Magnetic resonance imaging of the orbit and optic pathways.
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Biomedical subjects
Publications and source records attributed to B G Haik.
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A seven-year-old child developed a febrile rash, migratory arthritis, and neurologic signs. Three days earlier, the child had been bitten by a tick. An eye examination two months later revealed bilateral papilledema, although the cerebrospinal fluid pressure was 170 mm. The IgM antibody titer for the Ixodes dammini spirochete was 1:256. I dammini is the tick vector for the Borrelia spirochete that causes Lyme disease. The papilledema receded with treatment over a month's time, and bilateral pigment epithelial mottling at the fovea was present after one year.
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Twenty-three patients with parameningeal (including orbital rhabdomyosarcoma (RMS)) were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and January 1983. Twenty were children with a mean age of 6 and 3 were adults. In 6 patients, the primary tumor was from the orbit, whereas the remaining 17 had other parameningeal primary sites. The tumors were in a very progressive local stage, with extensive destruction of the facial bones in 19 patients. Eight patients were treated with T2 chemotherapy protocol and 15 received T6. Seven patients received 5,000 to 7,200 rad delivered to the primary tumor in 11-16 weeks, 15 patients received between 4,500 to 5,000 rad in 4-7 weeks, and 1 patient received 3,000 rad in 3 weeks for residual microscopic disease following surgery. Two patients were treated with radiation to the whole brain; no patients received radiation of the whole central nervous axis (CNA). Fifteen of the 23 patients (65%) are alive and well with a medical follow-up time of 5 years. Two patients died of therapeutic complications and six died of tumor spread. In five patients, involvement of the central nervous system (CNS) was the cause of death. The prognosis of orbital RMS with parameningeal involvement is no better than in other tumors of parameningeal sites. In those patients who had impaired vision because of optic nerve damage prior to treatment, the vision did not improve following treatment. There was no impaired vision seen due to radiation damage of eye structures except in the lens.
Leukocoria is an important clinical sign in ophthalmology. Conditions producing this white pupillary reflex must be differentiated from retinoblastoma to insure appropriate and timely treatment. Auxiliary diagnostic testing has been helpful in securing a clinical diagnosis. A new diagnostic modality, magnetic resonance imaging, provides similar morphologic information with the additional potential for biochemical characterization. A series consisting of 14 patients presenting with leukocoria as a result of retinoblastoma and simulating conditions was examined. The magnetic resonance imaging findings are discussed.
Nonrhegmatogenous retinal detachments occur in the pediatric population secondary to other ocular disorders. The most important cause to identify is retinoblastoma. Computed tomography has proven to be the most reliable ancillary diagnostic test in this pediatric population. A series of eighty patients, presenting with nonrhegmatogenous retinal detachments, was examined. The role of computed tomography and characteristic clinical findings are discussed.
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A 14-year-old girl had headaches and sudden loss of vision as a result of total retinal detachment in association with the morning glory optic nerve anomaly. Metrizamide cisternography with contrast dye introduced into the subarachnoid space disclosed migration of metrizamide with radiographic enhancement in the subretinal space. The demonstration of an abnormal communication between the subarachnoid and subretinal spaces suggested that the subretinal fluid is of cranial origin. The patient underwent surgical removal of a window of dura from the optic nerve sheath. This resulted in retinal reattachment and improvement of visual acuity to 20/200. The retina was still attached after a 15-month follow-up period.
The morning glory anomaly is a rare congenital clinical entity that results from abnormal optic nerve development. In our series of 30 patients, followed for a mean duration of 10.3 years, 11 developed associated retinal detachments. These detachments followed a variable clinical course, including spontaneous reattachment and even redetachment, and may result from an abnormal communication between the subarachnoid space of the optic nerve and the subretinal space, allowing cerebrospinal fluid to accumulate subretinally. The clinical findings (including ultrasonography, computed tomography, and electrophysiologic testing), natural history, complications, and therapeutic indications are discussed.
Retinoblastoma (Rb) is an uncommon childhood tumor of the neural retina with a significant genetic component in its etiology. A small proportion of patients have a deletion in chromosome 13 encompassing band 13q14, an observation which permitted the assignment of the RB1 locus to this region. About 20% of Rb tumors exhibit microscopic deletions of band 13q14 or monosomy 13. Trisomy 1q and i(6p) have also been reported in a high percentage of tumors. We analyzed the chromosome complements from direct preparations of 10 Rb tumors derived from seven patients. Modal chromosome numbers ranged from 45 to 48, and occasional duplications of the genomes were noted. In general, the tumors were chromosomally stable, although karyotypic evolution and random chromosome loss were encountered. Consistent abnormalities included trisomy 1q, i(6p), 6q-, and del(13)(q12----14). One patient with bilateral Rb had three tumor clones (two in one eye and one in the other) with chromosome abnormalities unrelated in origin. A second patient with unilateral Rb had two tumor clones with chromosome abnormalities again unrelated in origin. These two patients provide some of the first cytogenetic evidence for the multifocal origin of primary Rb. In the untreated tumor of a third patient, a homogeneously staining region (HSR) was detected in 1p32, indicating gene amplication in vivo; previously, an HSR at this site has been reported in the established Rb cell line Y79.
Retinoblastoma (Rb) cells may be more sensitive to X-irradiation than normal cells when assayed for clonogenic survival, suggesting that such sensitivity can be used as a trait for detecting RB1 gene carriers. We studied induction of chromosome damage in lymphocytes from patients with Rb by the radiomimetic antineoplastic antibiotic, bleomycin (BL). Lymphocytes were treated in G2 for 4 h at four concentrations of the drug (0.0, 0.3, 0.9, and 2.7 micrograms/ml), and chromosome damage was evaluated. No difference in levels of spontaneous or induced damage was detected among hereditary Rb, non-hereditary Rb, and normal lymphocytes. The role of X-ray treatment in the induction of second tumors in Rb patients is discussed.
The clinical records of 101 patients (biopsies done on 48) with capillary hemangiomas of the orbit, orbit and lids, and lids were analyzed. Lesions predominated in females and typically appeared at birth or within the first two months of life. The superior orbit and lids were definite sites of predilection. Telltale periocular cutaneous involvement occurred in one third of the cases. Pure proptosis caused by a deep orbital tumor without an anterior component was found in seven cases. Proptosis in general was mild, but in two cases was severe enough to cause corneal exposure. Findings suprortive of a diagnosis of an orbital or periorbital hemangioma include (1) change in tumor size on crying (46%); (2) a dark vascular coloration to the subcutaneous tissue (80%); and (3) the existence of nonperiocular strawberry marks (25). Diagnostic tests of most value were plain radiography, ultrasonography, computed axial tomography, and arteriography. The complication rate in the 50 patients followed up over five years was 80% (60% with amblyopia). Present treatment modalities do not appear to be achieving the desired goals of a good cosmetic and functional outcome. Radiotherapy and corticosteroids are both effective in individual cases, but many lesions are resistant to all forms of present treatment. Amblyopia therapy is difficult and often unsuccessful in these patients.
Corneal lesions 7.5 mm. in diameter were made with an ocular trephine in rabbits. The time periods studied were 0, 30 min., and 1, 2, 4, 8, 16, and 24 hr. At the end of the time period, the cornea was flooded with 4% glutaraldehyde, buffered with cacodylate, pH 7.4, and kept moist until removed. It was then fixed for 24 hr. Half of the sample was dehydrated in graded alcohols, critical-point-dried, coated with gold palladium alloy, and viewed in an AMR-1000 scanning electron microscope at an accelerating voltage of 20 kv. From 0 to 4 hr. cell trauma, debris, and retraction are seen at the margin of the lesion. From 8 to 24 hr. a significant number of polymorphonuclear leukocytes are present over the total surface but in especially large numbers at the wound margin. At 16 to 24 hr. evidence of cell movement is present. Cells show ruffling membranes a decreased number of microvilli, and a few filopodia along the advancing edge.
The history of radiography and orbital imaging begins in 1895 with Wilhelm Roentgen's discovery of x-rays. Over the next three quarters of a century, radiographic pioneers like Dr. William Sweet, who developed the Sweet method, and Dr. George E. Pfahler, who made the first successful pictures of a brain tumor, helped to bring radiography into the 20th century. With each new radiologic innovation producing a forward surge followed by a period of refinement, new methods were invented and utilized to their diagnostic limits. But perhaps none of the radiologic innovations of this century--the Coolidge tube, the Potter-Bucky diaphragm, tomography and angiography-will have more impact than computed tomography and magnetic resonance imaging.
Capillary hemangiomas are the most common orbital tumors in children. They typically arise early in life, grow rapidly during a proliferative phase and then slowly regress in an involutional phase. The tumors may present as small isolated lesions of minimal clinical significance or as large disfiguring masses that can cause visual impairment and systemic symptomatology. Capillary hemangiomas are managed effectively by establishing a secure diagnosis, outlining the extent of the tumor, and understanding the natural history of the lesion, as well as its response to therapy. The ophthalmic and systemic manifestations of capillary hemangiomas are discussed in detail, as are the histopathology, radiologic findings, differential diagnosis, and therapeutic alternatives.
To investigate the advantage and limit of contrast enhancement in the examination of orbital mass lesions, precontrast T1- and T2-weighted, and postcontrast T1-weighted spin-echo images were retrospectively compared by two experienced observers. Using contrast material, intraocular tumors were well detected and characterized, and several orbital tumors were characterized as to whether cystic or necrotic and intracranial involvement was better evaluated. On the other hand, tumor-fat interface lost conspicuity when tumors showed enhancement. We conclude that gadopentetate-dimeglumine-administered T1-weighted images were helpful in detecting, differentiating and characterizing tumors of the orbit. However, the loss of contrast with fatty tissue is a disadvantage to be considered.