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Biomedical subjects

L M Holbach

Publications and source records attributed to L M Holbach.

At least 37 records · Page 2Linked to original sources

[Orbito-palpebral emphysema due to ethmoidal fracture].

BACKGROUND: Orbito-palpebral emphysema is a frequent pathological entity due to head injury. We report on a patient and correlate the clinical view and computer tomography findings. CASE REPORT: A 17-year-old male was referred to our hospital after a blunt trauma to his head. Visual acuity was 1,0. Diplopia, restricted motility and exophthalmus about 4 mm were found. CONCLUSION: If swelling of the lid is found orbito-palpebral emphysema should be thought of to initiate appropriate therapy to avoid possible severe complications, for example, occlusion of the retinal central artery.

Adolescent↗

Multifocal corneal argyrosis after an explosion injury.

PURPOSE: To document the clinical and histopathologic corneal features of a patient who developed multifocal corneal argyrosis after a chemical explosion injury with unusual involvement of the corneal stroma and keratocytes. METHODS: The corneal button was investigated by light and transmission electron microscopy and scanning electron microscopy combined with energy-dispersive x-ray microanalysis. RESULTS: Clinically, the patient showed dark discoloration of the lids, periocular skin, episclera, and conjunctiva and had multiple brown dots in the superficial layers of the cornea. Microscopic examination of the cornea showed diffuse deposition of silver particles in the epithelial basement membrane, Bowman's layer, and Descemet's membrane. In the corneal stroma, silver granules accumulated intracellularly within lysosomal structures of degenerative keratocytes and extracellularly in association with collagen fibers and cellular debris. Energy-dispersive x-ray analysis showed peaks of silver and sulfur. CONCLUSION: The toxic influence of intracellular accumulation of silver in stromal keratocytes may lead to cell damage and necrosis and result in visual impairment.

Argyria↗

Oculocerebral non-Hodgkin's lymphoma with uveal involvement: development of an epibulbar tumor after vitrectomy.

Primary ocular lymphoma is the ocular manifestation of primary oculocerebral non-Hodgkin's lymphoma. We describe a 79-year-old woman with a 7-year history of bilateral uveitis and subsequent central nervous system lymphomas. Repeated diagnostic vitrectomy during the following 5 years failed to demonstrate intraocular lymphoma cells. Within 9 months after the second vitrectomy, an epibulbar tumor developed in the limbal region of the left eye at the site of the sclerotomy. The eye, blind and painful due to secondary angle-closure glaucoma, was enucleated. Histopathologically, the globe showed a diffuse large B-cell non-Hodgkin's lymphoma extending from the ciliary body outward through the sclerotomy. We conclude that, following vitrectomy, a primary ocular lymphoma may extend through the sclerotomy lesion and present as an epibulbar tumor. Uveal involvement may occur in oculocerebral non-Hodgkin's lymphoma.

Aged↗

Ocular findings in Fryns syndrome.

PURPOSE: To demonstrate the ocular histopathologic findings in Fryns syndrome, a multiple congenital anomaly syndrome, with characteristic features including Dandy-Walker malformation, cleft palate, diaphragmatic hernia, lung hypoplasia, distal limb anomalies and polyhydramnios. The prevalence is about 0.7 per 10,000 births. Reported ocular features include microphthalmus, "cloudy cornea", irregularities of Bowman's layer, thickened posterior lens capsule and retinal dysplasia. METHODS: Case report. The ocular histopathologic and ultrastructural findings in a male fetus with Fryns syndrome who died immediately after his birth at 26th week of gestation are shown. RESULTS: An abnormal Descemet's membrane was found in addition to "cloudy corneae". Electron microscopy demonstrated absence of the banded collagen fibrils in Descemet's membrane, indicating corneal endothelial dysfunction. Otherwise, the eye was morphologically normal for its age; none of the other reported ocular features of Fryns syndrome were found. CONCLUSION: Corneal endothelial dysfunction might cause abnormal composition of anterior Descemet's membrane and could contribute to the "cloudy cornea" known to occur in Fryns syndrome.

Abnormalities, Multiple↗

[Eyelid tumor as a recurrence of a gastric MALT-lymphoma].

BACKGROUND: MALT Lymphoma of the conjunctiva, eyelid and orbit represents a primary manifestation of the disease in most of the cases. CASE REPORT: A 58-year-old male presented with a tumor in the left inferior eyelid. A full-thickness incisional biopsy was performed. Histopathologically, a lymphoid infiltrate involved the eyelid in its full width, with reactive follicles surrounded by small cells with irregular nucleus. Follicular colonization and lymphoepithelial lesions were detected. The tumor was CD20 positive. The molecular genetic analysis disclosed a monoclonal B-cell proliferation. The patient had a history of gastric MALT lymphoma treated 9 years ago and he had been otherwise in good health. The histopathologic features of the gastric tumor were identical to those of the eyelid lesion. Since the eyelid tumor represented a recurrence of the disease, treatment consisted of systemic chemotherapy. Complete tumor eradication was obtained. CONCLUSION: Ocular adnexal MALT-lymphomas may represent a recurrence of a distant primary MALT-lymphoma after a long disease-free period and, therefore, be an indication for systemic chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗

[Langerhans-cell histiocytosis of the orbit; diagnosis, treatment and outcome in three patients -- children and adults].

BACKGROUND: Langerhans cell histiocytosis usually affects children. Systemic Langerhans cell histiocytosis may be present in 37% of affected children. Ten per cent of all children die of the disease. CASE REPORTS: Patient 1. A 9-year-old boy presented with painful upper lid swelling OD. Ultrasonography revealed a space-occupying lesion adjacent to a lytic defect of the frontal bone as shown on coronal CT. MRI showed marked contrast enhancement of the mass which came close to the intracranial fossa. The vascularized tumor was excised using an anterior orbitotomy with upper lid crease incision. No evidence of local or systemic recurrence was present three years postoperatively. Patient 2. A 19-year-old male presented with foreign body sensation, double vision OS and numbness of the left face of six weeks' duration. The ocular findings revealed a sixth nerve palsy OS. Otherwise, the eyes were unremarkable. Sensitivity of cranial nerve V2 was decreased. CT revealed an osteolytic process in the lateral orbital wall and sphenoid wing. MRI demonstrated a space-occupying lesion involving the temporalis muscle laterally, the pterygopalatine fossa medially with intracranial extension into the temporal lobe. Surgical excision was performed using a transcranial approach. Signs and symptoms had completely resolved six months after surgery with no evidence of local or systemic recurrence. Patient 3. A 30-year-old male presented with right-sided headache, painful upper lid swelling and vertical diplopia. Coronal CT scan revealed a lytic defect in the frontal bone laterally. Curettage and complete excision were performed using an infrabrow incision. Follow-up examination four years later revealed an intact ocular motility with no evidence of local or systemic recurrence. In all three cases the tumor showed characteristic light-microscopy and immunohistochemical features of Langerhans cell histiocytosis. CONCLUSION: Langerhans cell histiocytosis of the orbit is an important differential diagnosis of osteolytic and/or space-occupying lesions not only in children, but also in adults. A diagnostic biopsy followed by surgical excision and/or local and systemic corticoid- and chemotherapy are essential regarding the prognosis of local and systemic disease.

Adolescent↗

Merkel cell carcinoma: clinicopathologic correlation, management, and follow-up in five patients.

PURPOSE: To review the clinicopathologic features, management and follow-up in five patients with periocular Merkel cell carcinoma (MCC). METHODS: In this case series study we reviewed the clinical records and histopathologic findings of five consecutive patients with MCC, treated and followed between May 1991 and November 1998. RESULTS: Four patients were female and one was male. Their mean age at the time of surgery was 80 years (range: 69-86 years). Patients presented with a painless, nonulcerated, rapidly growing, solitary reddish or violaceous nodule, ranging in size from 11 to 21 mm, located in the upper eyelid in 4 patients and in the left eyebrow in one patient. Management included excision with frozen section control in three patients, and excision with wide surgical margins in two patients. Histopathologically, all tumors exhibited round cells of intermediate size and scanty cytoplasm, and large, round to oval vesicular nuclei with finely dispersed chromatin and one to three inconspicuous nucleoli. Mitotic figures were numerous. No patient had regional or extraregional metastases at diagnosis. Local recurrence or distant metastases were not detectable during a mean follow-up period of 45 months (range: 32-61 months). This outcome contrasts with the high incidence of local recurrence and early nodal metastasis reported in previous publications. CONCLUSIONS: Early diagnosis and aggressive, histologically controlled surgical treatment of this rare, highly malignant tumor may provide a longer disease-free period. Further investigation is necessary to determine the prognostic factors for recurrence and survival.

Aged↗

Adenoma of the nonpigmented ciliary epithelium mimicking a malignant melanoma of the iris.

Adenomas of the nonpigmented ciliary epithelium are rare tumors and are difficult to differentiate from amelanotic malignant melanomas of the ciliary body. We describe a 62-year-old woman with a nonpigmented, vascularized iris tumor with small satellites involving the anterior chamber angle. Clinically, an amelanotic, tapioca, malignant melanoma of the iris was suggested although the blood-aqueous barrier was intact. A block excision of 7.5 mm, a tectonic corneoscleral graft, and a simultaneous extracapsular cataract extraction with implantation of a posterior chamber lens were performed. Histopathologic and electron microscopic studies revealed an adenoma of the nonpigmented ciliary epithelium. Postoperative visual acuity was 20/40 OD after 6 weeks. An adenoma of the nonpigmented ciliary epithelium may mimic an amelanotic malignant melanoma of the iris and should be considered in its differential diagnosis.

Adenoma↗

Corneal stromal calcification after topical steroid-phosphate therapy.

Secondary corneal calcification involving the full thickness of the stroma is a rare potential complication of severe dry eye conditions, recurrent corneal ulcerations, chronic ocular inflammation, or multiple surgical procedures. We describe on a patient with unusual, hitherto unreported calcareous degeneration of the corneal stroma after topical steroid-phosphate therapy for chronic keratoconjunctivitis after Stevens-Johnson syndrome. The patient's serum levels of calcium and phosphorus were normal. Histopathologic and electron microscopic examination of the corneal button revealed mainly intracellularly located crystalline calcium deposits throughout all layers of the corneal stroma but sparing the Bowman layer. Energy-dispersive x-ray analysis confirmed the presence of calcium phosphate. The calcium deposits were closely associated with intracellular and pericellular accumulations of glycosaminoglycans. Our findings indicate that corneal stromal calcification may develop after topical steroid-phosphate medication, and suggest a possible role of alterations in the glycosaminoglycan metabolism of stromal keratocytes in the calcification process.

Adult↗

Ocular findings in ichthyosis follicularis, atrichia, and photophobia syndrome.

Ichthyosis follicularis, atrichia, and photophobia (IFAP) are typical features of a rare neuroichthyosis termed IFAP syndrome. We demonstrate the ultrastructural findings of the eyes from a 33-year-old patient with IFAP syndrome. Clinically, eyebrows and eyelashes were absent from birth, and photophobia was noted at the age of 1 year. The globes measured 28 and 29 mm, respectively, and both eyes showed a posterior staphyloma. Histopathologically, bilateral centrally located subepithelial avascular corneal scarring with secondary corneal amyloid deposition was found. In addition to already described ocular abnormalities in IFAP syndrome we demonstrate ultrastructural anomalies of desmosomes and tonofilaments in corneal epithelium; defects of basement membrane, Bowman layer, and anchoring fibrils; secondary corneal amyloid deposition; and keratocyte degeneration. A defective tear film, recurrent atopic keratoconjunctival inflammations, or a primary anomaly of corneal epithelial adhesion are potential causes for the corneal defects. Photophobia is most likely due to corneal abnormalities.

Abnormalities, Multiple↗

[Congenital divided compound nevi of the eyelids].

BACKGROUND: Congenital divided nevi of the eyelids are a rare melanocytic lesion. Only 30 patients are reported in literature. We report on a 6-year-old boy and a 41-year-old male with this lesion and give a short overview of the literature. CASE REPORTS: Patient 1: A 6-year-old boy presented with a pigmented divided nevus of the left eye involving the upper and lower lid naselly. The lesion had increased in size in the last one year. Otherwise the results of ophthalmological examination were unremarkable. The divided nevus was completely excised. Histopathological studies revealed a melanocytic compound nevus. Patient 2: A 41-year old male presented in our department with a divided pigmented nevus on his left eye involving the upper and lower lid laterally. The lesion had gradually increased since birth. Otherwise the results of ophthalmological examination were unremarkable. A subtotoal excision and debulking procedure was performed. The defect in the lower eyelid was covered using a transposition flap from the upper eyelid. Histopathological examination revealed a melanocytic compound nevus. CONCLUSION: Divided nevi of the eyelids may be disfiguring and include the possibility of deprivation amblyopia when reaching a certain size in childhood. Malignant changes of this lesion were not observed so far. Total excision or subtotal excision and a debulking procedure may be helpful.

Adult↗

Infection of the corneal endothelium in herpes simplex keratitis.

PURPOSE: To describe viral infection of the corneal endothelium in a patient with recurrent herpes simplex virus keratitis in the corneal graft. METHODS: Case report. A healthy 28-year-old man presented with necrotizing stromal keratitis and corneal perforation in the corneal graft. A second penetrating keratoplasty was performed. The corneal button was processed for histopathologic, immunohistochemical, and electron microscopic studies. RESULTS: Histopathologically, the corneal endothelium showed viral inclusion bodies. Herpes simplex virus antigens and viral particles were identified in stromal keratocytes and corneal endothelial cells. CONCLUSION: Productive herpes simplex virus infection of the corneal endothelial cells may contribute to corneal graft failure in recurrent herpes simplex virus infections.

Adult↗

[Molluscum contagiosum in immunosuppression with methotrexate: multiple warts with central depressions of the eyelids].

BACKGROUND: Poxvirus induced molluscum contagiosum is known to occur in children, elderly people and in patients with the acquired immune deficiency syndrome AIDS. PATIENT AND METHODS: We report on a 49-year-old patient who presented with a three-month history of multiple skin-coloured prominent warts on his right upper and lower eye-lid. On examination, the warts showed a central cavity and measured 2-6 mm in diameter. For his "mixed connective tissue disease" (MCTD) the patient took methotrexate in a weekly dosage of 7.5 mg since 3 years. Diagnosis of MCTD was made 7 years before. The warts were excised and studied histopathologically. One year postoperatively, there was no recurrence of the lesions. CONCLUSION: Drug induced immunosuppression as with methotrexate may be associated with multiple periocular lesions due to molluscum contagiosum.

Biopsy↗

[Epithelial giant inclusion cyst 50 years after enucleation without orbital implant].

BACKGROUND: Conjunctival cyst formation following enucleation may occur in 3% to 7% of patients receiving orbital implants, especially secondary implants. We present a patient with a giant epithelial inclusion cyst of the anophthalmic orbit 50 years after enucleation without orbital implant. PATIENT: A 54-year-old male presented with increasing proptosis of the ocular prosthesis, shallowing of the inferior fornix, a palpable orbital mass, and difficulty in retaining the ocular prosthesis. At the age of 1 year enucleation of the right eye without orbital implant was performed following perforating ocular trauma. Results of examination of the other eye were unremarkable. Transillumination of the orbital mass was possible. The clinical diagnosis of an epithelial inclusion cyst was confirmed using ultrasonography and CT scan. An incision of conjunctiva and cyst wall and subsequent marsupialization were performed. Nine months postoperatively there was no evidence of recurrence of the cyst. The ocular prosthesis was well in place. CONCLUSION: Conjunctival cyst formation following enucleation may rarely occur in patients receiving orbital implants, especially secondary implants. The presentation of a conjunctival cyst formation following enucleation without orbital implant 50 years after surgery is noteworthy in comparison to implantation cysts which are known to occur more commonly within the first two years following secondary orbital implants.

Blast Injuries↗

[Surgical management of periocular basal cell carcinoma using frozen section control and immediate plastic reconstruction--indications and methods in 106 patients].

PURPOSE: To review our first experiences with the surgical removal of basal cell carcinoma (BCC) utilizing frozen section control and immediate plastic reconstruction. PATIENTS AND METHODS: We analyzed the accuracy of clinical diagnosis, methods of surgical excision, plastic repair and recurrence rates in 162 consecutive patients who underwent surgical excision utilizing either frozen section (n = 106) or biomicroscopic control (n = 56) between January 1991 and June 1996. Specimens used for intraoperative frozen-section monitoring were also fixed, processed, and sectioned for permanent sections and served as postoperative controls for the frozen sections (n = 43). RESULTS: The accuracy of clinical diagnosis was 86% (n = 189). In 106 patients with BCC excised with frozen section control, there were no recurrences reported after a mean follow-up of 2.9 years. The mean surgical defect measured 55% of total eyelid length (range 10%-100%). The incidence of morphea pattern was 34%. Intraoperative re-excisions due to frozen sections positive for tumor were necessary in 31% of cases. Permanent postoperative sections of the tissue that had been used for intraoperative frozen sections confirmed in 97.5% of patients (n = 43) the preliminary findings made with frozen sections. In 56 patients with BCC excised with biomicroscopic control, three tumors recurred (5%) after a mean follow-up of 4.4 years. The mean surgical defect measured 42% of total eyelid length (range 16%-100%). The incidence of morphea type was 23%. CONCLUSION: Surgical removal utilizing frozen section control and immediate plastic repair appears to represent a reliable and effective option in the management of selected patients with periocular BCC.

Adult↗

[Infra-saccular lacrimal duct stenosis caused by bone thickening of the inner nasal bones in tuberous sclerosis].

BACKGROUND: Patients with tuberous sclerosis (Morbus Bourneville-Pringle) may show hamartomatous tumors of different organ systems. 50% of patients display astrocytic hamartomas of the retina. The skeletal system is affected in 40%: peri-, en- or exostoses, or cysts may occur. To the best of our knowledge, there are only three descriptions of an involvement of the facial skeleton in the literature. PATIENT: A 54-year-old man presented with recurrent dacryocystitis and lacrimal duct obstruction. Tuberous sclerosis and epilepsy were present since childhood. Besides typical skin lesions and intracranial calcified astrocytomas a retinal astrocytoma was detected in his left eye. Coronal CT scan revealed endonasal bone thickening involving the nasal floor, conchae and lateral walls. Evidence of maxillary and ethmoidal sinusitis was also present. CONCLUSION: The endonasal findings in our patient are most likely a manifestation of tuberous sclerosis of the facial skeleton. They may have favored the development of marked sinusitis and lacrimal duct obstruction.

Dacryocystitis↗