Home About us Current issue Back issues Submission Instructions Advertise Contact Login   

Search Article 
  
Advanced search 
 
Saudi Journal of Kidney Diseases and Transplantation
Users online: 2313 Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size 
 


 
Table of Contents   
CASE REPORT  
Year : 2019  |  Volume : 30  |  Issue : 4  |  Page : 982-984
Ruptured intracranial aneurysm and recessive polycystic kidney Disease: A Rare Association


1 Department of Medicine A (M8); Laboratory of Kidney Pathology (LR00SP01), Charles Nicolle Hospital; Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia
2 Department of Medicine A (M8), Charles Nicolle Hospital, Tunis, Tunisia
3 Department of Medicine A (M8), Charles Nicolle Hospital; Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia

Click here for correspondence address and email

Date of Submission25-Aug-2018
Date of Acceptance24-Sep-2018
Date of Web Publication27-Aug-2019
 

   Abstract 


Autosomal recessive polycystic kidney disease (ARPKD) is the most common childhood-onset ciliopathy. Intracranial aneurysms (ICA) are a serious complication of autosomal dominant polycystic kidney disease (ADPKD). However, there are only three reports about ICA in an adult patient with ARPKD. We describe a rare case of a 29-year-old man with ARPKD presenting with subarachnoid hemorrhage secondary to a ruptured intracranial aneurysm. The diagnosis of ARPKD was at the age of eight years based on typical ultrasonography findings with polycystic kidneys and liver disease. Magnetic resonance cholangiography showed a nonobstructive dilatation of intrahepatic bile ducts. Liver biopsy showed hepatic fibrosis. None of the family members was affected. At the age of 15 years, he had progressed to end-stage kidney disease, and hemodialysis was started. The patient had always a severe arterial hypertension. At the age of 29 years, he complained of headaches with an uncontrolled hypertension and disturbance of consciousness, computed tomography angiography showed subarachnoid hemorrhage and multiple cerebral aneurysms. Early neurologic screening of intracranial aneurysm should be recommended in ARPKD like in ADPKD patients.

How to cite this article:
Helal I, Handous I, Khadhar M, Bezzine H, Hamida FB, Abdallah TB. Ruptured intracranial aneurysm and recessive polycystic kidney Disease: A Rare Association. Saudi J Kidney Dis Transpl 2019;30:982-4

How to cite this URL:
Helal I, Handous I, Khadhar M, Bezzine H, Hamida FB, Abdallah TB. Ruptured intracranial aneurysm and recessive polycystic kidney Disease: A Rare Association. Saudi J Kidney Dis Transpl [serial online] 2019 [cited 2019 Sep 21];30:982-4. Available from: http://www.sjkdt.org/text.asp?2019/30/4/982/265479



   Introduction Top


Autosomal recessive polycystic kidney disease (ARPKD) is an inherited ciliopathy with incidence estimated at about 1/20,000.[1] ARPKD manifest at infancy with kidney or liver disorder.[2]

The association of intracranial aneurysm (ICA) and autosomal dominant polycystic kidney disease (ADPKD) has been established with the prevalence of 5%–10%.[3] Uncommonly, ARPKD was associated with intracranial aneu-rysm in three published cases.[4],[5],[6] We herein report the 4th case of a patient with ARPKD, presenting with subarachnoid hemorrhage secondary to a ruptured ICA.


   Case Report Top


We describe a rare case of a 29-year- old man presenting with subarachnoid hemorrhage secondary to a ruptured ICA complicating ARPKD.

His is a known case of chronic kidney disease since the age of eight years. The diagnosis of ARPKD was based on the typical findings in ultrasonography and computed tomography. Ultrasonography showed hepatorenal polycystic. Magnetic resonance cholangiography showed a nonobstructive dilation of intra-hepatic bile ducts. Liver biopsy showed hepatic fibrosis. Although none of the family members was affected, the diagnosis of ARPKD was made.

At the age of 15 years, the disease had progressed to end-stage kidney disease, and hemodialysis was started. The patient had always a severe arterial hypertension and a poor adherence to treatment, for those reasons, he was usually dialyzed without heparin.

At the age of 29 years, he complained of headaches with drowsiness. The blood pressure was 26/11 mm Hg. Despite optimal treatment, hypertension remains uncontrolled, and the patient presented consciousness disorders. Computed tomography angiography showed subarachnoid hemorrhage and multiple fusiform and saccular aneurysms in the branches of cerebral arteries were seen bilaterally [Figure 1] and [Figure 2]. The patient has been transferred immediately to a specialized neuro-surgical unit for endovascular intervention. Unfortunately, the outcome was fatal with brain herniation and sudden death of the patient.
Figure 1: Computed tomography angiography, 9.1 mm aneurysm at the right cerebral arterial.

Click here to view
Figure 2: Computed tomography angiography, multiples aneurysm at cerebral arteries.

Click here to view



   Discussion Top


Although cerebral aneurysm is a recognized feature of ADPKD, it is uncommonly reported in few cases of ARPKD.

We report multiple cerebral aneurysms in a 29-year-old man with ARPKD. In our case, those aneurysms expressed late in the disease course. To the best of our knowledge, it has been reported only three times in the medical literature one pediatric case and two adult’s 4-6 cases.[4],[5],[6]

Cerebral aneurysm may be a rare manifestation in ARPKD, and it can be also only a chance association in those patients. However, should this association exist, then the neurologic prognosis very bad with serious complications. Therefore, systematic screening of the brain should be recommended for those young patients with ARPKD, and this may increase the number of cases diagnosed as having intracranial aneurysms and highlight the exact incidence of ICA in ARPKD.

We conclude that ICA can occur in patients with ARPKD. Early neurologic screening of intracranial aneurysm should be recommended in ARPKD like in ADPKD patients.

Conflict of interest: None declared.



 
   References Top

1.
Zerres K, Rudnik-Schöneborn S, Steinkamm C, Becker J, Mücher G. Autosomal recessive polycystic kidney disease. J Mol Med (Berl) 1998;76:303-9.  Back to cited text no. 1
    
2.
Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: The clinical experience in North America. Pediatrics 2003;111:1072-80.  Back to cited text no. 2
    
3.
Chapman AB, Rubinstein D, Hughes R, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. N Engl J Med 1992; 327:916-20.  Back to cited text no. 3
    
4.
Neumann HP, Krumme B, van Velthoven V, Orszagh M, Zerres K. Multiple intracranial aneurysms in a patient with autosomal recessive polycystic kidney disease. Nephrol Dial Transplant 1999;14:936-9.  Back to cited text no. 4
    
5.
Lilova MI, Petkov DL. Intracranial aneurysms in a child with autosomal recessive polycystic kidney disease. Pediatr Nephrol 2001;16:1030- 2.  Back to cited text no. 5
    
6.
Chalhoub V, Abi-Rafeh L, Hachem K, Ayoub E, Yazbeck P. Intracranial aneurysm and recessive polycystic kidney disease: The third reported case. JAMA Neurol 2013;70:114-6.  Back to cited text no. 6
    

Top
Correspondence Address:
Imed Helal
Department of Medicine A (M8), Charles Nicolle Hospital, Tunis
Tunisia
Login to access the Email id


DOI: 10.4103/1319-2442.265479

PMID: 31464260

Rights and Permissions


    Figures

  [Figure 1], [Figure 2]



 

Top
   
 
 
    Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  
 


 
    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures
 

 Article Access Statistics
    Viewed119    
    Printed0    
    Emailed0    
    PDF Downloaded24    
    Comments [Add]    

Recommend this journal