Early Surgical Management of Anatomically Complete Vascular Rings Brief Title: Screening for Anatomically Complete Vascular Rings

結構上完整性血管環之早期診斷及外科處置經驗

10.30096/CSMJ.200606.0010

陳豐霖(Fong-Lin Chen)；余榮敏(Jung-Min Yu)；蔡宗博(Tsung-Po Tsai)

雙主動脈弓 ； 右側主動脈弓 ； 大規模篩檢 ； 結構完整性血管環 ； Double aortic arch ； Right aortic arch ； Mass screening ； Anatomically complete vascular rings

中山醫學雜誌

17卷1期（2006 / 06 / 01）

93 - 98

英文

背景 先天性血管異常常導致氣管食道的壓迫及動脈瘤破裂，而具有致命性的威脅，但在成年期卻容易被誤診為氣喘或反覆性上呼吸道感染，然而血管環卻是一種死亡率低且可完全治癒的疾病。因此早期診斷就成為早期手術治療的主要關鍵，只依賴臨床症狀上的高度懷疑而要求醫師作為診斷指標，常是早期診斷的一大挑戰。二維心臟超音波則已被證實是檢查心臟和及大血管的有效工具。 目的 本研究旨在探討二維心臟超音波於結構上完整性血管環之能力，並因此而早期手術，並藉由術後的併發症及死亡率來評估手術結果。 方法 藉由二維心臟超音波的檢查，共有96位學童被診斷出患有結構上完整性血管環（包括雙主動脈弓及右位主動脈弓併左側動脈導管韌帶），經過食道鋇劑攝影、螺旋式電腦斷層及（或）核磁共振檢查後，確認有血管環之存在。在基本資料、過去病史主訴及理學檢查完成後，再以回朔方法比較在診斷結構上完整性血管環時，與傳統上依賴臨床症狀的高度懷疑之診斷方法上的不同。這些氣管食道壓迫的學童，有明顯的臨床症狀時，才須接受外科治療。 結果 在所有經過診斷確認為結構上完整性血管環的病人中，有64位學童接受外科手術治療，右側主動脈弓合併柯氏憩室瘤(Kommerell's divertiuculum)及左側動脈導管韌帶，是最普遍的類型，佔了80.3%。與傳統依賴氣管食道壓迫的臨床症狀的高度懷疑之診斷方法比較，二維心臟超音波在診斷率上較為準確。藉由二維心臟超音波診斷結構完整性血管環，整體的敏感度及特異度均為100%。而反覆性上呼吸道感染及氣喘性咳嗽是氣管壓迫的主要症狀，這些病人經過外科手術後均復原良好，沒有合併症及死亡。 結論 二維心臟超音波作為主要診斷方法，具有高度的敏感度及特異度，是一項有效的診斷工具。應用於結構上完整性血管環之早期診斷，是安全且準確的。

Background Adults with potentially life-threatening congenital vascular anomalies due to tracheoesophageal compression and aneurysmal rupture are often misdiagnosed as having asthma or recurrent bronchopulmonary infection. However, the early recognition and treatment of these anomalies can help to achieve a low mortality rate. Early surgical treatment critically depends on early diagnosis, which still presents a challenge to doctors as it depends on an index of high clinical suspicion. We showed two-dimensional echocardiography (2DE) to be an effective tool for heart and great vessel examinations as it has a higher success rate and eliminates human error to a large extent. Purposes We explored the use of 2DE for the early diagnosis of anatomically complete vascular rings (ACVR) and its use as a tool to help plan a surgical strategy by determining possible surgical complications. Methods Two-dimensional echocardiography was used to diagnose anatomically complete vascular rings (including double aortic arch and right aortic arch with left ligamentum arteriosum) in 96 patients. They underwent a barium esophagogram, spiral CT and/or MRI to confirm the existence of the vascular rings. Demographics, historical objectives and the results of physical examinations were all taken into account when performing the clinical diagnosis, as opposed to the conventional methods that are used when diagnosing ACVR. Those patients with overt clinical symptoms of tracheoesophageal compression were then earmarked for surgical treatment. Results Among the patients diagnosed with ACVR, 64 received surgical treatment after ACVR was confirmed. Right aortic arch (RAA) with Kommerell's divertiuculum (KD) with left ligamentum arteriosum was found to be the most common type of ACVR (80.3% of cases). The diagnostic accuracy of 2DE was higher than conventional methods that depend on the clinical suspicion of symptoms of tracheoesophagal compression. Two-dimensional echocardiography was found to have a 100% overall sensitivity and a 100% specificity with regard to its ability to diagnose ACVR. Recurrent bronchopulmonary infections and asthmatic cough were the dominant symptoms of tracheal compression and all of these patients who underwent surgical treatment were well without morbidity and mortality. Conclusions Two-dimensional echocardiography is an effective primary diagnostic method with a high sensitivity and specificity. Early surgical management of ACVR is safe and easily achieved.