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Year : 2002  |  Volume : 12  |  Issue : 2  |  Page : 253-256
Successful treatment with therapeutic embolization of left renal arteriovenous malformation in a haemophilia patient

Department of Radiodiagnosis, King Edward Memorial Hospital, Sardar Moodliar Road, Pune 400011, India

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Keywords: Renal Arteriovenous malformation, haemophilia, haematuria

How to cite this article:
Pande S A, Shetty D D, Pawar A D. Successful treatment with therapeutic embolization of left renal arteriovenous malformation in a haemophilia patient. Indian J Radiol Imaging 2002;12:253-6

How to cite this URL:
Pande S A, Shetty D D, Pawar A D. Successful treatment with therapeutic embolization of left renal arteriovenous malformation in a haemophilia patient. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Dec 4];12:253-6. Available from:

   Introduction Top

Spontaneous haematuria is a common manifestation in haemophiliacs. Immune complex formation and renal cellular injury have been postulated as possible causes of haematuria in these patients. The incidence of congenital renal arteriovenous malformations (AVM) as a cause of haematuria in haemophiliacs is not known. In the past partial or total nephrectomy and arterial reconstruction were the most common methods of treatment. Transcatheter arteriographically directed embolization has now gained widespread acceptance. We report a case in which congenital renal AVM was the cause of haematuria in a haemophiliac, which was successfully treated with percutaneous transfemoral embolization and strict maintenance of factor VIII levels

   Case study Top

A 38 years old man presented with history of haematuria since 15 days. He had been previously diagnosed as having haemophilia A, when he had an episode of epistaxis a few years ago. His brother is also a known case of haemophilia. The patient on presentation had severe anaemia [HB=3.7 gm%], his partial thromboplastin time was prolonged [17 second] and his factor VIII levels were < 1%. His renal function tests were deranged with a serum creatine level of 1.7 mg% and serum uric acid of 10.2mg%. Abundant red blood cells were seen in the urine sample.

Sonographic examination during the present admission revealed a 2x2 cm calculus at the left uretero-vesical junction (which later turned out to be a blood clot)causing changes of left hydronephrosis. The urinary bladder showed trabeculated wall and a significant post-micturition residue. A 5 x 4.5 cm mobile mass was seen in the bladder. There was no renal parenchymal disease, calculus or renal mass. A cystoscopy and ureteroscopy, which was done after giving 3000 units of factor VIII revealed blood clots in the urethra and urinary bladder. No mass was seen in the bladder. The right ureter was normal. Blood clots were seen in the left ureter and fresh blood was seen trickling from the left kidney. An angiographic examination of the left kidney was undertaken to determine the cause of haematuria since haematuria was persistent even after administration of factor VIII. A right percutaneous transfemoral selective bilateral renal angiography was done using a 0.035 J tip guide wire and 5 F renal catheter. A small arteriovenous malformation was seen arising from the lower polar segmental artery of the left kidney. An instant blush and immediate filling of the renal vein and inferior vena cava was noted in the arterial phase [Figure - 1]. The feeding artery was occluded by using gelfoam and later by introducing a steel coil super selectively [Figure - 2]. Post-embolization films revealed complete occlusion of the feeding vessels and non-opacification of the arteriovenous nidus [Figure - 3]. Mild spasm of the other branches of the renal artery was noted. Follow up Doppler study revealed normal perfusion of the left kidney and non-visualization of the arteriovenous malformation.

The patient was asymptomatic after the procedure and his urine became clear within 12 hrs. of embolization. However he started having haematuria from the 7th postembolization day. Sonographic examination revealed a bright left renal cortex with enhanced corticomedullary differentiation which was suggestive of nephritis. On the 11th postembolization day, factor VIII level was markedly reduced to less than 1%. Hence 2400 units of factor VIII were re-administered and the level was maintained at 30%. Subsequently the haematuria subsided on the 11th post-embolization day, there was no further haematuria and the patient was discharged on the 28th post-embolization day.

   Discussion Top

Haemophilia A, an X linked disorder, with an incidence 1:10,000 males is characterized by bleeding diatheses and low plasma levels of factor VIII coagulant activity. The factor VIII deficiency is categorized as severe [<1%], moderate [1%-5%] or mild [5%-30%] according to the relative amount of factor VIII activity in the patients plasma [1]. Patients with severe disease bleed frequently without discernible trauma, patient with moderate disease bleed less frequently and those with mild disease bleed infrequently and almost always secondary to trauma [1]. In our patient the disease was of severe type as he had factor VIII level <1%, had no history of trauma and had massive haematuria. Majority of the patients of haemophilia have factor VIII levels below 5% [2].

The clinical manifestations include haemarthoses, central nervous system haemorrhage and muscular and soft tissue haematomas [1]. Haematuria also is a common manifestation which was the presenting symptom in our patient. The aetiology of spontaneous haematuria is unknown but immune complex formation and renal cellular injury in patients who have received multiple transfusions has been postulated [3]. Haematuria without trauma is uncommon in congenital deficiencies other than factor VIII and IX [3]. In such patients the only treatment needed is steroids and appropriate factor replacement.

Structural renal abnormalities tend to occur in all haemophiliacs secondary to haematuria irrespective of the degree of haematuria [4]. One of this was seen in our patient in the form of hydronephrosis resulting from obstruction of left pelvi-ureteric junction by blood clots. However functional renal impairment is confined mainly to those patients with severe haematuria [4]. The perplexing nature of this case was the presence of a congenital renal arteriovenous malformation (AVM) which was the cause of haematuria. The only other congenital anomaly to be reported was duplex kidney by Prentice which showed a higher incidence in haemophiliacs as compared to the normal population [4].

Congenital arteriovenous malformations in kidney are frequently silent, though they may present with haematuria, congestive cardiac failure, hypertension and abdominal pain [5]. Increased utilization of diagnostic angiography for renal diseases has resulted in more frequent diagnosis of AVMs involving kidneys [5]. On angiography, congenital AVMs are seen as one or more tortuous coiled vascular channels of varied sizes grouped in clusters which are supplied by multiple, slightly enlarged segmental or interlobar arterial branches. Malformations ranges in size from 15 x 15 mm to 30 x 40 mm, are more common in upper pole and arteriovenous shunting in almost always present. They usually involve peripelvic or medullary region and often produce a homogeneous stain on nephrogram phase of arteriographic studies. Proximal main renal artery and kidney size are usually normal [6].

Management of arteriovenous malformations should be based on the cause and associated symptoms. Most congenital arteriovenous malformations are small, asymptomatic and these close spontaneously. Therapeutic intervention is indicated in patients who have symptomatic arteriovenous malformations presenting with persistent microscopic haematuria or massive haematuria or frank rupture of the arteriovenous malformations [5]. In our case, as the patient was having frank haematuria, we decided to embolize the AVM. In the past partial or total nephrectomy and arterial reconstructive procedures have been the most common methods of treating symptomatic AVMs. Transcatheter arteriographically directed embolization has now gained wide acceptance for treatment of both congenital and acquired AVMs.

A haemophiliac can undergo the procedure as in a non-haemophiliac, as long as the pre-operative and two weeks post -operative level of coagulation factor activity is maintained at 50% or more of normal levels [7].

As the AVM in our patient was of medium size we decided to embolize it with gelfoam followed by introduction of a steel coil. Other embolization materials which can be used for large AVMs are gelatin sponge emboli, balloons and bucrylate. In case of small AVMs, autogenous clots are used because lysis of this embolized material within a few hours prevents renal infarction [5]. Potential complications of embolization include remote venous embolization, hypertension secondary to renal ischaemia and renal infarction and re-fistulization in the infarcted area [5].

Postembolization bleeding is a frequent problem encountered in patients with haemophilia and the most common cause is inadequate factor replacement [8]. Postembolization haemorrhage occurs mainly on the 1st to 11th postembolization day with the peak occurring on the 6th or 7th day [7]. In our case the patient started having frank haematuria on 7th postembolization day. It has been pointed out by Marintowitz et. al. [7] that in neurosurgeries for cerebral AVM in haemophiliac patients, the post operative haemorrhage may be associated with abnormalities of other coagulation factors or an excess of fibrinolytic activators in cerebral tissue. The probable reason for haematuria in our patient was non-maintenance of factor VIII levels in the post embolization period. The factor VIII levels had dropped to as low as 1% on the 11th postembolization day. Repeat USG study with color Doppler analysis had confirmed the occlusion of the AVM at this time. The haematuria subsided after administration of factor VIII and thus we succeeded in treating a haemophiliac patient presenting with haematuria by embolizing the renal arteriovenous malformation. This case highlights the following points:

1. The possibility of an underlying renal arteriovenous malformation, however remote is always likely in case of haematuria even in patients with haemophilia.

2. Percutaneous treatment of such renal AVMs can be successfully carried out in the patients with haemophilia and a surgical exploration can be avoided.

3. Even with percutenous embolization, the standard regime of maintaining factor VIII levels has to be strictly adhered to, as in case of embolization of central nervous system AVMs.

   Acknowledgements Top

We are thankful to Dr. Shashi Apte MD, Department of Haematology, K.E.M. Hospital, Pune.

   References Top

1.Kane WH, Davie EW: Blood coagulation factor V and VIII.: structural and functional similarities and their relationship to haemorrhagic and thrombotic disorders: Blood March 1988: 71, No. 3: 539 - 555.  Back to cited text no. 1    
2.Handin R.I.: Disorder of coagulation and Thrombosis, In: Fanci AS, Kasper DL, Hanser SL, Longo DL, eds Harrison's principles of internal medicine, 14th edition Harris, 14th edition vol. 1 New York, McGram Hill, 1998736-738, vol. 1:736-738  Back to cited text no. 2    
3.Corrigan JJ: Coagulation Disorders. In: Miller Dr, Baehner RL. Eds. Blood diseases of infancy and childhood, 6th edition, St. Louis: Mosby 1990: 856.  Back to cited text no. 3    
4.Prentice CRM, Lindsay RM, Barr RD; Forbes CD, Kennedy AC, McNicol GP, Douglas AS: Renal complications in haemophilia Christmas disease: QJM Jan 1971; 1577:47-61.  Back to cited text no. 4    
5.Schaeffer AJ, Greco FD: Other renal diseases of Urological significance. In: Walsh PC, retile AB, Stamey TA, Vanghan ED Jr. Eds. Campbells's Urology, 6th Edition. Philadelphia, WB Saunders, 1992:2070.  Back to cited text no. 5    
6.Cho KJ, Stanley JC: Nonneoplastic congenital and acquired renal arteriovenous amlformations and fistualas: Radiology Nov 1978; 129:333-343.  Back to cited text no. 6    
7.Nakau H, Maruishi M, Takiguchi H, Shima K: Successful surgical removal of a large AVM in a patient with haemophilla: Neurosurgery Dec. 1998; 43:1459-1462.  Back to cited text no. 7    
8.Bhushan V, Chandy M, Khanduri M, Dennison D, Srivastav A. Apter S. Surgery in patient with congenital coagulation disorders: The National Medical Journal of Indian Jan 1994; vol. 7: 8-12.  Back to cited text no. 8    

Correspondence Address:
S A Pande
Department of Radiodiagnosis, King Edward Memorial Hospital, Sardar Moodliar Road, Pune 400011
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Source of Support: None, Conflict of Interest: None

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[Figure - 1], [Figure - 2], [Figure - 3]

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