Ruptured intracranial aneurysm in a patient with autosomal recessive polycystic kidney disease

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Aneurysmal rupture can result in devastating neurological consequences and can be complicated by comorbid disease processes. Patients with autosomal recessive polycystic kidney disease (ARPKD) have a low rate of reported aneurysms, but this may be due to the relative high rate of end-stage illnesses early in childhood. Authors here report the case of a 10-year-old boy with ARPKD who presented with a Hunt and Hess grade V subarachnoid hemorrhage requiring emergency ventriculostomy, embolization, and decompressive craniectomy. Despite initial improvements in his neurological status, the patient succumbed to hepatic failure. Given the catastrophic outcomes of subarachnoid hemorrhage in young patients, early radiographic screening in those with ARPKD may be warranted.

ABBREVIATIONS ADPKD = autosomal dominant polycystic kidney disease; ARPKD = autosomal recessive polycystic kidney disease; EEG = electroencephalography; EVD = external ventricular drain; MRA = MR angiography; POD = postoperative day.

Article Information

Correspondence Michael M. McDowell: University of Pittsburgh Medical Center, Pittsburgh, PA.

INCLUDE WHEN CITING Published online October 12, 2019; DOI: 10.3171/2018.8.PEDS18286.

J.L.P. and M.M.M. contributed equally to this paper.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.



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    Axial head CT scans demonstrating diffuse intraventricular hemorrhage, subarachnoid hemorrhage, and bilateral gyrus rectus hemorrhage with ventriculomegaly.

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    Left internal carotid artery injection angiograms demonstrate an 8 × 4–mm irregular aneurysm at the junction of the left anterior communicating artery with the A1 and A2 segments: anteroposterior (A), oblique precoiling (B), and oblique postcoiling (C) views.

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    Postprocedural head CT demonstrating worsened intraventricular hemorrhage and ventriculomegaly.



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