To the Editors:
Cystic echinococcosis (CE), caused by Echinococcus granulosus, causes a long-lasting infection that affects humans and a wide range of livestock species.1 In the human host, cysts can develop in many anatomic sites, especially liver and lung. Because of the slow cyst growth, diagnosis of CE in children with multiorgan involvement is rare, even in endemic countries; the treatment strategy in CE remains an open question.2
We describe a case observed in September 2003 of a 9-year-old boy, living in a farm in the south of Italy, who was admitted to our hospital because of persistent cough and expectoration of bloody sputum. Thoracoabdominal computed tomography scans showed a large cyst (4 cm diameter) in the mediastinum near the left ventricle, multiple cysts in the lung, a cyst in the liver, and a very large cyst (8 cm diameter) in the pancreas; echocardiography showed an intrapericardial cyst (2 cm diameter). Nuclear magnetic resonance confirmed the CE diagnosis. Laboratory tests showed eosinophilia of 17%, anti-Echinococcus antibody seropositivity (hemagglutination test: antibody titer: 1:16,384), immunoblotting: antibodies against 8, 16, 20 kd (antigen B), and 55 to 65 kd (antigen 5) antigenic subunits.
Treatment was started with albendazole (10 mg/kg/d). One month after the start of anthelminthic therapy, an echocardiography showed a volumetric increase of the pericardial cyst (3 cm diameter). After 45 days of chemotherapy, complete surgical resection of heart cyst and concomitant cleaning of liver and lung cysts became necessary. The patient underwent elective operation in 1-stage surgery, through a median sternotomy incision and next xiphoumbilical incision. The cyst mass was seen clearly on the wall of the left atrium and completely dissected away. Lung cysts of left superior and right inferior lobes and the liver cyst of left lobe were removed. Finally, the peritoneum was raised and the cyst located in the pancreas was completely removed. Histologic examination of the surgical material confirmed the diagnosis of CE. The patient was extubated on second postoperative day and 1 week later discharged home with continuous albendazole therapy for 2 years (10 mg/kg/d). The postoperative period was uneventful.
Four years' clinical, serologic, and ultrasonographic follow-up has shown no recurrence. To note, although, enzyme-linked immunosorbent assays determining isotype antibody expression in response to E. granulosus hydatid fluid, showed no significant variations for total immunoglobulin (Ig) G and IgG1 concentrations, IgG4 and IgE concentrations dropped at completion of follow-up (IgG4:O.D.: 1.22 vs. 0.64; IgE:O.D.:0.18 vs. 0.008).
CE is endemic in Italy, in particular in the south of the country. Because the cyst requires many years to develop, the diagnosis of pediatric echinococcosis is rare, and the multiorgan localization in child is uncommon.3,4 In particular, cardiac and pancreatic involvements, which occur only after the larvae pass trough the barrier of liver and lung, are rare in children (0.2%–2%). In a Turkish study, a 13-year-old girl presented hydatid cyst of the right atrium, cyst in the kidney, and multiple cyst in the lungs.5 This is the first report of a child with hydatid with a cyst in the hearth, pancreas, lung, and liver. Although operating on 4 distinct organs is a high-risk procedure, we decided for a 1-stage surgery because we did not want to risk spreading the infection and the fatal complication from the growth and/or rupture of the cardiac cyst.
This noteworthy case of a young patient with multiorgan involvement shows that combined chemotherapy and surgery allow to successfully treating severe CE.
Maria Iannelli, MD
Department of Pediatric Medicine (DMP)
Pediatric Hospital Bambino Gesù, IRCCS
Alessandro Inserra, MD Roberto M. Di Donato, MD
Pediatric Hospital Bambino Gesù, IRCCS
Antonella Teggi, MD
Department of Infectious Diseases
Hospital Sant'Andrea
University of Rome, “Sapienza”
Alessandra Siracusano, PhD
Department of Infectious Parasitic and
Immune-mediated Diseases
Istituto Superiore di Sanità
Saverio Malena, MD
Pediatric Hospital Bambino Gesù, IRCCS
Renata Boldrini, MD
Pathology Unit-Laboratory Department (DL)
Pediatric Hospital Bambino Gesù, IRCCS
Cristina Russo, MD
Pediatric Hospital Bambino Gesù, IRCCS
Andrea de Zorzi, MD
Department of Pediatric Cardiology and Cardiac Surgery (DMCCP)
Pediatric Hospital Bambino Gesù, IRCCS
Alberto Villani, MD, PhD
Department of Pediatric Medicine (DMP)
Pediatric Hospital Bambino Gesù, IRCCS
Rome, Italy
REFERENCES
1. Eckert J, Deplazes P. Biological, epidemiological, and clinical aspects of echinococcosis, a zoonosis of increasing concern.
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2. Vuitton DA, Wen H. Treatment of cystic echinococcosis: a combination of general goals and rules, individual decisions and indications.
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3. Emilio C, Losurdo G, Mollero L, et al. Multiorgan echinococcosis in a pediatric patient.
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4. Olmez D, Babayigit A, Arslan H, et al. Multiorgan involvement in a pediatric patient with hydatid disease.
J Trop Pediatr. 2008;54:417–419.
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J Thorac Cardiovasc Surg. 2001;121:1009–1011.
