Neuroendocrine carcinoma of the common hepatic duct coexisting with distal cholangiocarcinoma: A case report and review of literature.

BACKGROUND: Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is very rare, and the treatment and prognosis are unclear. Herein, we report the case of a middle-aged female with primary large cell NEC (LCNEC) of the common hepatic duct combined with distal cholangiocarcinoma (dCCA). Additionally, after a review of the relevant literature, we summarize and compare mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) and pure NEC to provide a reference for selecting the appropriate treatment and predicting the prognosis of this rare disease. CASE SUMMARY: A 62-year-old female presented to the hospital due to recurrent abdominal pain for 2 months. Physical examination showed mild tenderness in the upper abdomen and a positive Courvoisier sign. Blood tests showed elevated liver transaminase and carbohydrate antigen 199 levels. Imaging examination revealed a 1-cm tumour in the middle and lower segments of the common bile duct. Pancreaticoduodenectomy + lymph node dissection was performed, and hepatic duct tumours were unexpectedly found during surgery. Pathology suggested poorly differentiated LCNEC (approximately 0.5 cm × 0.5 cm × 0.4 cm), Ki-67 (50%), synaptophysin+, and chromogranin A+. dCCA pathology suggested moderately differentiated adenocarcinoma. The patient eventually developed lymph node metastasis in the liver, bone, peritoneum, and abdominal cavity and died 24 months after surgery. Gene sequencing methods were used to compare gene mutations in the two primary bile duct tumours. CONCLUSION: The prognosis of MiNEN and pure NEC alone is different, and the selection of treatment options needs to be differentiated.

Primary intra-abdominal paraganglioma: A case report.

BACKGROUND: Paragangliomas are rare neuroendocrine tumors. We hereby report a case of a localized paraganglioma found in the abdominal cavity, and review the relevant literature to improve the understanding of this disease. CASE SUMMARY: A 29-year-old Chinese female patient was referred to our hospital due to an abdominal mass found on physical examination. Imaging revealed a mass in the left upper abdomen, suggestive of either a benign stromal tumor or an ectopic accessory spleen. Laparoscopic radical resection was subsequently performed, and histopathological analysis confirmed the diagnosis of a paraganglioma. The patient was followed up 3 months post-operation, and reported good recovery with no metastasis. CONCLUSION: Radical resection can effectively treat intra-abdominal paragangliomas, with few side effects and low recurrence risk. In addition, early and accurate diagnosis and timely intervention are essential for the prognosis of this disease.

Ocular microvascular alteration in Sjögren syndrome.

BACKGROUND: Sjögren syndrome (SjS) is a systemic disease affecting exocrine, including ocular lacrimal, glands. It is uncertain whether ocular microvascular alterations are associated with this disease. In this study, we evaluated retinal and conjunctival microvascular changes in SjS patients using optical coherence tomography angiography (OCTA). METHODS: Twelve SjS patients (24 eyes) and 12 normal controls (24 eyes) were recruited to this study. Three-dimensional conjunctival and retinal OCTA images of each eye were captured and microvascular density was calculated. Each image was analyzed by retinal area based on the early treatment of diabetic retinopathy study method (R, S, L, and I) hemisphere segmentation method (SR, SL, IL, and IR); and central wheel division method (C1-C6). Correlation analyses were used to look for associations between retinal and conjunctival microvascular densities. RESULTS: Superficial and deep retinal layer microvascular density was decreased in SjS patients compared with normal controls (P<0.05). This significant difference was found in both superficial and deep layers in S, L, SL, IL and C1-C3 regions, and additionally in the I and SR regions in the superficial layer. Conversely, in the conjunctiva microvascular density was higher in SjS patients than in controls. In SjS patients, a significant negative correlation was found between conjunctival and both superficial (r=-0.641; P=0.025) and deep (r=-0.958; P<0.0001) microvascular densities. CONCLUSIONS: The changed microvascular densities measured in deep and superficial retinal layers and in the conjunctiva demonstrate that OCTA is a promising method in differentiating the eyes from those with SjS.

Tooth-supported personalized template-assisted foramen ovale puncture system for trigeminal neuralgia treatment.

PURPOSE: To introduce a tooth-supported personalized template-assisted foramen ovale (FO) puncture system for trigeminal neuralgia (TN) treatment, analyze its advantages, and review other previously reported methods. METHODS: Sixty-seven patients were included. According to the preoperative digital design, the personalized puncture path was determined. Then, a tooth-supported personalized template was designed and manufactured. Finally, surgery (radiofrequency thermocoagulation or balloon compression) was carried out with the assistance of the template. The puncture effect and puncture-associated complications were evaluated, and the related literature was reviewed. RESULTS: The FO was successfully punctured in one attempt in all patients. The procedure was completed in 15 s in 35 (52.24%) patients and in 15-30 s in 28 (41.79%) patients. The required position was accurately reached in all patients, and the center point error range was within 1 mm. No complications associated with puncture occurred. CONCLUSION: The tooth-supported personalized template-assisted FO puncture system reported in this paper is an exceedingly simple, highly effective and safe FO puncture method that is worth popularizing.

Sensitivity of magnetic resonance tomographic angiography for detecting the degree of neurovascular compression in trigeminal neuralgia.

PURPOSE: Neurovascular compression (NVC) is hypothesized to be the main pathogenic factor of trigeminal neuralgia (TN). Microvascular decompression (MVD) has become a popular surgery for TN, and the success rate depends on the degree of NVC. As the routine examination before MVD, magnetic resonance tomographic angiography (MRTA) shows high sensitivity for detecting NVC. However, there are no reports on the sensitivity of MRTA for assessing the degree of NVC. METHODS: This study aimed to evaluate the sensitivity of MRTA for determining the degree of NVC by comparing preoperative MRTA and intraoperative endoscopy findings. A total of 480 patients who suffered from TN and underwent MVD were included. Their preoperative MRTA and intraoperative endoscopy findings were reviewed. The kappa test was used to identify similarities between the MRTA and endoscopy findings. RESULTS: The degree of NVC on preoperative MRTA was similar to that on endoscopy (kappa = 0.770). The number of offending vessels according to preoperative MRTA was coincident with that according to endoscopy (kappa = 0.722). CONCLUSION: MRTA had high sensitivity for detecting not only the presence of NVC but also the degree of NVC.

Parry-Romberg Syndrome With Hemimasticatory Spasm: A Rare Combination.

Parry-Romberg syndrome is a rare craniofacial disorder characterized by progressive hemifacial atrophy with systematic manifestations. The combination with hemimasticatory spasm is rare, with only 9 patients reported before. In this study, a study of a young male patient with Parry-Romberg syndrome and hemimasticatory spasm on his left side was presented. Radiologic examinations showed severe atrophy limited to subcutaneous tissue and electromyography demonstrated as hemimasticatory spasm. Injection of botulinum toxin type A was applied for the treatment of hemimasticatory spasm and after 2 separated injections, the patient was significantly relieved from symptom. The theory of focal demyelination of the trigeminal nerve peripheral brunches is the possible link between hemifacial atrophy and hemimasticatory spasm, although the pathogenesis of both diseases requires further study and current therapeutic methods are still limited to symptomatic treatments. Injection of botulinum toxin type A is an effective way to treat hemimasticatory spasm and autologous fat transplant is a promising solution to correct facial asymmetry.

Skull Base Erosion Resulting From Primary Tumors of the Temporomandibular Joint and Skull Base Region: Our Classification and Reconstruction Experience.

PURPOSE: We sought to introduce our classification and reconstruction protocol for skull base erosions in the temporomandibular joint and skull base region. PATIENTS AND METHODS: Patients with neoplasms in the temporomandibular joint and skull base region treated from January 2006 to March 2017 were reviewed. Skull base erosion was classified into 3 types according to the size of the defect. RESULTS: We included 33 patients, of whom 5 (15.2%) had type I defects (including 3 in whom free fat grafts were placed and 2 in whom deep temporal fascial fat flaps were placed). There were 8 patients (24.2%) with type II defects, all of whom received deep temporal fascial fat flaps. A total of 20 patients (60.6%) had type III defects, including 17 in whom autogenous bone grafts were placed, 1 in whom titanium mesh was placed, and 2 who received total alloplastic joints. The mean follow-up period was 50 months. All of the patients exhibited stable occlusion and good facial symmetry. No recurrence was noted. CONCLUSIONS: Our classification and reconstruction principles allowed reliable morpho-functional skull base reconstruction.

Modified Decompression of the Infraorbital Canal to Treat Trigeminal Neuralgia Affecting the Maxillary Branch.

PURPOSE: To modify the method of treating trigeminal neuralgia (TN) affecting the maxillary branch and to provide clinical evidence of an etiologic hypothesis of narrowing bone canal compression. MATERIALS AND METHODS: Forty patients who met the inclusion and exclusion criteria were included, and those with bony compression of the affected infraorbital canal (IOC) were selected by preoperative computer measurement. Modified decompression of the IOC was performed through the maxillary sinus with the assistance of a piezoelectric device and an endoscope. The results of the operation were evaluated. RESULTS: Fourteen patients (35.00%) had bony compression of the affected IOC. During a mean follow-up period of 29.4 months, 11 patients (78.57%) had complete pain relief (excellent); 1 (7.14%) had moderate relief with carbamazepine administration. The success rate was 85.71% (12 of 14). The postoperative numbness rate was 35.71% (5 of 14). CONCLUSION: Narrowing of the IOC might be a reason some patients with TN have no intracranial neurovascular compression and have pain localized to the distribution of the maxillary branch. Modified decompression of the IOC through the maxillary sinus is a safe, highly effective, and minimally invasive method to treat these patients.

Modified technique for preservation of inferior alveolar nerve during mandibulectomy.

BACKGROUND: The purpose of this article is to introduce the modified technique of preservation of the inferior alveolar nerve (IAN) during mandibulectomy for a benign lesion. METHODS: Five cases of osteofibrous hyperplasia and 3 cases of centricity osteomyelitis were included. During surgery, the IAN was marked using a planned cutting guide. Using an oscillating saw, the depth of the osteotomy along the IAN was controlled until the bone cortex was cut through. After splitting, the bony section was removed, leaving the neurovascular bundle intact. The sensation of the lower lip was evaluated using current perceptive threshold testing during follow-up. RESULTS: After follow-up for 6-27 months, no recurrence or secondary deformity was found. One patient had severe sensory disturbance. CONCLUSION: With the use of a cutting guide and osteotomy tricks, mandibulectomy with preservation of the IAN can be accurately performed.

Detection of exostosin glycosyltransferase gene mutations in patients with non-hereditary osteochondromas of the mandibular condyle.

Exostosin glycosyltransferase (EXT) 1 and EXT2 have been identified as causative genes in osteochondroma; however, it is not known whether these genes are also involved in condylar osteochondromas. The aim of this study was to identify EXT1 and EXT2 mutations in patients with non-hereditary osteochondromas of the mandibular condyle. DNA was obtained from resected tissues (cartilage cap) of 12 patients with solitary condylar osteochondromas. The exons, 3',5'-untranslated regions and intron-exon boundaries of EXT1 and EXT2 were amplified by polymerase chain reaction and the products were sequenced directly. Through direct sequencing, four genetic variations of EXT1 in 4 cases and three variations of EXT2 in 5 cases were identified. The intronic alteration of the EXT2 gene, occurring in 2 cases, was novel, whereas the other alterations had been previously reported. Nonsense somatic mutations were detected in tumor DNA. Our study extended the mutational spectrum in EXT1 and EXT2 and may facilitate a better understanding of the pathophysiology of condylar osteochondromas.

Elevated TGF-ß2 level in aqueous humor of cataract patients with high myopia: Potential risk factor for capsule contraction syndrome.

PURPOSE: To evaluate the level of transforming growth factor-ß2 (TGF-ß2) in the aqueous humor of highly myopic cataract patients and its correlation with capsule contraction syndrome. SETTING: Eye and ENT Hospital of Fudan University, Shanghai, China. DESIGN: Prospective comparative case series. METHODS: The highly myopic cataract patients were divided into the following 2 groups according to the Lens Opacity Classification System III: nuclear color (NC) 2 to 3 and NC 5 to 6. Aqueous humor TGF-ß2 concentrations were assayed in the highly myopic cataract and age-related cataract groups. The TGF-ß2, TGF-ßRII (the type II receptor for TGF-ß2), and α-smooth muscle actin (α-SMA) expressions in lens epithelial cells (LECs) were detected by real-time polymerase chain reaction and immunofluorescent staining. RESULTS: The study comprised 40 highly myopic cataract patients (40 eyes) and 20 patients (20 eyes) with age-related cataract as the control group. Compared with the control group, the highly myopic cataract group had significantly higher TGF-ß2 concentration in the aqueous humor and increased TGF-ßRII expression in LECs, especially in NC 5 to 6 cases. Expression of α-SMA was barely detectable in both groups. CONCLUSION: In highly myopic cataract patients, especially those with dark nuclei, elevated aqueous humor TGF-ß2 levels and the upregulated TGF-ßRII expression in LECs might contribute to the pathogenesis of capsule contraction syndrome through transdifferentiation of LECs into myofibroblasts. FINANCIAL DISCLOSURE: None of the authors has a financial or proprietary interest in any material or method mentioned.

Can a Novel Surgical Approach to the Temporomandibular Joint Improve Access and Reduce Complications?

PURPOSE: This clinical study investigated whether the vascular-guided multilayer preauricular approach (VMPA) to the temporomandibular joint (TMJ) could improve access and decrease complications. PATIENTS AND METHODS: This retrospective evaluation consisted of a consecutive series of patients who underwent TMJ surgeries through the VMPA from January through December 2013. Patients with a history of TMJ surgery were excluded. Clinical data, including operating times, subjective complaints of incision scars, functional conditions of the auriculotemporal nerve and facial nerve, and other complications, were recorded and analyzed. All patients in this study were followed for at least 6 months. RESULTS: All patients (606 joints) had successful TMJ surgeries through the VMPA. All incisions healed favorably with an uneventful recovery. No patient developed permanent weakness of the facial nerve or other severe complications. CONCLUSION: The VMPA can provide direct access and favorable visibility to the TMJ region and yield good esthetic and functional results. The VMPA can be considered the approach of choice for common TMJ surgeries.

Juxta-articular Myxoma of the Temporomandibular Joint.

The juxta-articular myxoma represents a benign mesenchymal neoplasm that arises from tissue within or adjacent to a joint space. There have been a number of reported cases involving myxomas of the knee, shoulder, elbow, wrist, and hip. To our knowledge there, however, have been no reported cases of juxta-articular myxomas of the temporomandibular joint (TMJ). This report describes the case of a 57-year-old woman with a juxta-articular myxoma of the left TMJ extending into the infratemporal fossa (ITF). Access to the tumor was accomplished via a preauricular incision and low condylar osteotomy which allowed for displacement of the condyle for direct visualization and excision of the tumor. The postoperative course was benign and the patient demonstrated no cosmetic or functional limitation. Likewise, follow-up at 30 months showed no evidence of recurrence. Benign encapsulated tumors of the ITF can be effectively accessed by means of a modified preauricular incision, low condylar osteotomy, and anterior meniscal release. This direct approach allows for excellent surgical exposure, minimal surgical site morbidity, and maintenance of physiologic joint function and occlusion.

Digital resection and reconstruction of TMJ synovial chondrosarcoma involving the skull base: report of a case.

Synovial chondrosarcoma (SCS) is a very rare malignant cartilaginous tumor. To the best of our knowledge, only three reported studies presented the involvement of the temporomandibular joint (TMJ). Hereby, we present a case of surgical management of a SCS of the TMJ, arising from SC and involving the skull base. The surgical procedure includes digital design, resection guided by digital templates, as well as immediate reconstruction with free iliac bone graft (IBG) and pedicled sternoclavicular joint (SCJ). At 1-year follow-up, the TMJ function and form were improved with no sign of local recurrence or metastasis to bone or other joints. However, its distant metastasis to lung was observed.

Osteochondroma of bilateral mandibular condyle: a case report.

Osteochondroma represents the largest group of benign tumors of bone, which usually develops in long bones and relatively uncommon in the craniofacial region. The condyle and coronoid tip are the most common sites of occurrence in the mandible, but both sides of condyle involved has never been reported. Here, we describe a case of osteochondroma arising from the bilateral mandibular condyle.

Pigmented villonodular synovitis of the temporomandibular joint with intracranial extension.

Pigmented villonodular synovitis is an uncommon benign tumor-like proliferative lesion with an undetermined origin. Involvement of the temporomandibular joint is uncommon. Although pigmented villonodular synovitis is a benign lesion, it can grow with an aggressive pattern, and it extends extra-articularly in most of the reported cases, about one-third of them exhibiting intracranial involvement. The authors reported an additional case of a 47-year-old woman with intracranial extension, who had a history of joint pain and trismus. The preoperative diagnosis was made with arthroscopy. The lesion was completely excised via preauricular approach and condylotomy. The bone defect was covered by the pedicled temporalis myofascial fat flap. The patient has been symptom-free for 40 months postoperatively.

Synovial chondromatosis of the tempromandibular joint: relationship between MRI information and potential aggressive behavior.

PURPOSE: To evaluate the types of synovial condromatosis (SC) of tempromandibular joint (TMJ) on MRI and their potential aggressive behavior. METHODS: 144 patients with pure SC of TMJ were included in. On MRI, 3 types of the lesion included loose body, homogeneous mass, and mixture of both loose body and homogeneous mass. Bony evaluations included 4 stages of erosion: without erosion, chondral breakdown, bony absorption and bony perforation. RESULTS: 47 (32.6%) cases were categorized in the type of loose body, 71 (49.3%) cases in the type of homogeneous mass, and 26 (18.1%) cases in the type of mixture. 80% of bony perforation belonged to the type of homogeneous mass and 20% belonged to the type of mixture. 2 patients were found recurrence. CONCLUSION: The types of homogeneous mass and mixture of both on MRI were more aggressive. Homogeneous mass with bony erosion on MRI had potential recurrence.

Post-infectious myositis ossificans in medial, lateral pterygoid muscles: A case report and review of the literature.

Myositis ossificans (MO) is a disease where heterotropic bone forms within a muscle or other type of soft tissue. MO is classified into two groups, MO progressiva and post-traumatic MO. It rarely occurs in the masticatory muscles and thus, only 20 cases involving the masticatory muscles have been reported since 2001. The majority of the reported cases occurred due to trauma, repeated injury or surgical manipulation. However, in a small number of cases, no specific traumatic event was identified as the cause of MO. To the best of our knowledge, this is the first case of post-infectious MO to be reported in the medial and lateral pterygoid muscles.