A case of prune belly syndrome, which was observed in a 3 month-old male, is presented. The congenital anomaly complex in which absence of abdominal muscle is associated with urinary tract abnormalities and abdominal testes has been well known. The literature is reviewed briefly.
Abdominal Muscles ; Humans ; Infant ; Male ; Prune Belly Syndrome* ; Testis ; Urinary Tract

In an attempt to obtain evidence in the mechanism of stone formation, the effectiveness of Amiloride. a K-sparing diuretic known to act on the distal nephron. on the kidney with oxamide-induced urolithiasis was investigated. Futhermore. the influence of stone formation on the distribution of intrarenal blood flow and the effect of Amiloride on them were also studied. Most prominent derangements of renal function observed during stone formation were decreases of both C(PAH) and C(cr) indicating the curtailment in renal hemodynamics. The oxamide-treated kidney also responded to Amiloride with typical natriuresis and antikaluresis. in the same fashion with normal kidneys. The experiments in which the intrarenal blood flow distribution was measured by PAH-extraction technique raveled that mainly the cortical blood flow was curtailed, whereas non-cortical blood flow (medullary flow) did not change during oxamide-stone formation. Amiloride did not influence the intrarenal b100d flow distribution both in normal and oxamide kidneys. These observations suggest that oxamide-stone formation was initiated not by tubular necrosis produced by the toxic action of oxalate on the tubules, but rather by mechanical obstruction of the tubules.
Amiloride* ; Hemodynamics ; Kidney* ; Natriuresis ; Necrosis ; Nephrons ; Renal Circulation ; Urolithiasis

Purpose: We report an unusual trichoblastoma of the lower eyelid.Case summary: A 74-year-old male presented with an asymptomatic 0.6 × 0.4-cm left lower eyelid tumor that had developed 3 years prior. On histological examination, a 0.3 × 0.3-cm intradermal tumor that was not connected to the epidermis was observed. The tumor was multilobular, and was surrounded by fibrous stroma and connective tissue. The tumor lobules consisted of basaloid cells, and a few keratinizing cysts were evident. These features are consistent with a trichoblastoma. We observed no recurrence at 1 year after complete resection. Conclusions Trichoblastoma (a rare, benign skin tumor) is histologically similar to basal cell carcinoma but the prognosis is excellent after complete resection. Trichoblastoma should be considered during the differential diagnosis of basal cell carcinoma of the eyelids.

Purpose: This study introduces a new machine learning-based auto-merge program (HydraVersion) that automatically combines multiple ocular photographs into single nine-directional ocular photographs. We compared the accuracy and time required to generate ocular photographs between HydraVersion and PowerPoint. Methods: This was a retrospective study of 2,524 sets of 250 nine-directional ocular photographs (134 patients) between March 2016 and June 2022. The test dataset comprised 74 sets of 728 photographs (38 patients). We measured the time taken to generate nine-directional ocular photographs using HydraVersion and PowerPoint, and compared their accuracy. Results: HydraVersion correctly combined 71 (95.95%) of the 74 sets of nine-directional ocular photographs. The average working time for HydraVersion and PowerPoint was 2.40 ± 0.43 and 255.9 ± 26.7 seconds, respectively; HydraVersion was significantly faster than PowerPoint (p < 0.001). Conclusions Strabismus and neuro-ophthalmology centers are often unable to combine and store photographs, except those of clinically significant cases, because of a lack of time and manpower. This study demonstrated that HydraVersion may facilitate treatment and research because it can quickly and conveniently generate nine-directional ocular photographs.

Purpose: To investigate the long-term outcomes of anterior chamber angle parameters in patients with primary angle closure glaucoma (PACG) after peripheral laser iridotomy (LI) combined with iridoplasty (PI) using a dual Scheimpflug analyzer. Methods: This retrospective study included 32 eyes in 32 patients diagnosed with PACG who underwent LI plus PI. Patients with an acute angle closure crisis were excluded. Dual Scheimpflug analyzer images were acquired before and 1 week after LI plus PI, and yearly afterwards. Anterior chamber depth (ACD) and volume (ACV), mean anterior chamber angle (ACA), and intraocular pressure (IOP) were also measured. Results: The mean follow-up was 32.28 ± 13.34 months. Baseline demographics were age 63 ± 7.9 years, 62.5% female, IOP 15.48 ± 4.79 mmHg, ACD 2.09 ± 0.19 mm, and visual field mean deviation -7.97 ± 8.49 dB. ACD increased from baseline to 2.15 ± 0.32 mm, but it was not significant (p = 0.136). ACV increased significantly from 78.32 ± 11.49 mm at baseline to 83.04 ± 11.16 mm at the last visit after LI (p = 0.011). The mean ACA increased significantly from 26.86 ± 2.53° at baseline to 28.82 ± 4.64° at the last visit (p = 0.022). IOP decreased significantly from baseline to 13.06 ± 2.21 mmHg at the last visit (p = 0.001). Conclusions The ACA parameters improved after LI combined with PI in patients with PACG and remained so on long-term follow-up. IOP was also significantly reduced for more than 2.5 years after LI plus PI.

PURPOSE: We report a case of fundus albipunctatus discovered in a young patient. CASE SUMMARY: A 7.6-year-old female showed numerous small whitish-yellow flecks in the perimacular area and retinal periphery. Dark adapted 0.01 electroretinography (ERG) and dark adapted 3.0 ERG were profoundly reduced. At 26 months after the first visit, the best-corrected visual acuities were 1.0 right eye and 0.9 left eye. There were no pigmented lesions, atrophic lesions, or vascular abnormalities in the retina. Humphrey and Goldmann visual field tests were performed, but neither of the tests revealed any scotomas or other visual field defect. The number and size of characteristic numerous small whitish-yellow retinal flecks seemed almost unchanged. In spectral domain-optical coherence tomography (SD-OCT), the subretinal hyper-reflective lesions spanned the retinal pigment epithelium and the external limiting membrane. ERG showed improved dark adapted responses (dark adapted 0.01 ERG and dark adapted 3.0 ERG) after prolonged dark adaptation (2.5 hours). No family member showed any abnormal findings. CONCLUSIONS: Fundus albipunctatus is a rare disease in Koreans. We report a case diagnosed using fundus photography, SD-OCT, visual field tests, and ERG after prolonged dark adaptation (2.5 hours).
Child ; Dark Adaptation ; Electroretinography ; Female ; Humans ; Membranes ; Photography ; Rare Diseases ; Retina ; Retinal Pigment Epithelium ; Retinaldehyde ; Scotoma ; Tomography, Optical Coherence ; Visual Acuity ; Visual Field Tests ; Visual Fields

PURPOSE: We present four cases of welding arc maculopathy as observed using spectral-domain optical coherence tomography (SD-OCT).CASE SUMMARY: Four patients, who performed welding without wearing protective eye gear, presented to the hospital due to poor visual acuity. The mean visual acuity of the patients was 0.6. Fundus photographs of the four patients revealed a yellowish retinal scar at the fovea. SD-OCT images of the four patients showed photoreceptor inner segment/outer segment junction (IS/OS junction) disruption and retinal pigment epithelium injury. We diagnosed the patients with welding arc maculopathy, and three of them were treated with oral steroids or antioxidants. The IS/OS junctions were restored in two patients, who had short welding arc exposures. The disrupted IS/OS junction recovered partially in one of the other two patients, who had a longer duration of exposure, and the IS/OS junction disruption remained in another patient. CONCLUSIONS We report four cases of welding arc maculopathy caused by welding light exposure evaluated using SD-OCT and treated with oral steroids and antioxidants.

Purpose: To evaluate the repeatability of retinal nerve fiber layer (RNFL) thickness and Bruch’s membrane opening-minimum rim width (BMO-MRW) measurements by spectral-domain optical coherence tomography (SD-OCT) in wet age-related macular degeneration (wAMD) and diabetic macular edema (DME). Methods: This was a prospective study. The RNFL thickness and BMO-MRW parameters for each sector and global average were measured twice by SD-OCT. Repeatability was evaluated using the intraclass correlation coefficient (ICC) and coefficient of variation (CV). If the optic disc membrane was confirmed, it was analyzed by dividing it into three groups based on severity. Results: A total of 99 eyes (48 with wAMD, 51 with DME) were included in the analysis. The ICCs of the global RNFL thickness and global BMO-MRW measurements were 0.996 and 0.997, respectively, in wAMD and 0.994 and 0.996, respectively, in DME eyes. The CV values of global RNFL thickness and BMO-MRW were 0.60% and 0.73%, respectively, in wAMD eyes and 1.10% and 1.21%, respectively, in DME eyes. The disc membrane on the optic nerve head significantly affected global BMO-MRW repeatability (B = 0.814, p < 0.001). Conclusions Both RNFL thickness and BMO-MRW measurements showed good repeatability in eyes with wAMD and DME. The severity of the optic disc membrane significantly affected the repeatability of BMO-MRW measurements in eyes with wAMD and DME. Therefore, physicians should examine the BMO-MRW in eyes with severe optic disc membrane.