Saudi Journal of Kidney Diseases and Transplantation

CASE REPORT
Year
: 2019  |  Volume : 30  |  Issue : 4  |  Page : 982--984

Ruptured intracranial aneurysm and recessive polycystic kidney Disease: A Rare Association


Imed Helal1, Insaf Handous2, Meriam Khadhar1, Hamida Bezzine3, Fethi Ben Hamida1, Taib Ben Abdallah3,  
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

Correspondence Address:
Imed Helal
Department of Medicine A (M8), Charles Nicolle Hospital, Tunis
Tunisia

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;30:982-984


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 2020 Jul 15 ];30:982-984
Available from: http://www.sjkdt.org/text.asp?2019/30/4/982/265479


Full Text



 Introduction



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



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}{Figure 2}

 Discussion



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

1Zerres K, Rudnik-Schöneborn S, Steinkamm C, Becker J, Mücher G. Autosomal recessive polycystic kidney disease. J Mol Med (Berl) 1998;76:303-9.
2Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: The clinical experience in North America. Pediatrics 2003;111:1072-80.
3Chapman AB, Rubinstein D, Hughes R, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. N Engl J Med 1992; 327:916-20.
4Neumann 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.
5Lilova MI, Petkov DL. Intracranial aneurysms in a child with autosomal recessive polycystic kidney disease. Pediatr Nephrol 2001;16:1030- 2.
6Chalhoub 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.