Combined Use of Endoscopic Techniques and Virtual Surgical Planning for Intraoral Approach for Hemi-mandibular Resection and Reconstruction.

Background: The study aimed to describe our experience in using endoscopic procedures to aid hemi-mandibular reconstruction with bone flaps through transoral approach. Methods: Five patients with huge benign mandibular tumors underwent transoral mandibulectomy and hemi-mandibular reconstruction, using endoscopy. Facial symmetry, occlusion, bone healing, and mandibular similarity were all evaluated postoperatively. The paired-samples t test was used to compare quantitative data, and a P value less than 0.05 was considered a significant difference. Results: All five patients who received transoral mandibular surgery recovered in terms of TMJ functionality, facial symmetry, and aesthetic results. Endoscopy monitored and ensured that bone flaps were correctly connected and fixed. The accuracy of endoscopy-guided mandibular reconstruction was confirmed by quantitative examination for four cases, which revealed no statistically significant variations between postoperative CT analysis and preoperative virtual surgical planning data. Conclusions: Endoscopy-assisted virtual surgery may resolve concerns with transoral hemi-mandibular reconstruction and broaden indications for mini-invasive mandibular reconstruction. However, only patients with benign mandibular tumors were included in our study, so surgeons should be very cautious if applying this technique to malignant lesions or bony tumors invading soft tissues.

Venous malformations of the lower limb with severe localized intravascular coagulopathy treated with radiofrequency ablation and resection.

Diffuse venous malformations (VMs) are relatively rare, especially the lesions locting special anatomical sites, and they are prone to casuse localized intravascular coagulopathy (LIC). Diffuse VMs can also cause bleeding and life-threatening disseminated intravascular coagulopathy (DIC) from trauma, surgery, and improper treatments. Thus, the treatment of diffuse VMs with LIC is quite tough. We report of a diffuse VMs with severe LIC that was treated with the combined use of minimally invasive treatment and open surgery.

Melanotic neuroectodermal tumor of infancy: Case report and literature review.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare benign tumor. Here, we report the diagnosis and treatment of 1 case of MNTI in the maxilla and discuss its clinical and pathological features, imaging features, treatment, and prognosis.

Children tongue leiomyosarcoma: A rare case and literature review.

Primary leiomyosarcoma of the tongue is a rare malignant mesenchymal tumor with high recurrence rate and metastatic potential. Through analysis of one case condition and literature review, this paper discusses the clinical characteristics and treatment methods and recommends that expanded resection surgery should be the first intervention. Postoperative adjuvant radiotherapy and combined chemotherapy should be administered if the case specifically requires such an approach.

Primary First Bite Syndrome of the Parotid Gland: Case Report and Literature Review.

OBJECTIVE: A case of primary first bite syndrome (FBS), diagnosed in a patient with nonspecific adenocarcinoma of the deep lobe of the parotid gland. DATA SOURCES: A Medline literature search was conducted on PubMed, using the keywords "first bite syndrome." REVIEW METHODS: Using primary FBS and existence of a definite etiology as inclusion criteria. RESULTS: We report on an unusual case of primary FBS, which had no surgical history. After multiple examinations, the pain was localized to a mass in the deep lobe of the parotid gland. After tumorectomy, the FBS pain was significantly relieved. The postoperative pathological examination determined that the excised mass was a nonspecific adenocarcinoma. Reviewing the literature, we found that primary FBS was mostly caused by malignant tumors in the inferior temporal fossa, the deep lobe of the parotid gland, and (or) the parapharyngeal space. Surgery was reported to be an effective treatment. CONCLUSION: The case highlights the critical importance of identifying the etiology of primary FBS. When manifested with a primary FBS, malignant tumors must be high on the differential diagnosis list, especially those in the region of the inferior temporal fossa, the deep lobe of the parotid gland, and the parapharyngeal space.

Embolization and sclerotherapy of maxillofacial arteriovenous malformations with the use of fibrin glue combined with pingyangmycin.

OBJECTIVE: The objectives of this study were to document the results of using fibrin glue (FG) combined with pingyangmycin (PYM) for the embolism and sclerotherapy of maxillofacial arteriovenous malformations (AVMs). STUDY DESIGN: We reviewed the associated clinical data from December 2012 to June 2017 for 25 patients with maxillofacial AVMs. The major treatment method was direct percutaneous puncture and injection of FG combined with PYM. Treatment outcomes were assessed through physical examination, Doppler ultrasonography, computed tomography, and 3-dimensional computed tomography angiography scans. Follow-up time ranged from 12 months to 3 years after the last treatment (mean 21 months). RESULTS: Of the 25 lesions, 80% showed greater than 90% reduction, 12% showed greater than 75% reduction, and 8% showed greater than 50% reduction. Superficial skin necrosis or mucous ulcer occurred in 3 patients and healed without intervention. Regrowth was observed in 3 patients with extensive lesions involving multiple anatomic regions. CONCLUSIONS: These data suggest that embolization and sclerotherapy with the use of FG combined with PYM are safe and effective for the treatment of small- to medium-sized, locally dilated maxillofacial AVMs. For AVMs involving multiple anatomic regions, combined application of this approach with other options should be considered.

NT-3/TrkC Axis Contributes to the Perineural Invasion and the Poor Prognosis in Human Salivary Adenoid Cystic Carcinoma.

The present study was aimed to investigate the role and mechanism of neurotrophin-3 (NT-3) and its specific receptor tropomyosin receptor kinase C (TrkC) in the perineural invasion (PNI) process of the salivary adenoid cystic carcinoma (SACC). The co-cultured system between SACC cells and Schwann cells (SCs) was employed to detect the expression of NT-3 and TrkC. The results of ELISA, qRT-PCR and western blot showed that NT-3 was noticeably elevated in the co-cultured SACC-83 cells, while TrkC was increased in the co-cultured SCs. The results of scratch wound healing, migration, and 3D co-culture assays showed that the directional migration abilities of the co-cultured SACC-83 cells and SCs were significantly increased. Under the stimulation of NT-3, the directional motor ability of SACC-83 cells and SCs was significantly improved, and the apoptosis of SACC-83 cells and SCs were obviously inhibited. In addition, blocking TrkC by its specific inhibitor AZD7451 could significantly inhibit these effects. Immunohistochemistry staining showed that the positive expression of NT-3 (88.5%) and TrkC (92.3%) was significantly correlated with the PNI in SACC specimens (P < 0.05). Additionally, the high expression of NT-3 was significantly associated with the poor prognosis of SACC patients (P < 0.05). The present study indicated that NT-3/TrkC axis contributed to the PNI progression and the poor prognosis of SACC via regulating the interaction between SACC cells and SCs. Interruption of the interaction between SACC cells and SCs by blocking the NT-3/TrkC axis might be an effective strategy for anti-PNI therapy in SACC.

The "Flap Suture Anchoring" Technique for Safe Oral Floor Reconstruction With Preservation of Alveolar Process.

Flaps-based microsurgery is routinely applied to reconstruct oral floor defects caused by oncologic resection. To prevent orocutaneous fistulae, flaps are frequently sutured with buccal vestibule mucosa after sacrificing the alveolar process. The patients suffered denture loss and irreversible structural damage. For reliable oral floor reconstruction with preservation of alveolar process, the authors introduced the flap "suture anchoring" technique. Oral floor, hemiglossal-oral floor, and tongue base-parapharyngeal wall-oral floor defects were included in this study. The flap anchoring technique involves structural oral floor reconstruction with a chimeric anterolateral thigh-free flap or radial forearm flap with adipofascial tissue extension. The dead space in oral floor is filled with vastus lateralis muscle or adipofascial tissue, then holes are drilled on the alveolar bone among tooth root, beneath the attached gingiva. Skin paddle is sutured with 4-0 sutures through the alveolar holes thus anchored to the mandible. By applying this technique, there was no wound infection or orocutaneous fistulae in all patients postoperatively, even experienced postoperative radiotherapy. In addition, a soft and natural jaw-tongue furrow could be formed to allow the free movement of tongue. Taken together, the flap anchoring technique offers a safe and reliable approach to recover oral function and preservation of occlusion.

[Chinese experts consensus on the use of oral propranolol for treatment of infantile hemangiomas].

Infantile hemangioma (IH) is one of the most common benign vascular tumors in children. A variety of treatment methods have been documented for the management of IH over the past years, including pharmacotherapy via oral administration or injection of corticosteroids, vincristine, alpha interferon and bleomycin; laser therapy, radionuclide therapy, cryotherapy and excisional surgery. The therapeutic efficacy of each treatment modality is variable, while adverse effects or complications are common and sometimes serious. Since the serendipitous discovery of propranolol, a nonselective beta-adrenergic receptor blocker, being very efficacious in treating IH in 2008, oral propranolol has earned a role as a first-line medical therapy for complicated IH. However, the appropriate drug dosage, dosing regimen, time for initiation, optimal duration, monitoring for side effects remains controversial. To standardize the use of propranolol in treating IH, avoid overtreatment or under-treatment, as well as minimize complications, a Chinese experts consensus on the use of oral propranolol for treatment of IH has been approved and written by a multidisciplinary experts group based on an up-to-date literature review and repeated discussion.

[Chinese expert consensus on the use of topical timolol maleate treatment of infantile hemangiomas].

Non-selective ß-blocker propranolol has been proved by FDA as the first-line agent for infantile hemangioma (IH) with dramatic response. To reduce the side effects caused by systemic administration of propranolol, timolol maleate treatment has been increasingly used as an alternative to systemic ß-blockers and watchful waiting for many IH patients in recent years. However, the appropriate indications, drug dosage, dosing regimen, time for initiation, optimal duration, monitoring for side effects still remains controversial. To standardize the use of topical timolol in treating IH, avoid overtreatment or under-treatment, as well as minimize complications, a Chinese expert consensus on the use of topical timolol treatment of IH has been approved and written by a multidisciplinary experts group based on an up-to-date literature review and repeated discussion, which can be used to reduce inappropriate variations in clinical practice and to promote the delivery of high quality, evidence-based health care for IH patients.

Genetic variation analysis in a Chinese Maffucci syndrome patient.

OBJECTIVE: To report on the molecular genetic analysis of a Chinese patient with Maffucci syndrome. METHODS: Using the genomic DNA extracted from the patient's hemangioma sample, the coding exons and exon/intron splice junctions of the IDH1 and IDH2 genes were amplified by polymerase chain reaction (PCR) and then sequenced. Genomic DNA was extracted from blood and a hemangioma sample from the patient, and also from her mother's blood, for chromosome microarray analysis (CMA) by Affymetrix CytoScan HD array. RESULTS: None of the known pathogenic mutations in the whole IDH1 or IDH2 genes was found in the patient's hemangioma sample. CMA detected 40 tumor-specific copy number variations (CNVs), and one copy number neutral loss of heterozygosity (LOH) region. Among the 73 known genes included in the 40 CNV regions, only 2 genes, CHEK2 (604373) located in 22q12.1 and EP300 (602700) located in 22q13.2, were found to be related to tumorigenesis. We did not find any CNVs at the IDH1 and IDH2 loci. CONCLUSIONS: This is the first molecular genetic analysis report on a Chinese patient with Maffucci syndrome and our data enrich the understanding of the genetic background of Maffucci syndrome in different ethnic groups. The relationship between CHEK2, EP300 and Maffucci syndrome needs to be further explored.

Cherubism misdiagnosed as giant cell tumor: a case report and review of literature.

Cherubism is characterized by progressive, painless, bilateral enlargement of the mandible and/or maxilla resulting from the replacement of bone with multilocular cysts composed of fibrotic stromal cells and osteoclast-like cells. Here we report one Chinese cherubism case that has been misdiagnosed for more than forty years. The patient displayed no typical clinical or radiographical signs of cherubism due to multi-surgical treatments. Her histopathologic examination revealed the proliferating fibrous connective tissue with few multinucleated giant cells. The family history suggested us to perform sequence analysis of the SH3BP2 gene, a candidate marker for cherubism, in the family, and it was found that both the proband and the son had a missense mutation in SH3BP2 in exon 9 (p. Arg415Gln). Here we emphasize the importance of gene testing in the diagnosis of suspected cherubism, especially for those cases with non-typical clinical, radiographic and histological presentations.

Lysosome-associated membrane glycoprotein 3 is involved in influenza A virus replication in human lung epithelial (A549) cells.

BACKGROUND: Influenza A virus mutates rapidly, rendering antiviral therapies and vaccines directed against virus-encoded targets ineffective. Knowledge of the host factors and molecular pathways exploited by influenza virus will provide further targets for novel antiviral strategies. However, the critical host factors involved in influenza virus infection have not been fully defined. RESULTS: We demonstrated that LAMP3, a member of lysosome-associated membrane glycoprotein (LAMP) family, was significantly induced in human lung epithelial (A549) cells upon influenza A virus infection. Knockdown of LAMP3 expression by RNA interference attenuated production of viral nucleoprotein (NP) as well as virus titers. Confocal microscopy results demonstrated that viral NP is colocalized within LAMP3 positive vesicles at early stages of virus infection. Furthermore, knockdown of LAMP3 expression led to a reduction in nuclear accumulation of viral NP and impeded virus replication. CONCLUSIONS: LAMP3 is an influenza A virus inducible gene, and plays an important role in viral post-entry steps. Our observations may provide insights into the mechanism of influenza virus replication and potential targets for novel anti-influenza therapeutics.

Bleomycin A5 sclerotherapy for cervicofacial lymphatic malformations.

OBJECTIVES: The purpose of this study was to document the results of bleomycin A5 sclerotherapy for cervicofacial lymphatic malformations (LMs), and the clinical data of 65 patients between October 2004 and October 2007 were reviewed. METHODS: Of the 65 patients in the study, 60 patients were given intralesional injection of bleomycin A5. Five patients underwent partial resection, and then an injection of bleomycin A5 for the remaining lesion. The outcomes were assessed by physical examination and Doppler ultrasonography scan. The follow-up time was from 6 months to 3 years after the last injection (mean, 16 months). RESULTS: Among the 65 patients, 41 were men and 24 were women (1.7:1 male:female ratio), the age range was 3 months to 45 years (mean, 12 years). Thirty-two lesions (49%) were macrocystic, 30 (46%) were microcystic, and 3 (5%) were combined. Each patient received 1 to 10 injections (mean, 3.0 injections) for the whole course of treatment, and the total dose of bleomycin A5 was from 8 to 80 milligrams (mean, 24.0 mg). Twenty-six of 32 macrocystic lesions (81%) showed greater than 90% reduction, whereas another 6 (19%) exhibited 50% to 90% reduction. Nineteen of 30 microcystic lesions (63%) showed greater than 90% reduction; 10 (33%) had 50% to 90% reduction; and 1 (4%) had less than 50% size reduction. Of the 3 combined lesions, 2 (67%) had greater than 90% shrinkage, and 1 (3%) had less than 50% reduction. The complications included ulceration of oral mucosa, minor soft tissue atrophy, mild fever, and hematoma. There was no recurrence throughout the follow-up period. CONCLUSION: These data suggest bleomycin A5 is a safe and effective intralesional agent for the treatment of macrocystic LMs, superficial oral mucosa LM, and localized deep microcystic lesions. For extensive macrocystic LMs involving contiguous anatomic areas and diffuse microcystic lesions involving deep tissues, bleomycin A5 injection combined with resection is necessary.

Application of an In-Cell Western assay for measurement of influenza A virus replication.

Influenza A pandemics present enormous challenges to modern medicine. To control such pandemics, quantitative assays characterised by rapidity, high sensitivity, and high-throughput are critical in determining the susceptibility of the influenza A virus to antiviral drugs and for screening chemicals that can inhibit viral replication effectively. In the present study, a rapid and quantitative method to determine influenza A virus replication was developed by an In-Cell Western (ICW) assay. This assay was found to be useful for monitoring the kinetics of influenza A virus replication, as viral nucleoprotein production could be correlated to both increasing doses of viral infection and to the lapse of time during viral infection. Compared to other conventional assays, such as TCID(50), quantitative real-time RT-PCR, and the indirect immunofluorescence assay, the ICW assay exhibits high accuracy, reproducibility, and ease of use. The antiviral effect of amantadine and ribavirin can be determined readily by the ICW assay in 96-well formats, providing a means of rapid antiviral drug screening. Thus, the ICW assay can be used for detecting viral replication, quantifying virus production, and assessing drug-susceptibility in high-throughput applications.

The 3C protein of enterovirus 71 inhibits retinoid acid-inducible gene I-mediated interferon regulatory factor 3 activation and type I interferon responses.

Enterovirus 71 (EV71) is a human pathogen that induces hand, foot, and mouth disease and fatal neurological diseases. Immature or impaired immunity is thought to associate with increased morbidity and mortality. In a murine model, EV71 does not facilitate the production of type I interferon (IFN) that plays a critical role in the first-line defense against viral infection. Administration of a neutralizing antibody to IFN-alpha/beta exacerbates the virus-induced disease. However, the molecular events governing this process remain elusive. Here, we report that EV71 suppresses the induction of antiviral immunity by targeting the cytosolic receptor retinoid acid-inducible gene I (RIG-I). In infected cells, EV71 inhibits the expression of IFN-beta, IFN-stimulated gene 54 (ISG54), ISG56, and tumor necrosis factor alpha. Among structural and nonstructural proteins encoded by EV71, the 3C protein is capable of inhibiting IFN-beta activation by virus and RIG-I. Nevertheless, EV71 3C exhibits no inhibitory activity on MDA5. Remarkably, when expressed in mammalian cells, EV71 3C associates with RIG-I via the caspase recruitment domain. This precludes the recruitment of an adaptor IPS-1 by RIG-I and subsequent nuclear translocation of interferon regulatory factor 3. An R84Q or V154S substitution in the RNA binding motifs has no effect. An H40D substitution is detrimental, but the protease activity associated with 3C is dispensable. Together, these results suggest that inhibition of RIG-I-mediated type I IFN responses by the 3C protein may contribute to the pathogenesis of EV71 infection.

A clinical study of ultrasound-guided intralesional injection of bleomycin A5 on venous malformation in cervical-facial region in China.

OBJECTIVES: To evaluate the therapeutic outcome of ultrasound-guided intralesional injection of bleomycin A5 on treatment of venous malformation (VM) in cervical-facial region. METHODS: Seventy-five patients (32 male, 43 female), ranging in age from 13 to 60 years old, suffering from VM in cervical-facial region were admitted to and treated at our hospital between June 2006 and February 2007. Of all the patients, 54 malformations were located in the facial region, eight in the submental region, 10 in the submandible region, and three in the cervical region; all were treated by ultrasound-guided intralesional injections of bleomycin A5. The size of the lesions ranged from 6 x 9 mm to 32 x 39 mm. Injection of bleomycin A5 on venous malformation was then carried out through the inspection of ultrasonography. Repeated course of bleomycin A5 injection was administrated for larger malformations. The amount was 8 mg each time. The therapeutic interval was two to four weeks. The therapeutic outcome on venous malformation was evaluated by physical examination and ultrasonography with Doppler according to the Shou standards, including four grades; cured, basically cured, improved, and invalid. The complications were also observed during and after injection. RESULTS: The duration of follow-up ranged from 6 to 24 months. The average times of treatment were 1.64 times. Among them, 42 patients (56%) received only one time of treatment, 21 (28%) patients received two times, nine (12%) patients received three times, and three (4%) patients received four times. According to criteria of therapeutic outcome, the results showed cured in 63 patients (84%), basically cured in 10 patients (13.33%), improved in two patients (2.67%), and none ineffective. Seventy-one patients (94.67%) had local swelling in injection region for several days and two patients (2.67%) developed temporary dizziness after treatment. There were no other complications recorded. CONCLUSIONS: Intralesional injection of bleomycin A5 establishes a promisingly effect way for patients suffering from VM in the cervical-facial region under ultrasound guidance.

Effects of nerve growth factor delivery via a gel to inferior alveolar nerve in mandibular distraction osteogenesis.

Inferior alveolar nerve (IAN) injury is a concern in mandible distraction osteogenesis (DO). We have previously demonstrated that repeated local injections of human nerve growth factor beta (NGF-beta) have significantly enhanced the histologic recovery of the IAN in a rabbit model of DO. This study was to further test the effect of a single injection of human NGF-beta delivered via a collagen/nanohydroxyapatite/kappa-carrageenan gel to the recovery of the IAN in DO. Rabbits underwent mandibular DO at a rate of 0.75 mm/12 h for 6 days. At the end of the distraction period, injections were performed near the IAN percutaneously as follows: group 1, human NGF-beta in the gel; group 2, human NGF-beta in saline; group 3, the gel alone; and group 4, saline alone. At 14 days after the end of distraction, IAN histologic findings and histomorphometric parameters were evaluated. Histologically, there were less myelin debris and more abundant regenerating nerve fibers in group 1 than the other groups. Both the myelinated fiber density and the myelinated axon area in group 1 were significantly higher than groups 3 and 4 (P < 0.01); the myelinated axon area in the group 1 was significantly higher than group 2 (P < 0.01). In conclusion, the delivery of human NGF-beta in the gel leads to a better acceleration of the IAN injury recovery over the saline delivery. It provides a possible way to enhance the recovery of nerve injuries in craniofacial DO clinically.

Bleomycin A5 plus dexamethasone for control of growth in infantile parotid hemangiomas.

OBJECTIVE: The purpose of this study was to evaluate the efficacy of bleomycin A5 (pingyangmycin) plus dexamethasone for control of growth in infantile parotid hemangiomas. STUDY DESIGN: We reviewed and analyzed the data of 31 cases undergoing therapy of intralesional injection with small-dosage and low-concentration bleomycin A5 plus dexamethasone between June 2004 and October 2007. Clinical manifestations, image characteristics, and therapeutic outcomes were reviewed. The therapeutic outcomes were evaluated by physical examination, photographs, and Doppler ultrasonography. The follow-up was from 6 months to 3 years after ending treatment. RESULTS: Twenty-five patients (80.6%) had a response rate greater than 90% reduction in tumor size. Three patients (9.7%) had a response rate between 75% and 90% reduction in tumor size. Another 3 patients (9.7%) had a response rate between 50% and 75% reduction in size. No patients had less than a 50% response rate. There was no recurrence, allergic reaction, pulmonary fibrosis, fever, or other complication during or after the course of treatment. CONCLUSIONS: The controlling therapy with small-dosage and low-concentration bleomycin A5 plus dexamethasone can treat the parotid hemangiomas of infants effectively, especially for lesions in the early phase and proliferative phase. Early control and long-term observation are the key aspects of treatment.

Preliminary study of fibrin glue combined with pingyangmycin for the treatment of venous malformations in the oral and maxillofacial region.

PURPOSE: To observe the outcome of using fibrin glue (FG) combined with Pingyangmycin (Tianjin Taihe Pharmaceutical Co Ltd, Tianjin, China) in the treatment of venous malformations (VMs) in the oral and maxillofacial region. MATERIALS AND METHODS: The treatment data of 7 patients with VMs from January 2005 to January 2006 were reviewed. All these patients were injected with FG combined with Pingyangmycin. The vital signs and symptoms were observed and recorded immediately after injections. Radiographic examination was used for the evaluation of pulmonary conditions. The therapeutic effect was evaluated by clinical examination and Doppler ultrasonography. The follow-up time was from 1 to 2 years. RESULTS: Of the 7 patients, 4 were male and 3 were female, with ages ranging from 10 to 62 years. Four of the 7 recovered to near-normal appearance, without any abnormal bloodstream detectable within the lesions. Two lesions were slightly asymmetrical in appearance, but no abnormal bloodstream could be detected in the lesions. Two patients had a fever during treatment, and one of them stopped treatment because of continuous high fever. No allergic reactions, pulmonary embolisms, or other complications were found during or after treatment. CONCLUSION: The therapeutic modality of FG combined with Pingyangmycin for VMs in the oral and maxillofacial region was effective, and the extent of morbidity was acceptable. However, the further long-term observation of severe complications such as deep venous thrombosis and pulmonary embolism should be performed in additional biological and clinical studies.