Ocular manifestations of nephropathic cystinosis. The French-Canadian experience in a genetically homogeneous population.

A large spectrum of ocular pathologic features have been described in infantile cystinosis. Reported symptoms may require corneal transplantation and therapeutic efforts to solubilize cystine crystals with hourly administration of cysteamine (mercaptamine) drops. French Canada has the highest incidence of cystinosis in the world. We studied 18 patients with cystinosis who appeared to have much milder ocular involvement than that in other reported series. No operative interventions were required. Relatively mild photophobia was the most common ocular manifestation of cystinosis.

Bimedial rectus recession measured from the limbus for congenital esotropia.

We reviewed the surgical results of bimedial rectus muscle recession measured from the limbus in 34 patients with congenital esotropia less than 1 year of age. The mean amount of recession was 10.8 mm. The procedure was successful (10 delta or less of horizontal deviation at 6 months) in 23 patients (68%). In all but one case failure was due to undercorrection. The success rate was 81% in the small-angle deviation group, compared with 29% in the large-angle deviation group (p less than 0.01). The average amount of deviation before surgery was 54.4 delta in the surgical success group and 65.0 delta in the undercorrection group (p less than 0.02). More surgery should be done in patients with larger deviations. Prospective randomized studies are needed to evaluate the efficacy of recession from the limbus versus recession from the insertion site.

Catheterization of the umbilical artery in neonates: surgical implications.

Catheterization of the umbilical artery is a common procedure in neonatal intensive care units. The authors studied the records of 100 consecutive newborns who underwent this procedure to review the indications for and complications of umbilical artery catheterization and to discuss preventive measures and alternative techniques. Only polyvinylchloride barium-impregnated catheters were used (nos. 3.5 and 5.0 French). The commonest indications were respiratory distress syndrome, asphyxia and congenital heart disease. Of the 100 infants, 75 weighed less than 2500 g. Ampicillin and calcium were the commonest medications infused (70 and 65 babies respectively). There were three major complications, two of which were gangrene of the lower extremity. Amputation of the foot was necessary in one and amputation of the toes in the other. The third complication was the development of gluteal necrosis. In all three cases, catheter placement was low and the infusion was ampicillin. In two of the babies, calcium was also administered. Minor complications were seen in 32 cases, with vascular spasm in the lower limb being the most common. All catheter tips were cultured; there was bacterial colonization in 13%, Staphylococcus epidermidis being the commonest organism. Proven necrotizing enterocolitis was seen in eight infants and was suspected in eight others. Blanching is a serious sign and was seen in the three infants with major complications. The infusion should be stopped immediately. When necrotizing enterocolitis is suspected, the catheter should be removed.

Risk factors for progression of visual field defects in medically treated patients with glaucoma.

A multivariate discriminant analysis of potential risk factors for the progression of visual field defects was performed in 70 patients with chronic open-angle or low-tension glaucoma. Male sex, low diastolic blood pressure and diabetes mellitus were found to be significant risk factors. When the patients with low-tension glaucoma were excluded, a high mean intraocular pressure was identified as a risk factor. It was concluded that there is no reliable way to predict future visual field loss in patients with glaucoma.