Hereditary anhidrotic ectodermal dysplasia is a rare condition characterized by underdeveloped ectodermal structure including the skin, teeth or skin appendages. The patient has characteristic feature of anhidrosis, hypotrichosis and defective dentition. We experienced a case of hereditary anhidrotic ectodermal dysplasia in a l-month-old male infant who had unexplained recurring fever, anhidrosis and characteristic facial feature, so we established the diagnosis with clinical feature and skin biopsy.
Biopsy ; Dentition ; Diagnosis ; Ectoderm ; Ectodermal Dysplasia* ; Fever ; Humans ; Hypohidrosis ; Hypotrichosis ; Infant ; Male ; Skin ; Tooth

The graft infection within the abdomen is a notorious condition, which usually develops serious complications of anastomotic rupture or distal embolism that ended in death. There has been many controversies in the treatment of an aortobiiliac graft infection and varying results have been reported. The authors treated a case of aortobiiliac graft infection after abdominal aortic aneurysm surgery. The operation was performed with re-aortobiiliac bypass using bilateral superficial femoral veins. The result was successful.
Abdomen ; Aortic Aneurysm, Abdominal ; Embolism ; Femoral Vein* ; Prostheses and Implants ; Rupture ; Transplants*

Purpose: To observe results in drusen patients with epiretinal membrane after a vitrectomy and membrane peeling. Methods: This retrospective, observational study included drusen patients diagnosed with epiretinal membrane after vitrectomy with membrane peeling. Twenty eyes had epiretinal membrane with drusen (group 1) and 112 eyes had epiretinal membrane without drusen (group 2). At the 6 month follow-up, central foveal thickness and changes in best-corrected visual acuity (BCVA) were evaluated. In Group I, the results with drusen size <65 μm and size >65 μm were compared. The largest drusen size change was measured and compared according to the existence of preoperative intraretinal cysts or ellipsoid zone disruption. Results: Preoperatively and at 6 months postoperatively, the average size of the largest drusen was 198.23 ± 27.34 and 192.23 ± 26.12 μm, respectively, and there was no significant change during the follow-up period. Group I patients with intraretinal cysts and ellipsoid zone disruption experienced low BCVA during the preoperative period; the BCVA had improved postoperatively at 6 months, but with limitations. Conclusions Postoperatively at 6 months, no significant change was observed in the largest drusen size after vitrectomy and membrane peeling. Thus, drusen patients with epiretinal membrane, as well as intraretinal cysts or ellipsoid zone disruption can expect an extended recovery period and slow BCVA improvement.

Purpose: To observe results in drusen patients with epiretinal membrane after a vitrectomy and membrane peeling. Methods: This retrospective, observational study included drusen patients diagnosed with epiretinal membrane after vitrectomy with membrane peeling. Twenty eyes had epiretinal membrane with drusen (group 1) and 112 eyes had epiretinal membrane without drusen (group 2). At the 6 month follow-up, central foveal thickness and changes in best-corrected visual acuity (BCVA) were evaluated. In Group I, the results with drusen size <65 μm and size >65 μm were compared. The largest drusen size change was measured and compared according to the existence of preoperative intraretinal cysts or ellipsoid zone disruption. Results: Preoperatively and at 6 months postoperatively, the average size of the largest drusen was 198.23 ± 27.34 and 192.23 ± 26.12 μm, respectively, and there was no significant change during the follow-up period. Group I patients with intraretinal cysts and ellipsoid zone disruption experienced low BCVA during the preoperative period; the BCVA had improved postoperatively at 6 months, but with limitations. Conclusions Postoperatively at 6 months, no significant change was observed in the largest drusen size after vitrectomy and membrane peeling. Thus, drusen patients with epiretinal membrane, as well as intraretinal cysts or ellipsoid zone disruption can expect an extended recovery period and slow BCVA improvement.

PURPOSE: To present differences in visual acuity and macular structure before and after surgery in patients with idiopathic epiretinal membrane (ERM) according to the presence of retinoschisis. METHODS: This retrospective observational study included 324 eyes with idiopathic ERM, that underwent pars plana vitrectomy with ERM and internal limiting membrane peeling, and were followed for more than 6 months. Subjects were classified into two groups according to the presence of retinoschisis using preoperative optical coherence tomography (OCT; group 1, ERM with retinoschisis; group 2, ERM without retinoschisis). Preoperative and postoperative macular structure changes and surgical outcomes were compared. RESULTS: Group 1 included 61 eyes, and group 2 included 263 eyes. Group 1 had a significantly higher preoperative and final postoperative best-corrected visual acuity compared with group 2 (p = 0.01, p = 0.02). Preoperative disorganization of retinal inner layers (DRIL) was significantly less in group 1 than group 2 (p = 0.01). Preoperative central macular thickness was not significantly different between the two groups. However, postoperative central macular thickness was significantly lower in group 1 than group 2 (p = 0.02, p = 0.01, p < 0.01). The ratio of the inner or outer layer in the total retinal thickness before surgery was significantly smaller in group 1 than in group 2 (p = 0.02, p = 0.04). CONCLUSIONS: Preoperative visual acuity was better and the occurrence of DRIL was less in idiopathic ERM with retinoschisis than without retinoschisis. Postoperative visual and structural outcome was better in idiopathic ERM with retinoschisis than without retinoschisis. Retinoschisis may have played a role in reducing the tractional force given to the inner and outer retina.
Epiretinal Membrane ; Humans ; Membranes ; Observational Study ; Retina ; Retinaldehyde ; Retinoschisis ; Retrospective Studies ; Tomography, Optical Coherence ; Traction ; Visual Acuity ; Vitrectomy

No abstract available.
Atrophy ; Pityriasis* ; Tinea Versicolor*

Limb salvage operation was introduced to treat, low grade malignant bone tumors about 100 years ago. However, amputation has been the most popular choice for primary malignant bone tumors. Recent improvement of chemotherapy, diagnostic methods and surgical techniques enabled us to treat more malignant bone tumors such as osteogenic sarcoma with limb salvage operation. 6 cases of malignant bone tumors were treated with wide resection and tumor prosthesis from Oct., 1987 to Jan., 1989 at Dept. of Orthopedic Surgery, Seoul National University Hospital. 4 cases of osteosarcoma, 1 case of parosteal osteosarcoma and 1 case of chondrosarcoma were identified. MRI was useful for determining the extent of tumor and surgical planning. All the patients osteosarcoma had neoadjuvant chemotherapy. The mean follow-up was 14.5 months ranging from 8 to 22 months, and theirresults are so far good except one pulmonary metastasis and one local recurrence. We present this as a preliminary report.
Amputation ; Chondrosarcoma ; Drug Therapy ; Follow-Up Studies ; Humans ; Limb Salvage ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Orthopedics ; Osteosarcoma ; Prostheses and Implants ; Recurrence ; Seoul

Purpose: To analyze the relationship between corneal morphological factors and polysomnographic factors in patients with obstructive sleep apnea (OSA). Methods: In all, 22 eyes of 22 patients with OSA were analyzed retrospectively under specular microscopy. The central corneal thickness (CCT), endothelial cell density (ECD), hexagonal cell percentage, and cell variation coefficient were measured using specular microscopy, and polysomnographic factors including the apnea-hypopnea index (AHI), respiratory disturbance index (RDI), oxygen desaturation index, and lowest O2 saturation were compared and analyzed between the OSA group and a control group consisting of 32 subjects. Additionally, we examined the statistical correlation between retinal nerve fiber layer (RNFL) thickness and the polysomnographic factors for the 12 OSA patients in which the RNFL thickness was measured. Results: The mean CCT and mean ECD were significantly lower in the OSA group than in controls (p = 0.033, p = 0.021, respectively). As the severity of OSA worsened, a significant negative correlation developed, such that the CCT decreased as the AHI and RDI increased (r = -0.519, p = 0.013 and r = -0.542, p = 0.009, respectively); in addition, the ECD decreased as the RDI increased (r = -0.454, p = 0.034). As OSA progressed, the nasal RNFL thickness decreased with the lowest O2 saturation (r = 0.703, p = 0.011). Conclusions CCT and ECD were significantly lower in OSA patients than in controls and there was a significant correlation between corneal morphological factors and polysomnographic factors. Due to the possibility of hypoxia and various ophthalmic clinical diseases associated with OSA, close observation of ophthalmic abnormalities in OSA patients is required.

Purpose: To analyze the relationship between corneal morphological factors and polysomnographic factors in patients with obstructive sleep apnea (OSA). Methods: In all, 22 eyes of 22 patients with OSA were analyzed retrospectively under specular microscopy. The central corneal thickness (CCT), endothelial cell density (ECD), hexagonal cell percentage, and cell variation coefficient were measured using specular microscopy, and polysomnographic factors including the apnea-hypopnea index (AHI), respiratory disturbance index (RDI), oxygen desaturation index, and lowest O2 saturation were compared and analyzed between the OSA group and a control group consisting of 32 subjects. Additionally, we examined the statistical correlation between retinal nerve fiber layer (RNFL) thickness and the polysomnographic factors for the 12 OSA patients in which the RNFL thickness was measured. Results: The mean CCT and mean ECD were significantly lower in the OSA group than in controls (p = 0.033, p = 0.021, respectively). As the severity of OSA worsened, a significant negative correlation developed, such that the CCT decreased as the AHI and RDI increased (r = -0.519, p = 0.013 and r = -0.542, p = 0.009, respectively); in addition, the ECD decreased as the RDI increased (r = -0.454, p = 0.034). As OSA progressed, the nasal RNFL thickness decreased with the lowest O2 saturation (r = 0.703, p = 0.011). Conclusions CCT and ECD were significantly lower in OSA patients than in controls and there was a significant correlation between corneal morphological factors and polysomnographic factors. Due to the possibility of hypoxia and various ophthalmic clinical diseases associated with OSA, close observation of ophthalmic abnormalities in OSA patients is required.

Purpose: We report a case of xeroderma pigmentosum in a pediatric patient with a progressive pterygium-like lesion.Case summary: A 2-year-old girl with photophobia and localized, scattered, small, round brownish macules on skin areas exposed to the sun visited hospital. During follow-up, at the age of 7, she was diagnosed with xeroderma pigmentosum in Japan, and pigmented freckles on the upper and lower eyelids of both eyes were observed. At the age of 11, a possible medial limbal pingueculum and lower lid telangiectasis of the right eye were observed via slit-lamp examination, and one year later, a pterygium-like lesion and gradual fibrovascular tissue growth were found in the same location of the right eye. At the last visit, the progressive pterygium-like lesion and, lower lid telangiectasis of the right eye, and lid pigmentations of both eyes (xeroderma pigmentosum) were observed. The size of pterygium-like lesion continues to increase, but the patient remains under observation because the lesion was too small to remove. Conclusions We report the first case of xeroderma pigmentosum in a pediatric patient with a progressive pterygium-like lesion that showed age-related degeneration. We suggest that the pterygium-like lesion may reflect the photosensitivity to ultraviolet radiation characteristic of xeroderma pigmentosum.