双胎胸腹联体畸形伴法洛四联症的早期外科手术纠治与随访

目的 探讨双胎胸腹联体伴法洛四联症(TOF)婴儿早期外科手术治疗的可行性及其效果.方法 术前经X线片、CT、MRI和B超等检查确诊,该联体婴儿共用1个肝脏,心包、膈肌、胸骨下段及第8～10前肋相连,胸膜紧贴,有2个心脏(B婴伴TOF)、各具独立的消化道和胆道系统.经积极术前准备,在营养和肝功能改善后,于出生第43天在多科协作下进行了联体分离手术.手术从一侧胸骨交界处至脐切开皮肤和腹壁进腹探查.术中见腹腔中有各自的消化道,共同肝相连的面积约6cm×5cm,将二婴胆总管作2条纵形线作为离断线,采用局部血流阻断共用肝离断方法,用超声刀进行肝脏分离.胸腔中见二婴儿的心脏共用一个心包,予以切开,直接缝合.胸骨、肋骨亦分别切开.伴TOF的B婴于分体术后0.5a实施Ⅱ期心脏畸形纠治术.术后随访患儿生长发育情况.结果 术后二婴儿皆存活,二婴儿于术后7h清醒,分别于术后24和26h拔除气管插管,术后二婴儿分别称体质量,A婴4100g,B婴3900g.B婴0.5a后的心脏畸形纠治手术顺利,恢复良好.术后随访3a,二婴儿生长发育与同龄儿童接近.结论 联体婴儿术前经正确评估、正确选用麻醉和手术方法、术后加强监护是手术成功的关键,分体术后0.5a后再行Ⅱ期心血管畸形纠治术是可行的.     

4例胸腹联体婴儿术前评估策略及手术时机探讨

目的总结胸腹联体婴的治疗经验,探讨联体婴儿的术前评估要点及治疗策略。方法回顾2009—2016年广州市妇女儿童医疗中心收治的4例胸腹联体婴儿的临床资料,分析影像学评估策略,并发畸形对手术时机的影响,手术分离技巧以及胸腹联体婴儿诊治流程。结果联体婴儿男童2例,女童2例,入院后均接受全面评估,4例均诊断为胸腹联体畸形。4例联体儿体桥内均可见肝脏相连,但有各自独立肝门、胆管及胆囊;胃肠道系统、泌尿系统完全独立无异常;均合并严重的先天性心脏畸形。1例因共用单个四腔心脏,放弃治疗后死亡;其余3例因循环不稳定导致肺炎,符合提前行分离手术指征,经多学科会诊后制定治疗方案;应用带蒂皮瓣转移联合补片方法修补体桥分离后的腹壁缺损,手术平均年龄为(35±6) d。成功分离6名个体,4名患儿术后出现伤口愈合不良,1名患儿于分离术后25 d行心脏畸形矫治术,平均住院时间(79±9) d。随访6个月至9年,1名于出院后半年因心脏畸形并发症死亡,其余5名患儿存活。结论通过对胸腹联体婴进行全面评估,多学科协作,严格掌握手术提前和急诊手术指征,制定精细体桥分离方案和序贯治疗并发畸形,可提高胸腹联体婴双胎存活率。     

联体双胎分离手术4例

目的总结4例联体儿分离手术的经验,为联体儿的诊治提供参考。方法回顾性分析2007年10月至2011年12月作者收治的4例联体儿治疗经过,其中3例为对称性联体,1例为不对性联体。共有3例手术成功,1例对称性联体儿未手术死亡。重点分析术前准备、手术时机、分离技术及术后监护。结果4例患儿中,胸腹联合畸形3例,骶尾部不对性联体1例;男性1例,女性3例。未手术的1例因复杂心脏畸形合并循环衰竭,丧失手术时机。1例不对称性联体儿分离手术在入院后3d进行,对称性联体儿分离手术时间分别为出生后16d、43d,均存活。一对现术后2年,1对现术后9个月,均发育良好。结论手术时机与分离方式必须依据每一对联体儿的具体情况而定,充分的术前准备可提高手术成功率。     

胸腹联体畸形的诊断与治疗进展

联体畸形是罕见的先天性畸形,其发病率约为1/5万～10万,其中胸腹联体发病率为73.5%,臀部联体为18.8%,坐骨联体为5.9%,头部联体为1.7%[1]。大多数联体儿在胚胎时或出生后即死亡,能成功进行分离,术后两婴儿存活的病例更罕见。充分的术前准备及合理的分离术式可提高患儿的存活率,现就胸腹联体畸形的诊断和治疗进展综述如下。     

影像学检查在臀部联体儿术前诊断中的价值

目的 探讨影像学检查在臀部联体儿术前评估中的价值,及其对分离手术方案和分离后患儿预后的意义.方法 对我院最近成功分离的1例臀部联体患儿,结临床查体选择进行B超、X线、CT、MRI检查,分析其影像学特点,以明确联体儿连接部位、范围、内容,以及是否伴有身体内部其他器官的畸形.结果 连接部前后径7.9 cm,上下径6.0 cm,位于腰骶部.双侧患儿腰3以下椎板裂,无骨性结构连接,但存在脊柱及骨盆的软骨连接.患儿下消化道及肛门独立,泌尿道独立.硬膜囊相连,但脊髓及马尾神经无相连及交叉支配.联体双患儿均存在动脉导管未闭,其中一侧患儿存在隐睾,未见其他畸形.术中所见证实影像学的结果.选择交叉V型皮瓣覆盖创面,直接缝合硬膜,联体儿成功分离,无术后感染及脑脊液漏,皮瓣愈合良好,无神经损伤,随访6个月,2例患儿发育及神经功能均正常.结论 根据联体儿分型,结合查体选择和利用各种影像学检查,能较全面地反映连接部位的范围、内容,对手术的难度预测、手术方案制定、术后预后评估有重要意义.     

先天性联体畸形

联体畸形是一种罕见的先天性畸形 ,临床上常根据连接部位分型 ,其中胸腹联体及剑脐联体比较多见。钡餐、B超、CT及MRI等检查可为内脏有无联接畸形提供影像参考依据。手术时机最好选择在出生 2～3个月进行。以两者胆总管为标志 ,在其中间均等位置设一条与两胆管平行的纵线为共用肝的离断线 ,可作为临床参考     

先天性联体畸形的外科处理

目的 总结4例联体儿分离术的经验,对联体儿诊治提供参考。方法 回顾性分析自1982～2001年,我院小儿外科共收治的4例先天性联体儿外科分离手术的临床资料,着重在手术时机、产前诊断、合并畸形、分离技术、术后监护、随访等方面。结果 4例联体儿畸形,其中胸部-脐联合畸形3例,坐骨联体1例,男性1例,余均为女性。均不同程度合并先天性心脏缺损,坐骨联体儿合并严重大血管畸形,泄殖腔畸形及Pieer-Robin综合征。2例产前超声检查获得诊断。2例紧急分离术时间为出生后7d内,存活1名。另2例延迟分离手术时间>1个月,均存活。随访存活的5名患儿,1982年分离的剑突-脐联体儿其中1名于术后4年“肺炎”死亡。另1名已20岁,健在。另一对胸部-脐联体儿术后1年发育良好。1例坐骨联体儿1名死亡,1名失随访。结论 手术时机与分离方式必需依据每一对联体儿局部环境及器官结构而定。紧急分离术存活率低,能延迟行分离术则存活率高。     

不阻断肝门小儿巨大肝肿瘤切除术探讨

目的小儿巨大肝肿瘤切除术中，常规行肝门阻断，本研究对不阻断肝门行巨大肝肿瘤切除术进行探讨。方法从2001年7月至2004年12月，我院对16例患儿施行不阻断肝门巨大肝肿瘤切除术，患儿年龄28d～14岁，平均3．4岁。其中男12例，女4例，肿瘤直径11～23cm，平均14．6cm，其中右半肝和左外叶内分别有一肿块1例。肝母细胞瘤9例，肝错构瘤4例，肝血管瘤3例。结果本组16例患儿手术全部成功，不阻断肝门行右半肝（Ⅴ～Ⅷ）切除8例，右三叶切除4例，左三叶切除1例，左半肝切除1例，第二肝门及右下叶肝段切除各1例。患儿术中无死亡，血流动力学指标稳定。本组良性巨大肝肿瘤患儿术后随访7个月～3．5年，目前均健康生存，无肿瘤复发，生长发育正常；肝母细胞瘤9例患儿术后常规化疗，患儿已经随访6～45个月，目前无瘤生存8例，2年以上4例，肝功能正常，正常生活。另外1例左外叶和右叶同时发现肝母细胞瘤，手术分别切除后5个月脑肺转移，于术后7个月死亡。结论不阻断肝门巨大肝肿瘤切除术，是一种安全可行的手术。术者熟练的肝脏解剖知识和肝切除技术、紧密结合术前和术中影像学技术了解肿瘤与大血管关系、彻底结扎肿瘤侧入肝肝动脉和门静脉及肝左右静脉共干或肝右静脉，是手术成功的关键。     

联合肝脏离断和门静脉结扎二步肝切除术在儿童肝肿瘤治疗中的应用

联合肝脏离断和门静脉结扎的二步肝切除(associated liver partition and portal vein ligation for staged hepatectomy,ALPPS)手术方案具有短期内残肝体积迅速增大的特点,在预估残肝体积(future liver remnant,FLR)不足的成人肝肿瘤手术中已获得推广。儿童肝脏肿瘤往往体积相对较大,尤其是肝母细胞瘤常侵犯多个肝段,或占据肝脏中央解剖部位,存在根治性肝切除术导致FLR不足的情况,有实施这一术式的价值。目前ALPPS在儿童肝脏肿瘤中的应用尚处于起步阶段。一般认为,术前评估FLR30%的病例可考虑实施本术式;术前应对患儿肝体积、肝功能以及肿瘤的可切除性进行精准评估;两次手术间隔时间以7~14 d为宜。手术并发症主要包括肝功能不足、出血和胆漏。关于儿童肝肿瘤中该术式的临床疗效评估尚待进一步总结。     

Ⅱ型Abernethy畸形4例诊治体会

目的评估Ⅱ型Abernethy畸形术前合并症、术后并发症, 探讨最佳治疗方法, 降低手术风险。方法回顾性分析2021年1月至2021年12月首都医科大学附属北京儿童医院收治的术前伴有严重合并症或术后出现严重并发症的4例Ⅱ型Abernethy畸形患儿的临床资料。其中男1例, 女3例;主要因血便、发绀、劳力性呼吸困难、转氨酶升高、肝脏肿瘤就诊。评估患儿术前合并症、术后并发症情况, 并对其进行随访。结果 4例患儿中, 3例有术前合并症, 其中肝脏肿瘤2例、肺动脉高压(术前均为中度)2例、肝性脑病2例、肝肺综合征(重度)1例。2例肝脏肿瘤患儿甲胎蛋白均在正常水平范围, 其中1例直径小未活检, 而另1例术前穿刺活检证实良性。4例患儿均行开腹分流关闭手术, 术中行阻断实验。分流阻断后, 3例患儿肠管无淤血, 门静脉压力在25 mmHg(1 mmHg=0.133 kPa)以下, 一期关闭分流;另1例患儿肠管淤血严重, 门静脉压力达36 mmHg, 分期关闭分流。术后常规给予肝素、低分子肝素序贯抗凝, 超声监测有无血栓。1例患儿术后第3天出现门静脉广泛血栓, 有轻微腹痛、腹胀, 给予全身溶栓、抗凝治疗...     

Congenital Heart Defects in Conjoined Twins: Outcome After Surgical Separation of Thoracopagus

In this study, we correlated cardiac malformations in the various types of conjoined twins with the proposed site of embryologic union and evaluated the possibility of survival after surgical separation. We conclude that surgical separation is only very rarely successful in twins with united hearts, whereas survival in twins with separate hearts is more dependent on the malformations of other viscera. This report deals primarily with conjoined twins in whom anomalies of the heart are the major factor in deciding whether surgical separation is feasible.     

Angiographic evaluation of conjoined twins

Two sets of conjoined twins were studied by angiocardiography and cerebral angiography. Conjoined heart was demonstrated in the thoracopagus twins and surgical separation was impossible. Cerebral angiography disclosed the separate circulations in craniopagus twins and surgical separation was performed.Presented at 14th Meeting of the European Society of Pediatric Radiology, Luzern, May 11–14, 1977     

Successful surgical repair of d-transposition of the great arteries in a separated conjoined twin

Abstract

Conjoined twins are very rare examples of congenital malformations. In 75% of thoracoomphalopagus conjoined twins, the intracardiac anatomy determines outcome and long-term survival.

Methods

We successfully separated one case of thoraco-omphalopagus conjoined twins, with one having d-transposition of the great arteries. After control of sepsis, the twin underwent an arterial switch and complete repair of her cardiac defect.

Results

She made a full recovery and was discharged from the hospital 20 days after surgery.

Conclusion

Careful planning, skillful surgical separation and cardiac surgery by a combined medical and surgical team is the key to save the twins in such a rare case.     

The contribution of modern imaging to planning separation strategies in conjoined twins.

BACKGROUND/AIM: An accurate knowledge of the often complex and unexpected anatomy of conjoined twins is required in order to devise appropriate surgical strategies for separation. The recent progress of imaging techniques prompts an assessment of their contribution to planning separation strategies. MATERIAL AND METHODS: From 1990, we treated 8 consecutive sets of conjoined twins: 1 acardiac-acephalus, 1 epigastric heteropagus, 2 omphalopagus, 2 omphalothoracopagus, 1 ischiopagus tetrapus and 1 parapagus tripus. The first two sets were separated straightforwardly after birth and were excluded. Ultrasonographic and plain and contrast X-ray studies were used in all the remaining sets, angiography in 5, CT in 4, and MRI in 4. Helical CT and magnetic resonance angiography (MRA) were used in 3 sets. RESULTS: Two sets of omphalopagus twins and one of omphalothoracopagus twins were separated after birth due to brain damage of one twin, cloacal exstrophy and large arterial shunting, respectively. They were assessed using barium studies (3 sets), IVP (3), angiography (2), CT (2) and MRI (1). Three of six babies survived after separation. In the second set of omphalothoracopagus ecocardiography and MRA revealed that the extent of cardiovascular sharing precluded separation. In the remaining two sets of twins respectively ischiopagus and parapagus, the extent of organ sharing was depicted using three-dimensional helical CT and MRI; the four babies are alive after separation. CONCLUSIONS: Conventional imaging contributes only modestly to separation planning. In contrast, advanced imaging techniques are amazingly efficient in depicting complex fusions that have to be accurately recognised when planning realistic separation strategies.     

Assessment of the cardiovascular system in conjoined thoracopagus twins

The thoracoomphalopagus conjoined twins comprise 75% of all conjoined twins. In the assessment of the organ systems of the twins, the cardiovascular system is important since union of this system in the twins has occurred in 75% of the cases. Of the five cases reported here, two had separate hearts and vessels with a common pericardium only. Two twins were joined at the right atrial level. The fifth pair were connected at both atrial and ventricular levels. It has been suggested that the presence of two separate QRS complexes in the ECG indicates complete separation of the two hearts. Case 4 shows that even in the presence of atrial union, the existence of an atrioventricular block in one twin may produce a very slow heart rate, while the other twin may have a normal rate that will not influence the ventricular rate of the other. In our experience, angiocardiography has been the most useful diagnostic procedure.     

Surgical management to conjoined twins in Shanghai area

Conjoined twins are very rare congenital malformation. The aim of this study was to summarize our experiences of surgical separation on seven sets of conjoined twins, and improve the treatment of conjoined twins in the future. A retrospective review of surgical separation included data of prenatal diagnosis, associated malformation, timing of separation, intra- and postoperative management, and follow-up of six sets of conjoined twins at Shanghai Xin-Hua Hospital from 1980 to 2005 and one set at Shanghai Children’s Hospital in 2002. Surgical separation was performed on seven sets of conjoined twins; six sets of thoracopagus–omphalapagus (including four sets of xipho-omphalopagus) and one set of ischiopagus. All sets presented varying degrees of severity of congenital cardiac malformations. Four sets were diagnosed prenatally by ultrasonography. Two sets of conjoined twins (case 2 and case 3) required emergent separation within 7 days after birth; both members of case 2 died within 2 days post operation, one member of case 3 died during operation while the other member survived. Five sets had scheduled separation undertaken more than 30 days after birth. One member of a set (case 6B) died 13 days after operation due to severe congenital cardiovascular anomalies. All other members of conjoined twins survived. Case 6A had a severe defect of the anterior thoracic cage and prosthesis of titanium alloy scaffold filled with silicone rubber was used to repair the defects successfully. Following up from 1980 to 2005, one member of a set (case 1A) died 4 years after operation due to pneumonia. Contact was lost to the surviving member of case 3 (ischiopagus). Other survivors of the separations had normal development. (1) Timing of operation and separation plan should be given according to the circumstances and the nature of the organ shared in each individual set of twins. (2) Prosthesis of titanium alloy scaffolds filled with silicone rubber may become one of viable methods for repairing severe defects of the thoracic cage.     

Fetal MRI of conjoined twins who switched their relative positions

Conjoined twinning is a very rare occurrence with no genetic predisposition. Twisting of conjoined twins around the axis of their connecting tissue bridge, close to the third trimester, has not been previously reported. We describe a unique case of in utero twisting of conjoined omphalopagus twins who survived without any adverse effects. Fetal US and fetal MRI played a vital role in the diagnosis and perinatal management of these twins.