Surgical management of scimitar syndrome: an alternative approach

OBJECTIVE: The scimitar syndrome is a congenital anomaly that consists in part of total or partial anomalous venous drainage of the right lung to the inferior vena cava. Surgical approaches to the scimitar syndrome have varied according to the anatomic and pathologic features presented in each case. The aim of this study was to present an alternative approach to the surgical correction of scimitar syndrome. METHODS: Nine patients with the scimitar syndrome were operated on between 1990 and 2000. They comprised 1 male and 8 female patients (mean age 11.5 +/- 17.6 years). All patients had symptoms, with recurrent pneumonia or respiratory tract infections and pulmonary/systemic flow ratios greater than 1.5:1.0. None of the patients had pulmonary hypertension or an atrial septal defect. All patients underwent repair of the anomalous scimitar vein by direct reimplantation into the left atrium without cardiopulmonary bypass. Two patients underwent concomitant resection of a right lower lobe sequestration. Follow-up was complete in all cases. RESULTS: There were no operative or late deaths, and no patients have required reoperation. At the time of follow-up (mean 55 +/- 46 months), echocardiography demonstrated a patent anastomosis in all patients without any evidence of restenosis. CONCLUSION: This clinical experience indicates that an alternative surgical approach to scimitar syndrome is direct anastomosis of the scimitar vein to the posterior aspect of the left atrium using a right thoracotomy without cardiopulmonary bypass. This procedure is safe and effective and obviates the need for long intra-atrial baffles and the use of the extracorporeal circuit.     

The crossover lung segment: congenital malformation associated with a variant of scimitar syndrome.

Three cases of a congenitally misplaced segment of lung are described. In one case the abnormal segment was identified at thoracotomy; it extended from an origin in the right upper lobe across the midline into the left hemithorax. The remaining two patients, with identical radiographic features, were identified in a review of 24 cases of bronchovascular malformation. In all three patients the crossover segment was associated with a variant of the scimitar syndrome: right lung hypoplasia with dextrocardia, aberrant systemic arterial supply to the right lower lobe, and anomalous venous drainage of the whole of the right lung, and in two patients with eventration of the right diaphragm.     

Pneumonectomy in scimitar syndrome —is it correct?

The scimitar syndrome is a rare congenital anomaly that is characterized by anomalous pulmonary venous drainage to the inferior vena cava causing a left-to-right shunt. Because the radiographic shadow of the anomalous vein resembles a curved turkish saber, this defect has been called as the “scimitar deformity”¹. Here we present a two months old male child with an anomalous drainage of the single right pulmonary vein draining the entire right lung into the inferior vena cava, direct arterial supply from the abdominal aorta to the right lung, horse-shoe lung and unilobar hypoplastic right lung. The child was treated surgically with a right pneumonectomy. The child had an uneventful postoperative recovery.     

Intralobal sequestration in children--a new concept from the form of bronchial tree in sequestrated lung]

Twenty cases of the intralobal sequestration of the lung in children have been experienced. The age of the patients were from eleven days to fifteen years. Intralobal sequestration was classified into two groups, (central type and peripheral type) by the way of reconstruction of the bronchial tree in the sequestrated lung. In 8 cases of central type, the site of lesion was variable. The bronchial trees of the sequestrated lung were running toward the hilus of the normal lung but the bronchi of the affected area in the normal lung were absent. The aberrant arteries were muscular in histology, and the drainage veins were pulmonary vein. In 12 cases of peripheral type, the lesion was in segment 10 area in every cases. The bronchial trees of the sequestrated lung were running toward the pulmonary ligament where an aberrant artery came from, and B10 of the normal lung were patent. The aberrant arteries were elastic in histology, and the drainage veins were pulmonary and/or azygos vein. It is conceivable that the peripheral type is the real intralobal sequestration and the central type is the mixture of some other diseases.     

Combined intracardiac and extracardiac repair of scimitar syndrome with anomalous pulmonary veins to both cavae

Anomalous pulmonary venous drainage from the right lung to the inferior vena cava (scimitar syndrome) is a rare finding. This paper briefly discusses this syndrome and presents a case of anomalous pulmonary venous return to both the superior and the inferior cavae, which was corrected with a combined extracardiac and intracardiac approach. Polytetrafluoroethylene was utilized for reconstruction of pathways to the left atrium. To our knowledge, this is the first time this technique has been used to correct this anomaly.     

Anomalous dual drainage of the right pulmonary veins in a patient with cor triatriatum: report of a case without scimitar sign

The majority of patients with partial anomalous drainage of pulmonary veins are asymptomatic during infancy and childhood. Patients with significant left-to-right shunt develop symptoms and benefit from early corrective surgery. Anomalous pulmonary veins draining into inferior vena cava is very rare and frequently encountered in association with scimitar syndrome. The purpose of this case report is to describe a non-scimitar patient with cor triatriatum who had anomalous dual drainage of right pulmonary veins into inferior vena cava/left atrium and anomalous connection of persistent left superior vena cava with a common pulmonary venous chamber. The patient underwent an operation with redirection of anomalous pulmonary venous drainage into left atrium and ligation of persistent left superior vena cava.     

Scimitar Syndrome: The Curved Turkish Sabre

Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies characterized by an anomalous pulmonary vein (scimitar vein) that drains into the inferior vena cava, a hypogenetic right lung, and dextroposition of the heart. It has been reported in 3% to 6% of patients with partial anomalous pulmonary venous connection. Patients are either diagnosed early with severe symptoms (infantile type) or late with minimal symptoms (childhood/adult type). In this chapter we reviewed the history, pathophysiology, presentation, diagnosis, and treatment methods of Scimitar syndrome in the current era.     

Upper thoracic extralobar pulmonary sequestration with anomalous blood supply from the subclavian artery

The authors report on a newborn boy with extralobar pulmonary sequestration in the right upper thoracic region. Preoperative angiography showed an anomalous large vessel from the right subclavian artery, supplying the sequestrated lobe. Right thoracotomy was performed to resect the sequestrated lobe, which was diagnosed as extralobar form. This is an extremely rare case of extralobar pulmonary sequestration in which anomalous blood supply from the subclavian artery was seen preoperatively on radiographs. The authors recommend angiographic examination, particularly in case of diagnostic difficulty.     

Pulmonary sequestration

A review of 41 patients over a 15-year period with a diagnosis of pulmonary sequestration was undertaken. The most common presenting complaint was repeated infections of the sequestrated segment. Two of our patients were symptomatic since the neonatal period. On an average, each patient was admitted three times to the hospital before undergoing surgery. All our patients had abnormal chest x-rays. Two patients had severe purulent infection needing emergency resection of the sequestrated lobe. In our experience, almost all cases of pulmonary sequestration require surgery in the long-term.     

Giant carcinoid tumor mimicking pulmonary sequestration

A 42-year-old woman who previously underwent two consecutive thoracotomies for a lower lobe mass in her right lung was referred to our clinic for further management. Both procedures were abandoned due to excessive bleeding. Computed tomographic angiography demonstrated an infra-diaphragmatic systemic arterial supply of the mass similar to pulmonary sequestration. However the lobe had a normal venous drainage to the left atrium. Then a right lower lobectomy was undertaken through a hemi-clamshell incision, and histopathology revealed an atypical carcinoid tumor. The patient was discharged home after a satisfactory postoperative period. She still remains disease free at 14 months follow-up.     

The multiple facets of pulmonary sequestration

PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. METHODS: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.     

Surgical repair of scimitar syndrome by direct anastomosis between right pulmonary vein and left atrium

A surgical case of 8-year-old boy with scimitar syndrome is presented. The patient was admitted to the hospital because of exertional dyspnea and underdevelopment. Cardiac catheterization revealed a large amount of left to right shunt and O2 step up at the level of the inferior vena cava below the diaphragm. Angiography showed that the pulmonary vein draining the right lower lobe pierced the diaphragm and emptied into IVC. At operation the right pleural cavity was entered and a large anomalous pulmonary vein trunk was found which headed downward and medially and eventually entered in the diaphragm. A direct anastomosis between the scimitar vein and left atrium was made. To avoid kinking or stenosis of this vein, the parenchyma of the right lung (S7) was divided to create a passway of the vein. Anastomosis was performed without excessive tension or kinking on it. We conclude that the method applied in this case might be a procedure of choice for the repair of scimitar syndrome especially when there is no associating atrial septal defect or scimitar vein drains into IVC at the level lower than hepatic vein.     

Bronchopulmonary foregut malformation in an adult

A 50-year-old man with repeated episodes of right epigastric pain was seen in our hospital. Chest computed tomography and angiography revealed several arteries feeding an enhanced large mass located in the right lower lobe region. A right lower lobectomy was done with a provisional diagnosis of an intralobar sequestration. A 5-mm duct that was lined with esophageal mucous membrane that tracked from the lower esophagus toward the sequestrated lung was detected. A bronchopulmonary foregut malformation (BPFM) was diagnosed based on the histological finding that the duct was composed of ciliated epithelium and smooth muscle layers. BPFM is a subgroup of pulmonary sequestrations that communicate with the gastrointestinal tract. In contrast to pulmonary sequestrations, 75% of BPFMs are located on the right side. Thus, a BPFM should be considered in patients with right-sided pulmonary sequestrations, and their gastrointestinal tracts should be examined.     

The spectrum of pulmonary sequestration

An intralobar sequestration with the unusual anatomical finding of both pulmonary and systemic (azygos) venous drainage is presented in detail. A review of the literature pertaining to pulmonary sequestration revealed a continuum of lung anomalies in which nearly every combination of pulmonary and systemic arterial supply, pulmonary and systemic venous drainage, normal and abnormal pulmonary tissue, gastrointestinal fistula, and defective diaphragm was described. Since no single anatomical mechanism can account for all these anomalies, the spectrum can best be explained at this time as a defect or defects of morphogenesis in the embryonic thorax. The surgeon must be aware of this spectrum of anomalies in order to remain alert to the possibility of unusual blood vessels and gastrointestinal fistula during operation for any cystic or suppurative lesion of the lungs.     

Scimitar syndrome with pulmonary arteriovenous fistulas.

Right abnormal pulmonary venous return into the inferior vena cava associated with abnormal fissure, dextrocardia, and systemic arterial supply of a variable degree, are the characteristics of the scimitar syndrome. We report on a patient in whom this rare syndrome was associated with pulmonary arteriovenous fistulas within the involved lung.     

Agenesis of the hepatic vena cava in a patient with scimitar syndrome

With the use of sophisticated imaging modalities, congenital anomalies of the inferior vena cava and its tributaries, such as agenesis of the hepatic vena cava, are becoming more and more commonly encountered. Scimitar syndrome, also called venolobar syndrome and hypogenetic lung syndrome, is a rare pulmonary vascular anomaly of the right lung that can sometimes be associated with some cardiovascular anomalies. We present here a case with agenesis of the hepatic vena cava associated with scimitar syndrome.     

Pulmonary sequestration with tuberculosis confined to the sequestrated lung

Pulmonary sequestration is a relatively rare malformation. The incidence of common pyogenic infection in this anomaly is very high. We describe a non-symptomatic, 19-year-old man who was misdiagnosed with left lower lobe pneumonia, which was treated with antibiotics for nearly one month. Contrast-enhanced computed tomography (CT) with multiplanar reconstruction showed an aberrant artery originated from the left side of the descending aorta, and went through the infiltration of the left lower lobe. The patient underwent surgical removal of the affected lobe. Microscopy demonstrated resected sequestrated lung tissue that was mainly composed of caseous necrosis with Langhans cells. And the tuberculosis was just confined to the sequestrated lung without any other sites of lung tuberculous infection. The patient received subsequent antituberculous chemotherapy after his operation. At 4-month follow-up, his clinical status was excellent. There are few reports of sequestration combined with tuberculosis. This case showed us two things: first, a persistent infiltration or consolidation in a same segment, especially in the lower lobe, reminds us of the possibility of sequestration; and second, even if the diagnosis of sequestrated lung is confirmed, we should consider whether the patient has any other diseases, besides the tuberculosis.     

Atypical infantile form of scimitar syndrome with bronchomalacia

A male infant presenting with severe heart failure and respiratory distress was diagnosed with a hypoplastic right lung, scimitar syndrome with pulmonary sequestration and multiple anomalous systemic arteries, left bronchomalacia, a large atrial septal defect and coarctation of the aorta. The infant underwent a successful combined treatment of surgical and transcatheter intervention, including coil embolization and endobronchial stenting.     

Infant with bilateral pulmonary sequestrations with portal venous drainage excised by video-assisted thoracic surgery

Introduction

Bilateral pulmonary sequestrations are rare congenital anomalies. Despite its benign nature, the potential complications of pulmonary sequestration (PS) are significant, including recurrent pulmonary infections, hemoptysis, congestive heart failure, and malignant potential. Therefore, the main treatment is surgical excision, even for patients with asymptomatic PS.

Case

We present an infant in whom an intralobar PS of the right lung and an extralobar PS of the left lung were diagnosed on prenatal screening ultrasonography. Both were found to have venous drainage into the portal vein. Surgical excision was performed via video-assisted thoracic surgery (VATS) at 14 months of age.

Conclusion

PS may present with unique vascular connections, including venous drainage into the portal vein. VATS resection for pulmonary sequestration is feasible and effective as an alternative to bilateral thoracotomies, in the setting of extensive preoperative planning and performance by an experienced thoracoscopic surgeon.     

Right heart bypass operation of complex cardiac anomalies in 4 patients with viscero-atrial heterotaxic syndrome

Four patients with viscero-atrial heterotaxic syndrome underwent a right heart bypass operation. 2 patients had right isomerism, and two had left isomerism. 3 patients had common atrio-ventricular canal, three had double outlet right ventricle, one had mitral valve hypoplasia, and one had double inlet right ventricle. All patients had anomalous systemic or pulmonary venous drainage. Redirection of systemic venous drainage was accomplished by total cavo-pulmonary shunt (TCPS) (2 patients), atrial baffle (1 patient), and intraatrial conduit (1 patient). In one patient underwent TCPS, regurgitant common atrioventricular valve was replaced with a prosthetic valve. One who had total anomalous pulmonary venous drainage (type Ib) died after cardiac repair. The three survivors are in improved condition. The right heart bypass operation, like total cavopulmonary shunt or connection, simplifies the redirection of systemic venous drainage and offers improved results in the surgical treatment of complex cardiovascular anomalies with viscero-atrial heterotaxic syndrome.