ER-to-Peroxisome Traffic of Peroxisomal Membrane Proteins.

Peroxisomal membrane proteins (PMPs) traffic to peroxisomes by two mechanisms: direct insertion from the cytosol into the peroxisomal membrane and indirect trafficking to peroxisomes via the endoplasmic reticulum (ER). In mammals and yeast, several PMPs traffic via the ER in a Pex3- and Pex19-dependent manner. In Komagataella phaffii (formerly called Pichia pastoris) specifically, the indirect traffic of Pex2, but not of Pex11 or Pex17, depends on Pex3, but all PMPs tested for indirect trafficking require Pex19. In mammals, the indirect traffic of PMPs also requires PEX16, a protein that is absent in most yeast species. In this study, we isolated PEX36, a new gene in K. phaffii, which encodes a PMP. Pex36 is required for cell growth in conditions that require peroxisomes for the metabolism of certain carbon sources. This growth defect in cells lacking Pex36 can be rescued by the expression of human PEX16, Saccharomyces cerevisiae Pex34, or by overexpression of the endogenous K. phaffii Pex25. Pex36 is not an essential protein for peroxisome proliferation, but in the absence of the functionally redundant protein, Pex25, it becomes essential and less than 20% of these cells show import-incompetent, peroxisome-like structures (peroxisome remnants). In the absence of both proteins, peroxisome biogenesis and the intra-ER sorting of Pex2 and Pex11C are seriously impaired, likely by affecting Pex3 and Pex19 function.

Correlative light and electron microscopy imaging of autophagy in a zebrafish infection model.

High-resolution imaging of autophagy has been used intensively in cell culture studies, but so far it has been difficult to visualize this process in detail in whole animal models. In this study we present a versatile method for high-resolution imaging of microbial infection in zebrafish larvae by injecting pathogens into the tail fin. This allows visualization of autophagic compartments by light and electron microscopy, which makes it possible to correlate images acquired by the 2 techniques. Using this method we have studied the autophagy response against Mycobacterium marinum infection. We show that mycobacteria during the progress of infection are frequently associated with GFP-Lc3-positive vesicles, and that 2 types of GFP-Lc3-positive vesicles were observed. The majority of these vesicles were approximately 1 µm in size and in close vicinity of bacteria, and a smaller number of GFP-Lc3-positive vesicles was larger in size and were observed to contain bacteria. Quantitative data showed that these larger vesicles occurred significantly more in leukocytes than in other cell types, and that approximately 70% of these vesicles were positive for a lysosomal marker. Using electron microscopy, it was found that approximately 5% of intracellular bacteria were present in autophagic vacuoles and that the remaining intracellular bacteria were present in phagosomes, lysosomes, free inside the cytoplasm or occurred as large aggregates. Based on correlation of light and electron microscopy images, it was shown that GFP-Lc3-positive vesicles displayed autophagic morphology. This study provides a new approach for injection of pathogens into the tail fin, which allows combined light and electron microscopy imaging in vivo and opens new research directions for studying autophagy process related to infectious diseases.