Emergency Situations

Following is information regarding potential emergency situations that can arise in someone with Loeys-Dietz Syndrome.

Loeys-Dietz syndrome is an aggressive aneurysm syndrome that significantly increases risk for AORTIC & ARTERIAL (head through pelvis) DISSECTION and other complications.

Cardiovascular

AORTIC or ARTERIAL DISSECTION is a potentially life threatening complication related to aortic aneurysm. Symptoms of aortic dissection include sudden severe chest pain, migrating to the chest, neck, back, abdomen and/or an extremity. Some individuals with aortic dissection have reported nausea, vomiting, shortness of breath, and collapse. Symptoms of aortic dissection warrant activation of the EMS (call 911), and transport to the nearest hospital for aortic IMAGING (CTA, MRA, echocardiography) to confirm or exclude dissection, for stabilization, and appropriate treatment. Stroke symptoms may present with neck artery dissections.

Pulmonary

SPONTANEOUS PNEUMOTHORAX results from rupture of pulmonary blebs. Blebs form due to increased air spaces in the lung. Symptoms of spontaneous pneumothorax include chest, neck or back pain exacerbated by deep breathing, or difficult breathing due to pain. HEMOPTYSIS of unknown etiology has also been seen in LDS.

Hollow organ rupture

Hollow organ rupture or tear of the spleen or uterus has been reported rarely. Bowel rupture may be a very rare complication.

Ocular

RETINAL DETACHMENT is associated with sudden visual field loss. Although retinal detachment is not life threatening, unrecognized/untreated detachment can result in blindness. Therefore, sudden visual disturbance warrants emergency evaluation.

 

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