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Show Journal of Neiiw- Ophllmlmoloay 18( 2): 99- 101, 1998. © 1998 Lippincott- Raven Publishers, Philadelphia Childhood Sarcoidosis Michael S. Vaphiades, D. o., and Eric Eggenberger, D. o. A 10- year- old black girl exhibited a bilateral lower motor neuron facial palsy and bilateral hearing loss. Gadolinium-enhanced magnetic resonance imaging of the brain showed enhancement of the cranial nerve VII- VIII complex bilaterally. Disc elevation developed in both eyes, and the patient exhibited facial swelling. Examination of a right parotid biopsy specimen demonstrated noncaseating granulomas, consistent with the diagnosis of sarcoidosis. Key words: Childhood sarcoidosis- Disc edema- Facial palsy. Sarcoidosis is an idiopathic, chronic, multisystem disease that has an overall incidence of 6 to 10 per 100,000. Although it is more prevalent worldwide in whites ( 79%), in the United States it is 6 to 10 times more common in blacks than in whites ( 1- 3). The peak incidence occurs in the adult population and rarely appears in childhood ( 4- 8). We present a case of biopsy- proven sarcoidosis in a 10- year- old girl and discuss childhood sarcoidosis. A review of the literature is also provided. CASE REPORT In October 1994, a 10- year- old black girl presented with a bilateral lower motor neuron facial palsy and bilateral decrease in hearing. Although the left facial palsy improved within 1 week, the palsy of the right face remained unchanged. Pediatric and otolaryngologic evaluation included audiograms which documented hearing loss in both ears. Magnetic resonance imaging ( MR1) of the brain demonstrated enhancement of the cranial nerve VII- V11I complex bilaterally without meningeal enhancement ( Fig. 1). Treatment was initiated with 20 mg prednisone three times a day for a presumed postviral syndrome. The patient continued on this dose for 1 Manuscript received December 5, 1996; accepted January 28, 1998. From ( he Department of Ophthalmology, University of Arkansas for Medical Sciences ( M. S. V.), Little Rock, Arkansas; and The Neuro- Visual Unit, Michigan Stale University ( F. E.), East Lansing, Michigan, U. S. A. Presented at the 28th annual Frank B. Walsh Society Meeting, 1996. Prepared from a case evaluated at Michigan State University. Address correspondence and reprint requests to Michael S. Vaphiades, D. O., Harvey & Bernice Jones Eye Institute, Department of Ophthalmology, University of Arkansas for Medical Sciences, 4301 West Markham, Mail Slot 523, Little Rock, AR 72205- 7199, U. S. A. month, during which her hearing improved. The prednisone was then abruptly discontinued. The patient subsequently experienced worsening of her hearing loss and facial diplegia, low- grade fevers, cephalalgia, and blurred vision. This prompted neuro- ophthalmologic consultation. She was the product of an uncomplicated term pregnancy, was born by spontaneous vaginal delivery, and had met developmental milestones as expected. Her medical history was otherwise notable for abdominal pain culminating in the diagnosis of cholelithiasis, which required cholecystectomy in June 1994. The neuro- ophthalmologic examination on December 20, 1994, revealed visual acuity of 20/ 25 OD and 20/ 30 OS, near vision of Jl in both eyes, and color vision of 10/ 10 in both eyes, using Hardy- Rand- Ritller pseudo-isochromatic plates. Goldmann perimetry showed enlarged blind spots in both eyes but was otherwise normal. Pupils were 6 mm in diameter with normal reactivity and no relative afferent defect. Motility findings were normal. The lower motor neuron facial palsy was more severe on the right than on the left ( Fig. 2). Slit lamp examination, trigeminal function, and exophthalmometry yielded normal findings. The optic discs appeared mildly FIG. 1. Pre- contrast ( upper) and postcontrast ( lower) coronal T1- weighted magnetic resonance image of the brain. The lower image displays bilateral enhancement of the cranial nerve VII- VIII complex. 99 700 M. S. VAPHIADES AND E. EGGENBERGER FIG. 2. External photograph of the patient after treatment with corticosteroids and atropine. Right more than left facial weakness is demonstrated. edematous. Besides the facial palsy, the neurologic findings were within normal limits. Cardiovascular, pulmonary, and dermatologic examinations were unremarkable. Investigation of the patient included a second MRI of the brain, which showed less enhancement of the cranial nerve VII- VIII complex bilaterally. Analysis of cerebrospinal fluid obtained with a lumbar puncture showed opening pressure of 140 mm H20; 0 red blood cells; 5 white blood cells; glucose, 66 mg/ dl; protein, 28 mg/ dl; and negative Cryptococcal antigen and cytology. Laboratory data: erythrocyte sedimentation rate, 130 mm/ h; white blood cells, 11.9 x 109 cells; hemoglobin, 10.1 g/ dl; hematocrit, 29.8%; normal white cell differential; angiotensin converting enzyme ( ACE), 4.8 nmol/ minute/ ml; normal findings in the urinalysis, Lyme test, and sickle cell screen; and normal levels of electrolytes, glucose, and antineutrophil cytoplasmic antibody. A chest radiograph was normal. At this point, prednisone was reinstituted and tapered more slowly, and the patient's hearing improved slightly during the next 2 months. Right lower facial fullness and sinusitis developed subsequently. A review of the pathologic specimen of the cholecystectomy did not reveal any granulomatous features. A right parotid biopsy was performed, and the pathologic examination revealed noncaseating granulomas and chronic inflammatory tissue consistent with sarcoidosis. With tapering doses of prednisone, the patient's facial palsy improved on the left, remained weakly present on the right, and the optic nerve edema resolved in both eyes. It was thought that the optic nerve edema represented papilledema caused by abrupt cessation of the prednisone versus an infiltrative process of both optic nerves. Optic nerve enhancement was never noted on MRI brain scan because there was no fat suppression of the orbits used. In late summer 1995, the patient experienced abdominal pain. A computerized axial tomographic scan of the abdomen and chest was obtained that showed abdominal and mediastinal lymphadenopathy. Analysis of biopsy specimen of the abnormal nodes showed noncaseating granuloma. In the fall of 1995, 1 year after the patient's initial presentation, uveitis developed with classic mutton fat keratic precipitates. The patient was treated with prednisone, tapered to 5 mg every other day, and was administered cycloplegic eye drops for the uveitis. By September 1996, her vision was stable. DISCUSSION Although sarcoidosis is rare in the pediatric population, reports highlight the differences between younger and older children ( 1,4- 6,8). The clinical manifestations of adult sarcoidosis differ from those in the pediatric age groups in the less than 4- year- old age range and in the 8 to 15- year- old age range. Among children who are less than 4 years old, a triad of maculopapular rash ( 81%), uveitis ( 77%), and arthritis ( 73%) predominates. An initial chest radiograph is abnormal in only 22% ( 1,8,9). These children must be differentiated from children who have juvenile rheumatoid arthritis ( 9). The typical patient in the 8 to 15- year- old age group with sarcoidosis is black ( 76%) and has initial symptoms of malaise, cough, and fever ( 5). Ocular lesions occur in TABLE 1. Signs and symptoms of sarcoidosis in different age groups < 4 years 8- 15 years Adults CXR Ocular symptoms Systemic symptoms Laboratory abnormalities Usually normal Uveitis Rash Arthritis Rare Abnormal ( 100%) Asymptomatic abnormal ( 36%) Uveitis, Vitritis, Choroidal granulomas Optic neuropathy, Keratic precipitates Retinal vasculitis Cystoid macular edema Rash Arthritis Splenomegaly and hepatomegaly Parotid enlargement CNS involvement Cardiac involvement Elevated serum ACE ( 80%) Hyperglobulinemia Hypercalcemia Eosinophilia Elevated ESR Elevated serum ACE ( 34- 88%) ./ Neitm- Ophllialmol, Vol. IS, No. 2, 1998 CHILDHOOD SARCOIDOSIS 101 24% of patients, manifesting most commonly as uveitis ( 5). Other reports have noted uveitis to be as high as 32%, along with other ophthalmologic involvement, similar to the incidence in the adult population ( Table 1) ( 4). The initial chest radiograph is abnormal in 100% of patients and usually displays hilar lymphadenopathy or, less commonly, a parenchymal infiltrate ( 5). Adults have pulmonary symptoms ( 20%) and ocular symptoms ( 20%) of uveitis, iritis, and keratic precipitates. Constitutional symptoms occur in 16% of adult patients, and 36% of adult patients are asymptomatic but have an abnormal chest radiograph ( 1,10). Serum ACE concentrations have proved useful in establishing the diagnosis of sarcoidosis, but levels differ in children and adults. They do not vary significantly in the normal pediatric to 15- year- old group, in which there is a mean serum ACE concentration of 46.7 ± 11.9 nmol/ minute/ ml ( 11). The normal adult serum ACE concentration is 32.1 ± 8.5 nmol/ minute/ ml ( Table 2) ( 11). Eighty percent of children have an increased ACE level at the time of diagnosis, whereas adults with sarcoidosis have increased ACE levels 34% to 88% of the time ( 11). Children with sarcoidosis have higher ACE concentrations than do adults with sarcoidosis ( 11). Other abnormalities in laboratory findings in adults include lymphocytopenia, mild eosinophilia, hyper-globulinemia, and an increased erythrocyte sedimentation rate. In older children, hypercalcemia, hyperpro-teinemia, and eosinophilia are the most common abnormalities detected in laboratory testing. Increased erythrocyte sedimentation rate has also been reported ( Table 1) ( 5,8). These abnormal findings are uncommon in younger children ( 8). Therapy consists of systemic corticosteroids. Low- dose methotrexate has been used in adults and recently has been shown to be of benefit in children ( 12). In summary, the diagnosis of sarcoidosis in a child can be challenging. A high percentage of children in the less than 4- year- old group have a rash and arthritis, whereas TABLE 2. Serum ACE activity in normal children and adults Normal ACE level ninol/ min/ ml Children ( 0- < 15 years) 46.7 + 11.9 Adults (> 18 years) 32.1+ 8.5 older children almost universally have an abnormal chest radiograph. Although histologic confirmation is required for diagnostic confidence, evaluation of possible childhood sarcoidosis is assisted by these guidelines and maintenance of a high level of clinical suspicion. REFERENCES 1. Seamone CD, Nozik RA. Sarcoidosis and the eye. Ophthalmol Clin North Am 1992; 5: 567- 76. 2. Bresnitz EA, Strom BL. Epidemiology of sarcoidosis. Epidemiol Rev 1983; 5: 124- 56. 3. Thomas PD, Hunninghake GW. Current concepts of the pathogenesis of sarcoidosis. Am Rev Respir Dis 1987; 135: 747- 60. 4. Hoover DL, Khan , IA, Giangiacomo J. Pediatric ocular sarcoidosis. Surv of Ophthalmol 1986; 30: 215- 28. 5. Kendig EL. The clinical picture of sarcoidosis in children. Pediatrics 1974; 54: 289- 92. 6. Fink CW, Cimaz R. Early onset sarcoidosis: Not a benign disease. J Rheumatol 1997; 24: 174- 7. 7. Jasper PL, Denny FW. Sarcoidosis in children../ Pediatr I968; 73: 499- 512. 8. Hetherington S. Sarcoidosis in young children. Am ./ Dis Child 1982; 136: 13- 5. 9. Rosenberg AM, Yee EH, MacKenzie JW. Arthritis in childhood sarcoidosis. J Rheumatol 1983; 10: 987- 90. 10. Obernauf CD, Shaw HE, Snydor CF, Klintworlh GK. Sarcoidosis and its ophthalmic manifestations. Am J Ophthalmol 1978; 86: 648- 55. 11. Rodriguez GE, Shin BC, Abcrnathy RS, Kendig EL. Serum an-giotensin- converting enzyme activity in normal children and those with sarcoidosis. J Pediatr 1981; 99: 68- 72. 12. Gedalia A, Molina JF, Ellis GS, Galen W, Moore C, Epinoza LR. Low dose methotrexate therapy for childhood sarcoidosis. J Pediatr 1997; 130: 25- 9. .1 Neuro- Ophlhalmol, Vol. IS, No. 2, 1998 |
