Challenges to cannulation for extracorporeal support in neonates with right-sided congenital diaphragmatic hernia

Right-sided diaphragmatic defects represent less than 20% of all congenital diaphragmatic hernias (CDH). Recent data suggest that right CDH (R-CDH) may carry a disproportionately high morbidity as well as increased rates of extracorporeal support when compared with left CDH. Treatment of infants with R-CDH may be further complicated by anatomical distortion unique to right-sided defects. We report 2 cases of azygous vein cannulation in neonates with large isolated R-CDH. Both infants had postnatal deteriorations within 48 hours, met our criteria for extracorporeal membrane oxygenation (ECMO), and underwent venoarterial cannulations through the right neck. In each case, the venous cannula passed directly into the azygous vein and failed to provide adequate ECMO support. Echocardiography confirmed both cases of azygous cannulation. In one child, the right atrium was successfully cannulated after 90 minutes of extensive cannula manipulation. This child survived a 5-day ECMO course and is alive at 22-month follow-up. In the second child, despite prolonged efforts at cannula repositioning, cannulation of the right atrium was not achieved. We did not offer central cannulation because of a rapidly deteriorating clinical course, with expiration in several hours. At autopsy, a dilated azygous vein was evident as a result of inferior vena cava compression by a malpositioned liver. The possibility of azygous vein cannulation may be increased in neonates with R-CDH and has not been previously reported. When evaluating infants with R-CDH for ECMO, clinicians must recognize the possibility of azygous cannulation and its potentially lethal consequences, and should anticipate alternative venous cannulation.     

Re-anastomosis of the anomalous segmental pulmonary vein during inferior bilobectomy

An anomalous pulmonary vein anatomy could represent a challenge for the thoracic surgeon. In these cases, the incidence of complications during lung surgery is increased, especially the need to perform a pneumonectomy due to a wrong section of the vascular elements. All attempts to reduce this risk must be undertaken, including techniques to restore normal venous drainage. We present a case of re-anastomosis of the posterior segmental vein of the right upper lobe draining into the lower pulmonary vein during a right lower bilobectomy for lung cancer.     

Interposition grafting of the lower lobe pulmonary artery with a cuff of azygous vein, following its accidental ligation and division

A patient with a mass lesion suggestive of a bronchial carcinoma in the right upper lobe was taken to theatre for a right upper lobectomy. The lower lobe artery, which was smaller than usual, was inadvertently divided. The arterial blood supply to the lower lobe was repaired with azygous vein as an interposition graft between the stump of the right apical segment arteries and the lower lobe artery. There were no signs of reperfusion injury post-operatively. We report a method that can be safely and relatively easily performed in these rare circumstances.     

Drainage patterns of middle lobe vein of right lung: an anatomical study.

OBJECTIVE: The purpose of the present study was to determine the variations in the drainage patterns of middle lobe vein of the right lung. METHODS: Right lungs of 30 formalin fixed cadavers, were dissected carefully to expose the variations in the venous drainage of their middle lobes. After identifying the pulmonary veins for each lobe, middle lobe vein (MLV) drainage patterns were followed to their openings. The diameters of the MLV and its lateral and medial parts were measured with a caliper. The length of the MLV trunk was also evaluated. RESULTS: Five different types of venous drainage patterns were observed. Type-I: Union of medial and lateral parts to form MLV as a trunk and opening of this vein to the right superior pulmonary vein (RSPV) (53.3%). Type-II: Opening of medial and lateral parts to the RSPV separately (16.6%). Type-III: Union of medial and lateral parts to form the MLV trunk and opening of this vein into the left atrium (16.6%). Type-IV: Opening of medial and lateral parts into the left atrium separately (10%). Type-V: Union of medial and lateral parts to form MLV trunk and opening of this vein to the right inferior pulmonary vein (3.3%). CONCLUSION: The venous drainage patterns of right middle lobe reveals great number of variations. Knowing the frequency of different types of drainage patterns classified in this study is extremely important for the surgeons performing pulmonary surgery, atrial fibrillation and imaging techniques.     

Surgical correction of a rare variant of cor triatriatum.

Cor triatratum is a rare congenital malformation. Problems in diagnosis and surgery arise due to associated anomalies such as atrial septal defects and anomalous pulmonary venous drainage. A 21-year-old man proved to have a rare variant of cor triatiatum in which both the upper and lower compartments of the left atrium communicated with the right atrium through two separate atrial septal defects. In addition, he had anomalous drainage of the right superior pulmonary vein into the superior vena cava. Intracardiac repair was undertaken successfully.     

Mediastinal hemangioma arising in setting of partial anomalous pulmonary venous drainage to azygous vein in a 57-year-old woman

Partial anomalous pulmonary venous drainage (PAPVD) to the azygous vein and benign posterior mediastinal hemangioma in adults are both rare entities in isolation. The coexistence of these two lesions in the same patient has not been reported. We describe a unique case of PAPVD to the azygous vein in an adult woman, where the anomalous left inferior pulmonary vein transited first through a large hemangioma, and then eventuated in the azygous vein.     

先天性心脏病伴体静脉异位引流的外科处理

为探讨先天性心脏病伴体静脉异位引流的特征和外科处理方法，回顾分析了１９８１～１９９６年１６年间４８例体静脉异位引流的类型和处理结果。其中永存左上腔静脉４０例，下腔静脉异位引流６例，肝静脉异位引流２例，随诊效果满意。作者认为，先天性心脏病合并体静脉异位引流，回流至右房者不需外科处理，但术中应建立良好的静脉引流；回流至左房者则具有外科意义。     

Pulmonary sequestration complicated by anomalies of pulmonary venous return

Five anomalies of pulmonary venous drainage were seen among 12 children operated for lung sequestration. In two children, venous drainage from the sequestrated lobe and the rest of the right lung was via a single channel into the inferior vena cava ("scimitar syndrome"). In one of these children, the sequestrated lobe was resected and repair of the scimitar syndrome was delayed; in the second patient, the anomalous pulmonary venous drainage was not recognized preoperatively and the vein was ligated, resulting in acute hemorrhagic infarction of the right lung and death of the patient. Three patients had less severe anomalies of pulmonary venous drainage. We recommend very careful evaluation of patients with lung sequestration with special reference to pulmonary venous drainage.     

Preliminary study of the anatomy of the venous drainage of the intrahepatic and extrahepatic bile ducts and its relevance to the practice of hepatobiliary surgery

Background : Although there have been many studies of the arterial supply of the biliary system, attempts to study the corresponding venous drainage have been few and all have been incomplete. The purpose of the present investigation is to describe the anatomy of the venous drainage of both the intrahepatic and extrahepatic bile ducts and to determine its relevance to hepatobiliary surgery. Methods : The intrahepatic and extrahepatic venous drainage of the bile ducts was investigated in seven specimens by injecting a solution of 10% gelatin coloured with Alcian blue into the portal vein or the superior mesenteric vein to outline the venous drainage. The specimens were dissected under loop magnification and representative drawings were obtained. Results : The surface of the intrahepatic and extrahepatic bile ducts was covered by a fine venous plexus. On the surface of the supraduodenal common hepatic duct and common bile duct the venous plexus drained laterally into marginal veins, usually two in number and known as the 3 o’clock and 9 o’clock marginal veins. Inferiorly the marginal veins and the venous plexus communicated with the pancreaticoduodenal venous plexus, which in its turn drained into the posterosuperior pancreaticoduodenal vein, a branch of the superior mesenteric vein. Superiorly the marginal veins divided into a number of branches. Some branches followed the left and right hepatic ducts into the liver, communicating with the venous plexus and the adjacent branches of the portal vein. Other branches of variable size entered either segment IV or the caudate lobe or process via the hilar venous plexus. A most important finding was that even after dividing the bile duct and all communicating veins at the upper border of the duodenum, the venous plexus and the marginal veins filled normally to the level of transection. This occurred almost certainly by retrograde filling from above. Conclusion : The satisfactory results of end‐to‐end anastomosis in whole liver transplantation depends partly on the presence of adequate venous drainage. This has been amply demonstrated by the injection studies. This would indicate that the poor results of end‐to‐end repair of the bile duct after surgical trauma results from other factors such as poor technique, devascularization of the cut ends due to trauma, and carrying out the anastomosis under tension. After resection of the hilum for cholangiocarcinoma the venous drainage of the left and right hepatic ducts and their branches depends mainly on the communications between the venous plexus on the ducts and the adjacent branches of the portal vein, even at a lobular or sinusoidal level. The satisfactory results obtained after anastomosis of the left and right hepatic ducts or their branches to a Roux loop of jejunum attest to this. This applies also to the transplantation of segments II and III in paediatric patients from related adult donors and in patients receiving split liver transplants. Finally, the venous drainage at the bifurcation of the common hepatic duct has been shown to enter the caudate lobe and segment IV directly. This suggests that a hilar cholangiocarcinoma may metastasize to these segments, and perhaps partly explain the significantly better long‐term results when the caudate lobe and segment IV are resected en bloc with the cholangiocarcinoma as part of modern radical surgery for this condition.     

Mixed type of total anomalous pulmonary venous return: a rare pattern of pulmonary venous drainage]

The occurrence of multiple drainage sites in total anomalous pulmonary venous return (TAPVR) has important implication in preoperative diagnosis and surgical treatment. We report a rare pattern of pulmonary venous drainage with the right upper pulmonary vein draining into the innominate vein and the other three pulmonary veins into the portal vein (Ib + III type). The preoperative diagnosis was made by echocardiography and confirmed by angiography. In operation, an anastomosis was made between the common pulmonary vein and the left atrium through posterior approach, but the right upper pulmonary vein was left uncorrected because the anomalously draining blood flow of a single pulmonary vein was about 20% of total pulmonary blood flow. The postoperative course was uneventful, however, the long-term follow-up is mandatory because of the right upper pulmonary vein being left uncorrected.     

成人间右半肝活体肝移植肝中静脉的处理

目的 总结成人间右半肝活体肝移植肝中静脉的处理经验。方法 回顾性分析两例成人间右半肝活体肝移植肝中静脉的处理方法，1例采取含肝中静脉的右半肝活体肝移植，肝中静脉移植供肝和剩余肝脏各保留一半，既有利于保证供体左肝内叶静脉回流，同时供肝右前叶静脉回流也不受到影响；另1例则采取不含肝中静脉的右半肝活体肝移植，术中重建供肝右前叶肝静脉回流通道，使供肝前叶淤血明显改善。结果 两例供体均存活，术后顺利出院，无并发症；受体1例存活，1例术后20d死于急性重度排斥反应。结论 成人间右半肝活体肝移植肝中静脉的处理十分关键，要注意保持供受体肝流出道的通畅，既要保证供体的绝对安全，又要尽量为受体提供足够的肝容量。     

Unusual total anomalous pulmonary venous connection; report of a case

We report a case of a 7-hour-old infant with total anomalous pulmonary venous connection having abberant origin of the vertical vein. His clinical condition presented hemodynamically severe pulmonary vein obstruction as common pulmonary vein atresia. Without making the definitive diagnosis, he underwent the operation at 11 hours after birth under cardiopulmonary bypass. During the operation, we could not identify the drainage vein. Although we ligated the vessel sized less than 2 mm in diameter draining into superior vena cava which was suspected to be the vertical vein. After the operation pulmonary edema was severe, but his clinical condition improved by using nitric oxide without extracorporeal membrane oxygenation (ECMO) support. Postoperative cardiac catheterization showed normal cardiac function without pulmonary venous obstruction, and moreover identified the drainage vein originated from right upper pulmonary vein. To the best of our knowledge, this is the first case where the vertical vein originated from right upper pulmonary vein. Immediate surgical treatment before the circuratory exacerbation improve the outcome for this congenital anomaly.     

Surgical treatment of the scimitar syndrome

Anomalous pulmonary venous drainage of the right lung into the inferior vena cava is an uncommon congenital defect. In 440 patients suffering from atrial septal defect (with or without anomalous pulmonary venous drainage) we could demonstrate the Scimitar-Syndrome in five cases. The described side-to-side-anastomosis between the anomalous vein and the left or right atrium is an alternative procedure to well-known operative methods.     

Branching Patterns and Drainage Territories of the Middle Hepatic Vein in Computer-Simulated Right Living-Donor Hepatectomies

Full right hepatic grafts are most frequently used for adult-to-adult living donor liver transplantation (LDLT). One of the major problems is venous drainage of segments 5 and 8. Thus, this study was designed to provide information on venous drainage of right liver lobes for operation-planning. Fifty-six CT data sets from routine clinical imaging were evaluated retrospectively using a liver operation-planning system. We defined and analyzed venous drainage segments and the impact of anatomic variations of the middle hepatic vein (MHV) on venous outflow from segments 5 and 8. MHV variations led to significant shifts of segment 5 drainage between the middle and right hepatic vein. In cases with the most frequent MHV branching pattern (n = 33), a virtual hepatectomy closely right to the MHV intersected drainage vessels that provided drainage for 30% of the potential graft, not taking into account potential veno-venous shunts. In individuals with inferior MHV branches that extend far into segments 5 and 6 (n = 10), the overall graft volume at risk of impaired venous drainage increased by 5% (p < 0.001). If this is confirmed in clinical trials and correlated with intraoperative findings, the use of liver operation-planning systems would be beneficial to improve overall outcome after right lobe LDLT.     

Management of congenital abnormalities of the donor lung

Congenital abnormalities were encountered in three donor lungs. A donor tracheal bronchus was incorporated into the right bronchial anastomosis. Anomalous pulmonary venous return of the right upper lobe to the superior vena cava and the left upper lobe to the innominate vein were managed by bridging the anomalous veins to the left atrial cuff with autologous pericardium and donor iliac vein, respectively.     

Congenital cystic adenomatoid malformation connected to an extralobar pulmonary sequestration in the contralateral chest: common origin?

Extralobar pulmonary sequestration (ELS) and congenital cystic adenomatoid malformation have been reported to coexist in several variations. This suggests a common embryologic origin. A 6-month-old boy presented with a history of recurrent pneumonias. The patient was diagnosed with a right lower lobe congenital cystic adenomatoid malformation (CCAM) and a left lower lobe ELS/CCAM. The diagnosis was made with the aid of a multidetector computed tomography (CT). Three-dimensional CT reconstruction showed the presence of a right lower lobe CCAM, a left lower ELS with an aberrant arterial supply from the celiac axis, and possible venous drainage into the right CCAM. The patient underwent a right thoracotomy. Intraoperatively, the lesions were discovered to be connected by a band of tissue. The right lower lobe CCAM and the left ELS were removed from the right chest. Histologic analysis confirmed the presence of a CCAM within the right lower lobe. The ELS had involvement of a type II CCAM within the sequestration. The connection between the right CCAM and left ELS/CCAM showed an anomalous conducting airway, anomalous vein, and anomalous artery connecting the 2 lesions. The authors present the first case of a CCAM connected to an ELS/CCAM in the contralateral hemithorax. The unique anatomic configuration of these lesions suggests a common embryologic origin of ELS and CCAM.     

Techniques of reconstruction of hepatic veins in living-donor liver transplantation, especially for right hepatic vein and major short hepatic veins of right-lobe graft

Living-donor liver transplantation (LDLT) is now widely accepted as a therapeutic option for adult patients with acute and chronic end-stage liver disease. In the early period, the left lobe was the major liver graft used in adult LDLT to ensure donor safety, especially in Eastern countries. However, the frequent extremes of graft-size insufficiency in left-lobe LDLT represented a greater risk of small-for-size graft syndrome in the recipient, which has focused attention on transplantation of the right lobe from a living donor. The major concern of right-lobe LDLT has focused on its safety for the donor and the necessity for including the middle hepatic vein (MHV) in the graft to avoid congestion of the right anterior segment. The MHV carries out important venous drainage for the right anterior segment and is essential for perfect graft function. The decision of whether to take the MHV with the liver graft (extended right lobe graft) or whether to retain it in the donor, with reconstruction of the MHV tributaries in the liver graft (modified right lobe graft) has been extensively discussed in numerous studies. However, adequate right hepatic vein and major short hepatic vein (middle and inferior right hepatic vein [RHV]) drainage of the liver graft is perhaps equally important as MHV outflow drainage for the integrity of right-lobe graft function. Herein, the author describes various techniques of venoplasty of the right hepatic vein (RHV) and the major short hepatic veins to obviate venous outflow obstruction in these veins.     

Relation between Hepatic and Portal Veins in the Right Paramedian Sector: Proposal for Anatomical Reclassification of the Liver

Although a right liver graft without a middle hepatic vein resulted in potential venous congestion in the right paramedian sector, the details of the hepatic venous distribution in the right paramedian sector have not been established. In this study, the ramification patterns of the hepatic veins draining the right anterosuperior segment (S8) and the relation between the hepatic and portal veins were assessed using multislice computed tomography in 44 patients without lesions in the liver. All 52 drainage veins of the ventral area of S8 joined the middle hepatic vein, and all 48 drainage veins of the dorsal area joined the right hepatic vein. The hepatic vein crossing between the ventral and dorsal areas was observed in each patient examined. Therefore, we propose a reclassification wherein the right paramedian sector is divided into ventral and dorsal segments. This new classification may contribute to the development of new and safer surgical procedures, including more limited resection and right lobe adult living donor liver transplantation to avoid graft congestion.     

Hepatic venoplasty in living-donor liver transplantation using right lobe graft with middle hepatic vein

Inclusion of the middle hepatic vein in a right lobe graft from a living-donor may improve venous drainage and avoid graft dysfunction, but reconstruction of the middle hepatic vein is technically difficult. We developed a hepatic venoplasty technique, which was applied in eight consecutive right lobe liver transplantations. The right and middle hepatic veins of the graft were joined together to form a triangular cuff for a single anastomosis to the recipient's inferior vena cava. Hepatic venoplasty was successful in all cases, and no interposition graft was required. Venovenous bypass was not used. All grafts showed immediate function, and no hepatic venous outflow obstruction was observed. There was no reoperation and the graft survival rate was 100%. This hepatic venoplasty technique can be applied systemically as a standard one in right lobe liver graft with the middle hepatic vein to simplify the recipient hepatectomy and to obviate venous outflow obstruction.     

Partial anomalous pulmonary venous connection in right lung cancer: Report of a case

A partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital anomaly which is frequently associated with congenital heart disease such as an atrial-level shunt. This report documents the case of an 81-year-old man with PAPVC which was incidentally discovered during a right upper lobectomy for lung cancer. Surgery was performed through a minithoracotomy of an auscultatory triangle using a video-assisted procedure (video-assisted thoracic surgery: VATS). Although the ramus lobi medii was connected normally, the right superior lobe vein was found to drain into the superior vena cava. The surgery was successful, and the patient had an uneventful postoperative course. Asymptomatic PAPVC without an atrial septal defect (ASD) is extremely rare. If the PAPVC is located in a different lobe, a pulmonary resection for lung cancer would precipitate an adverse outcome without a correction of the PAPVC. Surgeons should therefore be cautious regarding the potential existence of a PAPVC when a patient undergoes surgical procedures, especially VATS, for lung cancer.