Congenital hypertrophy of the retinal pigment epithelium. Electron microscopic and morphometric observations.

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a well-circumscribed, flat, pigmented fundus lesion that is stable and generally nonprogressive. Light and electron microscopy and morphometric analysis was used to study a lesion with the clinical characteristics of CHRPE found in an eye enucleated for a posterior segment malignant melanoma. These studies showed that the lesion was composed of tall, maximally pigmented RPE cells that had a density 1.7 times greater than the density of the adjacent normal peripheral RPE. These observations suggested that cellular hyperplasia and hypertrophy may contribute to CHRPE. Ultraviolet fluorescence microscopy showed no autofluorescent granules of lipofuscin in the CHRPE, suggesting that the lesion's constituent cells lack the capacity to phagocytose and digest photoreceptor outer segments. Photoreceptor degeneration in the overlying retina consequent to this functional defect could be responsible for the localized visual field defects that typically occur in patients with CHRPE.

Vesicovaginal fistula and its complications due to prolonged use of vaginal diaphragm.

The vaginal diaphragm is currently less popular than other contraceptive methods, such as the pill, IUDs or surgical sterilization. It is however, regarded as one of the safest methods of contraception available and complications of diaphragm use are rare. This report describes a case in which a vesicovaginal fistula and genitourinary tract infections complicated the use of this contraceptive method.

PIP: This article presents a case in which a diaphragm, in place in the vagina of a 61-year old woman for 37 years, caused vesicovaginal fistula and genitourinary tract infections. The patient presented with a history of foul smelling watery vaginal discharge for the preceding 10-15 years. The pathology specimen revealed vaginitis, cervicitis, endometritis, salpingitis with tubo-ovarian adhesions, and chronic cystitis in conjunction with a bladder calculus. The vertically positioned diaphragm was inspissated and calcified. The position of the diaphragm had not interfered with menstruation. A hysterectomy, bilateral salpingo-oophorectomy, cystectomy and anterior vaginectomy, right nephroureterectomy, and appendectomy were performed, and the left ureter had to be anastomosed to a right lower quadrant ileal loop. The postoperative course was uneventful. This case illustrates the serious complications that can accompany prolonged diaphragm neglect; this patient had no contact with a physician following diaphragm insertion.

Gastrointestinal hemorrhage in Turner syndrome. Long-term follow-up with postmortem examination.

A 57-year-old woman with Turner syndrome had severe recurrent gastrointestinal bleeding. Exploratory laparotomy at the age of 26 showed an extensive telanglectasia of the entire small intestine. Following death due to myocardial infraction at age 57, postmortem examination revealed only a 0.2-cm residual telangiectasia in the mucosa of the distal part of the ileum. Spontaneous regression of the intestinal telangiectasia observed in Turner syndrome may occur and account for the improved prognosis with age.