Intralobar pulmonary sequestration

Sequestration is defined as an area of abnormal pulmonary tissue not connected with the bronchial tree, supplied by an aberrant systemic artery and without a normal pulmonary function. Extralobar (ELS) and intralobar (ILS) forms are distinguished. During the year 2002 the authors diagnosed and operated upon two cases of the intralobar form of pulmonary sequestration, and in last 25 years five cases - 4 x ILS and 1 x ELS. Reported are a 35 year old man with relapsing infections of the sequester and a 21 year old woman where the sequestration was accidentally found without clinical symptoms. The focus was localized in both cases in the left lower lobe of the lungs, anomalous supply arteries derived from the thoracic aorta. Venous drainage of the sequester was different - in the man a systemic drainage via the v. azygos, in the woman via the pulmonary veins was found. In one case the diagnosis was made on the basis of angiography and computer tomography, in the other case it was made on the basis of multidetector CT angiography (MDCTA). Both findings were treated by primary surgical intervention lobectomy. The postoperative course was uneventful.     

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.     

Total anomalous pulmonary venous drainage

Forty-four patients with total anomalous pulmonary venous drainage underwent repair between 1979 and 1987. The anomalous drainage was supracardiac in 16, cardiac in 12, and infracardiac in 16. Median age at repair was 15 days and median weight, 3.3 kg. In 22 patients (50%) the venous drainage was obstructed. Emergency operation was necessary in 12 patients, and the condition of seven additional patients deteriorated while they were awaiting semiurgent operation. There was one hospital death (mortality rate 2.3%), occurring in a 2-week-old premature neonate with infracardiac drainage. In 17 of 44 patients, mean pulmonary artery pressure equaled or exceeded systemic arterial pressure immediately after repair. Four patients have required reoperation and two of these have died as a result of peripheral extension of pulmonary vein sclerosis. In the review period of from 1 month to 7 years, all survivors are well and receiving no medication.     

Extending the limits for modified Fontan procedures

During the early development of atriopulmonary anastomotic operations (Fontan-Kreutzer), a number of physiologic and anatomical limits were proposed by the Fontan group as selection criteria. Among 167 consecutive patients undergoing modified Fontan procedures from 1973 through 1985, 109 (65%) patients exceeded one or more of the original selection criteria in areas of age, anomalies of systemic or pulmonary venous connection, pulmonary artery distortion, and pulmonary artery pressure. Twenty-six patients had a mean pulmonary artery pressure greater than 15 mm Hg, with 16 operative survivors (62%). Nineteen patients had anomalies of systemic and/or pulmonary venous connection, and 16 survived (84%). There were 44 patients under the age of 4 years, and 26 survived (59%). Twenty-five patients were older than 15 years, and 23 (92%) survived the Fontan procedure. Pulmonary artery distortion, relating to prior palliative operations, was found in 34 patients. Seventeen of these 34 survived a modified Fontan procedure (50%). Twenty-six patients had a pulmonary arteriolar resistance more than 2 Wood units times square meter, and 14 survived (54%), whereas 81 of 93 with a pulmonary arteriolar resistance of less than 2 U X m2 survived (87%). Multivariate analysis showed that pulmonary arteriolar resistance and pulmonary artery distortion had a significant, negative impact on survival, but age and anomalies of systemic and/or pulmonary venous connection did not. Pulmonary artery pressure was not an independent predictor of outcome. The results show that the original criteria may be exceeded in the areas of age and anomalies of pulmonary or systemic venous connection. Pulmonary artery pressure alone should not contraindicate a Fontan procedure if pulmonary arteriolar resistance is low. Pulmonary artery distortion from a prior palliative operation and elevated pulmonary arteriolar resistance increase the risk of a Fontan procedure.     

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.     

Scimitar syndrome with left pulmonary hypertension and right pulmonary artery stenosis in childhood--report of a case of successful surgical repair

An one-year old child with Scimitar syndrome (anomalous drainage of the right pulmonary vein to inferior vena cava with right lung hypoplasia) associated with left side pulmonary hypertension and right pulmonary artery stenosis underwent surgical repair. The anomalous pulmonary vein was anastomosed to right atrium with 10 mm PTFE graft interposition and drained to left atrium through equine pericardial intra-atrial baffle. The stenotic portion of right pulmonary artery was enlarged with porcine pericardial patch. To our knowledge, this is the first successful surgical case of Scimitar syndrome with pulmonary hypertension in childhood reported in literature. A repair of anomalous pulmonary venous drainage to inferior vena cava in childhood is difficult, and a meticulous selection of surgical procedure is necessary.     

The beneficial hemodynamic effects of selective patent vertical vein following repair of obstructed total anomalous pulmonary venous drainage in infants.

OBJECTIVES: Postoperative low cardiac output may persist after repair of total anomalous pulmonary venous drainage (TAPVD) because of a relatively small and non-compliant left atrium and left ventricle. We examined the effects of selective vertical vein patency on postoperative hemodynamics. METHODS: Thirty-four patients less than 3 months of age with TAPVD were operated from July 1993 to June 2000. The mean age at operation was 21+/-8 days (range, 3-62 days) and the mean weight was 3+/-0.2 kg (range, 2-4.1 kg). Supracardiac type drainage was found in 12 (35%), cardiac in three (9%), mixed in one (3%), and infracardiac in 18 (53%) patients. Twenty-two patients (65%) had obstructed venous drainage. All operations were performed with deep hypothermic circulatory arrest. Supracardiac, mixed and infracardiac types were repaired through a posterior approach, whereas, in the cardiac type, the coronary sinus was unroofed and the atrial septal defect was patched. The decision whether to keep the vertical vein open was made at the end of the operation and was based on the hemodynamic state of the patient. RESULTS: There were no operative deaths. The suture on the vertical vein was released in 22 patients who had obstructed pulmonary venous drainage (infracardiac type, n=18; supracardiac type, n=3; and mixed type, n=1), resulting in a significant drop in the left atrial pressure from 19+/-2 to 12+/-2 mmHg (P<0.05), and in the mean pulmonary artery pressure from 42+/-6 to 35+/-3 mmHg (P<0.05), associated with an immediate increase in the mean arterial blood pressure from a mean of 46+/-3 to 60+/-4 mmHg (P<0.05). During a mean follow-up of 38+/-6 months (range, 8-71 months), there were no late deaths. Follow-up, two-dimensional echocardiography with Doppler studies demonstrated good left ventricular function and trivial or no left to right shunt through the vertical vein in those patients in whom the snare was released. CONCLUSIONS: Maintaining the vertical vein patent in a selective group of patients with infracardiac total anomalous venous drainage contributes to a favorable outcome following surgery.     

Pulmonary vein thrombosis after bilobectomy and development of collateral circulation

Pulmonary vein thrombosis is a rare but potentially life threatening complication following lobectomy or bilobectomy. We present a case of right upper pulmonary vein thrombosis after a middle and lower lobectomy diagnosed at transoesophageal echocardiography. The patient was treated with antibiotics and anticoagulation with good recovery. Pulmonary angiography was performed 35 days after surgery and revealed the venous return of the right lung through the intercostal veins. Despite double venous drainage of the lungs consisting of bronchial and pulmonary veins, pulmonary to systemic collaterals following pulmonary vein thrombosis have not previously been reported. The development of this shunt can prevent gangrene, and surgical resection of the lung segment involved can be avoided.     

Complications related to invasive hemodynamic monitors during adult liver transplantation

The rate of complications directly related to invasive monitors during liver transplantation (LT) was reviewed in 1206 consecutive adult LTs performed over 8.6 yr (1/1/2004–7/31/2012). The designated anesthesiologists placed intra‐operative monitors, including two arterial catheters (via the radial and the right femoral arteries), central venous catheters (a 9 Fr. catheter and an 18 Fr. veno‐venous bypass [VVB] return cannula in an internal jugular vein), a pulmonary artery catheter, and a transesophageal echocardiography (TEE) probe. A 17 Fr. VVB drainage cannula was placed via the left femoral vein. No Clavien–Dindo Grade V (death) or Grade IV (organ dysfunction) complication was identified. Nine Grade III complications (requiring surgical intervention) and 15 Grade II complications (conservative treatment) were noted. Seven (0.58% in 1206 cases) were related to a femoral arterial line with Grade III of four; seven (0.58%) were due to VVB return cannula in the femoral vein with Grade III of one; four (0.33%) were related to central venous catheters with Grade III of two; four (0.33%) were due to a TEE probe with Grade III of two; and two minor complications (0.17%) that were related to a radial arterial line. No complication was observed with a pulmonary arterial catheter. Current invasive monitors placed during LT have an acceptable risk.     

Partial nail plate removal after digital replantation as an alternative method of venous drainage

Partial nail plate removal, systemic anticoagulation, and the application of topical heparin to the exposed nail bed were used to provide and maintain venous drainage for 14 digital replantations in which an arterial repair but no venous repair was done ("artery only" replantation). In each case venous repair was not possible since no vein of an acceptable size could be found either because of a distal amputation level or because an avulsive or crushing injury had damaged the veins in the amputated part. The average operating time per "artery only" replantation was 2 1/2 hours. Ten of the 14 "artery only" replantations survived (71.4%). The active range of motion, sensibility, strength, activities of daily living, and hospitalization for this group of patients were comparable with replantations in which both arterial and venous anastomoses were done.     

Renal nutcracker syndrome: Surgical options

Nutcracker syndrome is one of the abdominal venous entrapments, caused by compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Occasionally a retro-aortic left renal vein is compressed between the aorta and the vertebral body (posterior nutcracker syndrome). The renal vein distal to the compression is dilated and renal venous flow can be diverted toward the pelvis through an incompetent, refluxing, left ovarian or spermatic vein, in addition to drainage through retroperitoneal venous collaterals. In this article, we describe the different surgical and endovascular techniques that are used to treat this syndrome.     

Total anomalous pulmonary venous connection with dysmorphic pulmonary vein: a risk for postoperative pulmonary venous obstruction

Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection remains potential and understanding of its mechanisms is warranted. Morphology of the pulmonary vein was qualitatively analyzed in 48 consecutive patients undergoing repair of non-isomeric total anomalous pulmonary venous connection. Pulmonary venous drainage was supracardiac in 26, cardiac in 7, infracardiac in 13, and mixed in 2. Nine had dysmorphic pulmonary venous confluence or tributary veins (Group A). Four had excessive (>or=5) tributary veins with a hypoplastic confluence (Type 1 abnormality). In the other four cases, the vertical vein was atretic (Type 2 abnormality). In a case with cardiac type, pulmonary veins had stenosis at orifices (Type 3 abnormality). The rest (n=39; Group B) had normal pulmonary vein. Eight patients (7 in Group A and 1 in Group B) developed postoperative pulmonary venous obstruction. Overall actuarial survival was 90.0% after 2.3 months up to 10 years. Actuarial freedom from pulmonary venous obstruction was 79.5% after 5.0 months up to 10 years. It was 22.2% at 1 year among Group A compared with 96.7% at 10 years among Group B (P<0.001). Morphological analysis of the pulmonary vein best predicted the incidence of postoperative pulmonary venous obstruction.     

Inadvertent aortic cannulation with a pulmonary artery catheter detected by transesophageal echocardiography

Attempted pulmonary artery catheterization via the left internal jugular vein resulted in a misdiagnosis of pulmonary hypertension before transesophageal echocardiography revealed the catheter positioned in the ascending aorta. Inadvertent aortic cannulation through an unusual type of partial anomalous pulmonary venous connection was confirmed with transcatheter fluoroscopy and later at autopsy. Partial anomalous pulmonary venous connection describes one or more of the pulmonary veins draining into the right atrium or its tributaries instead of the left atrium.     

Distribution,diagnosis, and treatment of pulmonary sequestration: Report of 208 cases

ObjectiveThis study was performed to explore the clinical features, typing, distribution, and treatment of pulmonary sequestration (PS), with the aim of improving the awareness and treatment of this condition.MethodsClinical data regarding surgical procedures, outcomes, and prognosis of 208 pediatric patients with PS who were treated in our center from January 2005 to October 2017 were retrospectively analyzed.ResultsPS was confirmed by ultrasonography, enhanced computed tomography (CT), and/or magnetic resonance imaging (MRI) before surgery, and the surgeries were smoothly performed in all 208 patients (138 males, 70 females; age, 1 month to 14 years; mean age, 19.70 ± 48.82 months). The operative time ranged from 10 to 230 min (mean, 70 ± 48.75 min), and the intraoperative blood loss volume ranged from 1 to 200 ml (mean, 5 ± 18 ml). PS was located in multiple sites of the thoracic cavity and was also found in some rare locations such as the neck and abdomen. The feeding arteries of the PS mainly arose from the thoracic aorta or abdominal aorta, and a few of them originated from other vessels in the systemic circulation. The venous drainage differed between intralobar and extralobar PS: in patients with intralobar PS, the venous drainage was mainly via the pulmonary veins, especially the lower pulmonary veins; in patients with extralobar PS, the venous drainage was via the azygos vein and hemiazygos vein or reached the right atrium via the vena cava. The infection rate in children with intralobar sequestration was 71.17% (79/111), and that in children with extralobar sequestration was 31.37% (16/51).ConclusionPS has increasingly been detected by prenatal ultrasonography, and enhanced CT and MRI are the main techniques for diagnosing PS. Once confirmed, PS should be surgically resected. We choose an age of 6 to 12 months for surgical resection. Minimally invasive video-assisted thoracic surgery has many advantages in the treatment of PS and can be the treatment of choice for this condition.Type of studyTreatment Study.Level of evidenceLevel III.     

荧光示踪法研究逆行岛状皮瓣静脉回流

目的探讨采用荧光示踪法研究逆行岛状皮瓣静脉回流的可行性，并初步观察静脉回流规律。方法20只新西兰大白兔，每只取耳静脉血0．ImL，分离RBC并用FITC标记。流式细胞仪检测已标记的RBC阳性率及荧光强度，倒置荧光显微镜观察其形态。取20只新西兰大白兔，在动物双侧后肢内侧分别建立4cm&#215;3cm隐动、静脉逆行岛状皮瓣模型（n=10）和顺行岛状皮瓣模型（n=10），血管蒂长3cm。将一侧后肢随机设定为实验组，皮瓣制备后注射已标记的RBC悬液5pL；对侧为对照组，不注射示踪剂。实验组按顺行和逆行皮瓣分成两组，即顺行皮瓣组和逆行皮瓣组，每组10个；再根据注入示踪剂途径不同，分为动脉和静脉2个亚组，每亚组5个皮瓣。注射示踪剂5S后取下皮瓣立即冷冻，取连续的3张冰冻切片（5～7pm），其中2张行HE染色和GENMED染色，另]张不染色直接压片，荧光显微镜观察荧光分布。结果流式细胞仪分析FITC标记的RBC阳性率在99％以上，荧光强度均≥10。；倒置荧光显微镜下标记的RBC呈均匀分布的绿色荧光，荧光强度均匀、稳定。冰冻切片显示实验组皮瓣蒂部均出现荧光，对照组未见荧光。顺行岛状皮瓣组荧光主要分布在静脉腔、静脉壁、动脉内膜和外膜；逆行岛状皮瓣组荧光分布在动脉内膜、外膜和静脉壁。结论荧光示踪剂可用于静脉回流研究，顺行岛状皮瓣静脉主要通过静脉腔、静脉壁、动脉内膜和外膜回流；逆行岛状皮瓣静脉主要通过动脉内膜、外膜和静脉壁的“迷宫式途径”回流。     

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.     

The anterior jugular venous system: variability and clinical impact

The anterior jugular venous system, with its interconnections to the subclavian and deep jugular veins, provides a collateral venous network across the midline of the neck area, which is especially important in unilateral occlusion of an innominate vein. We illustrate the variability of this system and its clinical impact on catheterization by three cases of landmark-guided central venous cannulation. Case 1: Cannulation of the left internal jugular vein with a central venous catheter and of the left innominate vein (LIV) with a pulmonary artery catheter resulted in correctly positioned catheter tips. However, these catheters were actually not placed in the innominate vein but coursed through the jugular venous arch. Case 2: Cannulation of the left subclavian vein was complicated by resistance of guidewire advancement at 13 cm. Occlusion of the LIV and enlargement of the jugular venous arch were present. Case 3: Insertion of a pulmonary artery catheter and a central venous catheter through the LIV. The pulmonary artery catheter was correctly placed. The tip of the central venous catheter was mistakenly positioned in the left anterior jugular vein. We describe the normal anatomy of the anterior jugular venous system and its role as a major collateral. Correct placement of central venous catheters may be possible via the anterior jugular venous system. Conversely, central venous catheters malpositioned in the anterior jugular vein can increase the risk for complications and should be removed.     

Intralobar Bronchopulmonary Sequestration with Mixed Venous Drainage in a 10-Year-Old Girl

Bronchopulmonary sequestration consists of a mass of abnormal lung tissue that has no normal connection with the bronchial tree and is supplied with blood from an aberrant artery mostly originating in the thoracic aorta. Two forms are recognized: intralobar and extralobar sequestration. The first is localized within the normal visceral pleura and has a venous drainage into the pulmonary system; the latter is localized without the normal lung in its own pleura with venous drainage into the systemic venous system. Intralobar sequestration is the most common form accounting for 75% of the cases. Intralobar sequestration usually presents in adolescence or adulthood with signs of recurrent pneumonia. Extralobar sequestration presents early in life with respiratory distress or feeding difficulties and is frequently associated with other congenital malformations. The diagnosis is confirmed by CT scan of the lungs and magnetic resonance angiography as demonstration of the aberrant vascular supply is essential for the diagnosis. Therapy consists in surgical removal.

We present a case of intralobar sequestration in a 10-year-old girl. The clinical symptomatology was typical. Arterial supply with two aberrant arteries and mixed venous drainage into the pulmonary and systemic systems were particular features.     

Carotid-cavernous fistula treatment with ethylene vinyl alcohol (onyx) exclusively through anterior venous approach

The type of venous drainage of a direct carotid-cavernous fistula is an important issue to consider for the endovascular therapeutic decision. In case of an inadequate posterior drainage associated with a good anterior drainage, the facial vein is a useful alternative. The exclusive embolization with ethylene vinyl alcohol (EVOH Onyx), arterial and/or venous via the internal carotid artery (ICA) occlusion has been used successfully, in a few cases until now. Nevertheless, the use of this method through anterior transvenous approach has not been previously described. Presented here is the case of a 13-year-old female patient with left posttraumatic carotid-cavernous fistula, with predominant anterior drainage, as well as carrier of traumatic occlusion of the contralateral ICA. The treatment was by means of a transvenous approach with transient occlusion of the left ICA.     

Technical modifications for improved results in total anomalous pulmonary venous drainage.

To delineate factors that contribute to improved surgical outcome in patients with total anomalous pulmonary venous drainage, we reviewed the records of 52 consecutive patients. Venous drainage was supracardiac in 25 (48%), cardiac in 12 (23%), infracardiac in 10 (19%), and mixed in five (10%). Preoperative pulmonary venous obstruction was present in 18 patients (35%). Median age at the time of repair was 35 days and weight, 3.7 kg. Repair was performed with deep hypothermia, low-flow cardiopulmonary bypass, and occasional short periods of circulatory arrest. In patients with coronary sinus drainage, the veins were tunneled to the left atrium through an enlarged atrial septal defect, with a mortality of 8% (1/12) and no postoperative stenosis. The approach in patients with supracardiac, infracardiac, and mixed drainage varied with time. In 16 patients, the condition was managed by apical or right-sided exposure of the common vein, anastomosis of the common vein to the left atrium with continuous sutures, and primary closure of the atrial septal defect (type I repair). In the other 24 patients the common vein was approached from the right side through the right atrium and the interatrial septum. Common vein-left atrium anastomosis was performed with interrupted sutures and a piece of pericardium used to augment the anastomosis, prevent common vein distortion, and close the atrial septal defect (type II repair). Mortality in type I repair was 25% (4/16) and in type II repair, 4% (1/24). Follow-up was 7.86 +/- 4.0 years with no late deaths. Postoperative stenosis occurred in five of 14 (36%) patients who had type I repair versus two of 23 (9%) who had type II repair. Multivariate analysis showed that type I repair was a positive risk factor for hospital mortality (p = 0.05) and restenosis (p = 0.04). The technique of transatrial exposure of the common venous chamber, interrupted suturing of the common vein to the left atrium, and pericardial patch augmentation significantly improves survival and decreases risk of restenosis.