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case report Disseminated peritoneal japonicum: a case report and review of the pathological manifestations of the helminth Salah Al-Waheeb,a Maryam Al-Murshed,a Fareeda Dashti,b Parsotam R. Hira,c Lamia Al-Sarrafd

From the aDepartments of Histopathology, and bSurgery, Mubarak Al-Kabeer Hospital, cDepartment of Microbiology, Kuwait University, dDepart-m ment of Radiology, Mubarak Al-Kabeer Hospital, Jabriyah, Kuwait

Correspondence: Salah Al-Waheeb, MD · Mubarak Al-Kabeer Hospital, PO Box 72, Code 71661, Jabriyah, Shamiyah City, Kuwait · T: +975-531- 2700 ext. 2188 · [email protected] · Approved for publication August 2008

Ann Saudi Med 2009; 29(2): 149-152

Schistosomiasis (also known as bilharzia, bilharziasis, bilharziosis or fever) is a human disease synd- drome caused by from one of several species of parasitic trematodes of the genus Schistosoma. The three main species infecting humans are S haematobium, S japonicum, and S mansoni. S japonicum is most common in the far east, mostly in China and the Philippines. We present an unusual case of S japonicum in a 32-year-old Filipino woman who had schistosomal ova studding the peritoneal cavity and forming a mass in the right iliac fossa.

chistosomiasis (also known as bilharzia, bilharziaa liver (Figure 1). CT examination showed multiple calac asis, bilharziosis or snail fever) is a human disease cific foci throughout the abdomen, particularly in the Ssyndrome caused by infection from one of several RIF. Prominent small bowel dilatation and fluid collecat species of parasitic trematodes of the genus Schistosoma. tion in the pouch of Douglas were also noted (Figure Approximately 200 million persons are infected with 2). The liver appeared surprisingly normal on the CT schistosomes worldwide.1 Most human study. Diagnostic laparoscopy was immediately conav is caused by S haematobium, S mansoni, or S japonicum. verted to an open laparotomy once a RIF mass with Less prevalent species such as S mekongi and S intercc pus involving the appendix was seen. Dilated small calatum may also cause systemic human disease. Less bowel loops were present in the RIF and next to the importantly, other schistosomes with avian or mamma spleen. The mass was dissected and released and the malian primary hosts can cause severe dermatitis in huma appendix was removed, after which the small intestian mans (e.g. swimmer’s itch secondary to Trichobilharzia nal obstruction subsided. Intraoperatively, multiple ocellata). The parasite S japonicum is found in the Far whitish yellow peritoneal nodules were noted coverai East, particularly China and the Philippines.1,2 The disae ing the abdominal peritoneal surface and the surface ease manifestations caused by S. japonicum in humans of the liver. The liver, however, did not appear to be have been extensively covered in the literature and will cirrhotic. On gross examination, the appendix was seav be reviewed in this paper. We present an unusual case verely congested, measured 6×2×1 cm and showed an of S japonicum where the patient had schistosomal ova area of perforation at the body. The adherent omentum studding the peritoneal cavity and forming a mass in the had focal areas of pus, and in other areas, firm yellow right iliac fossa (RIF). white nodules were present. The microscopic sections showed the appendiceal wall studded with schistoas Case somal ova (Figure 3). Furthermore polymorphonuclear A 32-year-old Filipino woman presented with a 5-day leukocytes were also seen in the wall along with areas history of generalized abdominal pain and a 3-day hista of suppurative inflammation and necrosis. Sections tory of nausea and vomiting. The pain settled in the from the adherent omental mass also showed suppuar RIF. She was admitted with a WBC count of 11×109/ rative inflammation and multiple foci of shistosomal L and a temperature of 38.5°C. Ultrasound studies of ova. The ova were oval to round in shape and showed the abdomen showed heterogeneous echogenicity of the outpouchings highly indicative of S japonicum species

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case report

Figure 1. Ultrasound of the liver shows heterogenous Figure 4. Schistosoma Japonicum ova with characteristic lateral echogenicity. outpouchings (hematoxylin and eosin stain ×200).

(Figure 4). No granulomatous response was present in the sections examined. Stains for bacterial, fungal and acid fast organisms were all negative. The histopathoal logic diagnosis was acute suppurative appendicitis and peritonitis with disseminated schistosomal ova involvai ing the appendix and omentum.

Discussion From a clinical perspective S japonicum usually causes an acute serum sickness also called ‘Katayama’ fever,3 which is associated with the onset of the female parasite laying eggs (approximately 5 weeks after infection) and Figure 2. CT scan shows multiple dilated small bowel loops with granuloma formation around eggs trapped in the liver multiple calcific peritoneal deposits. and intestinal wall. It manifests with hepatosplenomegaa aly and leucocytosis with . This phase of the infection is often asymptomatic, but when symptoms do occur they include fever, nausea, headache, an irritating cough and, in extreme cases, diarrhea accompanied with blood, mucus and necrotic material. These symptoms, if present, last from a few weeks, to several months and are not as commonly associated with S hematobium or S mansoni compared with those of S japonicum. The chronic phase of infection, however, is the more impa portant part of the infection.3 From a pathological peras spective, common morphological manifestations of the chronic phase are intestinal and hepatic schistosomiaas sis. Both manifest a number of years after infection. A large autopsy study involving 349 cases showed that the dominant pathology is evident in the liver.4 The pathoag genic reaction is a cellular, granulomatous inflammation Figure 3. Appendiceal wall studded with schistosomal ova around eggs trapped in the tissues, with subsequent fiba (hematoxylin and eosin stain ×200). brosis. Specific liver pathology was destruction of limitai ing plates, reparative hepatic lesions such as regenerata tion of the collapsed parenchyma, newly formed limitia ing plates and subsequent narrowing and disappearance

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schistosoma japonicum case report

of fibrous septa. In more chronic lesions, fibrosis and Kurniawan et al studied 52 S japonicum-infected pata cirrhosis are seen. Complications of liver cirrhosis and tients from an endemic area in Indonesia.11 All of these hepatocellular carcinoma related to viral hepatitis B patients exhibited signs and symptoms of chronic hepaat and/or C were also increased. Clonorchiasis was also to-splenic schistosomiasis. None of the patients showed consistently found.4 All areas of both the small and evidence of liver cirrhosis on histopathological examina large intestine may be involved, with the large intestine nation. However, varying degrees of portal fibrosis were showing the most severe lesions, whereas severe patholao exhibited and the authors concluded that liver biopsy ogy in the small intestine is only rarely observed, even has proved to be a valuable method of diagnosis in this though a large number of eggs may be deposited there.5 particular type of infection. S japonicum resides in the The theory is that the adult worms have a predilection mesenteric veins which drain to the liver, and therefore for inhabiting the branches of the inferior mesenteric causes liver fibrosis after depositing in that organ.12 A vein and superior haemorrhoidal vein and their eggs are study of the ultrasound and CT scan findings of paat deposited in much higher density in the large intestine, tients with biopsy-proven hepatic S japonicum revealed especially in the rectum, sigmoid and descending colon, characteristic findings.13 They observed a “network patat than in the small intestine.5 Gastrointestinal findings tern of calcification” on ultrasound and a “turtle-back” may include polyp formation. The friable vascular naat pattern on contrast CT scan. Laparoscopic findings of ture of these inflammatory polyps may lead to lower nine patients with chronic S japonicum were analyzed gastrointestinal bleeding.6 Large polyps, or bilharzioam and compared with histological findings from the same mas, may cause intestinal obstruction, intussusception, patients.14 In all nine patients laparoscopy revealed yelal or be confused with a malignancy.7 Colonic polyps are lowish, small speckles clustered over the liver surface, of higher prevalence in Egypt for reasons that are not which were later found to be the subcapsular calcified clear.8 Other cited gastrointestinal findings may include ova of S japonicum. While the liver surface was almost strictures, fistulae and bowel perforation.7,9 S japonicum smooth in mild schistosomiasis, multiple whitish markia eggs may also be deposited in the appendix and may ings and irregular, relatively wide, groove-like septums manifest as appendicitis. Schistosomal appendicitis acaq were seen in more advanced cases. In severe schistosoma quired in a traveler has been described.10 miasis, block-like formations of variable size, separated Liver-related and gastrointestinal complications by groove-like depressions, made the liver surface appa can be prevented with early recognition and therapy of pear like a tortoise shell on the CT scan. Other organs schistosomiasis. In a radiologic study of 12 patients that may rarely contain granulomas around eggs, like the underwent CT of the abdomen to detect what proved breast15 and fallopian tube.16 to be S japonicum by pathologic examination, the CT What is unusual and novel about our case from a demonstrated curvilinear or nodular calcification in the morphologic perspective is that there has not been a colon in 11 patients, in the appendix in 2, and in the reported case in the English language literature of S jacp distal ileum in 1 patient. Pathologic examination of the ponicum disseminating and covering the peritoneum in specimens showed calcified eggs of S japonicum deposia this. The patient was lost to follow-up and no colonosca ited more extensively in the submucosa and subserosa copy was done to rule out any colonic pathology which than in the muscularis propria, which led to the curvila may have been present in the patient. Similarly, the liver linear appearance.6 The pathology in the liver is similar might have been involved with S japonicum ova, as suspa to that seen in the intestine, with a cellular, granuloam picious nodular lesions were seen studding the surface matous inflammation around eggs trapped in the liver, of the liver intraoperatively, but no liver biopsy was peraf leading to fibrosis and hepatosplenic disease and subseaq formed. The liver enzymes were normal in the patient quent cirrhosis in chronic disease. Studies on the effect and no overt liver cirrhosis was present. The other unua of S japonicum and the liver have been recorded since usual feature was the lack of a granulomatous reaction the early 1970s from endemic areas. in the tissue examined in our laboratory.

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case report schistosoma japonicum

References 1. WHO Expert Committee. The control of tion. Radiol. 1994 ov;193(2):539-42. east Asian J Trop Med Public Health. 1976 schistosomiasis. Report of the WHO Expert 7. Cheung H, Lai YM, Loke TK, Lee KC, Ho WC, Jun;7(2):263-9. committee. World Health Organ Tech Rep Ser. Choi CH, Metreweli C. The imaging diagnosis of 12. Nash TE, Cheever AW, Ottesen EA, Cook JA. 1985;728:1e113. hepatic Schistosomiasis japonicum sequelae. Schistosome infections in humans: perspectives 2. Gryseels B, Polman K, Clerinx J, Kerstens L. Hu-m Clin Radiol. 1996 Jan;51(1):51-5. and recent findings. NIH conference. Vet Parasi-t man schistosomiasis. Lancet. 2006;368:1106e18. 8. Cheever AW, Kamel IA, Elwi AM, Mosimann JE, tol. 1984 Dec;17(1):47-64. 3. Ishii A, Tsuji M, Tada I. History of Katayama Danner R, Sippel JE. and S. 13. Cheung H, Lai YM, Loke TK, Lee KC, Ho WC, disease: Schistosomiasis japonica in Katayama haematobium infections in Egypt. III. Extrahepatic Choi CH, Metreweli C. The imaging diagnosis of district, Hiroshima, Japan. Parasitol Int. 2003 pathology. Am J Trop Med Hyg. 1978 Jan;27(1 Pt hepatic Schistosomiasis japonicum sequelae. Dec;52(4):313-9. 1):55-75. Clin Radiol. 1996 Jan;51(1):51-5. 4. Nakashima T, Kage M, Hirata M. A historical 9. Hayashi S, Ohtake H, Koike M. Laparoscopic di-a 14. Hayashi S, Ohtake H, Koike M. Laparo-s view of Schistosomiasis japonica in the Chikugo agnosis and clinical course of chronic Schistosom- scopic diagnosis and clinical course of chronic river basin. What can we learn from autopsy? miasis japonica. Acta Trop. 2000 Oct 23;77(1):133- Schistosomiasis japonica. Acta Trop. 2000 Oct Parasitol Int. 2003 Dec;52(4):327-34. 40. 23;77(1):133-40. 5. Chen MG. Relative distribution of Schisto-s 10. Weber G, Borer A, Zirkin HJ, Riesenberg K, 15. Varin CR, Eisenberg BL, Ladd WA. Mammo-g soma japonicum eggs in the intestine of man: Alkan M. Schistosomiasis presenting as acute graphic microcalcifications associated with schist- a subject of inconsistency. Acta Tropica. 1991 appendicitis in a traveler. J Travel Med. 1998 tosomiasis. South Med J. 1989 Aug;82(8):1060-1. Jan;48(3):163-71. Sep;5(3):147-8. 16. Beadles W, Wilks D, Monaghan H. Fallopian 6. Lee RC, Chiang JH, Chou YH, Rubesin SE, Wu 11. Kurniawan AN, Hardjawidjaja L, Clark RT. A tube carcinoma associated with schistosomia-s HP, Jeng WC, Hsu CC, Tiu CM, Chang T. Intestinal clinico-pathologic study of cases with Schisto-s sis. J Infect. 2007 Nov;55(5):e121-3. Epub 2007 Schistosomiasis japonica: CT-pathologic correla-t soma japonicum infection in Indonesia. Southe- Sep 10.

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