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Show J. elin. Ntwro-ophthaJmol. 4: 185-188, 1984 Rhabdomyosarcoma Causing Rapid Bilateral Visual Loss in Children LENORE A. BREEN, M.D. LANNING B. KLINE, M.D. WILLIAM M. HART, JR., M.D., Ph.D. RONALD M. BURDE, M.D. Abstract Rhabdomyosarcoma commonly involves the orbit resulting in unilateral proptosis, swelling.. and restricted ocular motility. We saw two unusual cases of children with nasopharyngeal rhabdo" myosarcoma which caused rapid bilateral visual loss due to simultaneous spread of the tumor to the orbital apices. Initial CT sans revealed the tumor, but were misinterpreted as "normal," causing a sig" nificant delay in diagnosis. We recommend high resolution CT scans of the nasopharynx, orbits, and suprasellar region in any child with rapid bilateral visual loss to rule out Ihis common childhood lumor. Rapid bilateral visual loss in a child is a rare clinical presentation. We present two cases of children with an initial diagnosis of ·optic neuritis" and "normar CT scans. Two months later the diagnosis of nasopharyngeal rhabdomyosarcoma was made in each case. Retrospective analysis of the scans in both cases revealed presence of the tumor on the initial study. Case Reports Case 1 A 2'year, 7-month-old boy presented with a 4day history of bilaterally decreasing vision progressing to blindness. In addition, the patient became lethargic, irritable, and anorexic. Two weeks prior to hospital admission, he had problems with constipation, rectal itching, and bowel incontinence. He was f;iven medication for From the Department of Neurology. University of Texas Health Science Center, Southwestern Medical School, Dallas, Texas (LAB); the Departments of Ophthalmology, Neurology, and Neurosurgery, University of Alabama School of Medicine, Binningham, Alabama (LBK); and the Departments of Oph. thalmology (WMH, RMB), Neurology, and Neurological Surgery (RMB). WaShington University School of Medicine, 51. Louis, Missouri September 1984 ·worms." Further history and review of systems were unremarkable. Physical examination revealed an alert, irritable boy with normal vital signs and with normal head circumference, weight, and height. General physical examination was remarkable for a widebased gait. Ophthalmologic examination revealed visual acuity of "light perception" in the right eye and "no light perception" in the left eye. Pupils were 5 mm in both eyes with an amaurotic pupil in the left eye. There was partial left abducens nerve weakness and absent optokinetic nystagmus. The optic discs appeared swollen, but oral fluorescein adminislration did not demonstrate visible leak· age of dye from disc vessels. Skull x-rays and a CT scan, with and without contrast, were reported as normal (Fig. 1). A lumbar puncture was done. Opening pressure was 130 mm H20. The cerebrospinal fluid glucose was 85 mg%, protein 51 mg%, and there were eight white blood cells/mm). Cerebrospinal fluid IgG/albumin ratio was 0.1, the IgG index was 0.71 (elevated), and oligodonal bands were negative. The patient was given 14 mg of intravenous methylprednisolone every 6 hours for 24 hours, then 2.0 mg of oral dexamethasone every 6 hours for 9 days, then 15 mg of oral prednisone twice a day on tapering doses. After no rl'Sponse to corticosteroid therapy occurrect, the patient was admitted for a metrizamide scan of the suprasellar region to look for a tumor. The study was interpreted as normal (Fig. 1). He was admitted again 1 month later with marked ptosis, ecchymotic lids, ophthalmoplegia, unimproved visual acuity, and paraplegia. CT scans of the head, abdomen, and myelography revealed tumor in the nasopharynx, orbits, left temporal fossa, presacral area, and epidural space at the ~ level. Biopsy of the tumor revealed a poorly differentiated malignancy with a pathological differential diagnosis of rhabdomyosarcoma, neuroblastoma, or histiocytic lymphoma. The child died a year later from sepsis after radiation therapy to the orbits and total 185 Rhabdomyosarcoma and Visual Loss ~ . Figure t. Case L Top left, initial CT scan without contTast reveals a soft tissue mass present in the orbital apices medial to the medial rectus muscle. Top right to bottom right, CT scans done on second admission. Top right, plain scan at the orbital level reveals moderate involvement of the orbits and nasopharynx by a soft tissue mass. Bottom left and righi, plain scans in suprasellar region without and with metrizamide are normal. neuraxis and chemotherapy with cis-platinum, vincristine, adriamycin, and cydosphamide. The final diagnosis on autopsy material from the meninges was alveolar rhabdomyosarcoma. Case 2 In November 1982, an 8-year-old girl was admitted to the hospital with a 3-week history of nausea, vomiting. and gradually worsening headaches. In addition, for 1 week prior to admission she complained of diplopia. General physical examination was unremarkable while neurologic testing demonstrated a left abducens nerve palsy. Cranial CT scanning done in early December 1982, was felt to be normal. Cerebrospinal fluid analysis revealed one lymphocyte/mm3 , protein of 23 mg/dl, and glucose of 67 mg/dl. In mid-December 1982, the patient was admitted to Childrens Hospital, Birmingham, Alabama. With the exception of a left abducens nerve palsy, physical examination was within normal limits. Cerebrospinal fluid examination demonstrated no cells, protein of 22 mg/dl, and glucose of 54 mg/ dl. Complete blood counl, serum chemistries, urinalysis and chest x-rays were within normal lim- "6 its. Westergren erythrocyte sedimentation rate was 8 mm/hour. Best-corrected visual acuity was 20/30 in the right eye and 3/100 in the left. Pupils were briskly reactive to light with a left afferent pupillary defect. No proptosis was present. Eye movement testing revealed a left abducens nerve palsy. Fundi were remarkable and spontaneous venous pulsations were seen bilaterally. Paltemreversal visual evoked testing was normal on the right. On the left, a response could only be obtained with flash stimulation, and the latency was prolonged (138.0 msec; normal = 112.36 ± 9.67 msec). Bilateral optic neuritis was suspected, and the patient was begun on 40 mg of prednisone daily. In early January 1983, the patient became listless and began losing weight. She developed a left oculomotor nerve palsy with pupillomotor involvement and lost all sight in the left eye. In addition, she rapidly lost vision on the right and developed a right abducens nerve palsy. At the time of admission 10 University of Alabama Hospital on January 10, 1983, best-corrected visual acuity was 2/400 on the right and ·no Iighl perception" on the left. The patient now had left oculomotor, trochlear, and abducens nerve pal- Journal of Clinical Neuro-ophthalmology Breen, Kline, Hart, Burde Figure 2. Case 2. CT ~ans with contrast reveals il large invasive tumor involving the orbital apices bilaterally (top. a:.ual view; bottom. coronal view). sies. The remainder of the neurologic examination was within normal limits. Skull films revealed a nasopharyngeal soft tissue mass with destruction of the sella turcica. Cranial CT scanning disclosed a large tumor at the anterior base of the skull invading the nasopharynx, sella, and parasellar regions (Fig. 2). On January 12, 1983, a nasopharyngeal biopsy was performed revealing embryonal rhabdomyosarcoma, The patient was treated with radiation therapy, 4,500 rads to the anterior base of the skull, and is presently receiving a chemotherapeutic regimen according to a randomized protocol for rhabdomyosarcoma patients. Discussion In a paper published in 1960, Kennedy and Carroll l reported a 25-year retrospective study of patients 15 years old and younger with optic neuritis. A total of 37 cases were found and reevaluated clinically. The optic neuritis was bilateral in 60% of the cases. Documented etiologies in this study and in other case reports include demyelinating diseases, malnutrition, diabetes, toxins, drugs, Leber's optic atrophy, and following viral infection or immunization.I -) The visual September 1984 loss often is less than 20/200, but usually begins recovering with proper treatment in the 3 weeks after onset. I. 2 The principal differential diagnosis in childhood. optic neuritis is increased intracranial pressure causing papilledema rather than papillitis, a tumor directly compressing the visual pathways, or functional visual loss. Our two cases have remarkable similarities. The bilaterality of the visual loss without signs of orbital disease or ophthalmoscopic changes led to the diagnosis of retrobulbar optic neuritis. In addition, the reportedly Mnormar CT scans, and in case 1 the elevated cerebrospinal fluid IgG, further aided the erroneous diagnosis. In retrospect the presence of abducens palsies in both children should have raised more questions. Rhabdomyosarcoma is the most l'ommon sarcoma in children and is third only to leukemia and neuroblastoma in overall tumor incidence.4.S The largest recent series of patients with rhabdomyosarcoma is the Intergroup Rhabdomyosarcoma Study group,S In the 780 patients reported, the leading primary site for rhabdomyosarcoma is head and neck, excluding the orbit (38%).5 The orbit is primarily involved in 10% of the cases.5The disease-free rate at the end of the 6 years is 51% for tumor arising in the head and 187 Rhabdomyosarcoma dnd Visual Loss neck and 77% in the orbit.tI Survival rates are 56% and 82%, respectively.tI Primary orbital rhabdomyosarcoma has a favorable prognosis due to the bony walls limiting spread and also the easy accessibility of the orbit for clinical examination leading to diagnosis at an early stage. Extraorbital sites in the head and neck, however, may be difficult to recognize, and the tumor may not be discovered until adjacent spread occurs with devastating consequences. In the Intergroup Rhabdomyosarcoma Study, of the 141 patients with primary head and neck tumors, 57 patients had tumor arising in a parameningeal location.? The meninges became involved in 20 of the 57 patients. All but two of these 20 patients died. The median time interval for meningeal extension to occur after diagnosis was 5 months, and the maximum time interval was 13 months. Therefore, diagnosis of parameningeal rhabdomyosarcoma at an early stage becomes extremely important. Clinically, orbital rhabdomyosarcoma will usually present with orbital swelling, proptosis, and in 25% of cases a palpable mass.8 Central visual loss, however, is an infrequent early or presenting symptom. In a clinical review article on sudden bilateral blindness in children, Duffner and Cohen9 state that although orbital tumors in children can lead to blindness, the onset of the visual loss is rarely acute or bilateral. Rhabdomyosarcoma was not initially suspected in our cases because the common signs of orbital rhabdomyosarcoma were not present on ophthalmic examination. The visual loss occurred due to compression of the optic nerves at the orbital apices by locally invading tumor. A retrospective analysis of the initial CT scans in both cases revealed the presence of a nasopharyngeal mass invading both orbits. A high suspicion of this possibility would have led us to an earlier diagnosis. Rapid, bilateral visual loss in children should alert the physician to suspect nasopharyngeal rhabdomyosarcoma and to perform high resolution, magnified CT scans of the orbits, nasophar+ ynx, and parasellar region. A delay in the diag- 188 nosis can significantly alter the child's morbidity and mortality. References 1. Kennedy, Co, and Carroll, F.D.: Optic neuritis in children. Arch. Ophtha/mol. 63: 747-755, 1960. 2. Meadows, S.P.: Retrobulbar and optic neuritis in childhood and adolescence. Trans. Ophtha/mol. Soc. U.K. 89: 603~638, 1969. 3. Carroll, F.D.: Symposium: Diseases of the optic nerve. TraIls. Am. Acad. Ophtha/mol. Otolaryngo/. 60: 74-82,1956. 4. Jaffe, N., Filler, R.M., Farber,S.. Traggis, D.G" Vawter, G.L Tefft, M., and Murray, J.E.: Rhabdomyosarcoma in children: Improved outlook with a multidisciplinary approach. Am. J. Surg. 125: 482487,1973, 5. Maurer, H.M.: The Intergroup Rhabdomyosarcoma Study: Update, November 1978. Natl. Cancer lust. MOllogr. 56: 61-68,1981. 6. Raney, RB., Donaldson, M.H., Sutow, W.W., lindberg, R.D., Maurer, H.M., and Tefft, M.: Special considerations related to primary sile in rhabdomyosarcoma: Experience of the Intergroup Rhabdomyosarcoma Study, 1972-1976. NaIl. Canctr II/st. MOIIQgr. 56: 69-74, 1981. 7. Tefft. M.. Fernandez, C, Donaldson, M., Newton, W" and Moon, T.E.: Incidence of meningeal involvement by rhabdomyosarcoma of the head and neck in children. A report of the Intergroup Rhabdomyosarcoma Study. Callcer 42: 253-258, 1978, 8. Weichselbaum, R.R., Cassady, l.R., Albert, D.M., and Gonder, J.R.: Mullimodality management of orbital rhabdomyosarcoma. bit. Ophtha/mol. Clin. 20(2): 247-259,1980. 9. Duffner, P.K., and Cohen, M.E.: Sudden bilateral blindness in children. Selected cases are presented and differential diagnosis is discussed. Clill. Pediatr. 17: 705-709, 1978. Acknowledgment This work was supported in part by a grant from Research to Prevent Blindness. Inc., New York, New York (Department of Ophthalmology, Washington University). Write for rcpriuts to: Ronald M. Burde, M.D., Department of Ophthalmology-Box 8096, 660 South Euclid Avenue, SI. Louis, Missouri 63110. Journal of Clinical Neuro-ophthalmology |
