Int J Colorectal Dis DOI 10.1007/s00384-015-2156-3
LETTER TO THE EDITOR
Schistosomal appendicitis with portal hypertension Ramesh Wijaya & Jia Hui Ng & Shoun Tan & Andrew Sy Wong
Accepted: 8 February 2015 # Springer-Verlag Berlin Heidelberg 2015
Dear Editor: Schistosomiasis is a waterborne acute and chronic parasitic disease resulting from infection by species of the trematode genus Schistosoma. Species of Schistosoma are prevalent in tropical and sub-tropical areas where poor and rural communities lack access to clean water. For instance, Schistosoma mansoni and Schistosoma haematobium is common in Africa and the Middle East, while Schistosoma japonicum is common in parts of China, Indonesia, and the Philippines. Although transmission is reported from 78 countries, it is rare in developed countries [1]. Schistosomiasis manifests itself mainly in the intestinal and urogenital system of the infected human body [1, 2]. In particular, acute appendicitis is a known but rare manifestation of intestinal schistosomiasis [2]. We report a case of appendiceal schistosomiasis in a 46-year-old Filipino foreign worker who presented in Singapore with clinical signs and symptoms of acute appendicitis as well as radiologic evidence of portal hypertension. The patient is a 46-year-old Filipino woman who works in Singapore. She presented with a day’s history of right iliac fossa pain. The pain was constant and associated with nonbloody, non-bilious vomiting. It was not associated with preceding fever or change of bowel habits. She did not have R. Wijaya (*) : A. S. Wong Department of General Surgery, Changi General Hospital, 2 Simei Street 3, Singapore 529889, Singapore e-mail: [email protected] J. H. Ng Department of Otolaryngology, Singapore General Hospital, Singapore, Singapore S. Tan Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore
pyrexia. On examination of the abdomen, there was right iliac fossa tenderness and also rebound tenderness with voluntary guarding. Digital rectal examination was unremarkable. Her full blood count showed only thrombocytopenia (129 × 103 μL), with normal total white, neutrophil, and eosinophil counts. Computed tomography (CT) scan of her abdomen and pelvis showed a slightly thickened appendiceal wall with wall enhancement of the appendix and minimal peri-appendiceal fat stranding, suggestive of early appendicitis. Cavernous transformation of the liver with chronic thrombosis in the hilar and right portal vein was evident, resulting in a lobulated liver with an atrophic right hepatic lobe with early evidence of portal hypertension represented by a tortuous splenic vein and splenomegaly. In view of the above findings, she underwent an open appendectomy for likely acute appendicitis on the same day as her CT scan. Intraoperative examination revealed a minimally inflamed and thickened appendix, measuring 5 cm in length. There was minimal clear peritoneal fluid noted. Postoperatively, the patient recovered uneventfully and was discharged 3 days later. Histological examination of the resected appendix revealed an infiltrate of neutrophils and eosinophils extending to the serosal surface from the ulcerated mucosa, consistent with acute appendicitis. There was no evidence of malignancy. Of note, there were multiple ova of a parasite in the lamina propria of the resected appendix, some of which exhibited granulomatous change with surrounding fibrosis. The most likely causative organism was identified to be S. japonicum. The patient was referred to an infectious disease specialist and was treated with praziquantel (60 mL/kg in three doses). Stool examination did not show any evidence of ova, cysts, or parasites. No schistosomal eggs were seen on urine examination. An ultrasound of the hepato-biliary system 2 months later showed coarse underlying echogenicity of the liver as well as prominence of the portal vein wall.
Int J Colorectal Dis
The patient was subsequently referred to a hepatogastroenterology specialist and investigated for the underlying etiology of her liver cirrhosis and portal vein thrombosis. Further history taking revealed no intake of hepatotoxic substances such as chronic ethanol intake. Hepatitis screen, autoimmune markers, metabolic screen, and prothrombotic screen were all unremarkable. Hepatic schistosomiasis was therefore the most likely etiology for her liver cirrhosis and portal vein thrombosis. Four years later, her liver function tests remain normal. Intestinal schistosomiasis is the result of schistosomal ova deposited in the gut wall. Host reaction to the eggs causes granulomatous inflammation and fibrosis, which is responsible for most of the manifestations of chronic schistosomiasis [1]. Polyposis secondary to progressive inflammation, fibrosis, and mucosal hypertrophy is the commonest intestinal manifestation [1, 2]. Schistosomal appendicitis is a rare manifestation of intestinal schistosomiasis even in endemic areas. It is reported to occur in 0.02–6.3 % of infected individuals, but represents up to 28.6 % of chronic appendicitis in these areas [2]. Hepatic schistosomiasis causes portal fibrosis which when severe, is termed Bpipe stem fibrosis^ for the appearance of the greatly enlarged fibrotic portal tracts. Portal hypertension, hepatosplenomegaly, and the development of esophageal varices develop as a consequence. There are marked differences from hepatic cirrhosis. There is no hepatocellular failure, normal liver lobular architecture is preserved, and nodular regenerative hyperplasia is not seen. Therefore, with early treatment, the fibrosis is reversible [1, 2]. As observed in the patient presented above, there is usually preservation of liver function until much later stage of disease [3]. It has been reported that ultrasound examination of subjects with hepatosplenic schistosomiasis yields different findings from
that in liver cirrhosis. Characteristically, there is echogenic thickening of the walls of the portal vein and its branches, indicative of periportal fibrosis, and echogenic enlargement of the gallbladder wall [3]. Again, on CT and MRI scans, periportal fibrosis is the most characteristic feature [3]. Prompt and effective treatment of infection is key to prevention of further damage and complications from portal hypertension. Especially in infection with S. japonicum, there may be rapid progression with little or no interval between acute and chronic hepatosplenic disease [4]. To this end, presumptive treatment with praziquantel is undertaken in endemic areas in patients with suggestive symptoms and signs even when definitive diagnosis has not been made. It is in developed countries where this disease is rare that diagnosis and treatment may be more easily overlooked. This report of a rare case of schistosomiasis in a first world country presenting clinically as acute appendicitis emphasizes the importance of vigilance against this disease even in first world countries, especially in light of the growth in immigration and global travel today. Effective treatment with praziquantel can prevent the severe multisystemic complications that arise in untreated late stage disease.
References 1. WHO: Schistosomiasis. Updated February 2014. http://www.who.int/ mediacentre/factsheets/fs115/en/. Accessed 6th June 2014 2. Elbaz T, Esmat G (2013) Hepatic and intestinal schistosomiasis: review. J Adv Res 4(5):445–452 3. Lambertucci JR (2014) Revisiting the concept of hepatosplenic schistosomiasis and its challenges using traditional and new tools. Rev Soc Bras Med Trop 47(2):130–136 4. Gryseels B, Polman K, Clerinx J, Kestens L (2006) Human schistosomiasis. Lancet 368(9541):1106–1118
LETTER TO THE EDITOR
Schistosomal appendicitis with portal hypertension Ramesh Wijaya & Jia Hui Ng & Shoun Tan & Andrew Sy Wong
Accepted: 8 February 2015 # Springer-Verlag Berlin Heidelberg 2015
Dear Editor: Schistosomiasis is a waterborne acute and chronic parasitic disease resulting from infection by species of the trematode genus Schistosoma. Species of Schistosoma are prevalent in tropical and sub-tropical areas where poor and rural communities lack access to clean water. For instance, Schistosoma mansoni and Schistosoma haematobium is common in Africa and the Middle East, while Schistosoma japonicum is common in parts of China, Indonesia, and the Philippines. Although transmission is reported from 78 countries, it is rare in developed countries [1]. Schistosomiasis manifests itself mainly in the intestinal and urogenital system of the infected human body [1, 2]. In particular, acute appendicitis is a known but rare manifestation of intestinal schistosomiasis [2]. We report a case of appendiceal schistosomiasis in a 46-year-old Filipino foreign worker who presented in Singapore with clinical signs and symptoms of acute appendicitis as well as radiologic evidence of portal hypertension. The patient is a 46-year-old Filipino woman who works in Singapore. She presented with a day’s history of right iliac fossa pain. The pain was constant and associated with nonbloody, non-bilious vomiting. It was not associated with preceding fever or change of bowel habits. She did not have R. Wijaya (*) : A. S. Wong Department of General Surgery, Changi General Hospital, 2 Simei Street 3, Singapore 529889, Singapore e-mail: [email protected] J. H. Ng Department of Otolaryngology, Singapore General Hospital, Singapore, Singapore S. Tan Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore
pyrexia. On examination of the abdomen, there was right iliac fossa tenderness and also rebound tenderness with voluntary guarding. Digital rectal examination was unremarkable. Her full blood count showed only thrombocytopenia (129 × 103 μL), with normal total white, neutrophil, and eosinophil counts. Computed tomography (CT) scan of her abdomen and pelvis showed a slightly thickened appendiceal wall with wall enhancement of the appendix and minimal peri-appendiceal fat stranding, suggestive of early appendicitis. Cavernous transformation of the liver with chronic thrombosis in the hilar and right portal vein was evident, resulting in a lobulated liver with an atrophic right hepatic lobe with early evidence of portal hypertension represented by a tortuous splenic vein and splenomegaly. In view of the above findings, she underwent an open appendectomy for likely acute appendicitis on the same day as her CT scan. Intraoperative examination revealed a minimally inflamed and thickened appendix, measuring 5 cm in length. There was minimal clear peritoneal fluid noted. Postoperatively, the patient recovered uneventfully and was discharged 3 days later. Histological examination of the resected appendix revealed an infiltrate of neutrophils and eosinophils extending to the serosal surface from the ulcerated mucosa, consistent with acute appendicitis. There was no evidence of malignancy. Of note, there were multiple ova of a parasite in the lamina propria of the resected appendix, some of which exhibited granulomatous change with surrounding fibrosis. The most likely causative organism was identified to be S. japonicum. The patient was referred to an infectious disease specialist and was treated with praziquantel (60 mL/kg in three doses). Stool examination did not show any evidence of ova, cysts, or parasites. No schistosomal eggs were seen on urine examination. An ultrasound of the hepato-biliary system 2 months later showed coarse underlying echogenicity of the liver as well as prominence of the portal vein wall.
Int J Colorectal Dis
The patient was subsequently referred to a hepatogastroenterology specialist and investigated for the underlying etiology of her liver cirrhosis and portal vein thrombosis. Further history taking revealed no intake of hepatotoxic substances such as chronic ethanol intake. Hepatitis screen, autoimmune markers, metabolic screen, and prothrombotic screen were all unremarkable. Hepatic schistosomiasis was therefore the most likely etiology for her liver cirrhosis and portal vein thrombosis. Four years later, her liver function tests remain normal. Intestinal schistosomiasis is the result of schistosomal ova deposited in the gut wall. Host reaction to the eggs causes granulomatous inflammation and fibrosis, which is responsible for most of the manifestations of chronic schistosomiasis [1]. Polyposis secondary to progressive inflammation, fibrosis, and mucosal hypertrophy is the commonest intestinal manifestation [1, 2]. Schistosomal appendicitis is a rare manifestation of intestinal schistosomiasis even in endemic areas. It is reported to occur in 0.02–6.3 % of infected individuals, but represents up to 28.6 % of chronic appendicitis in these areas [2]. Hepatic schistosomiasis causes portal fibrosis which when severe, is termed Bpipe stem fibrosis^ for the appearance of the greatly enlarged fibrotic portal tracts. Portal hypertension, hepatosplenomegaly, and the development of esophageal varices develop as a consequence. There are marked differences from hepatic cirrhosis. There is no hepatocellular failure, normal liver lobular architecture is preserved, and nodular regenerative hyperplasia is not seen. Therefore, with early treatment, the fibrosis is reversible [1, 2]. As observed in the patient presented above, there is usually preservation of liver function until much later stage of disease [3]. It has been reported that ultrasound examination of subjects with hepatosplenic schistosomiasis yields different findings from
that in liver cirrhosis. Characteristically, there is echogenic thickening of the walls of the portal vein and its branches, indicative of periportal fibrosis, and echogenic enlargement of the gallbladder wall [3]. Again, on CT and MRI scans, periportal fibrosis is the most characteristic feature [3]. Prompt and effective treatment of infection is key to prevention of further damage and complications from portal hypertension. Especially in infection with S. japonicum, there may be rapid progression with little or no interval between acute and chronic hepatosplenic disease [4]. To this end, presumptive treatment with praziquantel is undertaken in endemic areas in patients with suggestive symptoms and signs even when definitive diagnosis has not been made. It is in developed countries where this disease is rare that diagnosis and treatment may be more easily overlooked. This report of a rare case of schistosomiasis in a first world country presenting clinically as acute appendicitis emphasizes the importance of vigilance against this disease even in first world countries, especially in light of the growth in immigration and global travel today. Effective treatment with praziquantel can prevent the severe multisystemic complications that arise in untreated late stage disease.
References 1. WHO: Schistosomiasis. Updated February 2014. http://www.who.int/ mediacentre/factsheets/fs115/en/. Accessed 6th June 2014 2. Elbaz T, Esmat G (2013) Hepatic and intestinal schistosomiasis: review. J Adv Res 4(5):445–452 3. Lambertucci JR (2014) Revisiting the concept of hepatosplenic schistosomiasis and its challenges using traditional and new tools. Rev Soc Bras Med Trop 47(2):130–136 4. Gryseels B, Polman K, Clerinx J, Kestens L (2006) Human schistosomiasis. Lancet 368(9541):1106–1118
