Normal glomerular permeability and its modification by minimal change nephrotic syndrmone.

It has been suggested that the glomerular basement membrane restricts the passage of large molecules only, the barrier to filtration of smaller molecules being at the level of the epithelial slit pore. This hypothesis was investigated by measuring glomerular permeability to (125)I-labeled polydisperse polyvinyl pyrrolidone (PVP) in 16 children with idiopathic nephrotic syndrome (INS) and in 6 children of comparable age who had no evidence of renal disease. Studies were performed in the patients with INS before, during, and after treatment with steroids. PVP in blood and urine samples was separated according to molecular size by solumn chromatography, to permit the calculation of permeability to inert macromolecules of sizes ranging from 8,000 mol wt. In untreated INS, glomerular permeability to molecules > 40 A was normal; permeability to smaller molecules was markedly reduced, frequently to 20% or less of normal. There was an average decrease in inulin clearance (C(in)) of 24%. Glomerular permeability and C(in) returned to normal in INS treated with steroids only when proteinuria disappeared. The results support the concept, derived from studies with ultrastructural tracers, that the final barrier to filtration may be at the level of the epithelial slit pore. Thus fusion of the epithelial foot processed with obliteration of the slit pores was associated with impaired passage of smaller molecules of PVP into the urine. Reversal of the pathologic abnormality resulted in return of permeability to normal. The decreased C(in) seen in INS may not reflect true glomerular filtration rate, but may result from restricted passage of inulin molecules (mol wt 5,000) through the epithelial slit pore.

Descemet's membrane detachment by sodium hyaluronate.

The inadvertent injection of sodium hyaluronate (Healon) just anterior to Descemet's membrane during an extracapsular cataract extraction resulted in a corneal opacity. No extraordinary measures were taken to remove this substance. The opacity resolved over five months, with a final visual acuity of 20/25 and a reduction in specular microscopic cell density from 2,600 to 2,100/sq mm. Sodium hyaluronate was injected in an analogous manner into eight rabbit corneas. The eyes were enucleated, and histologic sections were examined 1, 5, 9, and 14 days later. Sodium hyaluronate injected just anterior to Descemet's membrane was found to incite an acute mild granulocytic response, which is followed by a macrophage invasion and ingestion of this substance. We recommend a conservative approach when the central portion of Descemet's membrane is detached by sodium hyaluronate during anterior segment surgery.

The effect of preoperative subconjunctival triamcinolone administration on glaucoma filtration. I. Trabeculectomy following subconjunctival triamcinolone.

Trabeculectomies were performed on 15 eyes because of uncontrolled glaucoma despite the maximum use of tolerated antiglaucoma medication. We considered all of these eyes to be at increased risk for episcleral cicatricial closure for one of the following reasons: neonatal glaucoma, a patient of age 40 years or less, previously failed glaucoma filtration surgery, or aphakia. A standard operation was performed except that triamcinolone acetonide (4 mg) was injected subconjunctivally at the intended trabeculectomy site one week before surgery in 12 eyes, the day of surgery in two eyes, and two days prior to surgery in one eye. During the follow-up period of six to 16 months, 14 of 15 eyes had an intraocular pressure (IOP) of 18 mm Hg or less. The average IOP for these 14 eyes was 12.4 +/- 4.6 (mean +/- 1 SD), and only three of the 14 required any antiglaucoma medication. One eye was a surgical failure. All eyes with controlled IOP had diffuse microcystic filtration blebs by slitlamp examination. The postoperative visual acuity was within one line of the preoperative level in all eyes, except for the one failure. The use of triamcinolone did not appear to add any additional risks to the surgical procedure or to the postoperative period.

Orbital apex syndrome.

A case is presented of a 16-year-old anticoagulated, immunocompromised male presenting with a blind, proptotic left eye, total ophthalmoplegia, and central retinal artery occlusion. Ophthalmic examination revealed other features of the orbital apex syndrome. Mucormycosis misdiagnosed as a traumatic orbital hemorrhage led to the patient's death. In this review, differential diagnosis of the orbital apex syndrome (loss of cranial nerves II, III, IV, ophthalmic division of V, and VI) is outlined, and features, diagnosis, and treatment of mucormycosis are discussed. Although mucormycosis is most commonly seen in diabetics, it should be considered in any immunocompromised patient presenting with the constellation of features of orbital apex syndrome.

Pediatric ocular sarcoidosis.

Sarcoidosis is an uncommon cause of childhood uveitis. However, the ophthalmologist familiar with the clinical features of childhood sarcoidosis can play a key role in the diagnosis and treatment of this disorder. Two subsets of pediatric sarcoidosis are identified. The 8-15 year age group has almost universal lung involvement, with the eye, skin, liver, and spleen involved in 30-40% of cases. Children 5 years of age and under are characterized by the triad of uveitis, arthropathy, and skin rash. The epidemiology, clinical features, diagnostic evaluation, and ocular management of pediatric sarcoidosis are reviewed. The clinical and laboratory findings that distinguish sarcoidosis from other causes of childhood uveitis are discussed.

Ophthalmoscopic findings in occult child abuse.

Two previously healthy infants initially presented with vomiting and lethargy. There was a history of whiplash shaken infant syndrome in one infant, but neither child had external or skeletal survey evidence of abuse. However, ophthalmoscopy revealed bilateral retinal hemorrhages, papilledema and vitreous hemorrhage. Subdural aspirations revealed occult hematomas. Both children required ventricular peritoneal shunts to control intracranial pressure. Ophthalmologists, pediatricians, and primary physicians should be aware of the possibility of child abuse in the differential of diagnosis of Terson's syndrome, especially in infants since this may be the only physical finding.

Results of a single lateral rectus resection for divergence and partial sixth nerve paralysis.

A single 5.5-millimeter to 8.0-millimeter lateral rectus resection eliminated diplopia in five of six adult patients with divergence paralysis and in all three patients with a sixth cranial nerve paresis. Preoperative distance esotropia ranged from 11 to 20 prism diopters in primary gaze. Prism therapy failed in seven of nine patients. Lateral incomitance was reduced in three patients with a mild limitation of abduction preoperatively, but was not induced in the six patients with divergence paralysis. Significant overcorrection of the near esodeviation did not occur. A single lateral rectus resection can effectively eliminate diplopia in selected patients with divergence and partial sixth nerve paralysis.

Intraosseous hemophilic pseudotumor of the orbit.

Intraosseous hemophilic pseudotumor involving the orbit has not been described previously. A 14-year-old white male presented with a rapidly progressive, expansile right orbital mass consisting of hematoma contained within bony cortex. Computerized tomography revealed findings consistent with intraosseous hemophilic pseudotumor. The patient responded to conservative therapy of factor VIII replacement.

Orbital and childhood sarcoidosis.

Orbital infiltration, anterior uveitis, disc edema, choroiditis, erythema nodosum, and polyarticular arthritis due to sarcoidosis occurred in a five-year-old child who presented with unilateral proptosis. Diagnosis was enhanced by computerized tomography of the orbit, and confirmed by synovial biopsy findings. This patient's course illustrates the importance of thorough and frequent ocular examinations in childhood uveitis associated with systemic disease and demonstrates an unusual finding of orbital sarcoidosis. Childhood sarcoid arthritis may be misdiagnosed as juvenile rheumatoid arthritis. Familiarity with the ocular and systemic findings may lead to the correct diagnosis.

Dinitrochlorobenzene and debulking therapy of conjunctival papilloma.

A conjunctival papilloma in a four-year-old boy had recurred despite one surgical removal, and three cryosurgical treatments. After sensitization of the child's lymphocytes to dinitrochlorobenzene (DNCB), the lesion responded rapidly and completely to debulking followed by painting the base of the tumor with DNCB. We feel that the effectiveness and simplicity of debulking and DNCB treatment of conjunctival papillomas, which have resisted previous standard forms of therapy, gives an easy, relatively inexpensive, and rapid method of treating these lesions.

Congenital absence of the superior oblique tendon.

Congenital absence of the superior oblique tendon occurred in 18% of patients with a diagnosis of congenital superior oblique palsy who were treated with an attempt at a tuck of the superior oblique tendon. The presence of horizontal strabismus and amblyopia in a patient with diagnosis of congenital superior oblique palsy should raise the possibility of absence of this tendon. Absence of the superior oblique tendon can be treated successfully with weakening of the antagonist inferior oblique, and either recession of the yoke inferior rectus or the ipsilateral superior rectus. The horizontal deviation can be treated with appropriate recession and resection of the horizontal recti.

Unilateral renal agenesis presenting as anuria.

The most common cause of sudden and total cessation of urine output is obstructive uropathy, usually at the bladder outlet. Bilateral ureteral obstruction is a much less common cause of anuria. In additioh, unilateral obstruction in the presence of a solitary kidney must be considered in the differential diagnosis. Primary renal parenchymal disorders and pre-renal azotemia occasionally may be anuric but more commonly are oliguric. A case of unilateral renal agenesis presenting as anuria and obstruction of the solitary kidney is described.

Dermal and glomerular deposition of IgA in anaphylactoid purpura.

During the period of acute anaphylactoid purpura, skin biopsies were performed on 14 patients with purpuric and nonpurpuric skin. In addition, four patients had renal biopsies. Examination of the tissue by immunofluorescence to anti-human immunoglobulins IgG, IgA, IgM, and IgE, fibrin/fibrinogen, complement Clq, C4, and C3 demonstrated predominant IgA, C3, fibrin/fibrinogen in the purpuric skin and glomerulus, without Clq and C4. These immunohistochemical findings are characteristic of anaphylactoid purpura and suggest that IgA is involved in the pathogenesis of anaphylactoid purpura and may operate through the alternate pathway of the complement system.

Autosomal-dominant inheritance of congenital superior oblique palsy.

PURPOSE: A pedigree comprised of five affected members is presented to demonstrate the genetic transmission of congenital superior oblique palsy. METHODS: A 2-year-old boy referred for strabismus was found to have bilateral congenital superior oblique palsy. The authors subsequently performed a complete ophthalmologic examination on all available family members to determine the inheritance pattern. The diagnosis of congenital superior oblique palsy was based on results of prism cover testing, ductions, and the Bielschowsky head tilt test, in addition to a history of early onset of symptoms and absence of preceding head trauma. RESULTS: The father, paternal grandfather, and a brother of the 2-year-old boy were found to have bilateral congenital superior oblique palsy. Evaluation of the paternal aunt showed right congenital superior oblique palsy. Bilateral absence of the superior oblique tendon was noted at the time of surgery in the 2-year-old boy. CONCLUSION: The occurrence of genetic transmission by an autosomal-dominant mode should be considered in patients with congenital superior oblique palsy.