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Biomedical subjects

S Rumelt

Publications and source records attributed to S Rumelt.

At least 37 records · Page 2Linked to original sources

Marginal corneal infiltrates: a possible new manifestation of sinus histiocytosis with massive lymphadenopathy.

PURPOSE: To describe unilateral deep marginal corneal infiltrates and chronic anterior uveitis in a young patient with sinus histiocytosis and massive lymphadenopathy. METHODS: A 20-year-old man with a painless enlargement of his cervical lymph nodes was followed for five years after debulking of these nodes on the right side for right chronic anterior uveitis and deep marginal corneal infiltrates. RESULTS: Histological sections of cervical lymph node confirmed the diagnosis of sinus histiocytosis with the typical emperipolesis. The uveitis partially responded to topical corticosteroid treatment but tended to recur upon its withdrawal. The marginal corneal infiltrates resolved gradually over a period of five years. CONCLUSIONS: Marginal corneal infiltrates and uveitis may presumably be associated with sinus histiocytosis. Patients with sinus histiocytosis should also be screened for rare ocular involvement, such as uveitis and corneal infiltrates.

Adult↗

Diagnosis of chlamydial infection by direct enzyme-linked immunoassay and polymerase chain reaction in patients with acute follicular conjunctivitis.

BACKGROUND: Acute follicular conjunctivitis is a clinical diagnosis common to multiple etiologies, of which chlamydial infection requires specific antibiotic treatment. PURPOSE: This prospective study was designed to evaluate Chlamydia trachomatis as the cause of acute follicular conjunctivitis by two sensitive tests: direct enzyme-linked immunosorbent assay (ELISA) and polymerase chain reaction (PCR). METHODS: Conjunctival scrapings from patients presented with untreated acute follicular conjunctivitis were examined by ELISA and PCR, and patients were followed up for prolongation of the disease course. RESULTS: All 36 consecutive patients presented with acute follicular conjunctivitis were negative for Chlamydia trachomatis by ELISA and PCR. None of the patients had a prolonged course of more than 4 weeks or required treatment with systemic antibiotics as would be expected from chlamydial infection. CONCLUSIONS: Chlamydia trachomatis was probably not responsible for the acute follicular conjunctivitis in this series, and ELISA and PCR may not be cost effective for evaluation of acute follicular conjunctivitis due to chlamydial infection. Further evaluation of the cost effectiveness of these tests is required in chronic follicular conjunctivitis.

Acute Disease↗

Aggressive systematic treatment for central retinal artery occlusion.

PURPOSE: To report the efficacy of an aggressive systematic regimen for the treatment of acute nonarteritic central retinal artery occlusion (CRAO). METHODS: Eleven patients who had unilateral CRAO with symptoms of fewer than 48 hours' duration were treated with an aggressive stepwise systematic regimen until retinal circulation improved or until all the treatment steps were performed. Five patients with unilateral CRAO and symptoms of fewer than 48 hours' duration were treated in the same institution in an arbitrary nonsystematic manner. The therapeutic steps of the aggressive treatment included ocular massage, sublingual isosorbide dinitrate, intravenous acetazolamide, intravenous mannitol or oral glycerol, anterior chamber paracentesis, intravenous methylprednisolone followed by streptokinase, and retrobulbar tolazoline. After each step, retinal flow was evaluated by three-mirror contact lens. The nonsystematic treatment was arbitrary and included one or several of the above. Visual acuity and complete eye examination data were recorded before and after treatment. RESULTS: Visual acuity and retinal arterial supply were improved in eight (73%) of the 11 patients treated in the stepwise systematic manner. All eight patients in whom visual acuity improved had symptoms for fewer than 12 hours, and the presumed cause was either platelet-derived or cholesterol embolus from atheroma or the patients had glaucoma. Patients in whom visual acuity did not improve had CRAO that was attributed to calcified emboli or primary antiphospholipid antibody syndrome and had symptoms more than 12 hours before treatment. Visual acuity did not improve in all five patients with the nonsystematic treatment regardless of the presumed cause or duration of the occlusion. The success of the treatment in the systematic treatment group was statistically significantly better compared with the outcome of the nonsystematic treated group (Fischer exact test, P = .01). CONCLUSIONS: In the treatment of CRAO, an aggressive systematic regimen including medical and mechanical means may reestablish retinal circulation and improve visual outcome. The cause of arterial occlusion, the nature of occlusive emboli, and the duration of retinal ischemia may determine the visual outcome, but a larger series is warranted to verify the effectiveness of the treatment and the prognostic factors.

Acetazolamide↗

Iridocorneal melanoma associated with type 1 neurofibromatosis: a clinicopathologic study.

OBJECTIVE: A clinicopathologic study of an iridocorneal melanoma associated with type 1 (peripheral) neurofibromatosis is presented. DESIGN: Case report with clinicopathologic correlation. PARTICIPANT: A 32-year-old white woman with type 1 neurofibromatosis presented with long-standing blindness of her right eye due to diffuse intrastromal brown corneal discoloration. INTERVENTION: The patient underwent penetrating keratoplasty and the corneal button was inspected. RESULTS: Histopathologic evaluation of the corneal button after penetrating keratoplasty revealed an intrastromal mixed-type malignant melanoma, which stained positively with HMB-45 and S-100 protein and spared the corneal epithelium and limbus. The corneal graft remained transparent, with best-corrected visual acuity of 20/30. Twenty-two months after surgery, the tumor involved the anterior chamber angle and the iris. Three years later, it caused refractory glaucoma necessitating enucleation. The iris tumor did not extend beyond the iris-lens diaphragm and showed the same cytologic features as the corneal stromal tumor. CONCLUSION: To our best knowledge, this is the first report of iridocorneal melanoma associated with peripheral neurofibromatosis. The location of the tumor in the deep corneal stroma, without initial macroscopic involvement of the angle or iris, may suggest that the corneal portion of the tumor may have developed "in situ" rather than as an extension of iris melanoma. The common origin of melanoma cells and Schwann cells from the neural crest and the proliferation of the Schwann cells in neurofibromatosis provides additional support for this hypothesis.

Adult↗

Prepartum mixed type cavernous-capillary hemangioma arising in nevus flammeus.

OBJECTIVE: Capillary hemangioma may appear de novo and involute during the first decade of life, but rarely during pregnancy. This study describes the clinical and histologic findings of an eyelid mixed type cavernous-capillary hemangioma arising in a nevus flammeus and discusses the differential diagnosis of this lesion. STUDY DESIGN: Clinicopathologic case report. INTERVENTION: A reddish, protruding eyelid mass arising from a nevus flammeus at the eyelid margin in a 26-year-old woman was monitored during her pregnancy. Postpartum, the mass was excised and examined histologically. RESULTS: The lobulated tumor recurred during the second pregnancy and partially regressed following delivery. It was composed of mixed elements of cavernous and capillary hemangioma that superficially resembled Kaposi sarcoma, set against the background of a nevus flammeus. CONCLUSION: The differential diagnosis of discrete prepartum vascular tumor arising in nevus flammeus includes mixed capillary-cavernous hemangioma, pseudo-Kaposi sarcoma, granuloma gravidarum, and angiodermatitis. A common stimulus during pregnancy may be the inciting factor for the development of these tumors.

Adult↗

Congenital unilateral buphthalmos in Walker-Warburg syndrome: a clinicopathological study.

BACKGROUND AND PURPOSE: Walker-Warburg syndrome is a congenital autosomal recessive oculocerebral disorder characterised by hydrocephalus, brain agyria, microphthalmos and retinal dysplasia with or without meningoencephalocele. We describe an unusual finding of congenital unilateral glaucoma and buphthalmos in one eye and microphthalmos in the fellow eye of two neonates with Walker-Warburg syndrome. PATIENTS: Two neonates with Walker-Warburg syndrome and unusual findings of buphthalmos in one eye and a microphthalmic fellow eye are presented. RESULTS: Histological examination of the buphthalmic eyes revealed the presence of mesenchymal tissue in the anterior angle covered by endothelium. No anterior chamber angle was identified in the microphthalmic fellow eye and the iris was adherent to the corneal periphery. CONCLUSIONS: Congenital buphthalmos may also appear in Walker-Warburg syndrome. The buphthalmos may result from later embryonal ocular developmental arrest than that of the microphthalmic eye.

Abnormalities, Multiple↗

Functional indications for enophthalmos repair.

PURPOSE: In general, orbital augmentation to correct enophthalmos is pursued to prevent or address an aesthetic deformity. In some cases, however, functional deficits may accompany enophthalmos and may serve as an indication for surgical intervention. The authors describe a series of patients with such deficits. METHODS: A retrospective review at a tertiary health care center of all patients with enophthalmos was conducted to identify a subset of cases in which the enophthalmos was associated with nonaesthetic, functional deficits that could not be attributed to muscular or neural dysfunction, or soft tissue scarring. RESULTS: Six patients with either traumatic enophthalmos (orbital fractures) or non-traumatic enophthalmos (sinus disease and orbital soft tissue atrophy) demonstrated nonaesthetic ocular dysfunction, including gaze-evoked diplopia, eyelid retraction, lagophthalmos, and exposure keratitis. The symptoms and signs resolved in the three patients who underwent orbital augmentation. CONCLUSIONS: In some patients with enophthalmos and globe ptosis, globe malposition may alter the underlying eyelid mechanics or extraocular muscle alignment, resulting in functional as well as aesthetic problems. In these patients, restoring the native orbital anatomy through orbital augmentation can reverse eyelid malposition, ocular surface exposure, and symptomatic diplopia, avoiding the need for eyelid or strabismus surgery.

Adult↗

Ocular trauma following penetrating keratoplasty: incidence, outcome, and postoperative recommendations.

BACKGROUND: The surgical wound after penetrating keratoplasty is more vulnerable to contusive trauma than the intact cornea. OBJECTIVES: To assess the incidence of ocular trauma following penetrating keratoplasty, and to evaluate its causative factors, management, and visual outcome. SETTING: Tertiary referral facility in a fairly closed population. METHODS: Retrospective study assessing ocular injuries of all 559 patients who underwent penetrating keratoplasty in the center between September 1986 and March 1993. RESULTS: Fourteen (2.5%) of 559 patients who underwent penetrating keratoplasty, over a period of 78 months, suffered surgical wound dehiscence because of contusive ocular trauma. The mean age of the patients (30.6 years) was significantly lower (P<.001) than that of the total number of patients who received transplants (49 years). The interval between transplantation and trauma ranged from 2 weeks to 2 years (mean interval, 6.7 months). In 11 of the 14 patients the trauma occurred prior to removal of sutures. Globe rupture occurred at the donor-recipient interface in all of the patients, accompanied by vitreous and lens loss in 8 patients (57%). In 2 patients (14%) trauma included disruption of retinal tissue resulting in poor visual outcome. The trauma occurred most often at home (in 7 patients). After follow-up periods of 1 to 6 years (mean, 29 months), the corrected visual acuity ranged from 20/120 to 20/20 in 12 patients (86%). CONCLUSIONS: Ocular injury following penetrating keratoplasty is not a rare event. Since corneal graft wound is vulnerable to ocular trauma, we recommend the constant use of protective eyewear and periodically instruct the patients on the long-term vulnerability of the graft wound. Patients should be repeatedly cautioned against high-risk environments and strenuous activity.

Adolescent↗

Four-eyelid sebaceous cell carcinoma following irradiation.

BACKGROUND: Sebaceous cell carcinoma is a distinctive tumor of the eyelid associated with a high rate of metastasis and mortality. Involvement of both upper and lower lids has been described; however, involvement of all 4 eyelids is extremely rare. OBJECTIVE: To describe the evaluation and diagnosis of the clinicopathologic features in a 74-year-old patient with 4-eyelid sebaceous carcinoma and a history of whole face irradiation for eczema. METHODS: Bilateral eyelid, conjunctiva, map, and fine needle biopsy of enlarged, submandibular lymph node specimens were stained with hematoxylin-eosin and oil-red-O for light microscopy and fresh-frozen sections were evaluated by electron microscopy. RESULTS: The biopsy specimens from the eyelids and conjunctiva showed sebaceous carcinoma cells in the meibomian glands and scattered within the conjunctival epithelium. The cells were moderately well differentiated. Similar cells stained with oil-red-O were found in the submandibular lymph nodes. CONCLUSIONS: The appearance of bilateral sebaceous carcinoma supports the concept of simultaneous occurrence of multiple primaries in specific patients, especially after radiation therapy. Patients with a history of facial irradiation and atypical eyelid lesions should be evaluated for malignant tumors such as sebaceous carcinoma.

Adenocarcinoma, Sebaceous↗

Implantation of glaucoma drainage implant tube into the ciliary sulcus in patients with corneal transplants.

The placement of glaucoma drainage implants may be complicated by tube-corneal touch and endothelial decompensation, particularly after corneal transplantation. We describe an innovative surgical approach to glaucoma drainage implant procedures that may decrease such complications. The approach involves placement of the shunt tube into the ciliary sulcus. This approach may serve as an alternative to anterior chamber angle or pars plana implant placement in pseudophakic or aphakic eyes with refractory glaucoma and a high risk for corneal decompensation.

Adult↗

Orbital computed tomographic characteristics of globe subluxation in thyroid orbitopathy.

OBJECTIVE: Axial globe subluxation may complicate thyroid orbitopathy. This acute event is defined by anterior displacement of the globe equator beyond the orbital rim, lid retraction behind the equator, and tethering of the optic nerve. The authors explored the factors leading to spontaneous globe subluxation in patients with thyroid orbitopathy. DESIGN: A clinical review. MAIN OUTCOME MEASURES: The medical charts and axial and coronal orbital computed tomographic (CT) scans of all the patients with spontaneous globe subluxation were reviewed. The patients were categorized according to their CT scans into type I ("lipogenic" variant) or type II ("myogenic" variant). RESULTS: Four (0.1 %) of approximately 4000 patients with thyroid orbitopathy presented with spontaneous globe subluxation. All of these patients had increased orbital fat without significant enlargement of the extraocular muscles. Thus, they represent type-I thyroid orbitopathy. CONCLUSIONS: Globe subluxation in thyroid orbitopathy requires compliance of the orbital soft tissues and extensibility of the extraocular muscles. These are characteristics of type-I orbitopathy. The increased fat content results in more compliance of the soft tissues, and the normal caliber of the muscles allows them to become more extensible. This permits the acute contraction of the eyelids posterior to the equator of the globe. Patients with type-I orbitopathy and extensive proptosis may be at relatively greater risk of having globe subluxation develop.

Acute Disease↗

Enhancement of the cosmetic and functional outcome of enucleation with the conical orbital implant.

OBJECTIVE: The authors evaluated a new design of a conical-shaped enucleation implant to help minimize the occurrence of superior sulcus defects and maximize motility of the prosthesis. The implant shape is a modification of a sphere. It has a posterior conical projection paralleling the orbital walls, a superior projection supporting the soft tissues of the upper eyelid sulcus, a flattened anterior surface, and channels for each rectus muscle. DESIGN: The study design was a consecutive case series from the Oculoplastics and Orbital Service of the Massachusetts Eye and Ear Infirmary. INTERVENTION/PARTICIPANTS: A total of 45 patients (average age, 40 years; range, 13-75 years) had placement of a conical implant (39 primary enucleations, 6 secondary implants) with a minimum of 1 year' follow-up (range, 12-36 months). All of the primary enucleations and two of the secondary procedures had the anterior portion of the implant covered with autologous fascia. Four of the secondary implants were covered with pseudocapsule harvested from the explanted primary implant. Prostheses were fit 6 to 10 weeks after surgery. MAIN OUTCOME MEASURES: Postoperative appearance of patients was assessed by qualitative appearance of the superior sulcus and prosthetic motility, and subjectively by patients' satisfaction. RESULTS: A total of 43 patients had minimal or no superior sulcus defect, whereas 2 patients had moderate defects. There were no severe sulcus defects. All patients were satisfied with their appearance and did not seek further surgery to correct any upper sulcus asymmetry. Prosthetic motility with small-angle ductions (<10 degrees) and saccades was good in all cases. There were two cases of conjunctival wound dehiscence. Both occurred within 4 weeks of surgery. One wound dehiscence was sutured, whereas the other healed spontaneously. There were no cases of implant extrusion, migration, or infection. CONCLUSION: The conical orbital implant provides appropriate reconstitution of orbital volume while minimizing superior sulcus defects with adequate prosthetic motility.

Adolescent↗

Sneddon's syndrome: neuro-ophthalmologic manifestations in a possible autosomal recessive pattern.

Sneddon's syndrome is a rare neurodermatologic disorder that is manifested by multiple cerebrovascular accidents and livedo reticularis. The authors describe two siblings from one family with Sneddon's syndrome, suggesting autosomal recessive inheritance. The propositus presented with internuclear ophthalmoplegia and ophthalmic artery occlusion. These manifestations as well as the autosomal recessive inheritance have not yet been reported in Sneddon's syndrome.

Adolescent↗

Giant epidermoid involving 3 cranial bones.

We describe a patient with an incidental finding of an expansile osteolytic lesion centered in the sphenoid involving 3 cranial bones-frontal, temporal, and sphenoid-and invading the brain parenchyma and orbit. Biopsy demonstrated an epidermoid. The lesion was excised through frontotemporal craniotomy. Despite the benign nature of the tumor, the epidermoid had a destructive pattern of growth. Complete resection was needed to adequately address the inflammatory mass effect of the large lesion.

Adult↗