Shaken baby syndrome.

PURPOSE: To examine the comprehensive ophthalmologic experience with the shaken baby syndrome at one medical center, including clinical findings, autopsy findings, and the outcome of survivors. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: One hundred twenty-three children admitted from January 1987 through December 1998 for subdural hematomas of the brain secondary to abuse were included. METHODS: Clinical features of eye examinations of the patients during their admission and after discharge and histopathologic observations for patients who died were retrieved from medical records and statistically analyzed. MAIN OUTCOME MEASURES: Visual response and pupillary response on initial examination, fundus findings, final vision, neurologic outcome of survivors, and death. RESULTS: Ninety percent of the patients had ophthalmologic assessments. Retinal hemorrhages were detected in 83% of the examined children. The retinal hemorrhages were bilateral in 85% of affected children and varied in type and location. Nonophthalmologists missed the hemorrhages in 29% of affected patients. Poor visual response, poor pupillary response, and retinal hemorrhage correlated strongly with the demise of the child. One child who died had pigmented retinal scars from previous abuse, a condition not previously observed histopathologically to our knowledge. One fifth of the survivors had poor vision, largely the result of cerebral visual impairment. Severe neurologic impairment correlated highly with loss of vision. CONCLUSIONS: Shaken baby syndrome causes devastating injury to the brain and thus to vision. Retinal hemorrhages are extremely common, but vision loss is most often the result of brain injury. The patient's visual reaction and pupillary response on presentation showed a high correlation with survival. Good initial visual reaction was highly correlated with good final vision and neurologic outcome. According to the literature, when retinal hemorrhages are found in young children, the likelihood that abuse occurred is very high. Nonophthalmologists' difficulty in detecting retinal hemorrhages may be an important limiting factor in identifying shaken babies so they can be protected from further abuse.

Endophthalmitis from Mycobacterium bovis-bacille Calmette-Guérin after intravesicular bacille Calmette-Guérin injections for bladder carcinoma.

PURPOSE: To present clinical and histologic findings of intraocular infection with Mycobacterium bovis-bacille Calmette-Guérin after intravesicular bacille Calmette-Guérin injections for treatment of bladder carcinoma. METHODS: A 77-year-old man was initially seen with visual acuity of 20/200, focal retinitis, vasculitis, and progressive vitreous opacity in the right eye and visual acuity of light perception, intraocular inflammation, and a dense cataract in the left eye 14 months after intravesicular injection of live bacille Calmette-Guérin organisms. RESULTS: Vitreous cultures in the right eye demonstrated growth of bacille Calmette-Guérin organisms. Bilateral loss of light perception occurred despite systemic antimy-cobacterial therapy. Histopathologic examination demonstrated nongranulomatous inflammation and acid-fast bacilli in both eyes. CONCLUSION: Delayed endogenous endophthalmitis may develop after intravesicular bacille Calmette-Guérin injection that may not respond to systemic agents. Intravitreal therapy may be indicated.

The costs versus the perceived benefits of an LCME institutional self-study.

PURPOSE: To calculate the costs versus the perceived benefits of an institutional self-study done to satisfy the requirements of the Liaison Committee on Medical Education's (LCME's) accreditation process. METHOD: From postcard questionnaires, the authors determined the hours spent over 18 months from 1994 to 1996 on the institutional self-study by 131 self-study committee members and 64 database compilers at the Medical College of Wisconsin. The committee members also rated the potential utility of the self-study process and the probability that the concerns identified by their subcommittees would be addressed. Administrative costs (self-study coordinating team's hours, supplies, and other expenses) were tracked using calendars and budget subaccount numbers. Personnel costs were calculated using salary data from the Association of American Medical Colleges and the College and Universities Personnel Administrators' survey. RESULTS: Supplies and equipment for the self-study cost $12,158, and the personnel costs, based on an 81% response rate, were estimated at $207,384, for a total of $219,542. The participants in the self-study rated the process as moderately useful, but believed that there was only a medium degree of probability that concerns they had identified would be addressed. CONCLUSION: Considering the costs of self-study, the process might be more useful if attention were focused less on identifying concerns and more on an institution's demonstrated ability to successfully respond to problems.

The in vivo effects of a prototype diode laser coagulator/vaporizing endoprobe used to perform retinectomy and retinotomy in rabbits.

BACKGROUND AND OBJECTIVE: A pilot study was conducted to evaluate the use of a prototype intraocular carbon-coated diode laser probe for performing single-instrument internal retinotomy and retinectomy. MATERIALS AND METHODS: A 20-gauge endolaser probe was used to perform retinotomies on 10 anesthetized rabbits. Two protocols were examined: one using low power and long duration, and one using high power and short duration. Retinotomies were performed on attached and experimentally detached retinas. RESULTS: Retinotomies were performed in all eyes using both protocols. The edges had photocoagulative effects in all cases. The eyes treated with the longer duration, lower power protocol had scleral damage, whereas the eyes treated with shorter duration, higher power did not. CONCLUSION: Although the diode laser coagulator/cutter may be used for retinotomies, it should be studied further.

Histopathologic study of autosomal dominant vitreoretinochoroidopathy in a 26-year-old woman.

The clinicopathologic findings were obtained from enucleated eyes, obtained post mortem, of a 26-year-old woman with autosomal dominant vitreoretinochoroidopathy. Light microscopy demonstrated atrophic, disorganized peripheral retina with retinal blood vessels obscured by pigmented cells surrounding periodic acid-Schiff-positive deposits. Peripheral retinal pigment epithelial cells showed multilayering and pigmentation, with a thickened basal lamina. By electron microscopy, the peripheral retinal vessel endothelium was replaced by an arrangement of morphologically polarized pigmented cells of presumed retinal pigment epithelial origin oriented with their basal surfaces toward a fibrillar matrix occupying the vessel lumen. The similarity of the findings in this young patient to those of an aged patient described previously suggest that autosomal dominant vitreoretinochoroidopathy is an early-onset dystrophy of the peripheral retina with minimal subsequent progression, characterized by a retinal pigment epithelial response that includes marked intraretinal migration and extracellular matrix deposition.

Screening for glaucoma with stereo disc photography.

PURPOSE: To determine the value of taking simultaneous stereo photographs of the optic nerve head as a basis for identification of patients with glaucoma. METHODS: Two hundred fifty-eight patients received complete ophthalmological examinations and were ranked on a scale of 1-5 regarding the likelihood of their having glaucoma. Each eye was also photographed using the NIDEK camera, providing stereo pairs of the optic nerve head. The same patients were reclassified by two independent masked observers on the same scale of 1-5, based solely on examination of the photographs. RESULTS: Examination of stereo photographs alone provided maximum sensitivity of 75% and specificity of 95% in identification of glaucoma patients when photographic readings were compared with all available clinical information. CONCLUSIONS: Stereo photographs of the optic nerve head can be used for glaucoma detection with an accuracy that is significantly greater than simple tonometry and with a sensitivity that is equivalent to screening with computerized perimetry.

Idiopathic sclerosing inflammation of the orbit: immunohistologic analysis and comparison with retroperitoneal fibrosis.

Idiopathic sclerosing inflammation of the orbit is clinically characterized by an insidious, chronic and progressive fibrosing process damaging orbital structures through entrapment and mass effect. Histologically, desmoplasia and a sparse infiltrate of lymphocytes, histiocytes, plasma cells, and occasional neutrophils and eosinophils are seen. An immune pathogenesis is suspected but presently poorly understood. To characterize the inflammatory infiltrate and to compare orbital and other inflammatory fibrosing lesions, immunoperoxidase studies using the streptavidin method were performed on 16 formalin or Bouins' fixed, paraffin-embedded orbital biopsy specimens and six specimens of retroperitoneal fibrosis. Positive staining of orbital tissue occurred as follows: T-cells (UCHL-1) 94% of cases, B-cells (L26) 40%, tissue macrophages (KP-1) 56%, HLA Dr positive antigen presenting cells and activated T-cells (LN3) 44%, and immunoglobulins (kappa, 80%; lambda, 63%, IgG, 73%, IgA, 44% and IgM, 31%). Results were strikingly similar for retroperitoneal fibrosis. These findings imply a cell mediated pathogenesis in idiopathic sclerosing inflammation of the orbit that is similar to retroperitoneal fibrosis and suggest therapeutic potential for agents modifying this facet of the immune system.

In vivo determination of the anisotropic diffusion of water and the T1 and T2 times in the rabbit lens by high-resolution magnetic resonance imaging.

PURPOSE: Several magnetic resonance imaging (MRI) "tools" for ophthalmologic research have recently been developed in this laboratory, including improved gradient and radiofrequency coils and pulse sequences for high-resolution and diffusion imaging (100-microns resolution). METHODS: These tools have been applied to the in vivo measurement of the relaxation parameters (T1 and T2) and the water diffusion coefficients (Dx and Dy) in the rabbit eye lens, both normal and cataractous. Maps of these parameters in the lens have been computer generated. RESULTS: In the normal lens, water diffusion is highly anisotropic and tends to be parallel to the surface. In the trauma-induced cataractous lens, an increase in spin-spin relaxation times (T2) consistent with edema and alteration of diffusion patterns was observed in a study conducted 2 weeks postsurgery. A partial reversal was observed 6 weeks postsurgery. The histologic data on the enucleated lens at 6 weeks showed a loss of normal lens architecture. Images are shown that display other small structures of the anterior segment with great clarity. CONCLUSIONS: An extension of this work, now underway, is the study of the formation of various types of cataract in animal models. It is hypothesized that these methods can be extended to humans as a quantitative alternative for the assessment of cataracts.

Blastomycosis of the eyelid and conjunctiva.

A 37-year-old man with a recurrent papillomatous lesion of the upper eyelid and four separate bulbar conjunctival ulcers was found to have North American blastomycosis. This represents the first reported case with conjunctival lesions that were not simply contiguous with eyelid involvement. This case underscores the importance of considering blastomycosis in the differential diagnosis of granulomatous conjunctivitis and when examining a lesion of the eyelid resembling a squamous cell carcinoma or atypical papilloma.

DNA flow cytometry of sebaceous cell carcinomas of the ocular adnexa: introduction to the technique in the evaluation of periocular tumors.

DNA content abnormalities are well recognized in tumor cell biology and kinetics. We introduce the technique of DNA flow cytometry through the study of the sebaceous cell carcinomas of the ocular adnexa. By correlating the data to the standard histopathologic parameters of pagetoid changes, degree of anaplasia, and stromal inflammation, significant associations are revealed. All aneuploid tumors demonstrate pagetoid spread, and more severe anaplasia and stromal inflammation. All diploid tumors are nonpagetoid and have lesser degrees of anaplasia and stromal inflammation. A complete review of the technique with a discussion of the implications and applicability to the study of ocular adnexal tumors is presented.

Orbital metastasis as the presenting sign of adenocarcinoma of the breast.

A 64-year-old woman presented with periorbital induration of 6 months' duration. Computed tomography (CT) revealed enlargement of extraocular muscles. A biopsy specimen of the orbicularis muscle and indurated preaponeurotic fat demonstrated an infiltrating carcinoma, and immunoperoxidase studies for surface antigens suggested a metastatic adenocarcinoma. Extensive evaluation revealed a 1-cm subareolar breast mass, with histopathological features identical to those of the orbital lesion. Orbital metastasis presenting before the diagnosis of a primary breast cancer is unusual, particularly in the absence of other disseminated disease.

Internuclear ophthalmoplegia in acquired immunodeficiency syndrome.

We describe the case of a patient with acquired immunodeficiency syndrome (AIDS) who had internuclear ophthalmoplegia (INO) and subsequent rapid neurologic deterioration. To our knowledge, this is the first report of a patient with AIDS and INO.

Antagonism of neurotensin induced miosis by thyrotropin-releasing hormone (TRH) in rabbits.

In previous studies we have shown that thyrotropin-releasing hormone (TRH) antagonizes many of the neural effects of neurotensin (NT). This study, evaluated the ability of TRH and two TRH analogs: 3 methyl-His-TRH and Phe2-TRH to affect NT-induced miosis in rabbits. In confirmation of previous findings, NT (30 micrograms) produced a significant miosis. The high (60 micrograms), but not the low (30 micrograms) dose of TRH significantly antagonized NT (30 micrograms)-induced miosis. Of interest was the observation that 3 methyl-His-TRH and Phe2-TRH were more effective than native TRH in blocking NT-induced miosis. The inhibitory effect of 3 methyl-His-TRH on the miotic response to NT exhibited long duration (approximately 60 min) when compared to native TRH and Phe2-TRH. TRH or the TRH congeners had no appreciable effects on pupillary diameter when administered alone. These findings indicate that TRH antagonizes the miotic response to NT, and suggest a hitherto undescribed peptide-peptide interaction involved in regulation of iris motility.

Pupillary response to atrial natriuretic factor in rabbits.

This study was designed to evaluate the pupillary and intraocular pressure (IOP) response to exogenously administered atrial natriuretic factor (ANF) in rabbits. Dose-response studies were conducted by administering intracameral (i.c.) ANF (0.1-5 micrograms). The effect of route of administration was evaluated by administering ANF (5 micrograms/kg) intravenous (i.v.) subcutaneous (s.c.) and intraperitoneal (i.p.). In a final study, normal rabbit serum or ANF antiserum were administered i.c. In all studies, pupillary diameter (PD) and IOP were evaluated. Intracameral administration of ANF (0.1-5 micrograms) produced a significant (P less than 0.01) dose-dependent unilateral miosis without affecting IOP. Peripheral (i.v., s.c., i.p.) administration ANF did not affect PD or IOP. Finally, ANF antiserum did not affect PD significantly. These data suggest that ocular, but not circulating ANF may contribute to regulate pupillary function. The mechanism of the miotic response to ANF probably involves interactions with other autonomic neurotransmitters because immunoneutralization of endogenous ocular ANF was without measurable effect.

Mycobacterium fortuitum keratitis.

Two of four cases of Mycobacterium fortuitum keratitis occurred after corneal surgery with contact lens wear, one was associated with extended contact lens wear alone, and one occurred after a foreign body injury. All cases were characterized by pain, conjunctival hyperemia, stromal inflammation, and ulceration. Diagnosis was made by culture and acid-fast staining of corneal scrapings. On the basis of published experience with amikacin for the treatment of nonocular M. fortuitum infections, three patients were treated with topical amikacin. Two patients responded clinically, but histopathologic examination of a penetrating keratoplasty specimen in one of the two disclosed persistent infection. One patient was cured of early disease by debridement alone. Rapid diagnosis and absence of corticosteroid use were the two most important determinants of successful therapy. In advanced cases, infection may be cured and useful vision restored by penetrating keratoplasty.

Blepharopigmentation: histopathologic observations and X-ray microanalysis.

Blepharopigmentation or permanent eyeliner is a popular cosmetic procedure currently performed by a variety of methods. All methods attempt to implant ferrous oxide pigment into the dermis. We performed histopathologic studies on a series of three eyelid specimens, including two by a previously undescribed multi-needle pigment delivery system. Light microscopy revealed the epidermis to be pigment-free and the dermis to contain pigment in clumps. Transmission electron microscopy (EM) demonstrated that the pigment was largely in macrophages. Energy-dispersive x-ray micro-analysis showed that in addition to iron and talc, titanium particles were also present. These findings suggest that the pigment is tolerated by the eyelid despite the fact that current pigments are contaminated.

False positive diagnosis of choroidal melanoma by magnetic resonance imaging.

Magnetic resonance imaging is a relatively new diagnostic modality that is used to an increasing extent as an aid to increase clinical diagnostic accuracy. We report a case of an erroneous diagnosis of malignant melanoma of the choroid associated with magnetic resonance imaging.

Magnetic resonance imaging of craniopharyngioma.

Craniopharyngiomas are common tumors located in the suprasellar region. Contrast enhancement, cyst formation, and calcification are the three characteristic features of craniopharyngiomas on computed tomographic scan. More than 90% of suprasellar craniopharyngiomas exhibit at least two of these three features, thus providing easy radiologic detection. We treated a 41-year-old man in whom a large suprasellar craniopharyngioma producing severe visual loss was not detected by computed tomography but was easily identified with magnetic resonance imaging. Thus, despite high-resolution computed tomographic scan, large suprasellar craniopharyngiomas can be missed. Magnetic resonance imaging may be superior to computed tomography in detecting these tumors.