Spontaneous Renal Artery Dissection in Ehler–Danlos Syndrome

Spontaneous renal artery dissection is a rare condition with an often non-specific presentation, resulting in a challenging diagnosis for clinicians. This is the case of a 39-year-old man who presented with an acute-onset right flank pain, mild neutrophilia and sterile urine. CT of abdomen and pelvis showed a patchy hypodense area in the right kidney originally thought to represent infection. He was treated as an atypical pyelonephritis with antibiotics and fluids. When his symptoms failed to improve, a diagnosis of renal infarction was considered and CT angiogram of the aorta revealed a spontaneous renal artery dissection. He was managed conservatively with systemic anticoagulation, antihypertensive treatment and analgesia and discharged home with resolution of his symptoms and normal renal function.

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Renal artery dissection as an unusual cause of unilateral loin pain

BJUI ◽

10.1002/bjuiw-2012-083-web ◽

2012 ◽

Author(s):

Chris King

Keyword(s):

Renal Artery ◽

Artery Dissection ◽

Loin Pain

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Internal carotid artery dissection in a patient with Ehlers-Danlos syndrome type IV: diagnosis and management

Jornal Vascular Brasileiro ◽

10.1590/s1677-54492013000200015 ◽

2013 ◽

Vol 12 (2) ◽

pp. 174-179 ◽

Cited By ~ 1

Author(s):

Michel Nasser ◽

Murilo Bucci Vega ◽

Luca Giovani Antonio Pivetta ◽

Ana Izabel Nasser ◽

Debora Gusmao Melo

Keyword(s):

Carotid Artery ◽

Internal Carotid Artery ◽

Internal Carotid ◽

Vascular Complications ◽

Carotid Artery Dissection ◽

Artery Dissection ◽

Internal Carotid Artery Dissection ◽

Ehlers Danlos Syndrome ◽

Type Iv ◽

Danlos Syndrome

Ehlers-Danlos syndrome (EDS) type IV, also known as vascular EDS, is an inherited connective tissue disorder with an estimated prevalence of 1/100,000 to 1/250,000. In EDS type IV, vascular complications may affect all anatomical areas, with a preference for large- and medium-sized arteries. Dissections of the vertebral and carotid arteries in their extra- and intra-cranial segments are typical. The authors report the case of a patient with EDS type IV for whom the diagnosis was established based on clinical signs and who developed internal carotid artery dissection at the age of 44 years. In the absence of a specific treatment for EDS type IV, medical interventions should focus on symptomatic relief, prophylactic measures, and genetic counseling. Invasive imaging techniques are contraindicated, and a conservative approach to vascular complications is usually recommended.

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Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome

Case Reports in Critical Care ◽

10.1155/2015/804252 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4 ◽

Cited By ~ 3

Author(s):

Filipa Pereira ◽

Teresa Cardoso ◽

Paula Sá

Keyword(s):

Renal Artery ◽

Cardiopulmonary Arrest ◽

Fatal Outcome ◽

Vascular Complication ◽

Autosomal Dominant Disorder ◽

Ehlers Danlos Syndrome ◽

Vascular Type ◽

Recent Diagnosis ◽

The Right ◽

Danlos Syndrome

Ehlers-Danlos syndrome(EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in theCOL3A1gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome.

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