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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 (CT) imaging of case 2 (A) before the first operation, (B) six months after the first operation. (C) The postoperative CT after thoracoabominal aortic replacement and (D) the postoperative CT after the David operation. (C) Two pairs of intercostal arteries were implanted to a parallel longitudinal graft (white arrow).
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f3-kjtcvs-47-536: Computed tomography (CT) imaging of case 2 (A) before the first operation, (B) six months after the first operation. (C) The postoperative CT after thoracoabominal aortic replacement and (D) the postoperative CT after the David operation. (C) Two pairs of intercostal arteries were implanted to a parallel longitudinal graft (white arrow).

Mentions: An 18-year-old male was referred to Seoul National University Bundang Hospital for a rapidly increasing thoracoabominal aortic aneurysm. Seven months prior, the surgeon in the referring center replaced the proximal half of the descending thoracic aorta based on the diagnosis of acute type B dissection, an aberrant right subclavian artery, and Marfan syndrome. The maximal diameter of the remaining thoracic aorta, which had been 31 mm at the time of the first operation, was found to have increased to 63 mm in a postoperative CT scan taken six months later (Fig. 3). A physical examination revealed bifid uvula, hypertelorism, and a clubfoot deformity that had required multiple orthopedic operations (Fig. 1B). Moderate dilatation of the aortic root (diameter 41 mm) along with marked tortuosity of the entire aorta and its major branches were noted in the CT scan. Those findings led to the diagnosis being revised to LDS.


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

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

Computed tomography (CT) imaging of case 2 (A) before the first operation, (B) six months after the first operation. (C) The postoperative CT after thoracoabominal aortic replacement and (D) the postoperative CT after the David operation. (C) Two pairs of intercostal arteries were implanted to a parallel longitudinal graft (white arrow).
© Copyright Policy
Related In: Results  -  Collection

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

f3-kjtcvs-47-536: Computed tomography (CT) imaging of case 2 (A) before the first operation, (B) six months after the first operation. (C) The postoperative CT after thoracoabominal aortic replacement and (D) the postoperative CT after the David operation. (C) Two pairs of intercostal arteries were implanted to a parallel longitudinal graft (white arrow).
Mentions: An 18-year-old male was referred to Seoul National University Bundang Hospital for a rapidly increasing thoracoabominal aortic aneurysm. Seven months prior, the surgeon in the referring center replaced the proximal half of the descending thoracic aorta based on the diagnosis of acute type B dissection, an aberrant right subclavian artery, and Marfan syndrome. The maximal diameter of the remaining thoracic aorta, which had been 31 mm at the time of the first operation, was found to have increased to 63 mm in a postoperative CT scan taken six months later (Fig. 3). A physical examination revealed bifid uvula, hypertelorism, and a clubfoot deformity that had required multiple orthopedic operations (Fig. 1B). Moderate dilatation of the aortic root (diameter 41 mm) along with marked tortuosity of the entire aorta and its major branches were noted in the CT scan. Those findings led to the diagnosis being revised to LDS.

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