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平陽霉素結合無水乙醇介入治療體表血管畸形的護理

目的 采用平陽霉素結合無水乙醇選擇性治療體表血管畸形，通過加強圍手術期的護理，從而提高患者依從性及臨床療效。 方法 2005年5月-2008年8月對53例體表血管畸形患者，使用平陽霉素與碘化油的混懸液結合無水乙醇進行治療。 結果 治愈32例， 治愈率為60.4%；好轉19例， 占35.8%；無效2例，占3.8%。未發現顯著的不良反應。 結論 選擇性治療體表血管畸形具有簡便、療效顯著、微創的特點，在介入手術全程，給予有針對性的圍手術期護理，可提高患者的依從性，有效預防并發癥，促進患者早日康復。

泡沫硬化劑治療皮膚血管瘤及血管畸形的臨床療效

目的觀察泡沫硬化劑注射治療皮膚血管瘤及血管畸形的療效。 方法2011年11月至2014年11月期間，對筆者所在醫院科室收治的53例不同類型的皮膚血管瘤及血管畸形患者采用泡沫硬化劑瘤內注射治療，根據患者年齡、瘤體大小和深度決定用藥劑量和注射次數，注射最大劑量不超過6 mL/次，2次注射的間隔時間為2～4周。 結果53例患者中男20例，女33例；年齡1個月～40歲，中位年齡1.6歲；瘤體大小為1.0 cm×（1.0～2.0）cm×5.0 cm。53例患者均完成全程治療，注射治療1～6次，平均注射治療3次，并經6個月隨訪，其中達到Ⅰ級療效者2 例（3.8%），Ⅱ級療效者5例（9.4%），Ⅲ級療效者10例（18.9%），Ⅳ級療效者36例（67.9%）；1例出現局部皮膚破潰，經換藥處理后創面愈合。 結論泡沫硬化劑注射治療皮膚血管瘤及血管畸形安全有效。

Value of DSA in Diagnosis and Interventional Treatment of Intestinal Vascular Malformalion

目的：探討DSA診斷小腸血管畸形的價值。方法：本文分析21例小腸血管畸形患者的臨床及DSA特征，其中男性14例，女性7例，所有患者均行腸系膜上、下動脈造影。結果：臨床特征：①急性消化道出血為主癥狀；②常規檢查一般為陰性；③血紅蛋白含量短期內降至4～6 g/mL。DSA特征：①動靜脈瘺；②局部腸壁染色增濃；③局部血管異常增多，結構紊亂。其中12例進行了動脈導絲栓塞，2例栓塞后出血，進行外科手術切除。結論：DSA是診斷血管畸形所致小腸出血的最有效的方法，動脈導絲栓塞是安全，有效的治療方法，同時為外科手術切除提供的正確部位。

后顱凹廣泛靜脈畸形致水腫誤診為視盤血管炎一例

Clinical analysis of unroofed coronary sinus syndrome with endocardial cushion defect

Objective To analyze pathologic features and surgical procedures for patients with unroofed coronary sinus syndrome (UCSS) associated with endocardial cushion defect (ECD). Methods The clinical data of 44 patients with UCSS and ECD from May 1998 to July 2016 were retrospectively reviewed. There were 18 males and 26 females with a mean age of 10.4±12.1 years (range: 5.0 months to 44.0 years) and mean weight of 25.2±20.9 kg (range 5.2-80.0 kg). According to the Kirklin and Barratt-Boyes classification, 28 patients were categorized into type Ⅰ, 5 typeⅡ , 4 type Ⅲ and 7 type Ⅳ. Among them 25 patients suffered partial ECD, 10 complete ECD, 9 transitional ECD, and 27 were associated with single atrium, 34 involved persistent left superior vena cava (PLSVC), and in 27 of the 34 patients PLSVC directly drained into the left atrium (LA). Among the 44 patients, 1 patient associated with complex anomalies underwent palliative operation, and other cardiac malformations were corrected simultaneously by surgical correction. PLSVC was ligated in 2 patients, and the intracardiac tunnels or baffles to drain PLSVC to right atrium (RA) were reconstructed in 25 patients. The associated cardiac lesions were corrected concomitantly. Results In-hospital death occurred in 2 patients, among whom 1 died of low cardiac output syndrome on postoperative day 8 and the other pulmonary infection on postoperative day 21. Thirty-one were followed up from 1 month to 10 years, and there was no death or severe complications. Conclusion When ECD is associated with PLSVC and a single atrium, UCSS may develop. Repair according to the type of UCSS is effective.

Perioperative Management of Anomalous Origin of the Left Coronary Artery from Pulmonary Artery

ObjectiveTo summarize perioperative management experience of 16 patients undergoing surgical correction of anomalous origin of the left coronary artery from pulmonary artery (ALCAPA). MethodsWe performed a retrospective analysis of 16 patients who received surgical correction of ALCAPA between January 2005 and December 2013 in Cardiovascular Center of Children's Hospital of Fudan University. There were 6 males and 10 females with their age ranging from 2 months to 13 years (mean age of 23.25±0.63 months) and body weight ranging from 5 to 30 kg (mean body weight of 19.77±0.75 kg). All the patients underwent direct implantation of the anomalous coronary artery into the ascending aorta, and received comprehensive management regarding left ventricular ejection fraction (LVEF), heart rate, blood pressure, arterial blood gas, urinary volume, blood lactic acid and central venous pressure. ResultsPostoperative complications were low cardiac output syndrome (LCOS) in 9 patients (including intractable LCOS in 2 patients) and arrhythmia in 1 patient. One patient with intractable LCOS and arrhythmia died postoperatively with the mortality of 6.25%. Cardiopulmonary bypass time was 85-260 (135.61±35.01) minutes, aortic cross-clamping time was 40-97 (57.32±16.02) minutes, mechanical ventilation time was 34-187 (106.34±41.62) hours, length of CICU stay was 2-21 (8.13±5.02) days, and hospital stay was 12-51 (22.14±5.00) days. Postoperative LVEF and left ventricular fractional shortening were significantly higher than preoperative values (P < 0.05). ConclusionKey points for successful surgical correction of ALCAPA include meticulous perioperative management, preoperative heart function improvement, prevention of postoperative complications and use of new techniques.

A PRELIMINARY STUDY ON CLINICAL FEATURE OF INFANTILE HEMANGIOMA AND VASCULAR MALFORMATION

Objective To study the cl inical features of infantile hemangioma and vascular malformation, to find out a proper strategy of deal ing with them. Methods From March 2000 to August 2007, 2 957 cases of infantile hemangioma and vascular malformation were treated, including 860 operative cases and 2 097 non-operative cases. There were 441 male and419 female patients in operation group, aging 6 months to 18 years (median 5 years). In 1 950 hemangioma patients of nonoperation group, there were 575 male and 1 375 female patients, aging 1 month to 14 years (median 6 months); in 147 vascular malformation patients of non-operation group, there 67 male and 80 female patients, aging 2 years to 17 years (median 7 years). In non-operative group, 147 vascular malformation patients and 1 525 infantile hemangioma patients were followed up without any medical intervention, while other 425 hemangioma patients recceived triamcinolone plus dexamethasone intralesional injection treatment. All the treatments and outcomes were recorded. Results Vascular malformation cases and infantile hemangioma cases presented totally different cl inical features. To the deadl ine of this study, 522 (34%) of 1 525 un-intervented hemangioma cases turned into involuted phase and 383 (90%) of 425 cases receiving triamcinolone plus dexamethasone intralesional injection treatment turned into involuted phase after injection treatment; no regression was noted in 147 cases of vascular malformation. The constituent ratio of infantile hemangioma in 860 operative cases was decreased gradually and the constituent ratio of vascular malformation was increased gradually as the age increasing. Conclusion Infantile hemangioma has a distinct l ife pattern. Except several specific cases need medical intervention for their special location or large ambit and unacceptable growth, most infantile hemangioma need no medical intervention. Most vascular malformations can not regress spontaneously, proper intervention is in need.

Brain Stem Cavernous Hemangioma: Clinical Manifestations and Prognostic Analysis

【摘要】　目的　探討腦干海綿狀血管瘤患者臨床表現及影響預后的相關因素。　方法　回顧分析2008年9月-2010年9月27例腦干海綿狀血管瘤患者臨床資料。　結果　20例（74.1%）患者CT顯示出血及血腫。急性或突然神經功能廢損22例，漸進性功能障礙15例，病情平穩4例。顯微手術12例，放射治療6例，保守觀察9例。長期隨訪25例，2例再次出血，無新發病灶，無患者死亡。手術組患者年齡（Plt;0.05）、術前病情嚴重程度（Plt;0.05）與術后KPS評分相關。非手術組不良預后評分與患者高齡及再出血有關。　結論　展神經麻痹和面癱患者功能恢復較差。年齡、術前病情嚴重程度及手術時機影響患者預后，術中全切病灶對復發和并發癥發生起主要作用，如何處理合并發生的靜脈畸形有待討論。立體定向放射治療明顯降低再出血風險，但其適應證尚有爭議。【Abstract】　Objective　To discuss the clinical manifestations and prognostic factors of brain stem cavernous hemangioma.　Methods　Based on the literature, the clinical data of 27 patients having brain stem cavernous hemangioma from September 2008 to September 2010 were reviewed and analyzed.　Results　Twenty patients (74.1%) presented with hemorrhage and hematoma in CT scan. Acute or sudden neurological deterioration occurred to 22 patients, progressive in 15 and stable in 4. Twelve patients underwent surgical removal of the lesion; 6 underwent radiosurgery; and 9 continued with conservative management. Twenty-five patients were followed up. Two patients had rehaemorrhagia. There were no de novo lesions or death. The risk factors indicative of a possible poor postoperative KPS score in the operative group included age (Plt;0.05) and the initial clinical condition (Plt;0.05). In the non-operative group, old age and rehaemorrhagia were obviously related to the poor outcome.　Conclusions　Patients with abducens and facial palsy have poor functional recovery. Age, the initial clinical condition and timing of operation are the major factors correlated to surgical outcome. The factor that affects recurrence and the occurrence of complications is complete resection during the operation. How to deal with the concomitant venous malformation should be further studied. The indications for stereotactic radiosurgery are still controversial, although it has confered a reduction in the risk of rehaemorrhagia.

下肢血管畸形的手術治療

目的 總結下肢血管畸形的手術方法及療效。 方法 2012 年 7 月—2015 年 7 月，收治下肢血管畸形患者 138 例。男 51 例，女 87 例；年齡 3～55 歲，平均 28.3 歲。靜脈畸形 98 例，動靜脈畸形 40 例。病變部位：臀部 24 例，大腿 30 例，臀部及大腿 23 例，小腿 35 例，大腿及小腿 9 例，累及整個下肢 17 例。其中 106 例行單純手術切除，32 例行術前髂外動脈球囊栓塞+手術切除。 結果 術后 4 例植皮區發生部分皮片壞死，經換藥后 Ⅱ 期愈合；余患者皮瓣及移植皮片均順利成活，創面及供區切口均 Ⅰ 期愈合。患者均獲隨訪，隨訪時間 4 個月～3 年，平均 18 個月。手術治愈率 84.8%（117/138）。復發患者均給予局部注射魚肝油酸鈉后，無再次復發。 結論 在嚴格掌握手術指征前提下，單純手術切除或聯合術前髂外動脈球囊栓塞可有效治療下肢血管畸形。

Features of Cardiacvascular Abnormalities and Clinical Results in Patients with Fontan Operation for Heterotaxy Syndrome

ObjectiveTo summarize the cardiac-vascular abnormalities and clinical results in patients with Fontan operation for heterotaxy syndrome. MethodWe retrospectively analyzed the medical records of 81 patients who underwent the Fontan operation with heterotaxy syndrome between September 2008 and September 2013. There were 49 male and 32 female patients at age of 3.79 (range 2.07-13.02) years with preoperative room air saturation of 81% (range 63%-97%) and weight of 14.8 (10.0-36.0) kg. ResultsThere were 70 patients in the right atrial isomerism group and left in 11 patients. Dextrocardia was seen in 16 patients, and single atrium in 50 patients. Single ventricle was found in 44 patients, conotruncal defects in 40 patients, pulmonary stenosis in 72 patients, pulmonray atresia in 8 patients, common atrioventricular valve in 61 patients, and bilateral superior vena cava in 54 patients. The staged Fontan procedure was applied in 57 patients and one stage in 24 patients. Operation strategies were included intra/extracardiac conduit (n=17), extracardiac conduit (n=48), lateral tunnel (n=14) and direct cavopulmonray connection (n=2).There were 15 early deaths. Postoperative complications included low cardiac output in 15 patients, hepatic insufficiency in 35 patients, renal insufficiency in 55 patients required peritoneal dialysis and arrhythmia in 28 patients. The room air saturation was 89% (range 78%-98%) before discharge and time of follow-up was from 2 months to 6 years in 64 patients. Thromboembolic events were found in 2 patients who had accomplished conduit replacement operation. ConclusionCompared with reported literatures in western countries, heteraotaxia patients are mostly with right atrial isomerism. Fontan palliation is still the main treatment option and strict indication was needed for satisfactory clinical results.