Retinal detachment in Ehlers-Danlos syndrome. Treatment by pars plana vitrectomy

Ehlers-Danlos syndrome (EDS) is an hereditary connective tissue disorder caused by defective collagen synthesis, the main features being hyperelasticity and vulnerability of the skin, recurrent bleeding from fragile blood vessels, and secondary deformities of the joints. Ocular involvement is a rare occurrence, e.g., corneal and scleral rupture from minor blunt injury, lens displacement, rhegmatogenous retinal detachment. To date, few reports exist concerning the treatment of retinal detachment in Ehlers-Danlos syndrome, all of them dealing exclusively with conventional scleral buckling surgery. PATIENT AND METHODS: We report on a 47-year-old male patient suffering from EDS type VI (so-called ocular type, lysine-hydroxylase deficiency). He presented with rhegmatogenous retinal detachment in his only eye. A scleral buckling procedure was not feasible because of marked scleral atrophy. A three-port vitrectomy was therefore carried out. RESULTS: During the operation, pronounced choroidal detachment and bleeding developed, subsiding within weeks postoperatively. Closure of the sclerotomies was difficult due to scleral thinning. Two revitrectomies were necessary because anterior PVR with traction retinal detachment occurred. The last revitrectomy was performed 18 months ago, and the retina has been completely reattached under 5000 cs silicone oil since then. Visual acuity is 0.1. CONCLUSION: Primary vitrectomy permits successful treatment of retinal detachment in EDS patients if a buckling procedure cannot be performed because of scleral atrophy. However, serious complications may occur. Surgical procedures other than primary vitrectomy should therefore always be carefully considered, e.g., pneumatic retinopexy, temporary balloon, dura patch with episcleral pocket.     

Glaucoma after pars plana vitrectomy and silicone oil injection for complicated retinal detachments

OBJECTIVE: To determine the incidence and associations of glaucoma after pars plana vitrectomy (PPV) and silicone oil injection (SOI) for complicated retinal detachments and the response to treatment. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: A total of 150 eyes of 150 patients who had completed a minimum of 6 months of follow-up were included in this study. Analysis included clinical records of all consecutive cases of complicated retinal detachment that underwent PPV with SOI between July 1991 and February 1996. INTERVENTION: Surgical intervention for vitreoretinal pathology included standard three-port PPV and additional procedures as appropriate for the retinal pathology, and SOI. Procedures for the control of glaucoma were silicone oil removal (SOR), trabeculectomy with mitomycin C, cyclocryotherapy, semiconductor diode laser contact transscleral cyclophotocoagulation (TSCPC) and anterior chamber tube shunt to encircling band (ACTSEB). MAIN OUTCOME MEASURES: Presence of glaucoma (predefined as intraocular pressure [IOP] > or = 24 mmHg, which also was > or = 10 mmHg over the preoperative level, sustained for > or = 6 weeks) and the result of medical and surgical management were the main outcome measures. Demographic, preoperative, intraoperative, and postoperative parameters including the age of the patient, etiology of retinal detachment, refractive status, pre-existing glaucoma, aphakia, diabetes mellitus, presence of silicone oil (SO) in the anterior chamber, emulsification of SO, rubeosis iridis, and anatomic success were evaluated by univariate and multivariate logistic regression analyses to assess their predictive value in the causation of glaucoma and to determine factors prognosticating response to treatment. RESULTS: The main indications for PPV+SOI were proliferative vitreoretinopathy (57%; 85 of 150), proliferative diabetic retinopathy (15%; 23 of 150), and trauma (14%, 21 of 150). Glaucoma occurred in 60 eyes (40%) at 14 days median (range, 1 day-18 months). Elevation of IOP could be attributed directly to SO in 42 (70%) eyes. Glaucoma was controlled in 43 (72%) of 60 eyes on treatment (with medicines alone in 30%; SOR and medicines in 25%; trabeculectomy with mitomycin C/ACTSEB/cyclocryotherapy or TSCPC in 17%); 28% (17 of 60) remained refractory. Independent predictive factors for glaucoma on multivariate analysis were rubeosis iridis (odds ratio, 10.76), aphakia (odds ratio, 9.83), diabetes (odds ratio, 6.03), SO in anterior chamber (odds ratio, 4.74), and anatomic failure (negative risk factor; odds ratio, 0.11). Poor prognostic factors for the control of glaucoma were SO emulsification (odds ratio, 15.34) and diabetes (odds ratio, 6.03). CONCLUSION: Glaucoma is a frequent and often a refractory complication of PPV with SOI and has a multifactorial etiology. Aggressive medical and surgical management with SOR, trabeculectomy with mitomycin C, glaucoma shunts, and cyclodestructive procedures shows modest success in controlling IOP.     

Pars plana vitrectomy with pars plana tube implantation in eyes with intractable glaucoma

Fas Ligand (FasL) can induce apoptosis of Fas-bearing cells. It is expressed on the cell surface of many tumor cells, immune-privileged tissues and activated lymphocytes. We report here that FasL can itself transduce signals, leading to cell-cycle arrest and cell death in CD4+ T cells. In vitro, FasL engagement inhibited CD4+ T-cell proliferation, cell-cycle progression, and IL-2 secretion. In vivo, FasL engagement prevented superantigen-mediated CD4+, but not CD8+, T-cell expansion. These findings demonstrate that FasL engagement regulates cell-cycle progression, and show that FasL engagement in vivo has a potent anti-inflammatory effect specific for CD4+ T cells.     

Fluorescein angiography of the fundus after pars plana vitrectomy

In diabetic patients with massive vitreous hemorrhage, fluorescein angiography after pars plana vitrectomy demonstrated varying degrees of vascular involvement depending on the stage of the basic disease process. Arteriolar occlusions, capillary bed drop-out, microaneurysms, and neovascularization were common findings. We concluded that diabetic retinopathy continued its course behind the cloudy vitreous, sometimes leading to retinal ischemia and subsequent spontaneous involution, macular disease, or retinal detachment. In addition, some of these patients were also subject to nondiabetic diseases, such as senile macular degeneration, that could not be detected preoperatively, but that reduced visual acuity postoperatively. In the nondiabetic patients with vitreous hemorrhage, visual acuity after vitrectomy usually depended on the degree of coincident macular disease.     

Early indications for pars plana vitrectomy in idiopathic macular holes

Soluble Fc gamma-binding components were detected in gingival fluid from periodontal lesions by incubation with biotinylated human Fc gamma fragments. Fc gamma III receptor was identified by incubation of gingival fluid with monoclonal antibody. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis and Western transfer showed that most of the Fc gamma-binding components had minimal mobility in a 4-15% gradient gel under nonreducing conditions. Under reducing conditions, the main band of Fc gamma-binding components in gingival fluid migrated corresponding to protein A of 49 kDa. The pattern of Fc gamma-binding components was similar in serum and gingival fluid except for the observation in gingival fluid of Fc gamma-binding components migrating like standard proteins of 19 to 20 kDa, a size that corresponds to the polypeptide part of Fc gamma II receptor and Fc gamma III receptor.     

Primary vitrectomy for bullous rhegmatogenous retinal detachments due to complex breaks

Sixty-five patients who underwent modified radical mastectomy were compared with 55 breast conservation patients, all of whom were in stage I, II or III of breast cancer and were under treatment at an oncology clinic in northern Israel. The main findings indicate no differences between the groups in physical, psychosocial, marital or medical interaction, according to the CARE scale. These findings reflect the results of similar comparative studies conducted during the 1980s and 1990s as documented in the literature. Sexual interaction showed statistical differences favoring the women who had undergone mastectomies.     

MR imaging for traumatic tears of the rotator cuff: high prevalence of greater tuberosity fractures and subscapularis tendon tears

OBJECTIVE: The purpose of this study was to determine whether occult bony injuries or other characteristic MR abnormalities are frequent in patients suspected of having traumatic tears of the rotator cuff. SUBJECTS AND METHODS: MR arthrography of the shoulder was performed in 24 consecutive patients with suspected traumatic tears of the rotator cuff. MR findings were analyzed with regard to abnormalities of the supraspinatus, infraspinatus, and subscapularis tendons; and the humeral head. A comparison group of 24 consecutive patients with symptoms of nontraumatic tears of the rotator cuff was included in the investigation. RESULTS: Radiographically occult fractures of the greater tuberosity were found in nine (38%) of 24 patients with clinically suspected traumatic tears of the rotator cuff (seven of which occurred in patients <40 years old). Nine partial-thickness and five full-thickness supraspinatus tears were found in the trauma group. In the comparison group, the corresponding numbers were 13 and 10, respectively. Seven partial lesions of the cranial border of the subscapularis and six complete subscapularis tears (all six in patients >40 years old) were found in the trauma group (nine and one in the comparison group). CONCLUSION: Occult greater tuberosity fractures and complete subscapularis tears are commonly seen on MR images in patients suspected of having traumatic tears of the rotator cuff. Greater tuberosity fractures should be looked for specifically in patients younger than 40 years, and subscapularis tears should be looked for specifically in patients older than 40 years.     

The restoration of the weight-bearing capacity of the lower extremities and the gait of patients with traumatic detachments

The functional recovery terms are estimated in 65 patients presenting extensive traumatic detachments (decollement) and defects of the lower extremities. The series includes 40 children (61.5 per cent), and 25 adult individuals (38.5 per cent). The distribution of patients by gender, age, surface involved by the lesion, its location and concomitant damage to deep anatomical structures is studied. The patients are subjected to 2-8 years postoperative follow-up. According to type of intervention, they are distributed in three groups, as follows: group one--patients with reimplantation, group two--reimplantation with ensuing necrosis, and group three--cases presenting granulation wounds on admission. The results are evaluated by the static loadbearing test of Buglaev and ichnography, and subjected to statistical processing by variation analysis. As shown by the results, maximal functional recovery in group one is attained by the end of the first year, in group two--by the end of the second year, and in group three--by the end of the fourth year. Application of early exercise therapy, individually prescribed, contributes to the positive outcome.     

Multi-organ ischemia due to traumatic lesion of the descending thoracic aorta--a case-report

This is a case of severe ischemic and reperfusion multi-organ failure due to descending thoracic aortic rupture. A 25 year-old man received a hard blow on his chest in a traffic accident. Chest X-ray, CT scans and aortogram elucidated descending thoracic aortic rupture. Emergency operation was performed 18 hours after the injury. The aortic rupture, 4/5 of the aortic circumference with intact adventitia was seen. The descending aorta 22 distal to the left subclavian artery was repaired under cardio-pulmonary bypass with selective cerebral perfusion. Ischemia due to aortic occlusion with thrombus and reperfusion injury after reconstruction of the aorta connected with rhabdmyolysis and severe multi-organ failure. Intestinal necrosis required laparotomy on the 9th and 15th post operative day. The patient died on 15th postoperative day of multi-organ failure.     

Treatment of pigment epithelial detachments due to age-related macular degeneration with intra-ocular C3F8 injection

AIMS: Epstein-Barr virus (EBV) is associated with a subset of gastric and head and neck carcinomas. While p53 mutation and overexpression is common in gastric cancer, in nasopharyngeal carcinoma p53 is overexpressed yet mutation is uncommon, leading to a proposed viral mechanism of p53 upregulation. We examined the expression of p53 protein in 18 EBV-associated gastric carcinomas (EBV-GA) and compared it with 29 age and sex matched EBV-negative gastric carcinomas (EBV0-GA) and 23 non-nasopharyngeal EBV-associated carcinomas (EBV-CAs) arising from various head and neck regions. METHODS AND RESULTS: Using two monoclonal antibodies (DO7 and PAb1801) with microwave pre-treatment, the p53 protein was scored according to the intensity and percentage of positive cells. The EBV0-GA showed a clear cut bimodal distribution of p53 levels, with either homogeneous intense staining of most tumour nuclei, or only very weak expression in a few cells. Nearly all the EBV-GA and EBV-CAs showed a weak to moderate p53 expression, characterized by heterogeneous intensity of staining in a variable proportion of tumour cells. CONCLUSIONS: The difference in p53 levels in the EBV0-GA and EBV-GA is statistically significant. The heterogeneous level of p53 in the EBV-GA and EBV-CAs and its difference from the EBV0-GA is suggestive of a non-mutational mechanism of p53 upregulation and underscores the role of the virus in the oncogenic pathway.     

Combined pars plana vitrectomy, lens removal and intraocular lens implantation for complications of diabetic retinopathy. Surgical results in 120 cases

We performed combined vitrectomy, lens removal and posterior chamber intraocular lens implantation for proliferative diabetic retinopathy in 120 eyes of 101 patients. Follow-up periods ranged from 3 to 63 months, with a mean of 17 months. Three lens removal methods were used: extracapsular cataract extraction (14 eyes), phacoemulsification and aspiration (49 eyes), and pars plana phacoemulsification (57 eyes). Preoperative rubeosis iridis or neovascular glaucoma was found in 21 eyes. Gas or temporary silicone oil tamponade was employed in 32 eyes. Surgical results were good, and the postoperative vision was finger counts or below only in 13 eyes. Thus the combined surgery proved to have no serious problems. Our results indicate two important points. (1) It is best to chose either of the following two methods for the lens surgery: phacoemulsification with continuous circular capsulorhexis, self sealing sclerocorneal incision, and in-the-bag fixation of the posterior chamber lens, or pars plana phacoemulsification leaving the anterior capsule, rub off and aspirating the lens epithelial cells, continuous circular capsulorhexis, and posterior chamber lens implantation in front of the anterior capsule from a self-sealing sclerocorneal wound. (2) It is mandatory to do complete vitrectomy and cut out the vitreous gels incarcerated in the sclerotomy site.     

Surgical management of retinal detachments related to coloboma of the choroid

We examined the influence of extruded chickpeas and wheat relative to casein and wheat in a dimethylhydrazine (DMH)-induced colon tumor study in male Sprague-Dawley rats. The three diets, based on a modified AIN76 rodent diet with fat present at 10 g/100 g dry matter (DM), were as follows: casein with wheat starch (Cas/S) as control, casein with wheat (Cas/W) and chickpeas with wheat (CP/W). All diets were fed from 5 wk of age throughout the 28-wk study. At 28 wk, there was a significantly lower incidence of large intestinal tumors in rats fed Cas/W relative to those fed CP/W ( 11 vs. 56%, chi-square test, P = 0.018). The colonic tumor burden (tumors/tumor-bearing animal) was not different in Cas/W-fed and CP/W-fed rats (1 vs. 1.7), but the tumor mass index was significantly lower in the former group (0.22 vs. 1.21, P = 0.026). Rats fed the CP/W diet had significantly lower plasma cholesterol concentration (P < 0.01) than rats fed the other two diets. The cecal contents of rats fed the CP/W diet had significantly greater relative weights (46%, P < 0.05) than those of the Cas/W-fed rats; this was associated with higher concentrations of all short-chain fatty acids. Fecal analyses showed significantly (P < 0.05) higher concentrations of total fat (54%), total steroids (83%) and secondary bile acids (179%) in the CP/W-fed rats relative those fed Cas/W. There were higher concentrations of nitrogen in the feces of CP/W rats relative to the Cas/W-fed rats (84%, P < 0.05), associated with greater fecal weights (67%, P < 0.05). Although wheat and its fibers have been shown to be protective against DMH-induced cancers in rats, this was not the case in this study in which chickpeas (45 g/100 g diet) provided the protein and were an important source of soluble fiber. Elevated fat, secondary bile acid concentrations and/or nitrogenous compounds could be responsible for the increased colon tumorigenesis seen and may reflect a legume effect.     

Surgical treatment of serous detachments of the macular neuroepithelium in diabetics

PURPOSE: Maculopathy in diabetes mellitus represents one of the most serious complications of the diabetic retinopathy. Retinal photocoagulations are often impossible and even dangerous. We propose a new method of surgical treatment for this macular serous detachment. MATERIAL AND METHOD: Thirteen eyes from 11 patients were treated using the same surgical procedure: vitrectomy, aspiration of subretinal liquid through a temporal retinotomy, fragmentation and extraction of the sub and intra retinal exsudates through one or several retinotomies, macular massaging with fluorodecaline, endophotocoagulations (focal on vascular anomalies and macular grid) and fluid-gas exchange (C3F8). RESULTS: Macular serous detachment and subretinal exsudates disappeared in all the cases, the fluorescein leakage decreased. A functional improvement was obtained in 11 eyes, a stabilization in 2. DISCUSSION: The clinical results of this new surgical treatment appear to depend on the preoperative macular ischemia and on the age of the detachment. This surgical procedure is very beneficial but could perhaps be technically improved.     

Retinal detachment in the rat

Retinal detachment is uncommon in the rat. Retinal atrophy, rosette formation and folding of the retina are some of the sequelae following reattachment.     

Compensatory effects of glutamatergic inputs to the substantia nigra pars compacta in experimental parkinsonism

The effect of transitory blockage of substantia nigra pars compacta glutamatergic inputs by intracranial injections of kynurenic acid were evaluated in two monkey treated with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). The MPTP protocol was designed to mimic the gradual evolution of human Parkinson's disease. No effects were observed before MPTP treatment or in the first stage of treatment. Once clinical signs appeared, however, motor abnormalities were significantly aggravated by blockage of these inputs (P < 0.001). Conversely, after full Parkinsonism was established, blockage no longer had any behavioural effect. These results confirm the postulated compensatory role of the glutamatergic pathways feeding the substantia nigra pars compacta. This added insight into the physiopathology of the basal ganglia, when compared with previous data on the presymptomatic revelation of experimental Parkinsonism, should help elucidation of the time pattern of evolution of Parkinson's disease.     

Pancreaticoduodenectomy due to cancer in the elderly

The elderly have traditionally been excluded from pancreaticoduodenectomy due to the high morbimortality of this procedure. Six cases of pancreaticoduodenectomy) 5 cephalic and 1 total) for periampullary tumors in patients over 70 are reported. There was no mortality. We conclude that, in selected cases, pancreaticoduodenectomy can be performed safely in the elderly.     

Ileus due to appendicitis in the puerperium

A report is given in a case of appendicitis after delivery in childbed. This was the third case of appendicitis within 17 657 deliveries in the gestation of the district hospital in Sangerhausen in the period from 1960 to 1975. The chronic relapsed appendicitis produced a paralytic Ileus. By using correspondent measures (infusional therapy, appendectomy, adhesion solutions) the patient could be cured without any complications. The patient feeled a Douglas-touch of pain that extended especially to the right. This was the only symptom that was noticed by the patient besides the Ileus that had been clinical well defined and secured with the help of x-ray photo.     

Technical considerations in the management of complex meniscus tears

Meniscal repair continues to be an important procedure in patients with meniscal tears. The orthopedic literature has recommended excision of complex tears or tears that are located in the central avascular region. For the past 13 years, the authors have repaired all meniscal tears that can be reduced anatomically and that have healthy tissue that will support the multiple sutures required for a stable repair, regardless of tear pattern or location. Correct patient selection criteria continue to be important. In all cases, the risks of complications and retear must be considered carefully and judged against the potential benefits of repair. A postoperative rehabilitation program that allows early range of motion, and progression of weight bearing is not detrimental to the healing process if the repair is stable. Close postoperative follow-up examinations are necessary to determine the incidence of meniscal symptoms; however, the absence of joint line pain, catching, locking, or effusion does not guarantee complete healing of the tear. Follow-up arthroscopy is currently the most accurate method to determine if complete or partial healing has occurred.