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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 18  |  Issue : 1  |  Page : 46-48

Bilateral pelvic kidneys in a haemophiliac


1 Department of Radiology, Rasheed Shekoni Teaching Hospital, Dutse, Jigawa State, Nigeria
2 Department of Radiology, Federal Medical Centre, Birnin Kudu, Jigawa State, Nigeria
3 Department of Radiology, Aminu Kano Teaching Hospital, Kano State, Nigeria

Date of Submission15-Jan-2020
Date of Decision26-Jan-2020
Date of Acceptance04-Aug-2020
Date of Web Publication4-May-2021

Correspondence Address:
Dr. Mohammed Aliyu Abdulmalik
Department of Radiology, Rasheed Shekoni Teaching Hospital, Dutse, Jigawa State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njbcs.njbcs_5_20

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  Abstract 


Renal migration anomalies are rare and often under-reported. Kidneys that fail to ascend to their normal location in the renal fossa are usually termed 'ectopic' kidneys, which can be unilateral or bilateral. Those that fail to ascend above the pelvic brim are commonly termed pelvic kidneys. We present a case of a 41-year-old male haemophiliac on routine investigation with a previous history of anaemia and bone pains, currently asymptomatic. He was found to have malrotated pelvic kidneys with vascular anomalies. Even though most patients with pelvic kidneys are asymptomatic, they are usually discovered in childhood or as young adults. This case is important because, in addition to the late discovery of the bilateral pelvic kidneys, there is coexistence of a bleeding disorder which may pose a challenge in patients' management. This is to also make the patient aware of his condition and avoid taken unnecessary risk which might make his condition worse.

Keywords: Haemophilia, malrotation, pelvic kidneys, vascular anomaly


How to cite this article:
Abdulmalik MA, Hafiz A, Mustapha MA. Bilateral pelvic kidneys in a haemophiliac. Niger J Basic Clin Sci 2021;18:46-8

How to cite this URL:
Abdulmalik MA, Hafiz A, Mustapha MA. Bilateral pelvic kidneys in a haemophiliac. Niger J Basic Clin Sci [serial online] 2021 [cited 2021 Jun 23];18:46-8. Available from: https://www.njbcs.net/text.asp?2021/18/1/46/315413




  Introduction Top


Renal anomalies can result from abnormal development, ascent, rotation and migration.[1] The most common type of renal migration anomaly is the pelvic kidney whose incidence is about 1 in 725 births or 1 in 2100–3000 autopsies.[2] The ectopic kidneys may pose a special treatment challenge, especially when there is a coexisting disorder.[3]

Haemophilia is an X-linked hereditary bleeding disorder characterised by deficiencies or full absence of functionally active Factor VIII or IX.[4]

There is very limited literature showing the prevalence of renal ectopia in haemophilia. However, certain common findings may be seen separately or coexisting in the two conditions, like haematuria, which is also a frequent finding and recognised complication in haemophiliacs.[5] Hepatitis and HIV infections acquired through blood transfusion or plasma-derived concentrates may occur, thus causing a spectrum of renal diseases.[6]

Imaging plays an important role in proper location of the kidneys and the renal vessels.[7] Apart from helping to make accurate diagnoses, imaging is used for follow-up during and after treatment and in the management of complications.

In this case, bilateral pelvic kidneys with anomalous origin of the renal arteries were seen coexisting with haemophilia.


  Case Report Top


The index case is a 41-year-old male known haemophiliac A, which was diagnosed about 10 years before presentation, on anti-factor VIII concentrate supplement. He also tested positive for hepatitis B virus antigen while on routine laboratory examination and yet to do the viral load count test due to financial constraint. There was a history of recurrent dull bone pains and weakness for about 2 years. Had two sessions of blood transfusion, last time was about 2 months before presentation. He is the fourth of five siblings, all males, with all except the first male having haemophilia.

General physical examination revealed mild parlour and bilateral mild bowing of the lower limbs. His blood pressure was normal. Other laboratory investigations showed packed cell volume to be 26%, normoglycaemic with normal electrolyte and creatinine parameters. Urine analysis was also normal.

On ultrasound scan, bilateral ectopic kidneys were found. Computed tomographic urogram (CT-IVU) also showed bilateral ectopic kidneys at the level of the sacrum with associated malrotation, though with good contrast excretion through both kidneys [Figure 1]. Other organs appeared normal.
Figure 1: Computed tomographic urogram showing bilateral pelvic kidneys seen at the sacral level with the left kidney higher than the right. Both showing good contrast excretion

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Contrast-enhanced computed tomography of the kidney-ureter-bladder regions with renal angiography showed bilateral ectopic kidneys in the pelvis measuring 10.2 and 11.1 cm in their bipolar length. Both kidneys showed dual arterial supply from their respective common iliac arteries, with one smaller in calibre than the other near the bifurcation of the aorta. The left renal artery originated from the posterolateral aspect of the left common iliac artery, while the right from the medial aspect of the right common iliac artery [Figure 2]. The renal veins came out of the hilum and entered into the proximal inferior vena cava. The patient was properly counselled regarding the bilateral ectopic kidneys and advised to continue follow-up with the haematologist regarding the hereditary bleeding disorder and associated viral infection.
Figure 2: Renal angiogram showing bilateral pelvic kidneys with both renal arteries originating from the common iliac arteries close to the aortic bifurcation

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  Discussion Top


The kidneys ascend to the lumbar region just below the adrenal glands, along the dorsal aorta, between the 6th and 9th weeks of intrauterine life.[3] In this case, bilateral pelvic kidneys were present just lateral to midline with the left kidney slightly higher than the right at the sacral level, though their sizes were normal.

Although our patient did not have any bleeding originating from the urinary tracts or haematuria, it is not a rare sign of haemophilia. A variety of gene defects associated with the development of haemophilia A is observed to cause a severe clinical form of haemophilia A with haematuria.[8] There is very scanty research on the association of haemophilia with ectopic kidney and the significant adverse effect of ectopic kidneys with haemophilia. However, since individuals with ectopic kidney are more predisposed to renal infection and formation of calculus,[9] It is common sense to think that if these agents are present in haemophiliacs with ectopic kidneys, haematuria might get worse.

Even though some cases of ectopic kidney may go undetected in life and get noticed post-mortem,[10] with coexisting haemophilia, more symptoms abound necessitating investigation and subsequent detection of the renal anomaly just like in our patient.

It is pertinent to properly investigate other congenital anomalies in such situation where one anomaly is seen coexisting with haemophilia.

Dr.

Bilateral pelvic kidneys associated with vascular anomalies in haemophiliacs are very uncommon. Clear knowledge on the association of the renal anomaly with the bleeding tendency in haemophiliacs will help in instituting appropriate management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Shweta BS, Shabana B, Suresh G. A human cadaveric study on incidence and morphology of anatomical variations of kidney and ureter with emphasis on its embryological, genetic and clinical significance. Int J Anat Res 2018;6:5892-910.  Back to cited text no. 1
    
2.
Bauer SB. Anomalies of the upper urinary tract. In: Wein A, Campbell-Walsh Urology, editors. 11th ed. Philadelphia: Saunders Elsevier; 2016. p. 2985-6.  Back to cited text no. 2
    
3.
Cinman NM, Okeke Z, Smith AD. Pelvic kidney: associated disease and treatment. J Endourol 2007;8:836-42.  Back to cited text no. 3
    
4.
Kyle AD, Joseph RS, Amy LD. Screening urinalysis demonstrates that haematuria is a frequent finding in persons with haemophilia treated at a paediatric haemophilia treatment centre. Haemophilia 2019;25:782-8.  Back to cited text no. 4
    
5.
Kulkarni R, Michael Soucie J, Evatt B. Haemophilia surveillance system project investigators. Renal disease among males with haemophilia. Haemophilia 2003;9:703-10.  Back to cited text no. 5
    
6.
Rao TK. Human immunodeficiency virus infection and renal failure. Infect Dis Clin North Am 2001;15:833-50.  Back to cited text no. 6
    
7.
Gutierrez MD, Rodriguez F, Guerra CJ. Renal anomalies of position, shape and fusion: radiographic analysis. Revista de la federacion Ecutoriana de Radiologia 2013;6:24-30.  Back to cited text no. 7
    
8.
Franchini M, Mannucci PM. Hemophilia A in the third millennium. Blood Rev 2013;27:179-84.  Back to cited text no. 8
    
9.
Rohit B, Dinesh S, Yash PS, Kshama N, Anurag S. An ectopic pelvic kidney. Pol J Radio 2015;80:425-7.  Back to cited text no. 9
    
10.
Dilip KP, Pritesh J, Manju B. Bilateral pelvic kidneys: A rare anomaly. J Case Reports 2016;6:411-14.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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