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Multiple aortic operations in loeys-dietz syndrome: report of 2 cases.

Na KJ, Park KH - Korean J Thorac Cardiovasc Surg (2014)

Bottom Line: Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often mis-diagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology.However, the differential diagnosis between these two connective tissue diseases is critical because they correspond to different surgical indications and surgical decision-making.We report two cases of successful thoracoabdominal aortic replacement in patients with previously undiagnosed Loeys-Dietz syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Thoracic and Cardiovascular Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine.

ABSTRACT
Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often mis-diagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology. However, the differential diagnosis between these two connective tissue diseases is critical because they correspond to different surgical indications and surgical decision-making. We report two cases of successful thoracoabdominal aortic replacement in patients with previously undiagnosed Loeys-Dietz syndrome.

No MeSH data available.


Related in: MedlinePlus

Computed tomography imaging of case 1. (A) 13 months before surgery, (B) at the time of rupture, and (C) postoperatively.
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f2-kjtcvs-47-536: Computed tomography imaging of case 1. (A) 13 months before surgery, (B) at the time of rupture, and (C) postoperatively.

Mentions: A thorough physical examination and review of previous CT images demonstrated hypertelorism, bifid uvula, arachnodactyly, and a tortuous aorta, which suggested Loeys-Dietz syndrome (LDS) rather than Marfan syndrome (Fig. 1A). Preoperative CT imaging showed a periaortic hematoma from the descending thoracic aorta to the abdominal aorta compatible with the concealed rupture of a thoracoabominal aortic aneurysm (Fig. 2).


Multiple aortic operations in loeys-dietz syndrome: report of 2 cases.

Na KJ, Park KH - Korean J Thorac Cardiovasc Surg (2014)

Computed tomography imaging of case 1. (A) 13 months before surgery, (B) at the time of rupture, and (C) postoperatively.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4279835&req=5

f2-kjtcvs-47-536: Computed tomography imaging of case 1. (A) 13 months before surgery, (B) at the time of rupture, and (C) postoperatively.
Mentions: A thorough physical examination and review of previous CT images demonstrated hypertelorism, bifid uvula, arachnodactyly, and a tortuous aorta, which suggested Loeys-Dietz syndrome (LDS) rather than Marfan syndrome (Fig. 1A). Preoperative CT imaging showed a periaortic hematoma from the descending thoracic aorta to the abdominal aorta compatible with the concealed rupture of a thoracoabominal aortic aneurysm (Fig. 2).

Bottom Line: Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often mis-diagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology.However, the differential diagnosis between these two connective tissue diseases is critical because they correspond to different surgical indications and surgical decision-making.We report two cases of successful thoracoabdominal aortic replacement in patients with previously undiagnosed Loeys-Dietz syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Thoracic and Cardiovascular Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine.

ABSTRACT
Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often mis-diagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology. However, the differential diagnosis between these two connective tissue diseases is critical because they correspond to different surgical indications and surgical decision-making. We report two cases of successful thoracoabdominal aortic replacement in patients with previously undiagnosed Loeys-Dietz syndrome.

No MeSH data available.


Related in: MedlinePlus