Williams–Beuren syndrome (WS) is a rare genetic disorder that results from microdeletion at chromosome 7, which harbors the elastin gene. Clinical findings include arteriopathy, aortic stenosis, hypertension, and laxities and contractures in different joints throughout the body. While many components of the temporomandibular joint (TMJ) normally contain elastin, there are few reports on TMJ manifestations of WS. This study reports a TMJ ankylosis case in a WS patient and shares insight on a possible link between development of TMJ ankylosis and elastin deficiency in WS patients. A WS patient presented with bilateral TMJ ankylosis and was successfully treated with TMJ gap arthroplasty. Hypermobility of TMJ and lack of elastin in retrodiscal tissue can induce anterior disc displacement without reduction. Due to lack of elastin, which has a significant role in the compensatory and reparatory mechanism of TMJ, WS patients might be prone to TMJ ankylosis.

The purpose of this study was to estimate average lacrimal gland size on computed tomography in korean. The subjects were 137 Koreans who were checked orbital CT in Department of Ophthalmology, Soonchunhyang University School of Medicine from June 1990 to August 1994. The average width of lacrimal gland was 17.15 +/- 1.75mm, A-P length was 9.81 +/- 1.65mm and thickness was 4.57 +/- 0.99mm. The average width, A-P length and thickness of lacrimal gland were not statistically different between males and females(p>0.05).
Humans ; Lacrimal Apparatus* ; Male ; Ophthalmology ; Orbit*

Although shock in sepsis is usually managed successfully by conventional medical treatment, a subset of cases do not respond and may require salvage therapies such as veno-arterial extracorporeal membrane oxygenation (VA ECMO) support as well as an attempt to remove endotoxins. However, there are limited reports of attempts to remove endotoxins in patients with septic shock on VA ECMO support. We recently experienced a case of septic shock with severe myocardial injury whose hemodynamic improvement was unsatisfactory despite extracorporeal membrane oxygenation (ECMO) support. Since the cause of sepsis was acute pyelonephritis and blood cultures grew gram-negative bacilli, we additionally applied polymyxin B direct hemoperfusion (PMX-DHP) to the ECMO circuit and were able to successfully taper off vasopressors and wean off ECMO support. To the best of our knowledge, this is the first adult case in which PMX-DHP in addition to ECMO support was successfully utilized in a patient with septic shock. This case indicates that additional PMX-DHP therapy may be beneficial and technically feasible in patients with septic shock with severe myocardial injury refractory to ECMO support.
Adult ; Cardiomyopathies* ; Endotoxins ; Extracorporeal Membrane Oxygenation ; Hemodynamics ; Hemoperfusion* ; Humans ; Membranes* ; Oxygen* ; Polymyxin B* ; Polymyxins* ; Pyelonephritis ; Salvage Therapy ; Sepsis ; Shock ; Shock, Septic*

We evaluated the histologic change of retina after gas compression of the vitreous followed by intravitreal injection of silicone oil in albino rabbits. The retinal architecture was well preserved and no abnormal findings could be demonstrated by light microscopic and electron microscopic examinaions 8 weeks after surgery.
Intravitreal Injections ; Rabbits ; Retina* ; Retinaldehyde ; Silicone Oils*

We investigated the influence of amniotic membrane application on corneal wound healing after excimer laser PRK in rabbits. PRK of -9.9D with the optical zone of 5.0mm was performed on each right eye of 34 pigmented rabbits and animals were divided into two groups. Preserved human amniotic membrane was applied in 17 eyes to cover the entire cornea for 48 hours(amniotic group) and the other eyes were used as control group. The area of epithelial defect, inflammatory cell infiltration, the number of anterior stromal keratocyte, and corneal haze were evaluated. The epithelium was healed completely within 3 days in all animals and there was no difference between two groups. At postoperative 12 and 24 hours, in amniotic group, the numbers of stromal inflammatory cells were significantly lower(p=0.009) and the numbers of anterior stromal keratocyte were significantly higher(p<0.05) than those in control group. At postoperative 2, 4, 6, 8, and 12 weeks, the scores of corneal haze in amniotic group were lower than those in control group(p<0.05) and , at postoperative 12 weeks, the number of anterior stromal keratocyte in amniotic group was significantly lower than that in control group(p=0.002). The application of amniotic membrane after PRK reduces keratocyte proliferation and corneal haze during corneal wound healing process, possibly by reducing infiltration of inflammatory cells and loss of keratocyte in the ablation area during the early postoperative period.
Amnion* ; Animals ; Cornea ; Epithelium ; Humans* ; Lasers, Excimer* ; Photorefractive Keratectomy* ; Postoperative Period ; Rabbits ; Wound Healing* ; Wounds and Injuries*

The studies about factors influencing the retroprostheic membrane formation, one of major complications after keratoprosthesis implantation, and about histological characteristics of the retroprosthetic membrane were performed. The methods to inhibit formation and proliferation of membrane were also tried. Formation of the membrane according to corneal trephination size and the effects of lens removal to membrane formation were studied. The influence by the design of keratoprosthesis was also studied. Corticosteroid and SIMP (synthetic inhibitor of metalloproteinase) were applied topically to see the inhibitory effects of drugs to membrane. To evaluate the postoperative intraocular inflammation. Interleukin-1beta was assessed in the aqueous humor at postoperative 3 weeks. Regardless of trephination size, all trephination sites were replaced with opaque fibrous membranes. Histologically, in small trephination group (under 6mm) the membrane was regeneration of cornea showing corneal stromal fibroblast migration and proliferation and mature collagen. In large trephination group (over 7mm), the periphery of the membrane was compatible with corneal regeneration, but central portion was filled with granulation tissue. The removal of lens didn`t influenced the formation of membrane formation. In disc type keratoprosthesis implantation group, all the implants were extruded within 1 month due to retroprosthetic membrane formation. In cylinder type keratoprosthesis implantation group, the physical barrier of cylinder stopped the retroprosthetic membrane proliferation and spared the posterior surface of optic portion. The implants were not extruded for average 10 weeks. Corticosteroid and SIMP showed the effects of decreasing mature collagen formation in the membrane. The level of Interleukin-1beta at postoperative 3 weeks showed no difference according to different drugs and the highest level was checked in cylinder type keratoprosthesis implantation group.
Aqueous Humor ; Collagen ; Cornea ; Fibroblasts ; Granulation Tissue ; Inflammation ; Interleukin-1beta ; Membranes* ; Regeneration ; Trephining

No abstract available.
Fontan Procedure* ; Heart Ventricles*

Malignant mixed Mullerian tumor of the ovary is extremely rare. This is heterologous neoplasms which apparently arise from undifferentiated mullerian stroma and is composed of mixture of malignant epithelial and stromal elements. We report a case of malignant mixed mullerian tumor of the ovary with brief literature review.
Female ; Ovary*

Most of keratoprostheses that had been developed did not show consistent successful results due to the high rates of complications including extrusion. We performed this study to develop a newly designed keratoprosthesis using internal scleral fixation and access the mechanical stability and biocompatibility of the keratoprosthesis (Seoul-type keratoprosthesis, S-Kpro). The S-Kpro has the advantage of double fixation-the Gore-Tex skirt is anchored to the cornea and the prolene haptic is anchored internally to the sclera, simultaneously. And the human amniotic membrane(AM) was covered over the cornea and prosthesis to improve the biocompatibility. The 24 rabbits were divided into four groups: group 1(S-Kpro) with scleral and corneal fixation and AM transplantation, group 2 with scleral fixation and AM transplantation but without corneal fixation, group 3 with corneal fixation and Am transplantation but without scleral fixation, group 4 with scleral and corneal fixation but without Am transplantation. The average retention period of group 1(S-Kpro) was 12.3 weeks, which was significantly longer than other groups. The implanted eyes withstanded the intraocular pressure of as high as 200 to 250mmHg. The retroprosthetic membrane was not observed in all cases but retinal detachment and proliferative vitreoretinopathy were developed, and they are thought to be related with the extrusion of the prosthesis. The fibrovascular tissue invasion was not found in the Gore-Tex pores. These results suggest that double fixation technique using internal scleral fixation and AM transplantation is helpful to improve the stability of the kratoprosthesis. The further experiment with total vitrectomy, different species of animals and Gore-Tex with the large-sized pores would be needed.
Animals ; Cornea ; Humans ; Intraocular Pressure ; Membranes ; Polypropylenes ; Polytetrafluoroethylene ; Prostheses and Implants ; Rabbits ; Retinal Detachment ; Sclera ; Vitrectomy ; Vitreoretinopathy, Proliferative

The objective visual acuity test is mandatory in certain cases, such as infants, nonverbal subjects and subjects who need legal judgements. To produce more reliable objective method, we made a new objective system for visual acuity test which is called Seohan visual acuity test(SVT). The SVT system has three elements such as stimuli, display and evaluation. For the visual stimuli, the computer program for optokinetic nystagmus(OKN)test was set up in personal computer to control the size, speed and direction of the stimuli easily. The visual stimuli are presented on HeadMounted-Display(HMD)to separate the stimuli from the environment to keep the uniform size and distance of screen to the eye of subjects and project the stimulus in full central field of patient. Electrooculography(EOG) was used to assess the visual acuity more objectively. To evaluate the usefulness of SVT, the smallest size of the stimulus to elicit OKN(objective visual acuity)with SVT was obtained in 10 eyes in each twelve grade of subjective visual acuity(decimal V). Objective visual acuity(VA)were distributed from 8.3+/-1.87 to 21.4+/-3.1 and showed high correlation(p<0.00 ). The relationship between the objective VA obtained from SVT and subjective VA make a regression line(y=-12.874X+21.303).Subjective VA could be obtained from conversion of objective VA with 95%confidence belt. In conclusion, the objective visual acuity with SVT is highly correlated with subjective visual acuity and SVT using the OKN response to stimuli presented on HMD by the computer program can be useful in assessing visual function objectively.
Electrooculography ; Humans ; Infant ; Microcomputers ; Nystagmus, Optokinetic* ; Visual Acuity*