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.
Aortic Aneurysm ; Connective Tissue Diseases ; Diagnosis, Differential ; Humans ; Loeys-Dietz Syndrome* ; Marfan Syndrome ; Pathology ; Prevalence

BACKGROUND: Early recognition of thoracic aortic disease is critical for managing the patients to reduce morbidity and mortality. In emergency department(ED), transesophageal echocadiography(TEE) has recently challenged traditional diagnostic modality for assessing thoracic aortic disease such as computed tomogram(CT) scan, aortography and magnetic resonance imaging(MRI) because of it's safety, rapid acquisition time, high sensitivity and portability. The purpose of this study was to evaluate the diagnostic role of TEE as the first imaging modality in nontraumatic and traumatic thoracic aortic disease in emergency department. METHODS: From May 1993 to Feburary 1995, twenty six patients(nontrauma : 13 cases, trauma : 13 cases) who were suspicious of thoracic aortic disease and received tee as the first diagnostic tool in emergency department were enrolledd. Indications of emergency TEE in the suspicious thoracic aortic disease were typical chest pain, mediastinal widening or massive left side hemothorax. RESULTS: Of the 26 patients undergoing TE, CT scan were performed sequentially in patients(except 4 patients who died immediately in ED). Aortic pathology was detected in 18 patients (65%). Aortic pathology of nontraumatic patients were as followings : DeBakey type I dissction was in 4 cases(36%), type II dissection in 3 cases (28%), and type III dissection in 4 cases(36%). Six of 13 traumatic patients (46%) had thoracic aortic injury. Findings of aortic injury were aortic tear(2 cases), aortic aneurysm and aortic dissection in each. Of six traumatic thoracic aortic lesions which were detected by TEE, 4 lesions (2 aortic tear, subintimal hematoma, and aortic aneurysm) were not detected dby CT scan. Only 4 cases(15%) underwent emergency operation after TEE. There was no significant hemodynamic deterioration or procedure-related dddeath during TEE. CONCLUSION: In emergency department, TEE may be considered as the first diagnostic tool in detecting acute thoracic aortic disease. In considering of it's high sensitivity, no contrast injection, short procedure time and portability at bedside, TEE should be prefered in patients with suspected thoracic aortic disease from trauma.
Aortic Aneurysm ; Aortic Diseases* ; Aortography ; Chest Pain ; Diagnosis* ; Echocardiography, Transesophageal* ; Emergencies* ; Emergency Service, Hospital ; Hematoma ; Hemodynamics ; Hemothorax ; Humans ; Mortality ; Pathology ; Tomography, X-Ray Computed

To assess the regional difference and influence of the biological variables on atherosclerosis in female, we analyzed 7 segments of aorta (2 ascending, 3 thoracic, and 2 abdominal) from 90 superficially healthy Korean women (39+/-14 yr of age) who died from external causes. Tissue specimens were macroscopically examined and histopathologically divided into 7 grades for scoring (ATHERO, from 0=intact, to 6=thrombi formation). Lumen diameter (LD), wall thickness (WT), intima thickness (INT), and media thickness (MED) were obtained by computed morphometry. Atherosclerosis was common in the distal infrarenal (C2), proximal thoracic (B1), and proximal ascending (A1) segments. Total 95.6% of all subjects had atherosclerosis of variable degree in one or more segments, but an aneurysmal change was not found. The number of atherosclerotic segments and atherosclerosis score in the 7 segments increased with aging. However, the body size did not affect the aortic size and ATHERO. With aging, LD and INT of the A1, B1 and C2 increased (p<.00001); WT of the B1 and C2 increased (p<.01); and MED of C2 decreased (p<.01). LD and WT of the B1 and C2 (p<.05), INT of the A1, B1 and C2 (p<.00001) increased, and MED of C2 decreased (p<.01) with ATHERO. These data suggest that age is simple but a reliable parameter for estimating the progression of atherosclerosis.
Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Aorta/*anatomy & histology/pathology ; Aortic Diseases/*diagnosis/*pathology ; Atherosclerosis/*diagnosis/*pathology ; Autopsy ; Cadaver ; Disease Progression ; Female ; Humans ; Korea ; Middle Aged

Gastrointestinal complications (GI) after thoracoabdominal aortic repair can be classified as biliary disease, heptic dysfunction, pancreatitis, GI bleeding, peptic ulcer disease, bowel ischemia, paralytic ileus, and aortoenteric fistula. Theses complications are associated with high post operative morbidity and mortality. Most of the aortoenteric fistulae after thoracoabdominal aortic surgery are found at the duodenum, near the surgical site. These rare complications are caused by an indirect communication with abdominal aorta that originated from an aneursymal formation ruptured into the duodenum. Such aorto-duodenal fistula formation is considered as a result of inflammatory change from secondary infection near the surgical instruments. Herein, we report two cases of massive upper GI bleeding from aorto-duodenal fistulae and spontaneous lower GI perforation related to cytomegalovirus infection after abdominal aortic aneurysmal repair operations.
Aged ; Aged, 80 and over ; Aorta, Abdominal/*surgery ; Aortic Aneurysm, Abdominal/complications/*surgery ; Aortic Diseases/*diagnosis/surgery/virology ; Cytomegalovirus Infections/*complications/diagnosis/pathology ; Endoscopy, Gastrointestinal ; Gastrointestinal Hemorrhage/etiology ; Humans ; Intestinal Fistula/*diagnosis/surgery/virology ; Intestinal Perforation/*diagnosis/virology ; Male ; Vascular Fistula/*diagnosis/surgery/virology

Gastrointestinal complications (GI) after thoracoabdominal aortic repair can be classified as biliary disease, heptic dysfunction, pancreatitis, GI bleeding, peptic ulcer disease, bowel ischemia, paralytic ileus, and aortoenteric fistula. Theses complications are associated with high post operative morbidity and mortality. Most of the aortoenteric fistulae after thoracoabdominal aortic surgery are found at the duodenum, near the surgical site. These rare complications are caused by an indirect communication with abdominal aorta that originated from an aneursymal formation ruptured into the duodenum. Such aorto-duodenal fistula formation is considered as a result of inflammatory change from secondary infection near the surgical instruments. Herein, we report two cases of massive upper GI bleeding from aorto-duodenal fistulae and spontaneous lower GI perforation related to cytomegalovirus infection after abdominal aortic aneurysmal repair operations.
Aged ; Aged, 80 and over ; Aorta, Abdominal/*surgery ; Aortic Aneurysm, Abdominal/complications/*surgery ; Aortic Diseases/*diagnosis/surgery/virology ; Cytomegalovirus Infections/*complications/diagnosis/pathology ; Endoscopy, Gastrointestinal ; Gastrointestinal Hemorrhage/etiology ; Humans ; Intestinal Fistula/*diagnosis/surgery/virology ; Intestinal Perforation/*diagnosis/virology ; Male ; Vascular Fistula/*diagnosis/surgery/virology