Objective: To explore the clinical characteristics and diagnosis and treatment plans of neurofibromatosis type 1 (NF1), and to provide references for clinical diagnosis and treatment. Methods : The clinical manifestations and treatment of an 8-year-old female patient with NF1 was reported. A literature review was conducted to summarize the clinical characteristics and therapeutic strategies of NF1. Multiple NF1s occurred on the right cheek, orbit, and eyelid, and recurred after surgical resection. The tumor caused ptosis, incomplete closure, and vision loss in the upper eyelid of the right eye. After a multidisciplinary assessment determined that radical resection was not feasible, selumetinib sulfate targeted therapy was adopted (25 mg, Po, bid), 28 days constitute one treatment course, and 14 courses have been completed, combined with symptomatic ocular treatments, such as Befusu. Result: The follow-up showed that the tumor volume did not continue to increase (stable disease), the uncorrected vision of the right eye improved (0.05 vs 0.1), and no drug-related adverse reactions occurred during the treatment period. The literature review summarizes the diverse clinical manifestations of NF1, with café-au-lait macules, multiple neurofibromas, and Lisch nodules being hallmark features. Currently, surgical intervention remains the most commonly employed and primary therapeutic approach for NF1; however, for patients who do not meet the criteria for surgery, alternative treatment strategies should be considered. MEK inhibitors, such as selumetinib, demonstrate significant efficacy in inhibiting the growth of NF1-associated plexiform neurofibromas, with tumor volume reductions of at least 20% observed in 70% of pediatric patients in the SPRINT clinical trial. Furthermore, these inhibitors exhibit favorable long-term safety profiles. Conclusion Café-au-lait macules, multiple neurofibromas, and Lisch nodules are hallmark features of NF1. Selumetinib is safe and effective for NF1 in the head and neck of children, and it is the preferred treatment option for patients who are not suitable for surgery. Long-term follow-up monitoring of tumor changes and drug safety is required.

Objective : To investigate the application of indocyanine green in near-infrared fluorescence imaging to determine the scope of necrotic bone resection in osteoradionecrosis of the jaw and to provide a reference for clinicians Methods : Eight patients with osteoradionecrosis of the jaws were enrolled. Indocyanine green was intravenously injected through the elbow vein 10 minutes before osteotomy. After conservative resection of necrotic bone lesions based on imaging results, the scope of potential dead bone resection in the area of low fluorescence intensity was gradually expanded at an initial distance of 0.3 cm. Near-infrared fluorescence imaging and fluorescence intensity determination of bone cross-section were performed before and after extended resection. Statistical differences were analyzed. All patients with osteonecrosis underwent regular follow-up to evaluate the postoperative efficacy Results: Indocyanine green was injected into all 8 patients with osteoradionecrosis for near-infrared fluorescence imaging and the scans were clear; the fluorescence intensity of fresh bone wounds with an expanded mandibular resection range of (0.95 ± 0.14) cm was (226.2 ± 15.8) au, which was higher than that based on intraoperative macroscopic observation and radiological results (108.8 ± 3.4) au, (t = 20.718, P<0.001). The postoperative follow-up improvement rate of 8 patients was 87.5%. Conclusion Near-infrared fluorescence imaging with indocyanine green can assist in the successful removal of necrotic bone until fresh bleeding of the jaw wound occurs, which has important clinical value in defining the resection range of osteoradionecrosis of the jaw.

Objective:To improve understanding and treatment of adult Hirschsprung's disease (HD) and Hirschsprung's disease allied disorders (HAD) by investigating the clinicopatho- logical features, diagnostic and treatment methods, and prognosis.Methods:This was a retrospective observational study. The study cohort comprised patients aged 18-65 years admitted to the Sixth Hospital of Sun Yat-sen University between January 2007 and December 2022 who were diagnosed with adult HD or HAD by postoperative pathological examination. Those with severe cardiovascular disease, diabetes mellitus, or cirrhosis of the liver were excluded, leaving 47 patients in the study cohort. Emergency open surgery was performed on patients with life-threatening manifestations, whereas those whose condition was stable received conservative treatment to stabilize them, following which they underwent a standard surgical procedure. Surgical procedures performed included the Duhamel procedure, Soave procedure, subtotal colonic resection, total colonic resection, and creation of a palliative stoma. Variables studied included clinicopathological characteristics, treatment modalities, postoperative complications, and long-term anal function. Complications were evaluated in accordance with the Clavien-Dindo criteria, and long-term anal function according to the 2005 Krickenbeck International Classification Criteria.Results:Of the 47 patients, 33 were men and 14 women, with a median age of 29 (18–51) years. HD was diagnosed in 41 (87.2%) patients and HAD in six (12.8%). The commonest initial symptom was dyspareunia (70.2%,33/47), followed by abdominal distension (57.4%, 27/47) and abdominal pain (44.7%,21/47). The detection rates of HD/HAD by barium enema + defecography, anorectal manometry, and preoperative rectal biopsy were 86.8% (33/38), 16/19, and 7/7, respectively. Three (6.4%) patients had discrepant preoperative clinical and postoperative pathological diagnoses. None of the three misdiagnosed patients had undergone preoperative rectal biopsy. Of the 47 study patients, three chose non-surgical treatment and 44 surgical treatment. All surgeries were successfully completed. Postoperative complications occurred in 19 patients (43.2%), including one death case who had undergone emergency surgery. The median duration of follow-up after surgery was 65 (12–180) months. Three patients in the surgical treatment group were lost to follow-up. Of the remaining 41 patients, 36, three, and two had excellent, good, and poor long-term anal function, respectively. The differences in outcomes between the surgical and non-surgical treatment groups (no patients, one, and two with excellent, good, and poor long-term anal function, respectively) ( Z=-3.883, P=0.001) were statistically significant. Of the 44 patients who underwent surgical treatment, 41 underwent standard surgeries and three emergency surgeries because their conditions were life-threatening. The difference in complication rate between standard surgery and emergency surgery groups (39.0% [16/41] vs. 3/3, χ 2=2.115, P=0.146) was not statistically significant. However, the rate of postoperative Grade III-V complications was lower in the standard surgery group (4.9% [2/41] vs. 2/3, Z=-2.668, P=0.008). Long-term anal function was significantly better in the standard surgery than emergency surgery group (94.7% [36/38] vs. 0/3, Z=-4.935, P=0.001). The 41 standard surgeries included 11 Duhamel's procedures, six Soave's procedures, 19 subtotal colonic resections, three total colonic resections, and two palliative colostomies. The incidence of postoperative complications was significantly superior in the Duhanmels procedures and palliative colostomies group(1/11 and 0/2, P=0.041). Of the 41 patients who underwent standard surgery, 23 underwent open surgery and 18 minimally invasive laparoscopic surgery. The incidence of postoperative Grade III–V complications and long-term anal function were significantly superior in the laparoscopic group than in the open group (all P<0.05). Conclusion:It is easy to misdiagnose adult HD and HAD, surgical treatment is safe and feasible, and its long-term efficacy is good.

Objective:To improve understanding and treatment of adult Hirschsprung's disease (HD) and Hirschsprung's disease allied disorders (HAD) by investigating the clinicopatho- logical features, diagnostic and treatment methods, and prognosis.Methods:This was a retrospective observational study. The study cohort comprised patients aged 18-65 years admitted to the Sixth Hospital of Sun Yat-sen University between January 2007 and December 2022 who were diagnosed with adult HD or HAD by postoperative pathological examination. Those with severe cardiovascular disease, diabetes mellitus, or cirrhosis of the liver were excluded, leaving 47 patients in the study cohort. Emergency open surgery was performed on patients with life-threatening manifestations, whereas those whose condition was stable received conservative treatment to stabilize them, following which they underwent a standard surgical procedure. Surgical procedures performed included the Duhamel procedure, Soave procedure, subtotal colonic resection, total colonic resection, and creation of a palliative stoma. Variables studied included clinicopathological characteristics, treatment modalities, postoperative complications, and long-term anal function. Complications were evaluated in accordance with the Clavien-Dindo criteria, and long-term anal function according to the 2005 Krickenbeck International Classification Criteria.Results:Of the 47 patients, 33 were men and 14 women, with a median age of 29 (18–51) years. HD was diagnosed in 41 (87.2%) patients and HAD in six (12.8%). The commonest initial symptom was dyspareunia (70.2%,33/47), followed by abdominal distension (57.4%, 27/47) and abdominal pain (44.7%,21/47). The detection rates of HD/HAD by barium enema + defecography, anorectal manometry, and preoperative rectal biopsy were 86.8% (33/38), 16/19, and 7/7, respectively. Three (6.4%) patients had discrepant preoperative clinical and postoperative pathological diagnoses. None of the three misdiagnosed patients had undergone preoperative rectal biopsy. Of the 47 study patients, three chose non-surgical treatment and 44 surgical treatment. All surgeries were successfully completed. Postoperative complications occurred in 19 patients (43.2%), including one death case who had undergone emergency surgery. The median duration of follow-up after surgery was 65 (12–180) months. Three patients in the surgical treatment group were lost to follow-up. Of the remaining 41 patients, 36, three, and two had excellent, good, and poor long-term anal function, respectively. The differences in outcomes between the surgical and non-surgical treatment groups (no patients, one, and two with excellent, good, and poor long-term anal function, respectively) ( Z=-3.883, P=0.001) were statistically significant. Of the 44 patients who underwent surgical treatment, 41 underwent standard surgeries and three emergency surgeries because their conditions were life-threatening. The difference in complication rate between standard surgery and emergency surgery groups (39.0% [16/41] vs. 3/3, χ 2=2.115, P=0.146) was not statistically significant. However, the rate of postoperative Grade III-V complications was lower in the standard surgery group (4.9% [2/41] vs. 2/3, Z=-2.668, P=0.008). Long-term anal function was significantly better in the standard surgery than emergency surgery group (94.7% [36/38] vs. 0/3, Z=-4.935, P=0.001). The 41 standard surgeries included 11 Duhamel's procedures, six Soave's procedures, 19 subtotal colonic resections, three total colonic resections, and two palliative colostomies. The incidence of postoperative complications was significantly superior in the Duhanmels procedures and palliative colostomies group(1/11 and 0/2, P=0.041). Of the 41 patients who underwent standard surgery, 23 underwent open surgery and 18 minimally invasive laparoscopic surgery. The incidence of postoperative Grade III–V complications and long-term anal function were significantly superior in the laparoscopic group than in the open group (all P<0.05). Conclusion:It is easy to misdiagnose adult HD and HAD, surgical treatment is safe and feasible, and its long-term efficacy is good.

Objective To study the vestibular function in patients with sudden deafness .Methods Retrospec-tive analysis of 436 cases of patients with sudden deafness ,being divided into two groups according to whether the merger of vertigo .147 cases with vertigo group ,75 cases of which were mild to moderately severe hearing loss .In-clud the rise type of 28 cases ,26 cases of flat type ,21cases of decline type .72 cases of which were severe hearing loss .In 289 cases without vertigo ,157 cases had mild moderate and severe hearing loss .Includ the rise type of 36 cases ,57 cases of flat type ,64 cases of decline type .One hundred and thirty two cases of which were severe hearing loss .Use video electronystagmogram (VNG) to test the vestibular function .Process the data with SPSS 13 .0 soft-ware .Results One hundred and twenty three(83 .7% )cases of the vertigo group suffered from vestibular function decline ,with 51 cases of mild to moderately severe hearing loss ,72 cases of severe and above .165 (57 .1% )cases of the without vertigo group suffered from vestibular function decline .There were 45 cases of mild to moderately se-vere hearing loss ,120 cases of severe and above .35 patients with vertigo patients with BPPV ,28 cases of which happened within 1 week in sudden deafness occured and 80% of which were vestibular function decline .Conclusion Patients with sudden deafness with vertigo suffered from vestibular function damage .Patients with sudden deafness without vertigo also suffered from vestibular function damage .And the more serious the hearing loss ,the higher the vestibular function decline opportunities .

Objective To illustrate the thoracic CT findings of Behcet disease and to assess the CT diagnostic role in patients with Behcet disease. Methods Thoracic CT images and medical records were retrospectively reviewed in 13 patients with thoracic Behcet disease. The diagnosis was based on the criteria set by the international study group for Behcet disease. Results Of 13 patients, subpleural patchy consolidations were showed in 3, bilateral diffuse ground glass opacities in 3, left low lobe collapse with a right small nodule in 1, subpleural solitary pulmonary nodule in 1, bilateral pleural effusion in 2 ,mediastinal lymphoadenopathy in 2, diffuse bilateral miliary lung nodules in 1 during treatment. Thoracic vascular involvement was presented in 8 patients, two with superior vena cava thrombosis, one with left subclavicle artery aneurysm, one with left low pulmonary artery aneurysm and mural thrombosis, one with bilateral low pulmonary arteries and left basilar artery aneurysm, three with right pulmonary artery thrombosis in which there were two with left pulmonary artery occlusion and one with left basilar artery branch aneurysm. Conclusions Thoracic CT findings of Behcet disease are variable and nonspecific. Contrast-enhanced CT scan can be helpful by showing thrombosis of the superior vena cava and pulmonary artery as well aneurysm of the pulmonary artery.

Objective To analyze the rationality of 16 or more multi-slice helical CT (MSCT) utilization for solitary pulmonary nodule (SPN) (<3 cm). Methods One hundred and fifty consultant cases with SPN from 133 different hospitals, examined with 16 or more MSCT, were selected in this study. The reconstructed slice thickness of routine CT scan, thin slice reconstruction thickness, 2D or 3D reconstruction, window wide and level setting, contrast enhancement effect were recorded from consultant films. Thoracic CT scan criteria for SPN were proposed according to the scan guidelines at Stanford University and PubMed articles. The rationality of 16 or more MSCT utilization in other hospitals for SPN was analyzed by two radiologists compared with scan criteria. The diagnostic accuracy in other hospitals was evaluated according to the pathologic and treatment results in our hospital. Results Sixteen-MSCT was performed in 92 cases, 64-MSCT in 54 cases and 40-MSCT in 4 cases. In routine thoracic CT scan, 9--10 mm reconstructed slice thickness was selected in 59 cases, 7--8 mm thickness in 12 cases and 5 mmthickness in 79 cases. Consecutive thin slice thickness (<3 mm) was used for SPN only in 46 cases. OnlyMPR and VR were filmed in 9 cases without thin slice reconstructed axial images. :53 cases (35.3%) were rescanned in our hospital due to the unsatisfied image quality. Of 150 cases, 78 cases had pathologic or treatment results in our hospital and 22 cases (14.7%) were proved to be misdiagnosed in other hospitals, and only 2 cases were misdiagnosed in our hospital. Conclusion There are many irrationalities of 16 or more MSCT utilization in our country which influence the diagnostic accuracy of SPN, Unified thoracic CT scan criteria in our country are needed.

Objective To evaluate the prevalence rate of pulmonary malignant disease detected by low dose spiral CT in people at high risk of lung cancer. Methods Low dose spiral CT scans and chest radiographs in 300 symptom free volunteers from an on going screening study were prospectively evaluated. The study has enrolled 240 smokers, aged 45 years or older, with at least 10 pack years of cigarette smoking and 60 individuals with chronic obstructive pulmonary disease, and without previous cancer history, who were medically fit to undergo thoracic surgery. Low dose CT scans were performed with SR 7000 scanner using spiral mode, 120 kV, 50 mA, pitch 2, 5mm thickness reconstruction and Lightspeed Plus multi slice scanner using spiral mode, 120 kV, 50 mA, pitch 6 to produce 2.5 mm thick image at 2.5 mm increments. All images were assessed with cine display mode on workstation monitor. Results Non calcified nodules were detected in 56 (19%) participants by low dose CT, compared with 9 (3%) by chest radiography. Malignant disease was detected in 4 (1.3%) by CT and 3 (1%) by chest radiography. All 4 cancers were stage I. Lobar or segmental bronchial abnormalities were detected in 9 (3%) participants by CT. Among them, 3 (1%) proved to be early central lung cancer. No bronchial abnormality was detected by chest radiography. The sensitivity and specificity of cancer screening was 43% and 89%, respectively for chest radiograph, 100% and 80%, respectively for CT. The sensitivity of CT was significantly higher than that of radiograph, whereas the specificity showed no statistical difference. Conclusion Preliminary screening study indicates that low dose CT can greatly improve the likelihood of detection for small non calcified nodules and mild bronchial abnormalities, and thus of peripheral and central lung cancer at an earlier stage.

Objective To study the CT findings of early central lung cancer and correlative pathologic basis, and to evaluate the role of CT in the differential diagnosis of central lung cancer Methods Seventeen patients with early central lung cancer and 10 patients with benign lesions resembling the central lung cancer were analyzed Thin section CT was performed with a section thickness of 3 mm or 1 5 mm at Philips SR 7000 or GE Lightspeed Plus scanner The visibility of the lesions was correlated with bronchoscopic and histopathologic findings Results All 17 lesions were visualized at CT Three lesions showed focal bronchial wall thickening and internal bronchial wall irregularity, bronchial mucous coarsening, redness and swollening at bronchoscopy Fourteen lesions showed focal narrowing or obliteration of the bronchial lumen, 4 complicated with subsegmental bronchial mucoid impaction, 10 with obstructive pneumonia or atelectasis Corresponding bronchoscopic view disclosed intraluminal polypoid protrusions The epithelia were involved in 1 case, subepithelia in 5, and cartilaginous involvement in 11 according to the depth of invasion of the bronchial wall at histopathology Among 10 patients with benign lesions, 6 cases were suspected as bronchogenic carcinoma and the possibility of bronchogenic carcinoma was not excluded at CT in 4 cases There were 5 patients with uneven thickening of internal bronchial wall, 2 with lumen blood clots, 1 with lumen nodule, and no abnormalities in 2 patients at bronchoscopy Conclusion Thin section CT has been proved to be a reliable method for demonstrating the mild changes of the bronchi and to be a valuable tool for the diagnosis of early central lung cancer

Objective To evaluate the efficacy of multislice helical CT(MSCT) in the diagnosis of coronary artery disease Methods 30 patients were studied with MSCT CT data were reconstructed to demonstrate the abnormalities of coronary artery and the results were compared with that of angiography Results In patients with heart rate less than 60 BPM, there was no difference to show the main branch of left coronary artery and left descending artery compared with more than 60 BPM( P =0 197 and 0 128,Fisher′exact);and obvious differences in showing left circumflex artery (? 2=5 88, P