Madabhavi i1, patel a1, anand a1, panchal h1, parikh s1. The may be due to obstruction external compression benign or malignant, fibrosis or thrombosis. Background superior vena cava svc syndrome is a set of clinical manifestations caused either by an svc. In both asymptomatic and symptomatic children with mediastinal masses, imaging plays a crucial role in making the diagnosis and developing a treatment plan. The majority of cases are caused by malignant tumors within the mediastinum, most commonly lung cancer and nonhodgkins lymphoma, directly compressing or invading the svc wall. A mass in the anterior superior mediastinum can cause. Recognition and management of superior vena cava syndrome. Rodeada by rigid structures and compress the upper originate vena cava syndrome svcs. Infants and children are more vulnerable to extrinsic airway compression than adults. Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. Jun 14, 2018 superior mesenteric artery syndrome can be severely debilitating and may require long term management, medications, costly parenteral nutrition intravenous feeding and rigorous followup.
Leiomyosarcoma in the mediastinum presenting as superior vena. Benign superior vena cava syndrome due to suppurative. Benign superior vena cava svc obstruction is an uncommon entity. A 5yearold boy presented with fever, cough, breathlessness and facial swelling. Mediastinal seminoma presenting with superior vena cava syndrome. Mediastinal masses may be nonvascular or vascular masses and represent congenital anomalies, infections, benign and malignant neoplasms, and pseudomasses. Index case report describes a middle adult, female, with superior vena caval syndrome and prevascular space mass on chest axial scan, and histological features revealing mrt of thymus and metastatic spread. Svcs, or superior vena cava syndrome can result from either partial or complete obstruction to blood flow via the svc to the right atrium by extrinsic compression or invasion.
Horner syndrome, phrenic nerve palsy, and palsy of the ipsilateral recurrent laryngeal nerve. Superior caval vein syndrome is the most severe complication of mediastinal syndromes and is considered a medical emergency. Superior mesenteric artery syndrome genetic and rare. Superior vena cava syndrome an overview sciencedirect topics. Superior vena cava svc obstruction most often is a complication of malignant tumors such as lung cancer or lymphoma.
Superior vena cava syndrome the american journal of medicine. It is a relatively common complication of lung cancer, and may constitute one of the early manifestations of this disease. The incidence of primary mediastinal bcell lymphoma pmbcl is disproportionately high in women of reproductive age. Surgical excision is the favorable treatment for ruptured. The patient being reported is a young male who presented. Anesthetic management of superior vena cava syndrome due to anterior mediastinal mass kapil chaudhary 1, anshu gupta 2, sonia wadhawan 3, divya jain 4, poonam bhadoria 3 1 department of anesthesiology and intensive care, maulana azad medical college and associated hospitals. Svco with significant and manifest tracheal compression with respiratory distress or failure while 1 presented. This article has been cited by other articles in pmc. Superior vena cava syndrome an overview sciencedirect. Primary mediastinal synovial sarcoma with subsequent development of primary adenoid cystic carcinoma of lung presenting as superior vena cava syndrome. Horners entity, it should be considered in the syndrome due to superior mediastinal schwannoma. Spontaneous rupture of a cystic mediastinal teratoma. Chest xray may detect the mass but does not evaluate compression or internal thrombosis of the superior vena cava, which is best assessed with ct with iv contrast.
A mass growing in the confined space of the mediastinum exerts pressure on existing structures, the veins being the mostsusceptible to external pressure are readily compressed, when obstruction becomes sufficient to occlude venous flow the patient develops superior vena caval syndrome. Us, ct, and mr imaging may allow detection of malrotation by showing the superior mesenteric artery and superior mesenteric vein in abnormal relative positions. Today most cases of superior vena caval syndrome re sult from infiltration of the mediastinum by malignant tumors,123 but, in the older literature, other causes. Pdf file of the complete article 421k, or click on a page image below to browse page by page. The common use of longterm indwelling central venous catheters also has added to the prevalence of svc obstruction. Malignant rhabdoid tumor mrt of the thymus is rare aggressive tumor and only 34 cases are reported in the literature till date. Pdf superior mediastinal syndrome due to intrathoracic. During the last century, progression in antibacterial therapies and improvement in. Primary mediastinal synovial sarcoma with subsequent. A superior mesenteric vein that is inverted relative to the superior mesenteric artery is highly indicative of malrotation. The patient died before theetiology of obstruction could be. Compression of mediastinal structures by any mass gives rise to a group of symptoms known as mediastinal syndrome mediastinal masses affect patients at any age and can be asymptomatic 3. Superior mediastinal syndrome sms is the term used when svcs coexists with obstruction of trachea.
The superior vena cava vcs is located in the anterior mediastinum, he carries venous blood from head, neck, upper extremities and upper. Superior vena cava syndrome is a clinical diagnosis, and associated imaging findings include widening of the mediastinum on radiographs and mediastinal soft tissue on ct, interruption of contrast material within the superior vena cava on contrastenhanced ct, and opacification of collateral vascularity fig. Superior vena cava syndrome in pediatric oncology sage journals. A positive pembertons sign is indicative of superior vena cava syndrome svc, commonly the result of a mass in the mediastinum.
Leiomyosarcoma in the mediastinum presenting as superior. Full text full text is available as a scanned copy of the original print version. Superior vena cava syndrome caused by mediastinal lymphoma. Mediastinal mass syndrome university of kwazulunatal. Mediastinal mass syndrome mms mediastinal mass syndrome refers to the clinical picture caused by the mediastinal mass effect in the anaesthetized patient. Superior caval vein syndrome is the most severe complication of mediastinal syndromes and is considered a. Notice the thickening ofthe tracheoesophageal stripe arrows. Herein we describe a case of svc syndrome caused by suppurative mediastinal lymphadenitis. Superior mediastinal syndrome with rowlandpayne syndrome.
Approximately 10% of tumours that occur in the anterior superior mediastinum compress the superior vena cava svc andor the proximal tracheal airway or main bronchus. He had an unusual constellation of findings not previously reported in neuroblastoma. Superior vena cava syndrome svcs, is a group of symptoms caused by obstruction of the superior vena cava svc, a short, wide vessel carrying circulating blood into the heart. We report the case of a caucasian 21yearold female, who presented with acute superior vena cava syndrome, having started 2 weeks before. Mediastinal masses can result in superior vena cava syndrome see figures 5179 through 5182. A pa and b lateral views of the chest show a widened upper mediastinal silhouette. Malignancies are predominant 95% while, in the past, infectious diseases used to be common. Hugh pemberton, who characterized it in 1946 the pemberton maneuver is a physical examination tool used to demonstrate the presence of latent pressure in the thoracic inlet. Superior vena cava syndrome svcs is a disease entity with notable signs and symptoms that cannot go unnoticed by clinicians. Superior vena cava syndrome in thoracic malignancies. This syndrome can result in acute cardiorespiratory decompensation that may present at any time during the intraoperative course and immediate postoperative period. Superior vena cava syndrome svcs refers to signs and symptoms caused by obstruction of the superior vena cava.
Anesth 18 5, 2006 superior vena caval syndrome in children a case report indira kumari, sunanda gupta and puneet pratap singhal introduction mediastinal masses in children are known to cause potentially life. The majority of cases are caused by malignant tumors within the mediastinum, most commonly lung cancer and nonhodgkins lymphoma, directly compressing or invading. Down syndrome trisomy 21 is the most common chromosomal abnormality among liveborn infants. The term superior mediastinal syndrome of ten used synonymously with svc syndrome in children in. Mediastinal fibrosis and superior vena cava syndrome. Owing to the fast growing nature of mediastinal bcell lymphoma, respiratory symptoms are pronounced at onset, with 30% to 50% presenting with clinical evidence of superior vena cava obstruction. Superior vena cava svc syndrome refers to the clinical manifestation due to an obstruction in the svc.
In patients with lymphoma, nonhodgkin lymphoma 80% was the most prevalent. To our knowledge, teratoma with rupture into the mediastinum complicated by superior vena cava syndrome has not been reported previously. A very large anterior mediastinal mass was identified and demonstrated to be an extragonadal seminoma. Superior mediastinal syndrome due to intrathoracic. Superior vena caval syndrome due to a mediastinal hematoma secondary to a dissecting aortic aneurysm thomas e. Chest radiographs showed superior mediastinal widening. Superior vena cava syndrome svcs superior mediastinal syndrome sms the svc is a thin walled vessel which is easily compressed by tumors or infection arising in adjacent lymph nodes and thymus. Anesthetic management of superior vena cava syndrome due. In children, malignant mediastinal masses are more common than benign mediastinal masses and they have relatively poor prognosis.
When vessel compression is associated with tracheal compression, it is termed superior mediastinal syndrome sms. Acute myeloid leukemia aml as a cause of sms is extremely uncommon in childhood. Superior mediastinal syndrome sms is the term used when svcs coexists with. Superior vena cava syndrome svcs refers to signs and symptoms caused by obstruction of the superior vena ca va. The following primary mediastinal tumours were found. Superior vena cava syndrome related to mediastinal lymphoma in. The organisms were anaerobic bacteria, which are part of the. Svcs is caused by compression, obstruction or thrombosis of superior vena cava. Paulson23 published a patient with superior vena caval syndrome in a series of 6i cases. With the clinical picture consistent with the superior vena cava svc syndrome, the patient was investigated with computed tomography of the chest. Superior vena cava obstruction secondary to mediastinal. Superior vena cava syndrome was caused by external compression of a loculated cystic. Comparison of benign and malignant mediastinal masses in.
A contrastenhanced computed tomog raphy ct scan of the chest demonstrated multiple venous collaterals figure 1b. Harlequin syndrome due to superior mediastinal neurinoma. In severe cases, malignant thymoma results in superior vena cava syndrome due to local invasion and compression. Superior mediastinal syndrome over 80% of mediastinal masses in children are malignant.
He was pale with distended veins over neck and chest, cervical and axillary lymphadenopathy and bilateral expiratory wheeze. Underestimated awakening faceedema caused by a mediastinal. During the last century, progression in antibacterial therapies and improvement in social conditions. Superior vena caval syndrome due to a mediastinal hematoma. Although the sign is most commonly described in patients with substernal goiters where the goiter corks off the thoracic inlet, 4 the maneuver is potentially useful in any patient with adenopathy, tumor, or. Leiomyosarcoma in the mediastinum presenting as superior vena cava syndrome ettayapuram v. Hence, the two terms are often used interchangeably in children. Etiology svc syndrome svcs may be related to various etiological factors. Uperior vena cava s v c syndrome arises from a mediastinal mass com pressing the svc and the veins that drain into it. Oct 10, 2012 superior vena cava syndrome svcs refers to signs and symptoms caused by obstruction of the superior vena cava. The authors hereby report a case of a child with aml who presented with sms at their hospital. The case is reported of a 70yearold man with clinical findings of superior vena caval obstruction. This report describes the first case of svc obstruction in a patient with cystic fibrosis due to extrinsic compression from benign reactive mediastinal.
The maneuver is achieved by having the patient elevate both arms usually 180 degrees anterior flexion at the shoulder until the forearms touch the sides of the face. However, it is important to recognize that a small percentage of svc syndromes are due to benign diseases such as mediastinal. Primary mediastinal synovial sarcoma presenting as superior. A mediastinal mass may also result in a compression of the superior vena cava superior vena cava syndrome, horners syndrome, or hoarseness due to compression of left laryngeal learn. Mar 23, 2019 the subgroup with superior mediastinal syndrome had relatively acute presentation and poor final outcome. Primary mediastinal synovial sarcoma presenting as. Diagnosis and management of superior vena cava syndrome. The commonest neoplastic cause of sms is non hodgkin lymphoma.
In children, a mediastinal pathology causing svcs generally results in sms as well, due to the limited chest volume. Superior vena cava syndrome due to mediastinal gardner. The obstruction can occur either due to tumour invasion of the vessel wall with associated thrombus or due to vessel wall compression by the tumour mass. Benign superior mediastinal syndrome sage journals. This report describes the case of a 35yearold male patient who presented with acute symptoms of dyspnoea, facial puffiness, voicehoarseness, and engorged neck veins. The longterm outlook prognosis can depend on whether the condition is diagnosed and treated in a timely manner. Case report open access superior mediastinal paraganglioma. Anesthetic management of superior vena cava syndrome due to. Feb 11, 2021 primary mediastinal thymic large bcell lymphoma is an unusual and aggressive nonhodgkin lymphoma that can present with superior vena cava syndrome. Hodgkins lymphoma and tb are the most common cause of malignant and benign mediastinal masses. Novella sanchez l, sanz herrero f, berraondo fraile j, et al. Superior mediastinal syndrome sms is an uncommon manifestation of malignant neoplastic disease in children. Oct 25, 2018 the superior vena cava svc located in anterior mediastinum, carries blood from the head, neck, upper extremities and upper chest. In the present study, three different cases of mediastinal syndrome, one of which was complicated by superior vena cava syndrome, are discussed.
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