Congenital fibrosis of extraocular muscles (CFEOM) syndrome is a genetically determined congenital disorder characterized by non-progressive ophthalmoplegia, restrictive ocular fixation, and ptosis. Its estimated incidence is approximately 1 in 230 000 to 250 000. This paper reports a family with type 3 CFEOM diagnosed at the Second Xiangya Hospital of Central South University. The proband was a 10-year-old female who presented with right esotropia and right upper eyelid ptosis. Whole-exome sequencing revealed a heterozygous c.904G>A mutation in the TUBB3 gene. Genetic testing of family members identified that the proband's mother carried the same mutation and exhibited left eyelid ptosis. The child underwent strabismus correction followed by ptosis repair, both of which led to marked postoperative improvement. For children presenting with congenital extraocular movement restriction and ptosis, genetic testing plays a crucial role in confirming the diagnosis and guiding family analysis. Additionally, individualized surgical intervention can significantly improve both ocular function and cosmetic appearance.
Humans ; Female ; Child ; Ophthalmoplegia/congenital* ; Fibrosis/congenital* ; Blepharoptosis/surgery* ; Mutation ; Tubulin/genetics* ; Pedigree ; Male ; Esotropia/genetics* ; Congenital Cranial Dysinnervation Disorders

Idiopathic Pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease with unknown cause, which is closely related to lung cancer. A serious complication called Acute exacerbation of IPF (AE-IPF) is prone to occur after lung resection. It progresses rapidly without effective treatment and has a poor prognosis. A typical case of AE-IPF after lung cancer surgery was reported, and its clinical characteristics, imaging features, diagnosis and treatment were summarized.
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Disease Progression ; Humans ; Idiopathic Pulmonary Fibrosis/surgery* ; Lung Neoplasms/surgery* ; Treatment Outcome

Objective: To investigate the correlation between the joint function and the histologic grading after total knee arthroplasty, to aid in the early diagnosis and prognostication of arthrofibrosis. Methods: A total of 29 patients including 22 females and 7 males were enrolled retrospectively from October 2015 to October 2020. These patients had a mean age of 63 years (range 41 to 79 years) and underwent total knee revision in Jishuitan Hospital due to joint contraction or loss of range of motion. Histologic assessment was carried out by utilizing immunohistochemistry (IHC) and the Masson staining to evaluate the fibrosis and inflammation of the samples. Results: By light microscopy, early stage arthrofibrosis showed massive proliferation of myofibroblasts and fibroblasts with SMA expression by IHC. In late stage arthrofibrosis, hyaline degeneration occured with extensive hyperplasia of fibrosis-related tissue. The arthrofibrosis samples appeared blue with Masson staining. Lymphocytes showed perivascular distribution. The arthrofibrosis tissue was mostly grade 3 (26 samples) in histologic assessment, moderate grade (25 samples) in ALVAL score, and grade 1 (23 samples) in lymphocyte grading. Fibrosis grading showed an overwhelming correlation with range of motion (ROM) of the joint. The ALVAL score was highly correlated with the WOMAC score. There was also a direct correlation between inflammatory cell infiltration and pain. The fibrosis grading joint with ALVAL score showed a good predictive value of joint function after joint replacement surgery. Conclusions: The histologic assessment score is closely correlated to the joint function with predictive values for the prognosis after joint replacement surgery.
Humans ; Adult ; Middle Aged ; Aged ; Retrospective Studies ; Knee Joint/surgery* ; Fibrosis

BACKGROUND: Critical patients with the coronavirus disease 2019 (COVID-19), even those whose nucleic acid test results had turned negative and those receiving maximal medical support, have been noted to progress to irreversible fatal respiratory failure. Lung transplantation (LT) as the sole therapy for end-stage pulmonary fibrosis related to acute respiratory distress syndrome has been considered as the ultimate rescue therapy for these patients. METHODS: From February 10 to March 10, 2020, three male patients were urgently assessed and listed for transplantation. After conducting a full ethical review and after obtaining assent from the family of the patients, we performed three LT procedures for COVID-19 patients with illness durations of more than one month and extremely high sequential organ failure assessment scores. RESULTS: Two of the three recipients survived post-LT and started participating in a rehabilitation program. Pearls of the LT team collaboration and perioperative logistics were summarized and continually improved. The pathological results of the explanted lungs were concordant with the critical clinical manifestation, and provided insight towards better understanding of the disease. Government health affair systems, virology detection tools, and modern communication technology all play key roles towards the survival of the patients and their rehabilitation. CONCLUSIONS LT can be performed in end-stage patients with respiratory failure due to COVID-19-related pulmonary fibrosis. If confirmed positive-turned-negative virology status without organ dysfunction that could contraindicate LT, LT provided the final option for these patients to avoid certain death, with proper protection of transplant surgeons and medical staffs. By ensuring instant seamless care for both patients and medical teams, the goal of reducing the mortality rate and salvaging the lives of patients with COVID-19 can be attained.
Aged ; Betacoronavirus ; Coronavirus Infections ; complications ; mortality ; Extracorporeal Membrane Oxygenation ; Humans ; Lung Transplantation ; methods ; Male ; Middle Aged ; Pandemics ; Pneumonia, Viral ; complications ; mortality ; Pulmonary Fibrosis ; mortality ; surgery ; Respiratory Distress Syndrome, Adult ; mortality ; surgery

BACKGROUND: To evaluate the versatility and reach of modified nasolabial flap used in reconstruction of defects created in and around the oral cavity. METHODS: A total number of 20 cases were selected. Out of which 13 were males and 7 females. The age of these patients ranged from 24–63 years. 29 modified nasolabial flaps were raised in twenty patients. Based on clinical and histopathological examination, out of 20 patients, 14 patients were diagnosed with oral submucous fibrosis, 3 with verrucous carcinoma, 1 with squamous papilloma, 1 with oro-antral fistula and 1 with traumatic loss of lower lip. RESULTS: Minimum preoperative interincisal distance (IID) was 0 mm and maximum was 15 mm with mean of 6.00±4.76 mm in patients with oral submucous fibrosis and 12 months postoperatively minimum IID was 16 mm and maximum was 41 mm with mean of 28.00±8.96 mm. In one case, dehiscence (3.4%) was noted on the anterior tip for which tip revision was done. Bulky appearance of the flap intraorally was observed in 2 cases (6.9%). Five (17.2%) among the 29 flaps had visible scar at the donor site postoperatively up to 3 months. CONCLUSION: Numerous reconstructive techniques have been employed in the reconstruction of small to intermediate sized defects of oral cavity. Modified nasolabial flap is a versatile flap which has robust vascularity and can be successfully used with minimal complications. It can be rotated intraorally to extend from the soft palate to the lip. Thus, it can be used efficiently to treat the small defects of the oral cavity as well as recreating lost lip structure.
Carcinoma, Verrucous ; Cicatrix ; Female ; Fistula ; Humans ; Lip ; Male ; Mouth ; Oral Submucous Fibrosis ; Palate, Soft ; Papilloma ; Precancerous Conditions ; Reconstructive Surgical Procedures ; Surgery, Oral* ; Surgical Flaps ; Tissue Donors

Increasing number of endovenous techniques are available for the treatment of saphenous vein reflux and endovenous laser ablation (EVLA) is a frequently used method. A newly developed alternative, based on thermal therapy, is endovenous microwave ablation (EMA). This study evaluated the effect of the two procedures, in terms of coagulation and histological changes, in occluding lateral veins in goats. Twelve animals were randomized into two group, with 6 treated with EMA (EMA group), and the rest 6 with EVLA (EVLA group). Results of coagulation, including coagulation, fibrinolysis and platelet activation, were assessed at three or four different time points: before, immediately after, 24 h (and 48 h) after ablation. The diameter change, a measure of efficacy, was ultrasonographically measured before and 1 month after the ablation. Histological changes were grossly and microscopically evaluated immediately, 1 and 3 month(s) after the ablation. The length of the ablated vein and preoperative average diameter were comparable between the two groups. In both EMA and EVLA groups, several coagulation parameters, fibrinolysis and platelet activation parameters only underwent slight changes. Ultrasound imaging displayed that the diameter reduction of the veins treated by EMA was significantly larger than by EVLA, in consistent with the results of macroscopic examination. Microscopic examination revealed necrosis and thickening of the vein wall, and occlusion of the lumen within 3 months after ablation in both EMA and EVLA groups. It is concluded that EMA is a minimally invasive therapy, which appears to be safe and effective for treatment of lateral veins in goats.
Animals ; Female ; Fibrinolysis ; Fibrosis ; etiology ; Goats ; Laser Coagulation ; adverse effects ; instrumentation ; methods ; Male ; Microwaves ; therapeutic use ; Necrosis ; etiology ; Platelet Activation ; Postoperative Complications ; Venous Insufficiency ; etiology ; surgery

OBJECTIVETo observe the fibrosis of skin after damage to the fat dome structure in skin of pig.

METHODSTotally 4 pieces of skin grafts of intermediate thickness in the size of 5 cm × 5 cm were obtained from both sides beside the spine of back in each of the 4 female red Duroc pigs with pedicle on one side with Humby knife performed by burn specialists, who were rich in clinical experience. These skin grafts were assigned as thin dermis group (TD). Pedicled tissue grafts in the size of 5 cm × 5 cm with the thickness of 1.5 mm were obtained within the wounds resulted from former incision with the same method mentioned above, and these tissue grafts were set as fat dome group (FD). The above-mentioned two groups of skin grafts were sutured back in situ immediately after completion of the former procedures. On post surgery day (PSD) 7, 14, and 21, 5 wounds were respectively selected according to the random number table for gross observation of the surgical areas. Tissue samples were obtained from corresponding surgical area deep to the deep fascia after gross observation at above-mentioned time points. Some of the tissue samples were used for observation of distribution of collagen fibers in the regions of operation of both groups of skin grafts with HE staining, and the breadth of fibrosis was measured; some of the tissue samples were used for observation of distribution of type I or III collagen fibers in the regions of incision of both two groups of skin grafts with Sirius red staining. Data were processed with two independent sample t test.

RESULTSA little scab on the edge of wounds was observed on PSD 7; all the wounds were healed on PSD 14; a few hairs were observed growing in the surgical area on PSD 21. HE staining showed that traces of incision were observed in the superficial layer of dermis and at the junction between dermis and fat dome at each time point; profuse hyperplasia of collagen fibers with parallel and orderly arrangement were observed in the region of incision of skin grafts in groups TD and FD at each time point. The breadth of fibrosis of the region of incision of skin grafts was respectively (251 ± 31), (240 ± 3 7), and (342 ± 69) µm in group TD, (239 ± 36), (286 ± 61), and (332 ± 28) µm in group FD on PSD 7, 14, 21, without significantly statistical difference (with t values respectively 0.750, -1.971, and 0.375, P values above 0.05). Sirius red staining showed that large amount of type III collagen fibers and small amount of type I collagen fibers arranging parallelly were present in the region of incision of skin grafts in groups TD and FD at each time point.

CONCLUSIONSUnder the circumstances of relatively intact restoration of dermal tissue, no excessive fibrosis was observed after simple incisional injury of fat dome in skin of pig.

Animals ; Burns ; surgery ; Dermis ; surgery ; transplantation ; Female ; Fibrosis ; complications ; Graft Survival ; Male ; Skin ; Skin Transplantation ; methods ; Skin, Artificial ; Swine ; Wound Healing

Bone Diseases, Developmental ; surgery ; Female ; Femur ; pathology ; surgery ; Fibrosis ; surgery ; Humans ; Middle Aged

BACKGROUNDRetroperitoneal fibrosis (RPF) is an uncommon disease that is characterized by development of fibrosclerotic tissues involving retroperitoneal structures. This study aimed to investigate the clinical features of 30 patients with RPF in a single center in Beijing in a 10-year period.

METHODSWe retrospectively analyzed clinical data on demographic characteristics, clinical manifestations, laboratory findings, radiological findings, modalities of treatments, outcomes and prognosis of 30 patients with RPF. Patients were treated in Beijing Chao-Yang Hospital between January 2003 and December 2013.

RESULTSThe mean age of patients with RPF was 56.7 ± 14.4 years. Twenty-three patients were men and seven patients were women. Acute phase reactants were elevated in most patients. Rheumatic factor was positive in 4/25 (16.0%) patients, and antinuclear antibody was positive in 6/22 (27.3%) patients. Elevation of IgG4 was observed in 9/22 (40.9%) patients. The most common type was I + III (n = 13), followed by I + II + III (n = 12). Five patients undertook an 18 F-fluoro-deoxy-D-glucose positron emission tomography examination and increased uptake was detected in four patients. Eight patients received combination therapy with glucocorticoids and tamoxifen. Surgical intervention treatments included intraureteral double-J stent implantation (n = 26), percutaneous nephrostomy (n = 2), open ureterolysis and intraperitonealization of the ureters (n = 5) and laparoscopic ureterolysis and intraperitonealization of the ureters (n = 5). Three patients underwent hemodialysis because of renal failure.

CONCLUSIONSClinical characteristics of RPF patients in our study are similar to those previously reported. Steroids and immunosuppressive therapy combined with ureterolysis could be a viable choice of treatment for RPF. More prospective, multi-center studies with a longer follow-up are warranted.

Adult ; Aged ; Female ; Glucocorticoids ; therapeutic use ; Humans ; Immunoglobulin G ; blood ; Male ; Middle Aged ; Retroperitoneal Fibrosis ; blood ; diagnosis ; drug therapy ; surgery ; Retrospective Studies ; Tamoxifen ; therapeutic use ; Treatment Outcome

STUDY DESIGN: Agreement study. PURPOSE: To validate the interrater reliability of the histopathological classification of the post-laminectomy epidural fibrosis in an animal model. OVERVIEW OF LITERATURE: Epidural fibrosis is a common cause of failed back surgery syndrome. Many animal experiments have been developed to investigate the prevention of epidural fibrosis. One of the common outcome measurements is the epidural fibrous adherence grading, but the classification has not yet been validated. METHODS: Five identical sets of histopathological digital files of L5-L6 laminectomized adult Sprague-Dawley rats, representing various degrees of postoperative epidural fibrous adherence were randomized and evaluated by five independent assessors masked to the study processes. Epidural fibrosis was rated as grade 0 (no fibrosis), grade 1 (thin fibrous band), grade 2 (continuous fibrous adherence for less than two-thirds of the laminectomy area), or grade 3 (large fibrotic tissue for more than two-thirds of the laminectomy area). A statistical analysis was performed. RESULTS: Four hundred slides were independently evaluated by each assessor. The percent agreement and intraclass correlation coefficient (ICC) between each pair of assessors varied from 73.5% to 81.3% and from 0.81 to 0.86, respectively. The overall ICC was 0.83 (95% confidence interval, 0.81-0.86). CONCLUSIONS: The postoperative epidural fibrosis classification showed almost perfect agreement among the assessors. This classification can be used in research involving the histopathology of postoperative epidural fibrosis; for example, for the development of preventions of postoperative epidural fibrosis or treatment in an animal model.
Adult ; Animal Experimentation ; Classification* ; Failed Back Surgery Syndrome ; Fibrosis* ; Humans ; Laminectomy ; Masks ; Models, Animal* ; Pathology ; Postoperative Period ; Rats, Sprague-Dawley