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Spontaneous Bilateral Hemothoraces in Type IV Ehlers-Danlos Syndrome FREE TO VIEW

Roopa Naik*, MD; Gary Wahl, MD
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Rochester General Hospital, Rochester, NY

Chest. 2012;142(4_MeetingAbstracts):1044A. doi:10.1378/chest.1382357
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SESSION TYPE: Miscellaneous Student/Resident Cases II

PRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM

INTRODUCTION: Ehlers-Danlos Syndrome (EDS) is a rare autosomal dominant disorder of collagen production. Type IV EDS is caused by a mutation in the gene for type III procollagen. Complications range from skin and joint abnormalities to spontaneous rupture of arteries and hollow viscera. We report a case of bilateral hemothoraces due to rupture of intercostal arteries from retching in a patient with previously diagnosed type IV EDS.

CASE PRESENTATION: A 58-year-old lady presented to an outside hospital with retching and vomiting with sharp peri-umbilical pain, radiating to the upper back. Medical history was notable for type IV EDS, multiple abdominal surgeries due to spontaneous intestinal ruptures and permanent colostomy, multiple shoulder dislocations, hypertension and rheumatoid arthritis. There was no known family history of EDS. She was transferred to our facility due to a sudden rise in liver enzymes compared to those from her admission, in the setting of unexplained hypotension which responded to fluid resuscitation. At the time of admission, examination revealed diminished breath sounds at the lung bases and minimal right upper quadrant tenderness with negative Murphy’s sign. Liver enzymes were deranged, with aspartate aminotransferase 1217 U/L, alanine aminotransferase 1561 U/L and normal bilirubin and alkaline phosphatase levels. Complete blood count revealed mild thrombocytopenia (110,000/μL), hemoglobin of 7.3g/dl from a baseline of 13.8g/dl, and a normal white cell count. Serum amylase and lipase was normal, ANA, anti-smooth muscle antibodies, hepatitis panel were negative. Review of a fairly unequivocal CT abdomen from the outside hospital revealed suspected bilateral hemothoraces which were confirmed on CT chest. Abdominal sonography revealed normal hepatic and splenic venous anatomy. Video assisted thoracic surgery drained 1.2 liters of bloody fluid and revealed multiple hematomas around the intercostal arteries without active bleeding. Chest tubes were placed with minimal drainage, which were removed after 4 days, just prior to discharge. Liver enzymes started to trend down immediately and were attributed to shock liver caused due to acute hemorrhage.

DISCUSSION: Patients with type IV EDS may present with aneurysms, dissection, rupture and fistulae involving middle to large sized vessels. To our knowledge this is the first reported case of hemothoraces due to rupture of intercostal arteries in this patient population.

CONCLUSIONS: Clinicians should thus consider this diagnosis while evaluating patients with unexplained hemothoraces.

1) Germain DP. Ehlers-Danlos syndrome type IV. Orphanet J Rare Dis. 2007;2:32

2) Selim B et al. Spontaneous Hemothorax and Recurrent Hemoptysis in a 26-Year-Old Man With Skin Lesions. Chest 2010 137:2

DISCLOSURE: The following authors have nothing to disclose: Roopa Naik, Gary Wahl

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Rochester General Hospital, Rochester, NY




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