Aortic arch anomalies associated with long gap esophageal atresia and tracheoesophageal fistula

PURPOSE: The purpose of this study was to determine whether aortic arch anomalies are associated with long gap esophageal atresia and tracheoesophageal fistula (EA-TEF). METHODS: The authors performed a retrospective review of all infants who had EA-TEF from 1980 to 1996 at two pediatric surgery centers. Two hundred three infants who had EA-TEF were identified. RESULTS: Twelve infants were noted to have both long gap EA-TEF defined as a gap length greater than 3 cm and aortic arch anomalies. Of these 12, 7 had aberrant right subclavian arteries originating from the descending aorta. Four of the seven infants who had aberrant right subclavian artery (SCA) had gap lengths greater than 4 cm. All four had their fistulae divided initially through a right thoracotomy with primary repair performed at a later date. The remaining five infants who had long gap EA-TEF had right-sided aortic arch with aberrant left subclavian arteries. All five initially underwent exploration through the right chest. On discovery of the long gap EA and concurrent vascular anomaly, the thoracotomies were closed, and the infants underwent definitive repair of both their EA-TEF and their vascular anomaly through a left thoracotomy. CONCLUSIONS: The authors find that aortic arch anomalies are associated with long gap EA-TEF. Patients who have these two anomalies tend to have a long gap. Preoperative diagnosis of these anomalies may alter the timing and technique of surgical intervention. The embryogenesis of these vascular lesions may account for this more severe form of esophageal atresia.     

Compression of esophagus and trachea by aortic arch anomalies in childhood

From February 1985 to November 1992, 14 children with aortic arch anomalies and tracheal and/or esophageal compression were treated at the Pediatric Surgery Division of the S?o Paulo University School of Medicine. There were 3 cases of double aortic arch, 3 of right aortic arch with ligamentum arteriosum and 4 of aberrant right subclavian artery. Accurate diagnosis was based only on barium esophagoradiogram and, eventually, tracheobroncoscopy. The surgical approach was made through a left posterolateral thoracotomy that allowed an adequate exposure of the malformations. There was no operative mortality. There were 3 late post-operative deaths, 2 of them caused by chronic pulmonary lesions owing to delayed diagnosis. The other child died from complications of a severe tracheomalacia which became symptomatic only after the corrective surgery. The diagnosis of the aortic arch anomalies should be early suspected in children with undetermined respiratory symptoms and can be easily made basically by esophagoradiogram and tracheoscopy.     

Lusorian artery lesion as rare cause of severe upper gastrointestinal tract bleeding

A patient in an intensive care unit experienced severe esophageal bleeding caused by erosion of a lusorian artery. The lusorian artery is a rare variant of the right subclavian artery. It originates in the descending aortic arch and crosses behind the esophagus to the right, sometimes generating esophageal compression. The patient's condition required respirator therapy and placement of a duodenal tube. At the point of crossing over of the lusorian artery and the esophagus, the duodenal tube caused esophageal necrosis, leading to erosion of the lusorian artery. This resulted in extensive esophageal bleeding, which at last required surgical intervention. To attain proper treatment and to avoid unnecessary diagnostic and therapeutic approaches, a lusorian artery lesion has to be included in the differential diagnosis of upper gastrointestinal bleeding.     

3 anatomical observations of a right subclavian artery originating from the aortic arch (arteria lusoria)

1. The dissection of 223 subjects has revealed 3 cases of A. lusoria (1.06%). In all these cases, the A. lusoria was the last branch of the aortic arch. 2. X-ray pictures were taken after injection of radio-opaque substance to elicit the topographical relations of A. lusoria with esophagus. 3. There were only small variations in the calibre and in the way of its branches. Right common carotid artery directly arose from the aortic arch. 4. The three Aa. lusoriae crossed the dorsal face of esophagus between the 4th and 2nd cervical vertebrae where they provoked at neat natch. 5. In one case, there was a marked narrowing at the origin of the artery. In another case, there was an initial widening of the artery. 6. Histological examination of the arterial wall from the portion between oesophagus and vertebral column demonstrated a decrease in number of elastic fibres towards the column. 7. In all cases, there was around A. lusoria a normal Ansa subclavia from the sympathetic trunk. 8. In one case, recurrent nervus were topographically normal on both sides. In both other cases, the right recurrent nerve did not pass under the right subclavian artery but went directly to the larynx without touching the right inferior thyroid artery and its branches.     

Brightest X-rays ever give researchers brand-new view of biological molecules

An aberrant right subclavian artery (ARSCA), also known as arteria lusoria, is the most common congenital anomaly of the aortic arch, with a reported prevalence ranging from 0.4 to 2%. The ARSCA arises as the last branch of the aortic arch and crosses the mediastinum from left to right indenting the oesophagus posteriorly. Aneurysms of this aberrant vessel, whether or not arising from a Kommerell's diverticulum, are rare. A 79-year-old woman is presented, in whom a partly thrombosed aneurysm of an ARSCA was diagnosed with magnetic resonance (MR) imaging.     

Diagnosis of subclavian steal in infants with coarctation of the aorta and interruption of the aortic arch by color-coded Doppler sonography

We report the noninvasive diagnosis of subclavian steal by color-coded Doppler ultrasonography in nine infants with congenital heart disease. The underlying cardiovascular malformations included coarctation of the aorta in four infants, interrupted aortic arch type B in three patients, truncus arteriosus communis type A4 (one patient), and isolation of the right subclavian artery (one patient). In all patients both vertebral arteries could be displayed through the anterior fontanel in coronal sections. Normally the flow in both vertebral arteries, as well as the flow in the basilar artery, is displayed in red. In eight patients with angiographically proved unilateral subclavian steal, an antegrade (red) flow could be shown in one vertebral artery, whereas the contralateral vertebral artery was displayed blue, indicating reverse flow. In one infant with interrupted aortic arch type B and associated aberrant right subclavian artery, both vertebral arteries and the basilar artery were displayed blue, indicating bilateral subclavian steal. Color-coded Doppler sonography seems to be a sensitive, noninvasive method for diagnosing congenital subclavian steal, especially in infants with obstruction of the aortic arch.     

Bronchogenic carcinoma associated with right aortic arch and postaortic left brachiocephalic vein--report of a case

61-year-old male was admitted to our hospital for surgical treatment of bronchogenic squamous cell carcinoma arising from left B8. The patient had right aortic arch with aberrant left subclavian artery and postaortic left brachiocephalic vein. Intraoperatively, left ligamentum arteriorsus forming vascular ring between the left subclavian artery and the pulmonary artery was found, however the ligamentum arteriorsus was not divided because no symptom of esophago-tracheal compression was observed. The left brachiocephalic vein was located between the ascending aorta and the arterial ligament. The lower lobe of the left lung was resected, and lymph nodes in the left side of the mediastinum were dissected easily because the aortic arch was positioned on the other side. Preoperative assessment of the type of branching and the course of arteries and veins is important for safe operation.     

An unusual cause of thoracic outlet syndrome

In an adult with severe dysphagia, diagnosis of aneurysmal aberrant subclavian artery was suspected on computed tomogram; no vascular ring was visible on arteriogram. A diverticulum of the aortic isthmus was surgically resected. Histologic analysis ruled out acquired disease. In conclusion, dysphagia related to esophageal compression may be caused by an isolated congenital aortic diverticulum.     

Subclavian arterio-esophageal fistula secondary to fish bone impaction: report of a case

A 48-year-old Indian man swallowed a fish bone and presented 1 week later with dysphagia, following a single episode of fresh hematemesis. A barium swallow demonstrated a horizontal mucosal tear at the level of the aortic arch, and computed tomography (CT) showed mediastinal emphysema and a bleeding point medial to the left subclavian artery which appeared to be contained by a surrounding hematoma. Subsequently, he developed sudden massive hematemesis and collapsed. Despite emergency surgery, the patient died. At the postmortem examination, a 1.2-cm fistula tract was found connecting the esophagus to the left subclavian artery. This case report emphasizes that a diagnosis of arterio-esophageal fistula should be considered if a patient presents with fresh hematemesis and a recent history of foreign body ingestion.     

Right eye blindness as the predominating clinical sign in a patient with aortic arch syndrome: a case report

A case is described of pulseless disease in 43 years old man with right-sided blindness as the main clinical sign. The brain was supplied with blood via the left vertebral artery. The cerebral circulation was additionally diminished by coexisting subclavian steal-syndrome with reversal of blood flow in the right vertebral artery.     

Geographic distribution of leukemia mortality in young people aged 0-24 years in France

Patients with significant stenosis at the carotid bifurcation are traditionally subjected to four vessel aortic arch angiography prior to consideration for carotid endarterectomy. The advent of the non-invasive vascular laboratory has necessitated a reappraisal of this approach. AIMS: 1. Determine the yield from aortic arch angiography and its influence on surgical management. 2. Evaluate the accuracy of clinical examination and the non-invasive vascular laboratory in the detection of aortic arch branch lesions. METHODS: One hundred and twenty-nine consecutive patients undergoing evaluation for carotid endarterectomy were prospectively enrolled into the study. The protocol entailed: 1. Clinical recording of upper limb pulses, blood pressure and supraclavicular bruits. 2. Duplex scan examination to evaluate proximal inflow into the carotid arteries. 3. Four vessel aortic arch angiography to detect aortic branch lesions. Data from the non-invasive tests were compared to angiography. Patients with aortic arch branch lesions were further evaluated to determine the proportion requiring additional surgery. RESULTS: Nineteen patients had angiographic evidence of aortic branch disease (14.7%); six involved the common carotid artery, three the innominate artery and 10 the subclavian artery. All of these lesions were detected by the combination of unequal blood pressure, pulse deficit, bruit or duplex scan. Seven patients underwent additional surgery (5.4%) which included carotid-subclavian bypass (five), aortoinnominate bypass (one) and innominate endarterectomy (one). CONCLUSION: In patients with significant stenosis at the carotid bifurcation undergoing evaluation for carotid endarterectomy, aortic arch angiography is unnecessary except in a small percentage of patients with abnormal clinical and non-invasive findings.     

Surgical treatment of an aortic arch aneurysm: reports of 2 cases with lesion of the arch vessels

A 48-year old woman underwent surgery for an aortic arch aneurysm with stenosis or dilatation of three arch vessels caused by aortitis syndrome. Total arch replacement and reconstruction of three arch vessels were performed with hypothermic selective cerebral perfusion (SCP). To avoid atheroembolism and malperfusion to the brain, the rt. common carotid artery was perfused via the rt. subclavian artery through the dacron vascular graft and the lt. subclavian artery was cannulated and perfused distally to stenosis. A 65-year-old man who had an atherosclerotic aortic arch aneurysm with severe stenosis of the brachiocephalic artery underwent operation. In the operation, extracorporeal circulation was instituted with the arterial return through the lt. subclavian artery. Same as case 1, total arch replacement and reconstruction of three arch vessels were performed under hypthermic SCP. In this case, the left common carotid artery was transected and cannulated directly into the vessel, instead of cannulation through the aortic lumen because of atheromatous plaques in the orifice of the left common carotid artery. The patients recovered uneventfully and doing well now.     

Iatrogenic vertebrobasilar insufficiency after surgery of the subclavian or brachial artery: review of three cases

OBJECTIVE AND IMPORTANCE: Vertebrobasilar insufficiency resulting from disease of the subclavian artery is well recognized. Usually, this occurs as the "subclavian steal" syndrome in the context of chronic subclavian stenosis and is consequently well tolerated because of collateralization. Acute disruption of the hemodynamics of the aortic arch vessels, however, can produce disastrous sequelae. CLINICAL PRESENTATION: We present three cases of iatrogenic vertebrobasilar insufficiency sustained as complications of surgery of the left subclavian artery or its distal continuation. The cases were chosen from a review of approximately 400 emergency neurosurgery consultations requested at the Los Angeles County Hospital between November 1995 and February 1996. INTERVENTION: The first patient underwent repair of a traumatic brachial artery occlusion and awoke postoperatively with bilateral cortical blindness, right hemiparesis, and multiple cranial nerve deficits that were most likely caused by acute subclavian steal. The second underwent removal of a subclavian embolus and developed bilateral cerebellar infarction leading to persistent coma, possibly from inadvertent embolization of the vertebral artery during surgery. The third underwent resection and bypass grafting of a subclavian aneurysm. Good backflow was reported when the vertebral artery was disarticulated from the subclavian artery, and this vessel was not reimplanted into the graft. The patient suffered massive cerebellar infarction leading rapidly to brain death. CONCLUSION: There are myriad ways in which the inherent redundancy of the vertebrobasilar system may be jeopardized, and when this protective mechanism fails, the results can be disastrous. Flow through the vertebral arteries may be compromised by thrombosis, embolization, dissection, inappropriate ligation, excessive head rotation, hypotension, vasospasm, or acute subclavian steal. These examples illustrate the importance of understanding the complex physiology of posterior fossa circulation as the basis of pre-, intra-, and postoperative management of patients undergoing surgery of the subclavian artery.     

Aortic coarctation. Treatment under one year (author's transl)

Fifteen patients operated upon for aortic coarctation under one year are reported. Nine of them survived. The E.C.G. was very useful as far as the surgical prognosis is concerned. Dead patients showed either right or biventricular hypertrophy, with alteration of the repolarisation. On the other hand 50% of survivors had left ventricular hypertrophy. In six of the operated patients a plasty between subclavian artery and aortic arch was performed. The remaining patients were treated by end-to-end anastomosis.     

Glucocorticoid-induced ketoacidosis in gestational diabetes: sequela of the acute treatment of preterm labor. A case report

Aortic dissection involving right aortic arch (RAA) is quite rare. A patient with RAA and aberrant left subclavian artery (type 3 RAA) developed type A dissection, but successfully underwent ascending and hemiarch replacement under hypothermic circulatory arrest with continuous retrograde cerebral perfusion. We approached the lesion through a midline sternotomy and could reconstruct the first two arch vessels involved by the dissection. We would have added bilateral thoracotomy, if the distal arch vessels had required reconstruction. To our knowledge, this is the first report of successful surgical repair for type A dissection involving RAA.     

Surgical treatment of common carotid artery occlusion

Eight patients with common carotid artery (CCA) occlusion underwent bypass with saphenous vein to either the carotid bifurcation (five), the internal carotid artery (two), or the external carotid artery (one). Indications included ipsilateral transient ischemic attack (two), recent nondisabling hemispheric stroke (two), and transient nonhemispheric cerebral symptoms (two). Two asymptomatic patients with CCA occlusion and contralateral internal carotid stenosis underwent prophylactic revascularization prior to planned aortic surgery. There were no perioperative strokes, occlusions, or deaths. Late ipsilateral stroke occurred in two patients, and one patient had a single transient ischemic attack after 2 years. The four patients with preoperative transient cerebral ischemia experienced relief of their symptoms. Duplex ultrasound is an accurate screening modality for distal patency. Collateral filling of the internal or external carotid artery can usually be demonstrated after aortic arch or retrograde brachial contrast injection. End-to-end distal anastomosis after endarterectomy eliminates the original occlusive plaque as a potential source of emboli. The subclavian artery is preferred for inflow on the left. The CCA origin is easily accessible for inflow on the right. Bypass of the occluded CCA is safe and may be effective in relieving transient cerebral ischemic symptoms, although long-term ipsilateral neurologic sequelae may still occur.     

Successful surgical treatment of an Edwards type IIIB right aortic arch aneurysm: report of a case

A true aneurysm of the right aortic arch which accompanies various branching characteristics is very rare. We report herein the successful surgical treatment of an elderly patient found to have an Edwards type IIIB right aortic arch aneurysm encircling and compressing the trachea. The complete right aortic arch and right subclavian artery were reconstructed through the inside of the aneurysm using selective cerebral perfusion. The patient recovered well, with no residual neurologic deficit and with resolution of the dyspnoic attacks he had suffered preoperatively.     

An experience of the modified Norwood's operation for hypoplastic left heart syndrome with aberrant origin of right subclavian artery and persistent left superior vena cava--the procedure without total circulatory arrest and cardiac arrest

We reported a successful case of the modified Norwood operation for a 21-day-old neonate with hypoplastic left heart syndrome (MS and AS) associated with an aberrant right subclavian artery and a persistent left superior vena cava. The modified Norwood operation was performed without total circulatory arrest and Cardiac arrest. A 4 mm Gore-Tex graft, which was anastomosed between the right carotid artery and the right pulmonary artery for systemic-pulmonary shunt, was used for cerebral perfusion during aortic arch reconstruction. Coronary perfusion was performed with a small cannula placed on the relatively large ascending aorta during anastomosis between the main pulmonary artery and the ascending aorta. Equine pericardial patch was used for aortic arch reconstruction and the ascending aorta was directly anastomosed to a part of the main PA. Postoperative course was uneventful and postoperative MRI revealed no stenosis of the aortic arch and the pulmonary artery.     

Cervical approaches to the surgery of cervicothoracic neoplasia

Seven cases are presented to illustrate four cervical surgical approaches to the upper mediastinum and thorax. These surgical approaches are described and illustrated, and we have termed these the retrosternal, retroclavicular, posterior, and lateral approaches. Two cases of thymic cyst, one of recurrent metastatic pretracheal adenopathy, a case of tracheal stenosis, a foreign body of the left subclavian artery, a large Zenker's diverticulum, and a neoglottic reconstruction are presented to illustrate the surgical anatomy, dissection technique, and postoperative complications.     

Abnormal origin of the vertebral artery from the common carotid artery

An abnormal origin of the vertebral artery from the common carotid artery (VA-CC) may occur on the right or left side with different embryonic mechanisms. We describe a patient with a double developmental anomaly, a right VA-CC and a right aortic arch. The rotation of the aortic arch caused a "twist" of the embryonic mechanisms of VA-CC and misdirected the differential diagnosis of the embryonic mechanisms at first glance. We discuss the pivotal points in differentiating the embryonic mechanisms of VA-CC.