May 2003 Schistosomiasis Chris McGuire, Harvard Medical School Year III
Overview Introduction to schistosomiasis Katayama syndrome Patient A Patient B Ultrasound detection Bladder infection Kidney infection Liver infection Conclusions 2
Schistosomiasis Widespread infection of animals and humans (>200 million people), causing 200,000 deaths/yr worldwide. Parasitic blood fluke endemic in Africa, S. America, and SE Asia. Four main species (S. haematobium, S. mansoni, S. japonicum, S. intercalatum) Varying predilection for GI tract, portal system, and urinary tract between the four species 3
Life cycle The worm breeds in standing freshwater, using snails as an intermediate host and can infect humans through skin inoculation. Next, they reside in the circulation (up to 30 years), and release thousands of eggs a day. The worms do not cause damage, rather, the immune response to the eggs. 4
Katayama Syndrome WHO/TDR/Sinclair Adult S.haematobium worm Occurs during first infection of any schistosome (i.e.traveler to endemic areas) Prodrome of skin erythema, urticaria swimmer s itch 3 to 9 weeks later, there can be headache, fever, chills, and cough that eventually resolves The chronic damage from infection takes place over the next 5-20 years 5
Katayama Radiograph Palmer, Seminars in Roentgenology 33:6-25 Young boy exposed 4 weeks earlier through swimming in African lake (that was designated schistosome free ) Coughing x 7 days, low fever, anorexia Note faint nodular pattern throughout both lungs, prominent hila Repeat radiograph after treatment was normal 6
Patient A 21 y.o. male presents with dull flank pain. He is from the SW mountainous area of Saudi Arabia, where there is rain and standing fresh water. An abdominal radiograph was ordered during the workup. Rural hospital with only plain film capabilities. 7
Abdominal Radiograph Notice bladder region. Courtesy Ferris Hall, M.D. BIDMC 8
Bladder Courtesy Ferris Hall, M.D. BIDMC Note calcifications 9
IVP The next step was to further investigate the findings with an IVP. 10
IVP Notice distal ureters Notice normal kidneys 11 Courtesy Ferris Hall, M.D. BIDMC
Distal Ureters Courtesy Ferris Hall, M.D. BIDMC Dilated, tortuous ureters Contrast filling indicates reflux 12
Patient B 36 y.o. male with persistent hematuria. From schistosomiasis endemic area of Egypt. On pilgrimage to Saudi Arabia and presents at hospital. 13
Patient B Abdominal Film Note bladder Courtesy Ferris Hall, M.D. BIDMC 14
Bladder and ureters Note bladder and ureter calcifications Next step was IVP Courtesy Ferris Hall, M.D. BIDMC 15
IVP of Patient B Note dilated, tortuous left ureter Left ureter Note lack of contrast in right ureter and enlarged kidney Courtesy Ferris Hall, M.D. BIDMC 16
Right Kidney Extremely large, dilated right kidney Courtesy Ferris Hall, M.D. BIDMC 17
Bladder and left ureter L ureter Possible bladder filling defect The next step is to confirm localization of defect by orthogonal view Courtesy Ferris Hall, M.D. BIDMC 18
Oblique Bladder Bladder filling defect Courtesy Ferris Hall, M.D. BIDMC 19
Bladder Mass At surgery, the bladder filling defect was traced to a large mass. Pathology demonstrated that the mass was squamous cell cancer. 20
Bladder Cancer Squamous cell cancer is the most common form of cancer in individuals infected with schistosomiasis. This is in contrast to transitional cell cancer, which is the most common cancer in nonendemic countries. 21
Bladder infection Right sided bladder calcification The calcifications are due to ova in submucosa and may not indicate the current or past state of infection. Palmer, Seminars in Roentgenology 33:6-25 22
Bladder infection Calcification in fundus of bladder Entire bladder is calcified Palmer, Seminars in Roentgenology 33:6-25 Palmer, Seminars in Roentgenology 33:6-25 23
Ureters Ureters are irregular, beaded, and dilated Infection can lead to ureter damage, and is best seen with contrast urography. Palmer, Seminars in Roentgenology 33:6-25 24
Ultrasound Good way to observe changes in bladder, kidney, and liver Economical for many developing countries Standards published by the WHO for survey (www.who.int/tdr/publications/publications/ ultrasound.htm) 25
Ultrasound WHO/TDR/Crump Health worker in Tanzania uses ultrasound to examine young boy with hematuria www.who.int Different transducer cuts with liver u/s from WHO handbook 26
Ultrasound of Bladder Thickened bladder wall Palmer, Seminars in Roentgenology 33:6-25 Almost all forms of schistosomiasis can affect the GU system, but S. haematobium primarily affects. In some endemic areas, 40% of the population will have bladder lesions. These lesions are important to document and follow, as there is a higher rate of bladder cancer. 27
Ultrasound of Kidney Hydronephrosis Hydroureter The hydronephrosis and hydroureter are secondary pathology due to the distal ureter stricture Palmer, Seminars in Roentgenology 33:6-25 28
Liver changes on ultrasound Periportal fibrosis can progress to portal hypertension. Portal vein and splenic vein size are important early markers. U/s is accurate enough to replace liver biopsy for diagnosis. 29
Chris McGuire Liver Fibrosis Palmer, Seminars in Roentgenology 33:6-25 Pathology specimen showing vein fibrosis Palmer, Seminars in Roentgenology 33:6-25 Fibrosis on ultrasound 30
WHO bladder U/S exam standards The important findings on bladder exam are: 1. Wall irregularity, thickening, masses, and pseudopolyps 2. Ureter dilation or thickening 3. Dilated renal pelvis Ultrasound in Schistosomiasis, www.who.int 31
WHO bladder U/S exam standards Further standards are: 1. Bladder wall calcifications 2. Residual urine 3. Renal pelvis fibrosis Ultrasound in Schistosomiasis, www.who.int 32
WHO liver U/S findings Normal starry sky rings and pipe-stems ruff around portal bifurcation bird s claw patches Ultrasound in Schistosomiasis, www.who.int 33
Portal Hypertension- Angiography Paucity of portal circulation Proper hepatic artery Splenic artery Palmer, Seminars in Roentgenology 33:6-25 34
Liver Changes on CT Palmer, PES. The Imaging of Tropical Diseases. Palmer, PES. The Imaging of Tropical Diseases. Note low density patterns in liver 35
Portal Hypertension Gross changes Palmer, PES. The Imaging of Tropical Diseases. Chinese woman infected with S. japonicum Palmer, PES. The Imaging of Tropical Diseases. Enlarged liver at surgery 36
Conclusions Schistosomiasis is an endemic disease worldwide, and important to physicians serving in those countries. Travellers to and immigrants from endemic countries can also become infected, and present in American clinics. Main findings to recognize are: Bladder calcifications and/or masses Dilated/tortuous distal ureter Fibrotic changes on liver u/s Bladder masses or wall thickening on u/s Hydronephrosis secondary to ureter changes 37
Warning Schistosomiasis is an entirely treatable disease. However, there can be a delay in diagnosis, leading to many complications. In endemic countries (S. America, Africa, SE Asia), do not enter any freshwater source, even if it is safe. 38
References Centers for Disease Control. Schistosomiasis in U.S. Peace Corps Volunteers- Malawi, 1992. MMWR 1993; 42:565-69 Colley, DG. Ancient Egypt and Today: Enough Scourges to Go Around. Emerging Infectious Diseases 1996; 2:362-63 Palmer, PES. Schistosomiasis. Seminars in Roentgenology 1998; 33:6-25 Palmer, PES. 2001. Schistosomiasis in The Imaging of Tropical Diseases. Berlin: Springer- Verlag. World Health Organization. Ultrasound in Schistosomiasis 1996, www.who.int/trd/publications/publications/ultrasound.htm 39
Acknowledgements Ferris Hall, MD Larry Barbaras and Cara Lyn D amour our Webmasters Pamela Lepkowski 40