British Journal of Radiology (2007) 80, e105-e106
© 2007 British Institute of Radiology
doi: 10.1259/bjr/82836722
Focal renal infarction: an unusual cause of haematuria in a patient with sickle cell trait
B Hedayati, MB BS, MRCS
1
K M Anson, MBChB, MS, FRCS(Urol)
2 and
U Patel, MBChB, MRCP(UK), FRCR
1
Departments of 1 Radiology and 2 Urology, St George's Hospital, Tooting, London SW19 0QT, UK
Correspondence: Dr B Hedayati, Department of Radiology, St George's Hospital, Tooting, London, SW17 0QT, UK. E-mail: bijan.hedayati{at}stgeorges.nhs.uk
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Abstract
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A 29-year-old woman with sickle cell trait developed persistent haematuria. Intravenous urography, ultrasound, cystoscopy and selective renal angiography revealed focal renal infarction, but in the absence of papillary necrosis. There are no prior reports of focal renal infarction as a cause of haematuria in patients with sickle cell trait.
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Introduction
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We describe a case of haematuria in a patient with sickle cell trait, caused by focal renal infarction in the absence of papillary necrosis: this rare phenomenon has not been reported previously.
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Case report
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A 29-year-old female with sickle cell trait presented with loin pain and frank haematuria, associated with clots. Her haemoglobinopathy had been asymptomatic prior to this episode. In the past she had undergone an uncomplicated laparoscopy for infertility. There was no other medical history of note. Her full blood count, clotting profile, urea and electrolytes, and urine microscopy and culture were normal.
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Investigations
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Intravenous urography showed no evidence of calculi, obstruction or papillary necrosis (Figure 1
). An ultrasound scan demonstrated normal kidneys without scarring, but with a diffusely altered echogenic texture and a mixture of sediment and thrombus in the bladder. Flexible cystoscopy revealed active bleeding. However, the exact site could not be identified due to the poor views obtained. Selective renal angiography showed two regions of decreased vascularity, in the upper and interpolar regions of the left kidney, compatible with scarring following infarction (Figure 2). The right kidney (not shown) was normal. We are unsure as to the cause of this infarction, as the upper pole wedge defect is rather large, yet the arterial pattern is preserved to the very edge of the defect. Views of branch veins are poor in the late phase of the arteriogram and venous infarction is possible. No arteriovenous malformations or sites of active bleeding were identified. The haematuria resolved slowly over the next week without treatment.
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Figure 2. Digital subtraction renal angiography showing two hypovascular foci. One wedge-shaped defect in the upper pole and a small parenchymal defect in the interpolar region of the left kidney. (a) The arterial tree and the vessels can be followed up to the edge of the abnormality without any major occlusion. (b) A late acquisition, but this only showed the main renal vein well.
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Discussion
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Haematuria is a recognized clinical presentation of patients with sickle cell trait, usually due to papillary necrosis said to originate more commonly from the left kidney. Harrow et al [1] suggest that the many tributaries of the left renal vein may lead to an elevated venous pressure on that side with stagnation and sickling. The pathophysiology of papillary necrosis is related to hyperosmolarity of the fluid in the interstitium of the renal medulla. Fluid re-absorption from Henle's loop concentrates the red cells of the vasa recta. Once sickling commences, microthrombosis and infarction ensue within the capillaries. These events together can lead to papillary necrosis [2, 3]. Haematuria occurs because of increased capillary permeability, allowing red blood cells to leak into the collecting system.
Excretion urography has a central diagnostic role, particularly in the demonstration of the early changes of papillary necrosis [4]. Radiographically, renal papillary necrosis in sickle cell trait can be classified into medullary and papillary types. The former type describes necrosis centrally in the papilla, with a central contrast medium pool referred to as an "egg in cup" appearance. Papillary necrosis refers to tracks extending from the calyceal fornices. If these cross the base of the papilla, the result is papillary sloughing and a blunted calyx on the urogram.
Alternatives, such as ultrasound, CT or MRI, have not yet been evaluated in the diagnosis of early papillary necrosis. As these changes are subtle on urography, the likelihood is that these alternative modalities with their lower resolution are unlikely to be useful, although fine section multislice CT may prove accurate.
In the absence of papillary necrosis the patient with sickle cell disease/trait should undergo the normal route for investigation of gross haematuria. After cystoscopy, this would be CT if ultrasound and urography were normal. If all these investigations are normal then arteriography may be used to rule out unusual causes such as an arteriovenous malformation.
In our case renal infarction was an unexpected finding. In one previously reported case of massive haematuria associated with sickle cell trait, nephrectomy was necessary to arrest life-threatening haemorrhage. Subsequent histology revealed changes closely resembling infarction, but were by no means typical [5].
Our case report highlights a unique case of focal renal infarction, in the absence of papillary necrosis in a patient with sickle cell trait, postulated to be of venous origin.
Received for publication August 11, 2003.
Revision received April 30, 2004.
Accepted for publication September 2, 2005.
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References
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- Harrow BR, Sloane AJ, Leibman NC. Roentgenolic demonstration of renal papillary necrosis in sickle cell trait. N Engl J Med 1963;268:969–75.[Medline]
- Zadeii G, Lohr JW. Renal papillary necrosis in a patient with sickle cell trait. J Am Soc Nephrol 1997;8:1034–40.[Abstract]
- Oksenheldler E, Bourbigot B, Desbazeille F, Droz D, Choquenet C, Girot R, et al. Recurrent haematuria in 4 white patients with sickle cell trait. J Urol 1984;132:1201–3.[Medline]
- Pandya KK, Koshy M, Brown N, Presman D. Renal papillary necrosis in sickle cell haemoglopinopathies. J Urol 1976;115:497–501.[Medline]
- Bennett MA, Heslop RW, Meynell MJ. Massive haematuria associated with sickle cell trait. Br Med J 1967;1:677–9.[Free Full Text]
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Abstract
Introduction
