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ediatric Urology

ltrasound and Computed Tomography Findingsf Spontaneous Intramural Hemorrhage of Renalelvis and Ureter in Patient With Hemophilia A

smail Kirbas, Esra Ozgul, Zekai Avci, Mehmet Coskun, and Namik Ozbek

ntramural renal pelvic and ureteral hemorrhage is seen most commonly in patients treated with anticoagulant therapy.owever, spontaneous intramural hemorrhage of the ureter seen in patients with hemophilia is a rare entity and has

een reported only in 2 cases. Computed tomography is a valuable imaging method in the diagnosis and follow-up. Weeport the ultrasound and computed tomography findings of spontaneous intramural renal pelvic and ureteral

emorrhage in a patient with hemophilia A. UROLOGY 72: 1030–1032, 2008. © 2008 Elsevier Inc.

Fsand dilation of renal pelvis and proximal ureter (arrows).

ntramural hemorrhage of the renal pelvis or uretercan occur as a complication of anticoagulant the-rapy.1-5 It also is 1 of the common complications of

emophilia.6,7 However, spontaneous intramural hemor-hage of the ureter in patients with hemophilia is a rarentity and has been reported in only 2 cases.6,7 We reporthe ultrasound and computed tomography findings ofpontaneous intramural renal pelvic and ureteral hemor-hage in a patient with hemophilia A.

ASE REPORT13-year-old boy was admitted to our emergency de-

artment with left-sided flank pain. He had been diag-osed with severe hemophilia A (factor VIII level 1%)hen he was 5 months old. He had no history of trauma,

ever, or blunt injury to the abdomen. On physical ex-mination, tenderness was present on the left flank andower abdominal quadrant. The other systemic evalua-ions were normal. The results of the complete bloodount revealed hemoglobin 11.8 g/dL, white blood cellount 12.4 � 109/L, platelet count 238 � 109/L, acti-ated partial thromboplastin time 118.9 s, and factor VIIIevel of 0.3%. No factor VIII inhibitor was present.

acroscopic and microscopic hematuria were absent.rinary tract ultrasonography revealed increased wall

hickness of the renal pelvis and proximal ureter. Pelvi-aliceal and proximal ureteral dilation were also visibleFig. 1). Abdominal computed tomography (CT) waserformed (Volume Zoom, Siemens, Erlangen, Ger-any). Nonenhanced CT scans revealed high-attenua-

ion thickening of the left pelvic ureteral wall caused by

rom the Department of Radiology, Fatih University Faculty of Medicine, Ankara,urkey; and Departments of Radiology and Pediatric Hematology, Baskent Universityaculty of Medicine, Bahcelievler-Ankara, TurkeyReprint requests: Esra Ozgul, M.D., Department of Radiology, Baskent University

aculty of Medicine, Fevzi Cakmak Cad 10. sok No: 45, Bahcelievler-Ankara 06490

urkey. E-mail: dresrayam@gmail.comSubmitted: April 1, 2008, accepted (with revisions): June 2, 2008

030 © 2008 Elsevier Inc.All Rights Reserved

igure 1. (A) Longitudinal and (B) axial urinary tract ultra-ound scans of left kidney showing increased wall thickness

0090-4295/08/$34.00doi:10.1016/j.urology.2008.06.012

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emorrhage (Fig. 2A). Contrast-enhanced CT scansemonstrated delayed nephropyelography and hydrone-hrosis due to obstruction in the left kidney (Fig. 2B,C).he radiologic diagnosis was spontaneous intramural re-al pelvic and ureteral hemorrhage. The patient wasiven 500 U of factor VIII concentrate intravenouslywice daily for 4 days (to maintain a factor VIII level of5%-30%). Because of the severe pain, the patient re-eived intravenous meperidine, hyosine-N-butyl bromidespasmolytic), and tenoxicam. Microscopic and macro-copic hematuria did not occur in the following days. By

igure 2. (A) Nonenhanced CT scan revealing high-attenuaB,C) Contrast-enhanced CT scans demonstrating delayed nidney.

he fourth day of therapy, the patient’s complaints had o

ROLOGY 72 (5), 2008

isappeared. Subsequently, 500 U of factor VIII concen-rate were administrated once daily for 4 days, and theatient was discharged from the hospital. Follow-up CTas performed 1 month after discharge and showed re-ression of all findings (Fig. 3).

OMMENTemophilia A is the most common inherited bleeding

isorder caused by defects in the F8C gene that encodesoagulation factor VIII.8 This X-linked recessive disorder

thickening of left pelviureteric wall caused by hemorrhage.opyelography and hydronephrosis due to obstruction in left

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ccurs in approximately 1:5000 males. Spontaneous

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leeding episodes are seen in patients with severe hemo-hilia and can involve the major joints, such as thenees, muscles or soft tissue.9,10

Intramural renal pelvic and ureteral hemorrhage iseen most commonly in patients treated with anticoagu-ant therapy.1-5 The hemorrhagic incidence in these pa-ients is 4%-24% and urinary tract hemorrhage is seen in0% of these patients.5 Hemophilia is another cause ofreteral intramural hemorrhage, but spontaneous hemor-hage is rare. Only 2 cases of spontaneous ureteral hem-rrhage with hemophilia have been reported.6,7 Sponta-eous renal pelvic or ureteral hemorrhage can also occur

igure 3. (A,B) Follow-up CT scans performed 1 monthfter treatment showing regression of all findings.

n renal neoplasms.4 Thus, these patients must be eval-

032

ated completely with nonenhanced and enhanced ab-ominal CT, including the arterial, portal venous, andate phases. Follow-up CT within 1 month must beerformed to exclude a neoplasm. Generally, renal intra-ural hemorrhage regresses spontaneously within 2-6eeks.1,4,5

Ultrasonography can show a thickening wall of theenal pelvis and/or ureter. However, it cannot distinguishemorrhage from edema or fluid.4 CT is the most valu-ble imaging method for these patients.1,4,5 Nonen-anced CT scans will show high-attenuation thickeningf the pelvic and/or ureteral wall.5 The findings willesolve within 3-4 weeks and follow-up CT will show novidence of hemorrhage.4,5

Factor replacement therapy in the presence of ureteralemorrhage occurring in association with hematuriaould be contraindicated because it can cause coagula-ion and obstruction in the urinary tract. However, inatients with only intramural hemorrhage, such as in ourase, factor VIII replacement therapy must be adminis-ered. CT is important in the diagnosis and follow-up andan help guide therapy. Our patient had a good clinicalutcome with factor VIII replacement therapy.

ONCLUSIONSntramural spontaneous renal pelviureteric hemorrhage israre bleeding complication of patients with hemophilia.his diagnosis should be kept in mind when patients withemophilia present with sudden-onset flank pain withoutematuria. CT is a valuable imaging method in theiagnosis and follow-up.

eferences1. Kossol JM, Patel SK. Suburothelial hemorrhage: The value of

preinfusion computed tomography. J Comput Assist Tomogr. 1986;10:157-158.

2. Smith WL, Weinstein AS, Wiot JF. Defects of the renal collectingsystems in patients receiving anticoagulants. Radiology. 1974;113:649-651.

3. Kaiser JA, Jacobs RP, Korobkin M. Submucosal hemorrhage of therenal collecting system. AJR Am J Roentgenol Radium Ther NuclMed. 1975;125:311-313.

4. Fishman MC, Pollack HM, Arger PH, et al. Radiographic mani-festations of spontaneous renal sinus hemorrhage. AJR Am J Roent-genol. 1984;142:1161-1164.

5. Phinney A, Hanson J, Talner LB. Diagnosis of renal pelvis subep-ithelial hemorrhage using unenhanced helical CT. AJR Am JRoentgenol. 2000;174:1023-1024.

6. Ameri A, Martin R, Vega R, et al. Successful management ofintramural ureteral hemorrhage in a patient with factor VIII defi-ciency and high-titer inhibitor. J Thromb Haemost. 2004;2:2273.

7. Kashiwai H, Kawata Y, Hirayama A, et al. A case of acquiredhemophilia A discovered by right renal bleeding. Jpn J Urol. 1999;90:928-931.

8. Castaldo G, D’Argenio V, Nardiello P, et al. Haemophilia A:Molecular insights. Clin Chem Lab Med. 2007;45:450-461.

9. Venkateswaran L, Wilimas JA, Jones DJ, et al. Mild hemophilia inchildren: Prevalence, complications, and treatment. J Pediatr He-matol/Oncol. 1998;20:32-35.

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UROLOGY 72 (5), 2008