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Background: Prostatic carcinoma (PCa) represents the second most common cancer, and the fifth leading cause of cancer death among males worldwide. PSCA is a GPI-anchored cell surface protein. It belongs to the Thy-1/Ly-6 family which shows a functional diversity ranging from T-cell activation to apoptosis regulation. Snail is one of zinc finger proteins which are transcriptional repressors of E-cadherin. Aim: To study PSCA and Snail expression in different prostatic lesions to evaluate their roles in PCa. Material and Methods: This retrospective study was done upon 80 different prostatic lesions; 17 cases of BPH, 13 cases of HGPIN, and 50 cases of PCa. PSCA and Snail immunostaining was done and assessed for each case. Results: There was a highly significant statistical correlation between both PSCA and Snail expressions and histopathological type (P-value

Background: Transitional cell carcinoma remains a challenge in the oncology field, representing an ideal candidate for research on biomarkers that could identify patient’s progression and prognosis. D2-40 and CXCL5 have been implicated in progression of many cancers, but their significance in urinary bladder TCC remains unclear. The aim of this study is to assess their possible significance in urinary bladder transitional cell carcinoma.Methods: Immunohistochemistry was performed to examine the expression of D2-40 and CXCL5 in 50 cases of urinary bladder TCC and 10 cases of normal urothelium taken as a normal control. Statistical analysis methods were used to evaluate the relationship between D2-40 and CXCL5 and various clinicopathological parameters.Results: D2-40 was expressed in the lymphatic endothelial cytoplasm of all cases highlighting the tumor emboli in cancer lymphatic vessels. CXCL5 was found to be expressed in all urinary bladder TCC cases but not in normal control. The two markers significantly correlated with associated necrosis, tumor grade, T, N stage and presence of H&E detected LVI. CXCL5 was also positively correlated with presence of associated necrosis and CIS while D2-40 was insignificantly correlated with both. D2-40 detected intra-lymphatic tumor emboli are associated with lymph node metastasis in a highly significant correlation. Conclusions: The results suggested that CXCL5 might be involved in urinary bladder TCC carcinogenesis. It could be concluded that D2-40 and CXCL5 may have a possible role in urinary bladder TCC progression and prognosis. D2-40 might be considered as more reliable method in predicting tumor spread and lymph-node metastasis.

Background: Preservation of homeostasis status in the skin needs an equilibrium of keratinocyte proliferation, differentiation, necrosis and apoptosis. Disturbance of these regulatory mechanisms may lead to keratinocyte neoplastic and hyperproliferative diseases. Pigment epithelium-derived factor is a glycoprotein that is endogenously produced in different tissues and has a variety of biological effects in different diseases. Objective: To evaluate the keratinocyte expression of pigment epithelium-derived factor in normal skin and three epidermal hyperproliferative diseases, namely, psoriasis, verrucae and squamous cell carcinoma. Methods: This study included skin biopsy samples from 80 participants who were divided into four equal groups; each containing 20 samples. The first group included skin biopsies from normal skin, the second group from psoriatic lesions, the third group from verruca vulgaris and the fourth group from squamous cell carcinoma. All tissue samples were stained with hematoxylin and eosin stain and later immunohistochemically for pigment epithelium-derived factor expression. Results: Scores of pigment epithelium-derived factor expression were lower in squamous cell carcinoma and verruca and psoriasis than normal skin with a significant difference (P = 0.04). In addition, the pattern of pigment epithelium-derived factor expression was mainly cytoplasmic in normal skin with a significant difference with that seen in psoriasis, squamous cell carcinoma and verruca vulgaris (P = 0.001). Conclusion: Pigment epithelium-derived factor may play a role in keratinocyte differentiation.

Background: Sutureless phacotrabeculectomy technique has varied deliberations among ophthalmologists. Improvements in surgical techniques have made sutureless phacotrabeculectomy better in terms of success and postoperative complications. Objective: To describe and evaluate the results of sutureless phacotrabeculectomy with intraocular lens implantation. Methods: The present study was conducted in the Postgraduate Department of Ophthalmology, Sher-i-Kashmir Institute of Medical Sciences, Medical College and Hospital, Bemina Srinagar. The study included 40 eyes of 40 consecutive patients, suffering from primary open angle glaucoma with co-existing cataract. Intraoperative complications and postoperative visual acuity, intraocular pressure (I.O.P), Bleb status and complications were evaluated for a follow up period of 6 months. Results: The mean preoperative I.O.P was 32.53mmHg and mean postoperative IOP was 11.96+3.27mmHg at 2 months and 14.45+4.43 mmHg at six months. The reduction in mean IOP at the end of six months was 55.6%(p

Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.75 years and early seizure onset (6.8 months), whereas group B was composed of older patients (mean age, 15.3 years) with later onset of seizures (3.7 years). Neurologic disease was more severe in group A, but group B had more widespread structural brain defects — shown on computed tomographic scans and magnetic resonance imaging — and metabolic brain defects — shown on hexamethylpropyleneamine oxime and [18F] fluorodeoxyglucose single photon emission computed tomographic scans. Six group A cases had hypoperfusion at baseline and five of nine had worsening of perfusion and glucose metabolism 1 year later. A total of 119 stroke-like episodes occurred in six group A cases and eight group B cases; there were 65% fewer strokes in children treated with aspirin. The data suggest that progressive hypoperfusion and glucose hypometabolism are associated with neurologic deterioration in Sturge-Weber syndrome. Longitudinal studies are needed to better define the natural history of disease and to evaluate the safety and efficacy of aspirin therapy. ( J Child Neurol 1998;13:606-618).

Joubert syndrome is characterized by episodic hyperpnea and apnea, developmental delay, hypotonia, truncal ataxia, ophthalmologic abnormalities, and vermian dysgenesis. We studied 15 patients with the diagnosis of Joubert syndrome to (1) more fully define the syndrome's clinical features, and (2) correlate the clinical features with magnetic resonance imaging (MRI) findings. Eight of 15 patients had a history of episodic hyperpnea and apnea. All patients had developmental delay and hypotonia. Of the 13 patients receiving detailed neuro-ophthalmologic evaluations, three had optic nerve dysplasia, pendular nystagmus, and gaze-holding nystagmus. All 13 patients had a normal vestibulo-ocular reflex based on head thrust, but had absent to poor ability to cancel the vestibulo-ocular reflex horizontally and vertically. Twelve of 13 patients had impaired smooth pursuit. Twelve of 13 patients had defects in initiation of saccades and quick phases. Two of the most consistent radiologic features were absent or hypoplastic posterior cerebellar vermis, and deformed midbrain and pontomesencephalic junction, which based on ocular motor physiology correlate with the vestibulo-ocular reflex cancellation/pursuit defect and saccade initiation defect, respectively As a result of midbrain, vermian, and superior cerebellar peduncle abnormalities, axial neuroimaging showed a unique "molar tooth" appearance of these structures. These results indicate that Joubert syndrome results from maldevelopment of the midbrain and cerebellar vermis, producing a pathognomonic sign on MRI. (J Child Neurol 1997;12:423-430).

The authors examined a patient who had neovascular glaucoma (NVG) shortly after ipsilateral carotid endarterectomy and followed the patient's clinical course. The authors speculate that the sudden reperfusion after endarterectomy that occurs in patients with combined central retinal artery occlusion and carotid occlusive disease may enhance the circulation of preformed angiogenesis factors, leading to the development of neovascularization. The authors recommend frequent and careful surveillance of patients with central retinal artery occlusion associated with carotid occlusive disease if ipsilateral carotid endarterectomy is performed. [Ophthalmic Surg Lasers 1996;27:881-884.]

Background: Upper eyelid retraction in thyroid eye disease may be caused by proptosis, levator and Muller's muscle infiltration with fibrosis or individual fiber enlargement, excessive sympathetic innervation, abnormal adhesions between levator palpebrae muscle and surrounding tissues, or fixation duress. Fixation duress refers to upper eyelid retraction while fixating with an eye with inferior rectus muscle restriction due to excessive simultaneous firing of the ipsilateral superior rectus and levator palpebrae muscles. Methods: The authors prospectively examined six patients with strabismus and thyroid eye disease associated with inferior rectus restriction and upper eyelid retraction in whom the eyelid retraction was suspected clinically to be a result of fixation duress. All six patients underwent recession of both inferior recti ranging from 3 to 6.5 mm (mean, 4.5 mm) to improve the ocular motility and alignment, reduce the diplopia, and mitigate the upper eyelid retraction. Results: Reduction of upper eyelid retraction measured as the preoperative versus postoperative difference in corneal light reflex — upper eyelid margin measurements was achieved in all patients postoperatively, ranging from 1.5 to 5.5 mm (mean, 3.2 mm). Only one patient showed significant residual retraction to warrant consideration of upper eyelid surgery. Conclusion: Fixation duress plays a significant role in upper eyelid retraction of thyroid eye disease in a subset of patients with restriction of the inferior rectus muscle. In this selected group of patients, the upper eyelid retraction may be reduced or eliminated upon proper recession of the tight inferior rectus muscle(s).

Gliomas that arise in the brain stem have been associated with a poor prognosis. Diagnostic neuroimaging readily identifies the tumor as it extends between normal brainstem structures. Histologic sampling of tumor with stereotactic methods is notoriously unreliable in establishing a definitive prognosis. Clinical trials that incorporate high-dose chemotherapy, autologous bone marrow rescue, and irradiation hold promise of better tumor control by overcoming the inaccessibility of the central nervous system to standard doses of chemotherapy. We review the pathology, clinical features, neuroimaging features, and current therapeutic concepts relative to brainstem glioma. The pediatric neurologist has a pivotal role in identifying and monitoring children with this malignancy. ( J Child Neurol 1993;8:112-128).

It is well established that children with infantile esotropia have deficient stereopsis and asymmetrical monocular optokinetic (OKN) nystagmus, even despite successful surgical alignment. On the ba...

Mechanisms that underlie overdepression of the eye in adduction, the so-called superior oblique overaction, are multiple. The various pathophysiologic processes involved remain incompletely defined...

The efficacy of tissue coating with sodium hyaluronate solution or oxidized regenerated cellulose (Interceed®) sleeves in reducing the occurrence and severity of postoperative adhesions following strabismus surgery was tested in rabbits in vivo and compared with the efficacy in this regard of treatment with balanced salt solution. The use of Interceed sleeves significantly increased formation of postoperative adhesions, but coating with sodium hyaluronate solution prior to and during surgery significantly reduced such formation.

Intracranial complications as a result of otogenic infections occur even in the antibiotic era. Meningitis is the most common reported intracranial complication, followed by brain abscess and lateral sinus thrombosis. The purpose of this study is to review our experience and management of these serious complications.A retrospective chart review was performed at a tertiary referral medical center for the period from 1998 to 2007. Charts with acute or chronic otitis media as primary diagnosis were reviewed, and intracranial complications secondary to either were included in the study. Age, sex, clinical presentation, radiographic findings, management, and outcome were studied. Patients with meningitis or petrous apicitis were not included in the study.Ten cases reviewed had intracranial complications. Five patients had brain abscesses, 1 patient had a subdural empyema, and 4 patients had lateral sinus thrombosis. All patients received broad-spectrum intravenous antibiotics for 6 weeks. Mastoidectomy was performed in all patients, but not all patients were treated with direct drainage of the intracranial abscess, especially if clinical and serial radiographic response was favorable.Otogenic intracranial complications can be fatal if not managed appropriately. Broad-spectrum intravenous antibiotics for 6 weeks is usually sufficient treatment. Management of the intracranial disease takes precedence, but direct drainage of the abscess may not be necessary if a patient's symptoms, neurologic status, and radiographic findings progress favorably. A high index of suspicion should be maintained on all patients presenting with symptoms not typically seen with routine otitis media.

The determination of temperature distribution in blood perfused tissue is important in many medical therapies and physiological studies. Examples are found in cryosurgery, frost bite, hyperthermia, skin burns and body thermal regulation and response to environmental conditions and during thermal stress. The key to thermal modeling of blood perfused tissue is the formulation of an appropriate heat transfer equation. Such an equation must take into consideration three factors: (1) blood perfusion, (2) the vascular architecture, and (3) variation in thermal properties and blood flow rate. The problem is characterized by anisotropic blood flow in a complex network of branching arteries and veins with changing size and orientation. In addition, blood is exchanged between artery-vein pairs through capillary bleed-off along vessel walls, draining blood from arteries and adding it to veins. Energy is transported between neighboring vessels as well as between vessels and tissue. Thus, heat transfer takes place in a blood perfused inhomogeneous matrix undergoing metabolic heat production. The search for heat equations modeling this complex process began over half a century ago and remains an active topic among current investigators. Over the years, several bioheat transfer equations have been formulated. A brief description of some of these equations will be presented and their limitations, shortcomings and applicability outlined. Before these equations are presented, related aspects of the vascular circulation network and blood flow and temperature patterns are summarized.

The authors analyzed the racial distribution of optic nerve head drusen and nonar-teritic ischemic optic neuropathy in a clinical setting. Blacks had a significantly much lower incidence of these two disorders than whites. Knowing previously that both disc drusen and nonarteritic ischemic optic neuropathy are associated with small discs, and that blacks have a larger disc area than whites, the authors conclude that blacks have a lower incidence of optic nerve head drusen and nonarteritic ischemic optic neuropathy than whites, partly because of racial variation of the optic disc size.

A case-control study of patients with and without confirmed UTI was performed to identify risk factors for nosocomial UTI. Duration of hospitalization, unit of admission, history of diabetes mellitus or debilitating diseases, and duration and number of urinary catheters were independently associated with increased risk of nosocomial UTIs.

A 72-year-old woman who had a history of arthritis and cerebral vascular accident 4 years earlier underwent hand surgery. Because regional anesthesia was not successful, general anesthesia was administered. Shortly before extubation, proptosis developed in her left eye, and she was unable to open it. The condition was due to spontaneous orbital hemorrhage and resolved with conservative treatment.

Purpose To explore the appropriate treatment of acute comitant esotropia in patients with Chiari I malformation. Design Interventional case reports and literature review. Participants Two patients with Chiari I malformation presenting with acute comitant esotropia are described. Intervention Strabismus surgery, then neurosurgical decompression of the Chiari I malformation was performed. Main outcome measure Both patients were evaluated for resolution of esotropia and other ocular motility problems. Results After initially successful strabismus surgery, both patients developed recurrent esotropia with diplopia, which resolved on suboccipital decompression. Conclusion Comitant esotropia may recur and other eye movement disorders may develop after initially successful strabismus surgery in patients with Chiari I malformation. The data suggest that the appropriate sequence of treatment should first be suboccipital decompression, then strabismus surgery if spontaneous realignment does not occur, but further studies are needed to confirm this impression.

Background The tonic ocular tilt reaction (OTR) consists of vertical divergence of the eyes (skew deviation), bilateral conjugate ocular torsion, and paradoxical head tilt. The head and superior pole of both eyes are rotated toward the hypotropic eye. Objective To describe ocular motility and torsion findings in 5 patients with OTRs that mimicked superior oblique palsies (SOPs). Results In 5 patients, results of the 3-step test suggested an SOP (bilateral in 1 patient); however, no patient had the expected excyclotorsion of the hypertropic eye. Two patients had conjugate ocular torsion (intorsion of the hypertropic eye and extorsion of the hypotropic eye), and 2 patients had only intorsion of the hypertropic eye. All had other neurologic features consistent with more widespread brainstem disease. Conclusions Vertical ocular deviations that 3-step to an SOP are not always caused by fourth nerve weakness. When a patient with an apparent fourth nerve palsy has ocular torsion that is inconsistent with an SOP, OTR should be suspected, especially if vestibular system or posterior fossa dysfunction coexists. The rules for the 3-step test for an SOP may be fulfilled by damaging the otolithic projections corresponding to projections of the contralateral anterior semicircular canal. Because results of the Bielschowsky head tilt test may be positive in patients with the OTR, the feature distinguishing OTR from an SOP is the direction of torsion. We advocate use of a fourth step—evaluation of ocular torsion—in addition to the standard 3 steps.

Abnormal cerebral venous drainage is associated with hypoxia and glucose deprivation, which can account for progressive neurologic deterioration in Sturge-Weber syndrome. Although developmental delay is common in Sturge-Weber syndrome, bihemispheric calcification is uncommon. Computed tomography (CT) and magnetic resonance imaging (MRI) were used to study the neuroanatomy, while single photon emission computed tomography (SPECT) was used concurrently to evaluate perfusion and glucose metabolism using99mTc hexamethylpropyleneamine oxime (HMPAO) and [18F] fluorodeoxyglucose (FDG), respectively. Ten patients (10 to 22 years of age) with previously diagnosed Sturge-Weber syndrome, port-wine nevi, and clinical evidence of seizures or stroke-like episodes were studied. Five children with onset of seizures in the first year of life had overall clinical severity comparable to that of children with later-onset seizures. Calcification was present in both hemispheres in one patient; six additional patients had other radiologic evidence of bihemispheric disease; SPECT studies detected bihemispheric disease in four cases. Our study is the first to concurrently evaluate structure, perfusion, and glucose metabolism in Sturge-Weber syndrome and to show a mismatch between functional and structural brain imaging in both cerebral hemispheres. Widespread abnormalities of cerebral perfusion and glucose metabolism might explain the high prevalence of developmental delay associated with Sturge-Weber syndrome. Longitudinal studies are needed to define better the natural history of neurologic deterioration and radiologic progression that relates to central nervous system circulatory dysfunction in Sturge-Weber syndrome. ( J Child Neurol 1998;13:595-605).

Background: A form of skew deviation, called alternating skew on lateral gaze, resembles bilateral superior oblique overaction. Oblique muscle overaction has been recently speculated to result from loss of fusion with subsequent "free-wheeling" of the torsional control mechanisms of the eyes, causing sensory intorsion or extorsion with attendant superior or inferior oblique muscle overaction, respectively. We wanted to investigate whether loss of fusion plays a role in the pathogenesis of alternating skew on lateral gaze. Subjects and methods: We examined seven consecutive patients with posterior fossa tumors, enrolled in a multi-disciplinary pediatric neuro-oncology program, who displayed alternating skew on lateral gaze. All patients underwent a thorough ophthalmologic evaluation. Results: Visual acuities in the study patients ranged from 20/20 to 20/40. Five of the seven patients were orthotropic, and showed 40 sec of arc stereopsis. Three patients showed associated downbeat nystagmus. No ocular torsion was found in any of the five patients who showed normal stereopsis upon inspection of fundus landmarks on indirect ophthalmoscopy. Conclusion: Patients with alternating skew on lateral gaze often have normal binocular vision and stereopsis, and lack ocular intorsion so typical of superior oblique overaction. Alternating skew on lateral gaze is neurologically mediated, with no role for defective fusion in its pathogenesis.

Children with neuro-ophthalmologic disorders often develop anomalous head postures (torticollis) or rhythmical movements of the head. The neuroophthalmologic evaluation of torticollis is greatly simplified by the probable association of a head tilt with superior oblique palsy and a horizontal or vertical head posture with incomitant strabismus or nystagmus. When strabismus and nystagmus are absent, the differential diagnosis includes a long list of ocular and systemic conditions. Head nodding in children usually signifies spasmus nutans or congenital nystagmus; however, an awareness of other rare causes is necessary to provide a complete evaluation. Abnormal head movements are less likely to be overlooked by parents than are abnormal head positions.

Pediatric nystagmus differs clinically and pathophysiologically from adult-onset nystagmus. Acquired nystagmus in adulthood is usually associated with an acute neurological lesion involving the ocular motor pathways within the brain stem and/or cerebellum. Based upon the clinical characteristics of the nystagmus and the associated neurological signs, a causative lesion can often be inferred clinically and confirmed by neuroimaging.

Background: Children with certain neurologic diseases (hydrocephalus, meningomyelocele, or cerebral palsy) have been reported to manifest a high frequency of A-pattern strabismus and superior oblique overaction. However, it is not generally recognized whether children with strabismus who have superior oblique overaction are more likely to have concurrent neurologic diseases than those without superior oblique overaction. In this study, the authors examine this issue. Methods: The authors retrospectively reviewed the medical records of all patients (n = 168) with overdepression of the downturned eye in adduction, who were examined between October 1989 and March 1992. A randomly selected population of children with strabismus who did not have overdepression of the eye on infraduction and adduction served as controls (n = 98). Patients with simulating or confounding conditions such as pseudo-superior oblique overaction, inferior rectus skew deviation (alternating skew on lateral gaze), and restrictive or paralytic strabismus, and who were older than 20 years of age, were excluded. Results: One hundred twelve patients with true superior oblique overaction were analyzed. Of these 112 patients, 45 (40.2%) had concurrent neurologic abnormalities, compared with less than one fifth (17.3%) of control subjects (17 of 98) ( P ≤ 0.001). Conclusions: Children with strabismus who have superior oblique overaction were found to have higher prevalence of concurrent neurologic diseases than control subjects. Superior oblique overaction may represent a clinical marker for an associated neurologic dysfunction, possibly representing a form of skew deviation in some cases.

Periodic alternating esotropia (PAE) is a rare ocular motility disturbance observed in association with periodic alternating gaze deviation (PAG) or periodic alternating nystagmus. We examined a 9-month-old developmental^ delayed girl who showed PAE occurring synchronously with PAG. The ocular motility disorder consisted of rhythmic alternating fixation with the right eye in abduction, a left face turn and esotropia of the left eye (90 seconds), a changeover phase (10 to 15 seconds) during which the eyes are straight and the head is upright, then fixation with the left eye in abduction, a right face turn, and esotropia of the left eye (90 seconds). There was no spontaneous jerk nystagmus present. These findings continued incessantly during a follow-up period of 18 months. Magnetic resonance imaging revealed pronounced cerebellar vermis hypoplasia. Only three clinically similar cases have been previously reported, but none had been studied with modern neuroimaging techniques.

Oculomotor nerve paresis, ocular hypotony, anterior segment ischemia, and the ocular ischemic syndrome are uncommon manifestations of giant cell arteritis. Four patients with these findings had giant cell arteritis documented by temporal artery biopsy. Cerebral angiography or ultrasonography, or both, performed in three patients, excluded hemodynamically significant stenosis of the internal carotid artery as the cause of ocular ischemia and cerebral aneurysms as the cause of oculomotor nerve paresis. Corticosteroid treatment, administered to three patients, resulted in resolution of the oculomotor deficits and the clinical signs of ocular ischemia, although the visual acuity in one patient improved from 20/400 to 20/60. Giant cell arteritis should.be considered in the differential diagnosis of the ocular ischemic syndrome.

Anatomic features unique to periocular tissues alter the clinical appearance of angioedema, making it difficult to distinguish from cellulitis and other inflammatory conditions. Two patients had prominent periocular manifestations of chronic angioedema with necrotizing vasculitis, a systemic disease often associated with multiorgan involvement. The diagnosis was established by exclusion of other inflammatory disorders and confirmed by biopsy. The scarcity of reports in the ophthalmic literature on chronic angioedema of the eyelids may be caused by its underrecognition. The distinction between chronic angioedema and typical angioedema or urticaria is important because of differences in their diagnostic evaluation, treatment, and prognosis.

Unlike acquired oculomotor nerve palsy in childhood, the congenital form is usually considered to be an isolated disorder, not associated with other neurologic or systemic disease. In a series of 14 patients with this diagnosis, 10 had associated neurologic disorders. Clinical-neuroradiologic correlation identified the brainstem as the site of the lesion in some patients. The ipsilateral pupil was involved in 12 patients and was miotic with a trace or no reaction to light on the side of the palsy in 8 of the 12 patients. In two of these eight patients, the pupil was documented to be dilated and fixed in early infancy and subsequently became miotic at several months of age, concurrent with emergence of other signs of aberrant reinnervation. Pupillary miosis as a result of aberrant reinnervation appears to be much more frequent after congenital than acquired pupillary-involving oculomotor palsy. Four patients showed better visual acuity in the paretic eye, an unexpected finding. The paretic eye appeared to have a fixational advantage if the patient had nystagmus, presumably due to asymmetric dampening of the nystagmus. Significant anisometropia and astigmatic errors in the paretic eye were present in essentially all patients.

Strabismus presenting after cataract surgery is etiologically related to a heterogenous group of disorders. Clinical data from 63 patients so affected revealed four broad etiologic categories: 1) pre-existing disorders that preceded the cataract surgery, but were rendered asymptomatic by the occluding cataract (e.g., thyroid eye disease, cranial nerve palsy, myasthenia); 2) disorders precipitated by prolonged occlusion by a cataract (e.g., sensory deviations, decompensation of heterophorias, and central disruption of binocular vision); 3) disorders resulting from surgical trauma to extraocular muscles and orbital soft tissues. Traumatic injury to the inferior rectus muscle secondary to retrobulbar anesthesia injection, a specific subset, is postulated to result from a Volkmann's type ischemic contracture, a well-known osseofascial compartment syndrome occurring in peripheral skeletal muscles. 4) Disorders related to resulting aphakia/pseudophakia and associated optical aberrations (e.g.; anisophoria, ocular dominance reversal, and color/brightness disparity). The diagnostic and therapeutic implications of these findings are discussed.

Strabismus presenting after cataract surgery has been attributed to a variety of disorders, some of which are unrelated to the surgery. When thorough ophthalmologic and neurologic examinations fail to elucidate a definitive etiology, the motility disturbance is often ascribed to operative trauma to the orbital soft tissues. In a series of 58 patients with strabismus presenting after cataract surgery, eight (14%) were found to have previously unsuspected thyroid eye disease. Three of the eight patients had past histories of systemic dysthyroidism, but none reported diplopia prior to cataract extraction. Three of the remaining five patients showed normal thyroid function tests. Absence of diplopia prior to cataract extraction in these patients may be due to: 1) poor vision precluding diplopia until surgical restoration of sight; 2) intraoperative aggravation of the inflammatory process in otherwise subclinical thyroid eye disease; or 3) onset of clinical thyroid eye disease that was temporally associated with cataract surgery by chance. Thyroid eye disease should be included in the differential diagnosis of all patients presenting with binocular diplopia following cataract surgery. Suitable neuroimaging studies may be needed to confirm the diagnosis.

T he article byJeffery et al. 1 on pages 159-67 of this issue of the Journal of AAPOS raises interesting points regarding pediatric Horner syndrome. On the basis of a retrospective review of 73 patients less than 18 years old seen over 10 years at two large referral centers, the authors provide a diagnostic paradigm emphasizing that seemingly idiopathic cases should be thoroughly investigated for the possible presence of malignancy, chiefly neuroblastoma. Their diagnostic workup would vary with the clinical profile and would include testing for urinary catecholamine breakdown products and imaging of the head, neck, chest, and abdomen. Generally, an ideal diagnostic paradigm is highly sensifive and specific for the particular clinical situation, safe, convenient, and cost effective. For pediatric Horner syndrome, the usual evaluation variably includes a physical examination, ophthalmologic examination, urinary assay for catecholamine breakdown products, and imaging studies. Thoughtful acquisition of data in each component of this workup can help make the overall diagnostic paradigm approach the ideal above. Underlying the fear of missing or delaying the diagnosis of neuroblastoma is the observation that the age at diagnosis is the most important prognostic factor, with children less than 1 year old having the best prognosis. Evidence now suggests that this is so, at least in part, because some of the tumors detected in early life are biologically more benign and some are perhaps destined to spontaneously involute without treatment. This may have been the case in cases 20 and 21 of the authors' report. Early diagnosis, although very desirable, may sometimes detect tumors destined for spontaneous involution, a lesson now well learned from the Japanese studies on mass screening for neuroblastoma. 2, 3 The physical evaluation should seek to exclude known causes of Homer syndrome and estimate the age of onset (inspection of old photographs is helpful). It should include palpation of the neck and abdomen for masses.

Background: Upper eyelid retraction in thyroid eye disease may be caused by proptosis, levator and Muller's muscle infiltration with fibrosis or individual fiber enlargement, excessive sympathetic innervation, abnormal adhesions between levator palpebrae muscle and surrounding tissues, or fixation duress. Fixation duress refers to upper eyelid retraction while fixating with an eye with inferior rectus muscle restriction due to excessive simultaneous firing of the ipsilateral superior rectus and levator palpebrae muscles. Methods: The authors prospectively examined six patients with strabismus and thyroid eye disease associated with inferior rectus restriction and upper eyelid retraction in whom the eyelid retraction was suspected clinically to be a result of fixation duress. All six patients underwent recession of both inferior recti ranging from 3 to 6.5 mm (mean, 4.5 mm) to improve the ocular motility and alignment, reduce the diplopia, and mitigate the upper eyelid retraction. Results: Reduction of upper eyelid retraction measured as the preoperative versus postoperative difference in corneal light reflex — upper eyelid margin measurements was achieved in all patients postoperatively, ranging from 1.5 to 5.5 mm (mean, 3.2 mm). Only one patient showed significant residual retraction to warrant consideration of upper eyelid surgery. Conclusion: Fixation duress plays a significant role in upper eyelid retraction of thyroid eye disease in a subset of patients with restriction of the inferior rectus muscle. In this selected group of patients, the upper eyelid retraction may be reduced or eliminated upon proper recession of the tight inferior rectus muscle(s).

PURPOSE To evaluate the use of Scheimpflug photography in the examination of iris configuration and anterior chamber depth in eyes with pigment dispersion syndrome (PDS) and pigmentary glaucoma (PG). METHODS The Nidek EAS 1000 Scheimpflug camera was used to photograph the anterior segment of 12 eyes with PDS/PG, as well as 12 age-matched controls and four eyes with primary open-angle glaucoma (POAG). Anterior chamber depth, iris insertion angle, and iris configuration were evaluated. Five eyes of three patients were serially photographed over 10 min while blinking was prevented. RESULTS Patients with PDS/PG showed deeper anterior chambers than age-matched controls or patients with POAG. Nine of 12 PDS/PG eyes showed a concave iris configuration. There were no concave irides noted in control or POAG eyes. Preventing blinking resulted in significant anterior excursion of concave irides in three of the five eyes studied. CONCLUSIONS Scheimpflug photography is a useful modality to study iris configuration. These photographs document the presence of a concave iris configuration in some patients with PDS/PG, and support the hypothesis that blinking may be significant in producing this configuration.

• Optic nerve aplasia is a rare congenital defect invariably associated with other ocular or systemic disorders. We examined a 3-year-old girl with monocular microphthalmos who had optic nerve aplasia on histopathologic examination of the eye after enucleation. Magnetic resonance imaging verified the presence of unilateral optic nerve aplasia, and demonstrated hemichiasmal hypoplasia on the affected side and bilateral optic tracts. The visually evoked cortical response revealed increased signals over the occipital cortex ipsilateral to the aplastic nerve, suggesting misdirection of axons from the temporal retina of the normal eye. The visual pathway in unilateral optic nerve aplasia may assume a primitive form of neuronal organization characterized by an increase in contralateral retinogeniculostriate projection.

• The mechanisms by which optic nerve-sheath fenestration is effective remain speculative. Possibilities include surgical production of a cerebrospinal fluid filtration outlet or scarring in the subarachnoid space around the nerve in the vicinity of the fenestration site, with shifting of the pressure gradient from the nerve head to the retrobulbar portion. Two patients who underwent successful optic nerve-sheath fenestration developed a cystlike structure contiguous to the fenestration site, apparently in direct communication with the optic nerve sheaths. This was shown on magnetic resonance imaging (one patient) and orbital echography (both patients). These previously unreported observations may support the hypothesis that fenestration works by creating a filtration apparatus that controls the intravaginal pressure in the subarachnoid space surrounding the orbital segment of the optic nerve.

To the Editor. —Danazol is an androgenic compound used in the treatment of endometriosis, benign cystic breast disease, and some hematologic disorders. Danazol therapy has been implicated in the pathogenesis of pseudotumor cerebri in some patients. 1,2 We describe a patient who developed symptoms and signs of increased intracranial pressure (ICP) shortly after cessation of danazol. Report of Case. —A 36-year-old woman was prescribed 200 mg/d of danazol for endometriosis. She received the drug from December 1989 to July 1990. Two days after discontinuing danazol, she developed bifrontal headaches with nausea and tinnitus, accompanied by transient visual obscurations. She was examined 3 weeks later because of progressive worsening of her symptoms. Corrected visual acuity was 20/25 in each eye. Results of pupillary and slit-lamp examination and applanation tonometry were normal. Fundus examination showed bilateral papilledema, peripapillary hemorrhages, and absent spontaneous venous pulsations. The blind spots were enlarged on Goldmann visual

We studied four patients with HIV-associated optic neuropathies. One had syphilitic optic perineuritis, which responded promptly and completely to penicillin therapy. The second had cytomegalovirus papillitis and visual acuity subsequently deteriorated to no light perception. The third showed varicella zoster optic neuritis, which improved after intravenous acyclovir treatment. The fourth patient developed cryptococcal retrobulbar neuritis and died shortly thereafter. Optic neuropathy was among the initial symptoms of HIV infection in two of the four cases.

Intracranial hypertension with papilledema occurred in two patients receiving danazol therapy for either cyclic neutropenia or immune hemolytic anemia. Results of clinical, laboratory, and neuroradiologic studies showed no apparent cause for the condition in Case 1 and the papilledema resolved one month after discontinuing danazol. Carotid angiography in Case 2 demonstrated cerebral venous sinus thrombosis; the papilledema showed gradual improvement after cessation of danazol. An additional seven cases of pseudotumor cerebri presumed secondary to danazol therapy have been reported to the Food and Drug Administration. The papilledema resolved in all seven cases soon after discontinuing danazol. A drug-induced complication should be suspected, and alternative therapy sought, in patients who develop intracranial hypertension associated with administration of danazol.

Unlike keratoacanthomas occurring on the skin, those found on mucous membranes are decidedly rare. We present two cases of keratoacanthoma of the limbal area.

Ocular motility manifestations of focal brainstem dysfunction were the initial clinical features in three patients with human immunodeficiency virus (HIV) infection. These included conjugate gaze palsy with ipsilateral facial paresis, bilateral abducens palsy and a gaze paresis, and homolateral internuclear ophthalmoplegia and abducens nerve paresis. Two patients had focal brainstem lesions as evidenced on neuroimaging. The third showed concurrent infection with Treponema pallidum.

We reviewed the records of 38 consecutive patients who had persistent binocular diplopia after cataract surgery. The patients were divided into ten categories based on the suspected conditions underlying the diplopia. Of 16 patients who underwent strabismus surgery, five achieved the therapeutic goal of single binocular vision in the primary and reading position, and four attained this with prismatic or botulinum toxin therapy in addition to surgery. Seven patients continued to have diplopia despite strabismus surgery and adjunctive therapy. Even small residual deviations were often intolerable, because of highly diminished fusional amplitudes.

A muscle sleeve fashioned from polyglactin 910 Vicryl* mesh was used in a rabbit animal model to evaluate its efficacy in decreasing postoperative adhesions encountered in extraocular muscle surgery. Clinical observations, as well as gross and histological comparison of the experimental and control groups, revealed a significant reduction in postoperative adhesions under conditions of normal surgical trauma. Where there was extensive trauma, however, no significant reduction was observed. No evidence of toxicity of the material to ocular tissues was found.

We examined three patients who developed optic neuropathies while undergoing chronic hemodialysis. One patient developed severe bilateral deterioration of vision, but recovered totally four weeks after discontinuing deferoxamine chelation therapy. Another patient had bilateral visual loss associated with chronic papilledema of idiopathic increased intracranial pressure. A third showed atypically severe consecutive anterior ischemic optic neuropathy. The latter two patients showed little improvement with high-dose intravenous methylprednisolone therapy combined with more vigorous hemodialysis. These cases, in addition to those previously described, underscore the heterogeneity of optic nerve disease in patients with uremia.

• Two patients had protracted blind spot enlargement, photopsia, and no optic disc edema. Both had fundus and fluorescein angiographic findings typical of multiple evanescent white dot syndrome that resolved within several weeks, leaving a persistent enlarged blind spot and photopsia. These clinical findings and an analysis of the literature data suggest that acute idiopathic blind spot enlargement without optic disc edema may be a subset of multiple evanescent white dot syndrome.