ObjectiveTo evaluate the effectiveness of Cross-Union surgery for the treatment of pseudarthrosis of the tibia (PT) with neurofibromatosis type 1 (NF1). MethodsThe clinical data of 8 children of PT with NF1 who met the selection criteria between January 2018 and December 2023 was retrospectively analyzed. There were 5 boys and 3 girls, and the operative age ranged from 1.8 to 13.3 years with a median age of 3.5 years. According to Paley classification, there were 2 cases of type 2a, 2 cases of type 3, 2 cases of type 4a, and 2 cases of type 4c. There were 5 cases of first operation and 3 cases of re-fracture after previous operation. Six cases had leg length discrepancy before operation, and 2 of them had shortening over 2.0 cm. Except for 1 case of ankle fusion, the other 7 cases had ankle valgus. Preoperative coronal/sagittal angulation was recorded. Postoperative pseudarthrosis healing and refracture were observed. Leg length discrepancy and tibiotalar angle were measured and recorded before operation and at last follow-up. Inan imaging evaluation criteria was used to evaluate the imaging effect. ResultsAll patients were followed up 12-37 months (mean, 23.5 months). One pseudarthrosis failed to heal at 12 months after operation and healed at 3 months after reoperation, while the other pseudarthrosis healed with a healing rate of 87.5% and a healing time of 4-8 months (mean, 5.3 months). No refracture occurred during the follow-up. At last follow-up, there were 2 new cases with leg length discrepancy, which were 0.7 cm and 1.3 cm, respectively. In 2 cases with the leg length discrepancy more than 2.0 cm before operation, the improvement was from 4.1 cm and 12.6 cm to 2.1 cm and 9.0 cm, respectively. There was no significant difference in leg length discrepancy between pre- and post-operation in 8 cases (P>0.05). At last follow-up, 6 patients still had ankle valgus, and there was no significant difference in the tibiotalar angle between pre- and post-operation (P>0.05); the tibial coronal/sagittal angulation significantly improved when compared with that before operation (P<0.05). According to Inan imaging evaluation criteria, 1 case was good, 6 cases were fair, and 1 case was poor. Conclusion Cross-Union surgery is an effective method for the treatment of PT with NF1 in children, can achieve good bone healing results with a low risk of re-fracture. The surgery may not have significant effects on leg length discrepancy and ankle valgus, and further treatment may be required.
ObjectiveTo observe the changes of peripapillary vessel density and retinal nerve fiber layer parameters (RNFL) in diabetic mellitus (DM) patients with early diabetic retinopathy (DR).MethodsA retrospective clinical study. From January to December 2018, twenty-eight DM patients (47 eyes, DM group) and 20 normal subjects (40 eyes, control group) in Eye Hospital of Wenzhou Medical University at Hangzhou were included in the study. There was no significant difference between the two groups in age (t=－1.397, P=0.169) and sex composition ratio (χ2=0.039, P=0.843). The optic nerve head was scanned by OCT angiography (OCTA) with HD 4.5 mm ×4.5 mm imaging scanning mode for all subjects. The peripapillary radial peripapillary capillaries vessel density (ppVD) and peripapillary retinal nerve fiber layer (pRNFL) thickness were measured. The changes of ppVD and pRNFL thickness between the two groups were observed. Pearson correlation analysis was used to analyze the correlation between ppVD and pRNFL in each quadrant.ResultsCompared with the control group, the mean ppVD and superior-hemi part, inferior-hemi part, superior, nasal, inferior and temporal quadrant ppVDs of DM group were all significantly lower than those of control group (t=5.107, 4.360, 3.713, 4.007, 2.806, 4.046, 2.214; P＜0.05). The mean and all quadrants pRNFL thickness were lower in eyes of DM group compared with the control, and the superior and inferior quadrant pRNFL thickness were statistically significant (t=2.117, 2.349; P＜0.05), while the mean pRNFL and superior-hemi, inferior-hemi part, nasal and temporal quadrant were not statistically significant (t=1.867, 1.717, 1.869, 0.720, 0.303; P＞0.05). Pearson correlation analysis showed that the significant high-positive correlation was found between ppVD and pRNFL thickness in the nasal quadrant (r=0.734, P＜0.001).ConclusionIn early DR patients, ppVD decreased and pRNFL thinned.
ObjectiveTo investigate the relationship between optic disc hemorrhage and localized retinal never fiber layer defects (RNFLDs) in norma l tension glaucoma.MethodsIn 83 patients with normal-tension glaucoma, the cumulative frequency and quadrantal distribution of optic disc hemorrhages were retrospectively analyzed. The neighboring relation between optic disc hemorrhages and RNFLDs in a same quadrant and the changes of correspondin gretinal never fiber layer (RNFL) after the occurrence of optic disc hemorrhages were observed by tridimensional photochromy of ocular fundus.Results(1) The occurrences and distribution of optic disc hemorrhages: 29of83(34.94%) patients (33 eyes) had totally 58 occurrences, including 39 in infer iotemporal area, 14 in superiotemporal area, and 5 in other area. (2) The relati onship of neighborhood between optic disc hemorrhages and RNFLDs: in the availab le tridimensional photochrome, 23 occurrences in 15 patients (16 eyes) were foun d with cuneiform RNFLDs in the same quadrant, in which 22 was near the border of cuneiform RNFLDs. (3) The changes of corresponding retinal never fiber layer (R NFL) after the occurrence of optic disc hemorrhages: the photochromes of 24 occurrences in 20 patients (21 eyes) were kept well in the initial and the 2-year follow-up periods, while the changes of RNFL were found in each region correspon ding to the 19 occurrences (in inferiotemporal or superiotemporal area) in the initial photochrome, including 7 cuneiform defects with various sizes, and 12 developed localized RNFLDs next to the initial hemorrhages in the optic disc. No obvious localized RNFL corresponding to the other 5 occurrences (1 in inferiotempo ral, 1 in superiotemporal, and 3 in other areas) were found in the follow up period.ConclusionOptic disc hemorrhages in normal-tension glaucoma occur mostly in inferiotemporal area, and secondly in superiotemporal area of optic disc, and the appearance of optic disc hemorrhages may suggest that the localized RNFLDS would develop in the associated regions.(Chin J Ocul Fundus Dis，2004，20：339-342)
Objective To observe the changes of retinal nerve fiber layer (RNFL) thickness in patients with Alzheimer's disease (AD). Methods Twenty eyes of 40 patients with mild and (or) moderate AD confirmed by clinical examination (AD group) were included in the study. There were 11 males and 9 females with an average age of (72.75±8.25) years. Age and gender-matched normal 20 objectives were in the normal control group. Among them, there were 11 males and 9 females with a mean age of (71.05±7.08) years. There was no significant difference in gender composition, age and intraocular pressure between the two groups (P＞0.05). There were significant differences in visual acuity, cup disc ratio and mini-mental state examination score (P＜0.05). All eyes underwent high-resolution optical coherence tomography (OCT) examination. With a diameter of 3.4 mm and a center on the center of the optic disc, circular fast scans on optic disc were performed to obtain an average disc RNFL thickness, signal threshold ＞6. Computer image analysis system was used to measure the RNFL thickness from superior, inferior, temporal and nasal quadrants, and the average RNFL thickness. The changes of RNFL thickness between the two groups and between different eyes of the same group were compared. Results Compared with the normal control group, the average (t=5.591), superior (t=8.169, 8.053) and inferior (t=12.596, 11.377) thickness of RNFL in both eyes in AD group were thinner, the differences were significant (P＜0.05); the temporal (t=1.966, 0.838）and nasal (t=2.071, 0.916) thickness of RNFL in both eyes of AD group were thinner, but the difference was not statistically significant (P＞0.05). There was no significant difference of the mean and different quadrant RNFL thickness between different eyes in AD group and normal control group (AD group: t=0.097, 0.821, 0.059, 0.020, 0.116; normal control group: t=0.791, 1.938, 1.806, 2.058, 1.005; P＞0.05). Conclusion The RNFL thickness around the optic disc in AD patients is thinner; This occurs first in superior and inferior quadrants of the optic disc.
Neurofibromatosis type 1 (NF1) is an autosomal dominant neoplastic disease caused by mutations in the NF1 gene and one of the most challenging diseases to treat. Patients have a characteristic phenotype with neurofibromas as the main features in different forms, including numerous cutaneous neurofibromas, plexiform neurofibromas involving the primary nerves, or malignant peripheral nerve sheath tumors with a very short survival period after malignant transformation. NF1 patients also suffer from multi-system involvement, with a high rate of deformity and disability, making complete surgical resection more difficult. Currently, there is no consensus on the diagnosis and treatment of NF1 in China, and different disciplines have different understandings of NF1. Multidisciplinary systematic evaluations and cooperative treatments are the keys to improve the treatment, quality of life, and prognosis of NF1 patients. In 2020, the Department of Plastic Surgery of the Ninth People’s Hospital of Shanghai Jiaotong University School of Medicine led the establishment of the first multi-center collaboration group for NF1 in China. Furthermore, the group had worked with renowned experts from the various departments including surgical oncology, medical oncology, dermatology, reproductive medicine, et al. in China to formulate the “Expert consensus on diagnosis and management of neurofibromatosis type 1 (2021 edition)”, aiming to promote standardized and homogeneous treatment covering the whole life cycle of NF1 patients and improve the treatment level and outcome of NF1 patients in China.
ObjectiveTo investigate the time relationship of the change, and diagnostic accuracy and sensitivity between retinal light threshold fluctuations (LTF) and retinal nerve fiber layer (RNFL) and ganglion cell complex(GCC) thickness on high-risk primary open-angle glaucoma (POAG). MethodsTotally 319 patients (319 eyes) with high-risk in POAG from the First Affiliated Hospital of Kunming Medical Universityand during December 2009 and December 2017, 50 healthy individuals (50 eyes) as control were collected in this longitudinal cohort study. Visual field and OCT were reviewed every 6 months on the high-risk group and every 12 months on the control group. High-risk groups inclusion criteria: vertical C/D≥0.6; early visual field defect (according to glaucoma visual field damage GSS2 quantitative grading standards, mean deviation and pattern standard deviation of central field exceeds the border as an early visual field defect); continuous repeatable results. The first field and OCT results in the absence of visual field defects and C/D≥0.6, which were conformed reliability indicators and removed learning effects as a baseline. When patients achieve POAG diagnosis criteria first time which was recorded as a turning point. And they were divided into early group meanwhile were ended of follow-up. After the last follow-up, the inspection data was segmented counted in yearly interval. The changes of LTF, thickness of RNFL and GCC during the follow-up period in the early POAG group and the control group were observed. The loss rate and change rate in each period were compared for the assessment of their trends with time. Followed by calculation of the area under receiver operating curves (AUC) to compare the predicted value of POAG and the sensitivity at 95% specificity in each period. ResultsAfter last follow-up, totally 67 patients 67 eyes (early POAG group, 37 males and 30 females) were entered the turning point. The mean follow-up of the early POAG group and the control group were 6.6 and 6.4 years. The average RNFL thickness was 79.05±8.09 μm, GCC thickness was 71.58±8.41 μm, LTF was −6.05±7.02 dB in early POAG group. The average RNFL thickness was 93.49±6.24 μm, GCC thickness was 79.72±6.32 μm, LTF was −0.31±0.58 dB in the control group. The differences of LTF and the thickness of RNFL and GCC were statistically significant (t=−5.97, −10.42, −5.60; P＜0.001). The AUC of RNFL, GCC thickness and LTF increased with time in the early POAG group. The sensitivity was gradually increased at 95% specificity: 5th year before to at turning point, RNFL thickness AUC was 0.15, 0.65, 0.71, 0.77, 0.85, 0.92, and sensitivity was 20%, 56%, 61%, 65%, 70%, 76%, respectively; GCC thickness AUC was 0.12, 0.53, 0.69, 0.74, 0.82, 0.90, and sensitivity was 14%, 53%, 69%, 74%, 82%, 90%, respectively; the AUC of LTF was 0.10, 0.21, 0.33, 0.75, 0.86, 0.91, and sensitivity was 7%, 17%, 44%, 65%, 78%, 87%, respectively. ConclusionsThe earliest time of structural functional damage of POAG is at the 4th year before confirmed, simultaneous RNFL diagnosis accuracy and sensitivity are better than GCC and LTF. The earliest time of visual functional damage of POAG is at the 2th year before confirmed, simultaneous LTF diagnosis accuracy and sensitivity are better than RNFL and GCC.
Objective To summarize the latest developments in neurosurgical treatments for neurofibromatosis type 1 (NF1) and explore therapeutic strategies to provide comprehensive treatment guidelines for clinicians. Methods The recent domestic and international literature and clinical cases in the field of NF1 were reviewed. The main types of neurological complications associated with NF1 and their treatments were thorough summarized and the future research directions in neurosurgery was analyzed. Results NF1 frequently results in complex and diverse lesions in the central and peripheral nervous systems, particularly low-grade gliomas in the brain and spinal canal and paraspinal neurofibromas. Treatment decisions should be made by a multidisciplinary team. Symptomatic plexiform neurofibromas and tumors with malignant imaging evidence require neurosurgical intervention. The goals of surgery include reducing tumor size, alleviating pain, and improving appearance. Postoperative functional rehabilitation exercises, long-term multidisciplinary follow-up, and psychosocial interventions are crucial for improving the quality of life for patients. Advanced imaging guidance systems and artificial intelligence technologies can help increase tumor resection rates and reduce recurrence. Conclusion Neurosurgical intervention is the primary treatment for symptomatic plexiform neurofibromas and malignant peripheral nerve sheath tumors when medical treatment is ineffective and the lesions progress rapidly. Preoperative multidisciplinary assessment, intraoperative electrophysiological monitoring, and advanced surgical assistance devices significantly enhance surgical efficacy and safety. Future research should continue to explore new surgical techniques and improve postoperative management strategies to achieve more precise and personalized treatment for NF1 patients.
Objective:To observe the changes of the thickness of reti nal nerve fiber layer (RNFL) of optic disc in rats with chronic glaucoma continuously dete cted by optic coherence tomography (OCT). Methods:A total of 48 Wist ar rats (24 males and 24 females) were randomly divided into 3 groups with 16 ra ts (32 eyes) in each group. The right eyes were the photocoagulation eyes and the left ones were as the control. Laser photocoagulation with the wavelength of 532 nm was perfo rmed on the trabecular network of the right eyes to induce the chronic middlelevel oc u lar hypertension. The changes of the intraocular pressure (IOP) were observed. O pticdisc linear scanning of OCT was performed 3, 6, and 9 weeks after IOP incr e ased, and the thickness of RNFL of optic disc was detected by the computer. Eight rats in each group were killed and retinal histology slic es were used to detect the thickness of RNFL. The flatmount s of retina from the right eyes of the other 8 rats in each group were stai ned by 1% toluidine blue. The density of retinal ganglion cells (RGC) was calcul ated and the results were compared and analyzed. Results:IOP o f the rats increas ed chronically and moderately after photocoagulation. IOP of the experimental ey e 3,6, and 9 weeks after photocoagulation was obviously higher than which of the control eyes, respectively （P＜0.001）. The results of OCT showed that the thickness of the RNFL of the experimental eyes was (67.39plusmn;5.91) mu;m, (53.4 2plusmn;5.64) mu;m，and (44.35plusmn;5.76) mu;m 3, 6, and 9 weeks after photocoagulation, and the corresponding thickness in the control eyes was（80.32plusmn;5.87）, （79.69plusmn;5.69）, and （80.78plusmn;5.84）mu;m, respectively. The thickness of the retinal fiber layer detecte d by histological method was （64.38plusmn;6.54）, （51.47plusmn;6.4）, and （42.10 plusmn;6.10）mu;m in the experimental eyes 3, 6, and 9 weeks after photocoagulation, and (76.23plusmn;6.78), (78.64plusmn;6.15), and (77.64plusmn;6.63) mu;m in the control eyes. Regression analysis of the thickness detected by the two methods was made, and the regression coefficients was 0.932（P＜0.001）.The differ ence of the ave rage density of RGC between the two groups was significant (P＜0.05）. Conclusi on:Glaucoma model in Wistar rats may successfully set up b y photocoagulating the trabecular meshwork. The thickness of retinal nerve fiber layer of the optic disc in rats with chronic glaucoma detected by OCT and obser ved by the light m icroscope is accordant. The changes of the thickness of RNFL in rats with chroni c glaucoma could be continuously detected by OCT to investigate the progress of the glaucomatic retinopathy in rat model.