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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.

Brain Stem Cavernous Hemangioma: Clinical Manifestations and Prognostic Analysis

【摘要】　目的　探讨脑干海绵状血管瘤患者临床表现及影响预后的相关因素。　方法　回顾分析2008年9月-2010年9月27例脑干海绵状血管瘤患者临床资料。　结果　20例（74.1%）患者CT显示出血及血肿。急性或突然神经功能废损22例，渐进性功能障碍15例，病情平稳4例。显微手术12例，放射治疗6例，保守观察9例。长期随访25例，2例再次出血，无新发病灶，无患者死亡。手术组患者年龄（Plt;0.05）、术前病情严重程度（Plt;0.05）与术后KPS评分相关。非手术组不良预后评分与患者高龄及再出血有关。　结论　展神经麻痹和面瘫患者功能恢复较差。年龄、术前病情严重程度及手术时机影响患者预后，术中全切病灶对复发和并发症发生起主要作用，如何处理合并发生的静脉畸形有待讨论。立体定向放射治疗明显降低再出血风险，但其适应证尚有争议。【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.

下肢血管畸形的手术治疗

目的 总结下肢血管畸形的手术方法及疗效。 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；年龄 3～55 岁，平均 28.3 岁。静脉畸形 98 例，动静脉畸形 40 例。病变部位：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，累及整个下肢 17 例。其中 106 例行单纯手术切除，32 例行术前髂外动脉球囊栓塞+手术切除。 结果 术后 4 例植皮区发生部分皮片坏死，经换药后 Ⅱ 期愈合；余患者皮瓣及移植皮片均顺利成活，创面及供区切口均 Ⅰ 期愈合。患者均获随访，随访时间 4 个月～3 年，平均 18 个月。手术治愈率 84.8%（117/138）。复发患者均给予局部注射鱼肝油酸钠后，无再次复发。 结论 在严格掌握手术指征前提下，单纯手术切除或联合术前髂外动脉球囊栓塞可有效治疗下肢血管畸形。

泡沫硬化剂治疗皮肤血管瘤及血管畸形的临床疗效

目的观察泡沫硬化剂注射治疗皮肤血管瘤及血管畸形的疗效。 方法2011年11月至2014年11月期间，对笔者所在医院科室收治的53例不同类型的皮肤血管瘤及血管畸形患者采用泡沫硬化剂瘤内注射治疗，根据患者年龄、瘤体大小和深度决定用药剂量和注射次数，注射最大剂量不超过6 mL/次，2次注射的间隔时间为2～4周。 结果53例患者中男20例，女33例；年龄1个月～40岁，中位年龄1.6岁；瘤体大小为1.0 cm×（1.0～2.0）cm×5.0 cm。53例患者均完成全程治疗，注射治疗1～6次，平均注射治疗3次，并经6个月随访，其中达到Ⅰ级疗效者2 例（3.8%），Ⅱ级疗效者5例（9.4%），Ⅲ级疗效者10例（18.9%），Ⅳ级疗效者36例（67.9%）；1例出现局部皮肤破溃，经换药处理后创面愈合。 结论泡沫硬化剂注射治疗皮肤血管瘤及血管畸形安全有效。

平阳霉素结合无水乙醇介入治疗体表血管畸形的护理

目的 采用平阳霉素结合无水乙醇选择性治疗体表血管畸形，通过加强围手术期的护理，从而提高患者依从性及临床疗效。 方法 2005年5月-2008年8月对53例体表血管畸形患者，使用平阳霉素与碘化油的混悬液结合无水乙醇进行治疗。 结果 治愈32例， 治愈率为60.4%；好转19例， 占35.8%；无效2例，占3.8%。未发现显著的不良反应。 结论 选择性治疗体表血管畸形具有简便、疗效显著、微创的特点，在介入手术全程，给予有针对性的围手术期护理，可提高患者的依从性，有效预防并发症，促进患者早日康复。

Forensic Pathological Analysis of Death from Cerebrovascular Malformation

ObjectiveTo provide references in the forensic identification of injury and cerebrovascular malformation involved death cases, and to reduce the relevant medical dispute by exploring the forensic pathological features, identification of medical dispute as well as relationship between injury and disease. MethodsWe collected 33 cases of cerebrovascular malformation from January 2006 to December 2014 in West China Center of Forensic Medicine, including details of cases, clinical medical record and forensic pathology examination, and then the cases were retrospectively analyzed. ResultsIn the 33 cases, the average age of the individuals was 37.4 years old, and the male/female ratio was 23/10. Nineteen patients (57.6%) died within 1 hour. Seventeen patients with mixed pathological type of cerebrovascular malformation dominated (51.5%). Medical dispute happened in 7 cases (21.1%), 4 of which were identified to be led by medical fault and 3 with no medical fault. Relationship between injury and disease was analyzed in 11 cases (33.3%), in which injury was identified to take full responsibility in 1 case, inductive cause of death in 9 cases, and no relationship between injury and death in 1 case. ConclusionComprehensive and systematic investigation of forensic pathology plays an important role in the proper settlement of medical disputes as well as the identification of cause of death and relationship between injury and disease.

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.

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.

脊髓出血

脊髓出血是脊柱外科急症之一，该病较为罕见，病情复杂，来势凶险，不及时给予恰当的治疗，会导致患者死亡或者永久性神经功能障碍。及早抢救治疗有望降低病死率，减少后遗症，故临床医生对该病应有一定的认识并保持高度的警惕。现对脊髓出血的病因和发病机制、临床表现、辅助检查、诊断及治疗等进行归纳总结，为早期发现治疗脊髓出血提供科学的理论依据。

后颅凹广泛静脉畸形致水肿误诊为视盘血管炎一例