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Susan Mary Stuart, DO; CDR Michael James Nowicki, MC, USN
From the Department of Pediatrics (Dr Stuart), and the Division of Pediatric Gastroenterology, Department of Pediatrics (Dr Nowicki), Naval Medical Center, Portsmouth, Va.

Arch Pediatr Adolesc Med. 1998;152:823-824.

A PREVIOUSLY healthy 5-year-old girl was referred for evaluation of "spiking" fevers (temperature to 41.1°C) that had occurred for 6 weeks. Fever aside, she was asymptomatic. There were no complaints of abdominal pain, rashes, weight loss, joint pain, or night sweats. There was no history of foreign travel.

On initial evaluation the results of the physical examination were normal except for a nontender 1x1x1-cm left axillary lymph node. The liver and spleen were not enlarged. Laboratory data included the following values: white blood cells, 8.8x10⁹/L, with 0.60 neutrophils, 0.29 lymphocytes; erythrocyte sedimentation, 84 mm/h; alkaline phosphatase, 168 U/L; alanine aminotransferase, 37 U/L; -glutamyltranspeptidase, 37 U/L; lactate dehydrogenase, 785 U/L. Findings were negative for heterophile antibody analysis; normal, urinalysis;negative, rheumatoid factor; negative, antinuclear antibody; negative, serology for cytomegalovirus; and normal, chest radiograph. Results of blood, urine, and throat cultures were negative for organisms.

On presentation to our hospital further testing was completed. Results of a purified protein derivative skin test were negative. Titers for human parvovirus, toxoplasmosis, and Borrelia burgdorferi were all negative. Three blood cultures obtained during fever spikes were negative for organisms. Denver panel findings (antinuclear antibody, anti–double-stranded DNA, anti–SS-A, anti–SS-B, antiscleroderma) were normal. Radioisotope bone scan findings were normal as were those from sinus radiographs. An abdominal computed tomographic scan (Figure 1, left and right) and ultrasound scans (Figure 2) were performed.

Figure 1.

Figure 2.

Denouement and Discussion: Cat-scratch Disease With Hepatic and Splenic Involvement

Figure 1. Top and bottom abdominal computed tomograms have multiple well-defined low-attenuation lesions of the liver parenchyma and a single low-attentuation lesion of the spleen.

Figure 2. Real-time ultrasound examination of the liver shows clearly defined hypoechoic liver lesions (arrows) that are not fluid containing.

Serum titers for Bartonella (formerly Rochalimaea) henselae were positive (IgM 1:60, IgG > 1:1024). A diagnosis of cat-scratch disease (CSD) with hepatic and splenic granulomas was made. The patient remained asymptomatic and subsequently the fevers subsided.

A well-recognized, self-limited cause of fever and regional adenopathy, CSD may present in a number of atypical fashions. Involvement of the spleen and liver have been reported increasingly. Margileth et al,¹ reported splenomegaly in 12% of patients in whom the diagnosis was made by skin testing. Splenic involvement may occur with, or independent of, hepatic involvement.^1-5 Hepatic involvement has been recognized since 1950.⁶ Initial reports identified hepatomegaly in association with a classic presentation of CSD,^7-8 in association with adenopathy,^2-3,9-14 and as the sole focus of involvement in fever of unknown origin.^4-5,13-16 Hepatomegaly may be identified on physical examination,^{5, 9-10,13} but in most cases, hepatic involvement was noted only on radiological studies.^{3-4,11, 13-16} Liver function test results are often normal^{4-5,11, 13, 15-16} or show mild elevation of aminotransferase levels.^{3, 9-10,14} Following early description of macroscopic hepatic lesions, the typical ultrasound features are multiple, round, hypoechoic lesions of varying size.^{9, 15-16} Computed tomographic features are multiple, scattered, well-circumscribed, low-attenuation lesions of variable size, which become isodense with surrounding hepatic tissue after intravenous contrast enhancement.^{7, 15-16} The differential diagnosis for these radiological findings includes other granulomatous disease, pyogenic abscesses, and neoplastic disease. In reported cases, hepatic tissue was obtained by biopsy because of concerns of possible malignancy or unrecognized infectious process. The diagnosis in most reported cases of CSD with hepatic involvement has been established by liver biopsy and/or skin testing with cat-scratch antigen.^{3-5,9-11,13, 15-16} Cat-scratch disease causes a granulomatous hepatitis with a histological appearance similar to the granulomas in lymph nodes of individuals with CSD. The typical histological appearance is epithelioid granulomas with central stellate necrosis and many polymorphonuclear lymphocytes.¹⁶ The presence of pleomorphic bacilli by Warthin-Starry silver stain, present in lymph nodes of patients with CSD, has been variable in hepatic lesions. Because of the invasive nature of liver biopsy and the low chance of identifying organisms this way, it is not optimal for diagnosing CSD. Skin testing with cat-scratch antigen is a good alternative diagnostic test and has been the basis for diagnosis in many previously reported cases with hepatic involvement.^{5, 9-11,13, 15} An unfavorable feature of this test is that it exposes the patient to potential infectious risks. Since identification of organism(s) causing CSD, safer methods of diagnosis are the polymerase chain reaction for the detection of B henselae DNA^{14, 17} and antibody testing to confirm the diagnosis of CSD.¹⁴ The use of clinical history, typical radiological findings, and antibody testing allow for a safe means of confirming the diagnosis.

AUTHOR INFORMATION
Accepted for publication April 3, 1998.

Reprints: Michael J. Nowicki, MD, Division of Gastroenterology, Department of Pediatrics, Naval Medical Center, 620 John Paul Jones Cir, Portsmouth, VA 23708.

REFERENCES
1. Margileth AM, Wear DJ, English CK. Systemic cat scratch disease: report of 23 patients with prolonged or recurrent severe bacterial infection. J Infect Dis. 1987;155:390-402. ISI | PUBMED 2. Cox F, Perlman S, Sathyanarayana A. Splenic abscesses in cat scratch disease: sonographic diagnosis and follow-up. J Clin Ultrasound. 1989;17:511-514. ISI | PUBMED 3. Delahoussaye PM, Osborne BM. Cat-scratch disease presenting as abnormal visceral granulomas. J Infec Dis. 1990;161:71-78. ISI | PUBMED 4. Lenoir AA, Storch GA, DeSchryver-Kecskemett K, et al. Granulomatous hepatitis associated with cat scratch disease. Lancet. 1988;2:1132-1136. 5. Malatack JJ, Altman HA, Nard JA, et al. Cat-scratch disease with adenopathy. J Pediatr. 1989;114:101-104. FULL TEXT | ISI | PUBMED 6. Inglis JA, Tonge JI. A disease with visceral granulomatous lesions of unknown etiology. Med J Aust. 1950;1:433-436. PUBMED 7. Kinlaw WB. Cat scratch disease: report of a case presenting with hepatosplenomegaly. Conn St Med J. 1954;18:960-962. 8. Korbitz BC. Systemic cat scratch disease with hepatosplenomegaly, multiple lymphadenopathy, and hepatic dysfunction. Rocky Mt Med J. 1973;70:23-27. 9. Rocco VK, Roman RJ, Eigenbrodt EH. Cat scratch disease: report of a case with hepatic lesions and a brief review of the literature. Gastroenterology. 1985;89:1400-1406. ISI | PUBMED 10. Greenbaum B, Nelson P, Marchildon M, Donaldson M. Hemolytic anemia and hepatosplenomegaly associated with cat-scratch fever. J Pediatr. 1986;108:428-430. FULL TEXT | ISI | PUBMED 11. Cohen-Abbo A, Cheatham S, Edwards K. Disseminated cat-scratch disease simulating neuroblastoma. Pediatr Infect Dis J. 1992;11:1056-1060. 12. Larsen CE, Patrick LE. Abdominal (liver, spleen) and bone manifestations of cat scratch disease. Pediatr Radiol. 1992;22:353-355. FULL TEXT | ISI | PUBMED 13. Malatack JJ, Jaffe R. Granulomatous hepatitis in three children due to cat-scratch disease without peripheral adenopathy: an unrecognized cause of fever of unknown origin. Am J Dis Child. 1993;147:949-953. FREE FULL TEXT 14. Dangman BC, Albanese BA, Kacica MA, Wallach MT. Children presenting with fever of unknown origin: imaging features and association with a new causative agent, Rochalimaea henselae. Pediatrics. 1995;95:767-771. FREE FULL TEXT 15. Rappaport DC, Cumming WA, Ros PR. Disseminated hepatic and splenic lesions in cat-scratch disease: Imaging features. AJR Am J Roentgenol. 1991;156:1227-1228. FREE FULL TEXT 16. Port J, Leonidas JC. Granulomatous hepatitis in cat-scratch disease: ultrasound and CT observations. Pediatr Radiol. 1991;21:598-599. FULL TEXT | ISI | PUBMED 17. Anderson B, Sims K, Regnery R, et al. Detection of Rochalimaea henselae DNA in specimens from cat scratch disease patients by PCR. J Clin Microbiol. 1994;32:942-948. FREE FULL TEXT

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