quantities was aspirated from the cysts, together with many daughter cysts. This fluid did not resemble at all the fluid which was obtained by thoracocentesis. After aspiration of the cysts and injection of formaldehyde, the cysts were excised. The postoperative course was uneventful, with complete recovery after one month.
Superior Vena Cava Syndrome Caused by Bronchogenic Cyst· Gumpeni Rammohan, M.D.; Herbert W. Berger, M.D., F.C.C.P.; FOtUJd La;am, M.D.; and Wilfrido ]. "Buhain, M.D., F.C.C.P.
DISCUSSION
The etiology and pathogenesis of chyliform pleural effusion are poorly understood. It is assumed that chylifonn pleural effusion is formed from degenerating cells in longstanding encysted effusions which have a high cell content. This is usually associated with chronic pleural effusions, such as those in tuberculosis, nephrotic syndrome, or neoplasm. 5, 7 Chyliform pleural effusion was also described in rheumatoid arthritis and other collagen diseases." However, we could not find a description of chyliform pleural effusion associated with echinococcosis in the English-language medical literature. Regarding the origin of this chyliform fluid, we have no definite explanation. However, the following two elements may be considered as causative factors: (1) a temporary damage to the right lymphatic thoracic duct that occurred while the Echinococcus cyst spread from the liver to the right pleural space (this damage was later healed, resulting in a longstanding, noncommunicating chyliform effusion); or (2) a coincidental finding of a longstanding, encapsulated pleural effusion. In this case, cell destruction and lipid degeneration of mesothelial cells resulted in the formation of the milky fluid.
1 Sors C, Debesse B, Heintz C, et al: Metastatic pulmonary echinococcosis of hepatic origin. Presse Moo 79 :2521-2525, 1971 2 Sarris MC: Secondary echinococcosis of the pleura following ruptured hydatid cyst of the lung: Report of a case. Dis Chest 43: 106-108, 1963 3 Lopez-Majano V: Biological diagnosis of pulmonary hydatidosis. Respiration 28:472-479, 1971 4 Jesiotr M, Romanoff H: Pneumothorax following rupture of a primary pleural hydatid cyst. J Thorac Cardiovasc Surg 63:594-596, 1972 5 COO JE, Aikawa JK: Cholesterol pleural effusion. Arch Intern Med 108:163-174, 1961 6 Klepser RG, Berry JF: The diagnosis and surgical management of chylothorax with the aid of lipophilic dyes. Dis Chest 25:409-426, 1954 7 Bower GC: Chylothorax: Observations in 20 cases. Dis Chest 46:464-468, 1964 8 Bower GC: Chyliform pleural effusion in rheumatoid arthritis. Am Rev Resp Dis 97 :455-459, 1968
CHEST, 68: 4, OCTOBER, 1975
A patient with superior vena cava syndrome due to 811 infected benign mediastinal bronchogenic cyst is reported. Although bronchogenic cyst is a rare cause of the superior vena cava syndrome, it should be considered because it is a potentially treatable lesion. Patients with bronchogenic cysts should have elective surgery before major compHcations develop. cysts rarely cause the superior vena cava Bronchogenic syndrome. We recently treated a patient who de-
veloped superior vena cava obstruction due to a large benign bronchogenic cyst arising from the anterior mediastinum. A mediastinal mass was first detected 33 years ago. We report this case to illustrate that bronchogenic cysts may cause the superior vena cava syndrome, that not all cases of superior vena cava syndrome are due to malignant disease, and that elective surgery should be performed on bronchogenic cysts before serious complications develop. CASE REPORT
A 47-year-old woman was admitted to the Mount Sinai Hospital Services-City Hospital Center at Elmhurst on October 25, 1972, complaining of right pleuritic chest pain, shortness of breath, and nonproductive cough. She was known to have a right anterior mediastinal mass since the age of 14 years. A diagnosis of bronchogenic cyst was made at our institution four years previously when she was admitted for treatment of nonspecific chest pain. She refused surgery at that time and was followed-up in the chest clinic. During this period, the size of the lesion decreased and the patient remained asymptomatic until 17 days prior to admission. Physical examination on admission to the hospital revealed an orthopneic, tachypneic, and anxious-looking woman with marked jugular neck vein distension. Prominent superficial veins were present on the upper chest. Her blood pressure was 140/80 mm Hg in both upper arms, temperature 37.5°C, pulse rate 110 per minute, and respiratory rate 30 per minute. The central venous pressure was 40 em H20. Breath sounds were absent at the base of the right lung. The rest of the physical examination was unremarkable. Admission chest xray film (Fig 1) revealed a right anterior mediastinal mass measuring 10 em in diameter. This mass was larger than in the previous examination. A small "right pleural effusion was also present. Electrocardiogram showed sinus rhythm and incomplete right bundle branch block. The hematocrit was 30 percent and white blood cell count 8200 per cu mm with 68 percent polymorphonuclears, 25 percent lymphocytes, and 7 percent monocytes. Urinalysis revealed 2+ albumin. Because °From the Departments of Medicine and Surgery, Mount Sinai Hospital Services-City Hospital Center at Elmhurst, Elmhurst, New York, and The Mount Sinai School of Medicine of the City University of New York, New York City. Reprint requests: Dr. Berger, Mount Sinai Hospital Seroices, City Hospital Center at_Elmhurst, Elmhurst, New York 11373
SUPERIOR VENA CAVA SYNDROME 599
FIGURE 1. PA view of chest showing mass in superior mediastinum and small right pleural effusion.
of the sudden increase in the size of the mass and the evidence of superior vena cava obstruction, right thoracotomy was performed on Octoher 31, 1972. At surgery, a large fluid-filled cyst approximately 12 em in diameter was found adjacent to the right upper lobe. The medial wall of the cyst WlL~ adherent to the ascending aorta, right main hronchus, and the pulmonary artery . The superior vena cava and the azygos vein were dilated and measured about 5 em and 2.5 em across respectively. Complete removal of the cyst WlL~ not possihle due to adhesions. Aspiration of the cyst WlL~ performed with a Garlock trochar and 200 ml of cloudy, nonfoul smelling fluid was evacuated. Immediately after aspiration there was marked reduction in the size of the superior vena cava and azygos vein and both returned to normal size. Most of the lateral, anterior, inferior, and superior margins of the cyst were excised. However, the posterior wall of the cyst WlL~ left intact and the edge cauterized . The remaining medial wall was irrigated with hydrogen peroxide solution. The straw colored pleural fluid and the cloudy fluid from the cyst grew Staphylococcus aureus on culture . The pathologic diagnosis was bronchogenic cyst. The postoperative course was uneventful. On follow-up examination April 17, 1974, the patient WlL~ asymptomatic, without any evidence of superior vena cava syndrome. DISCUSSION
Bronchogenic cysts are congenital, usually benign lesions which arise in the lung parenchyma or mediastinum. The cysts result from an abnormal budding or branching of the tracheobronchial tree and are lined by ciliated columnar epithelium, the wall containing mucous glands, cartilage, elastic tissue, and smooth muscle. The pulmonary parenchyma is the most common location and there is a predilection for the lower lobes. Rogers and Osmer- reported 46 pathologically proved cases seen over a period of 15 years : 32 were within the pulmonary parenchyma and 14 in the mediastinum. Mediastinal bronchogenic cysts may be arbitrarily divided into the following groups: (I) paratracheal, (2) carinal, (3) hilar, (4) paraesophageal, and (5) miscellaneous; the hilar group is the most common.s The clinical course depends upon the size and location of the cysts. Symptoms may be produced by pressure on adjacent structures such as tracheobronchial tree,
600 RAMMOHAN ET AL
esophagus, or blood vessels. Many patients are asymptomatic and the bronchogenic cysts are found on routine chest roentgenograms. Pressure on the trachea or large bronchi may lead to chronic infection of the respiratory passages. The majority of pulmonary bronchogenic cysts have a patent bronchial communication. Usually this communication is small, creating a stagnant pool of secretions behind it; therefore, secondary suppurative infections within these cysts are frequent and lead to cough, chest pain, and fever.' The mediastinal variety rarely communicates with the tracheobronchial tree; it is always solitary, but may be multiloculated. The cyst may increase in size rapidly due to hemorrhage or infection. Because of these potential dangers, a bronchogenic cyst should be removed when first diagnosed . Superior vena cava syndrome due to benign bronchogenic cyst is very uncommon. In the report by Dabbs and colleagues," of two cases of intrapericardial bronchogenic cysts, one patient had evidence of superior vena cava obstruction which was relieved after removal of the cyst. . Mclntire and Sykes' collected 502 cases of superior vena cava syndrome in 1949 and found that the three most common causes were malignant intrathoracic tumor, aortic aneurysm, and mediastinal fibrosis . A more recent review indicated that superior vena cava obstruction was secondary to primary intrathoracic malignancy in 76 percent of cases, 65 percent of which were bronchogenic carcinornas.s Obstruction of the superior vena cava gives rise to edema of the head, neck, and upper extremities, cyanosis, prominent superficial veins of the chest wall, and an increased venous pressure in the upper extremities. Benign intrathoracic lesions rarely cause this syndrome, but mediastinal fibrosis, dermoid cyst," intrathoracic goiter, 7 aortic aneurysm, and mediastinal cystic lymphangioma" have been reported. One should be aware of these benign causes since symptomatic venous obstruction is commonly a sign of inoperable malignancy. When the cyst cannot be completely resected, partial excision with destruction of the lining mucosa of the remaining portion gives good results." In our patient, the cyst might have been resected completely if surgery had been performed when the initial diagnosis was made four years previously. In our patient, the superior vena cava syndrome was due to a longstanding benign bronchogenic cyst which suddenly increased in size due to infection and produced superior vena cava obstruction. We are not aware of a similar report in the literature. REFERENCES
Rogers LF, Osmer JC: Bronchogenic cyst. A review of 46 cases. Am J Roentg 91 :273-283, 1964 2 Maier HC : Bronchogenic cysts of the mediastinum . Ann Surg 127:476-502, 1948 3 Dabbs CH, Berg R j-, Peirce EC II : Intrapericardial bronchogenic cysts. Report of two cases and probable embryologic explanation. J Thorac Surg 34:718-735, 1957 4 Mcintire FT, Sykes EM Jr : Obstruction of the superior
CHEST, 68: 4, OCTOBER, 1975
5
6 7 8
9
vena cava : A review of the literature and report of two personal cases. Ann Intern Med 30 :925-960, 1949 Banker VP, Maddison FE : Superior vena cava syndrome secondary to aortic dis ease. Report of two cases and review of th e literature. Che st 51 :656-662, 1967 Case records Mass. C en Hosp : N Eng J Med 224 :207-210, 1941 Silverst ein GE , Burke G, Goldberg D, et al : Superior ven a caval system obstruction caused by benign endothoracic goit er. Ch est .'56 :519-523, 1969 Daniel TM , Staub EW, Clark DE : Symptomatic venous compression from a mediastinal cystic lymphangioma. Ch est 63 :834-835, 1973 Adam s WE, Thornton TF : Bronchogenic cysts of the mediastinum with a report of three cases. J Thorac Surg 12:503-516, 1943
Right Cervical Aortic Arch Associated with a Ventricular Septal Defect* Margo M. Schleman, M.D .;oO Leslie A. Koru, M.D .;t Norman Gootman, M.D .;: and Dan iel Silbert , M.D.§
A patient with a right cervical aortic arch and ventricular septal defect is presented. This is the second reported case to have this rare combination of defects and the first to have survived corrective surgery. The x-ray film findings are discussed, and the similarity to the right posterior aortic arch is stressed. °From the Departments of Pedi atrics and Radiology, Long Island Jewish-Hillside Medical Center, New Hyde Park, NY, and the School of Medicine, Health Sciences Center, State University of New York at Stony Brook. ° ° Instructor in Pedi atri cs. tAssociate Prof essor of Radiology. : Associate Prof essor of Ped iatrics. §Assistant Profes sor of Pediatrics. Reprint requ ests: Dr. Schleman, Long Island Jewish Medi cal Cent er, New Hyde Park, New York 11040
T
h is case report is of a child with a ventricular septal d efect a nd a right cervical aortic arch. This is the 16th known case of a cervical arch but only the second to have an as sociated intracardiac lesion. The purpose of this report is to familiarize the reader with the clinical features of the cervical arch syndrome and to pl ace the cervical arch into an accepted aortic arch clas sifica tion . CASE REPORT
After a normal pregnancy and delivery, the child was noted at birth to have a systolic cardiac murmur, Catheterization at two week s of age revealed a large ventricular septal defect, severe pulmonary hypertension, and increas ed pulmonary vascular resistance. The aortic angiogram showed the aortic arc h to be tortuous and to the right of th e trach ea. At six months of age, pulmonary artery banding was perform ed because of continued high pulmonary vascular resistance and poor weight gain . Cardiac catheterization was repeated at three years of age. The ventricular septal defect was aga in found to be larg e. The righ t ventricular pre ssur es were at systemic levels, but th e pulmonary pres sures distal to the band were normal, An aortic angiogra m demonst rated the arch to be quite unusual. It ascend ed on the right to T-I , passed behind the trachea and esophagus at T -3, and descended to the left of the vertebral column. The gre at vessels came off in the following sequence : left common carotid , right external carotid, right internal carotid, and right subclavian (Fig 1 and 2) . The left subclavi an came off last from a diverticulum of the descending aorta. No compression of the trachea was noted. A year later , the child retu rned to the hospital for successful correc tion of her ventricular sep tal defect and removal of the pulmonary art ery band. Postoperatively, the child was noted to have a right sup raclavicular bruit and thrill wh ich had not been appreciated preoperati vely. Th is finding was interpreted as transmission of the cervical aortic puls e. DISCUSSION
Cervical aortic arch is a rare anatomic finding that characteristically reaches in to the upper thorax or cer-
FIGURE lA (le ft ). Aort ogr am . Posteroanterior view shows high cervical position of arch and successive takeoff of left carotids, right external, and internal carotids. FIGURE IB (right) . Labeled drawing of Figure lA .
CHEST, 68: 4, OCTOBER, 1975
RIGHT CERVICAL AORTIC ARCH 601
Superior Vena Cava Syndrome Caused by Bronchogenic Cyst· Gumpeni Rammohan, M.D.; Herbert W. Berger, M.D., F.C.C.P.; FOtUJd La;am, M.D.; and Wilfrido ]. "Buhain, M.D., F.C.C.P.
DISCUSSION
The etiology and pathogenesis of chyliform pleural effusion are poorly understood. It is assumed that chylifonn pleural effusion is formed from degenerating cells in longstanding encysted effusions which have a high cell content. This is usually associated with chronic pleural effusions, such as those in tuberculosis, nephrotic syndrome, or neoplasm. 5, 7 Chyliform pleural effusion was also described in rheumatoid arthritis and other collagen diseases." However, we could not find a description of chyliform pleural effusion associated with echinococcosis in the English-language medical literature. Regarding the origin of this chyliform fluid, we have no definite explanation. However, the following two elements may be considered as causative factors: (1) a temporary damage to the right lymphatic thoracic duct that occurred while the Echinococcus cyst spread from the liver to the right pleural space (this damage was later healed, resulting in a longstanding, noncommunicating chyliform effusion); or (2) a coincidental finding of a longstanding, encapsulated pleural effusion. In this case, cell destruction and lipid degeneration of mesothelial cells resulted in the formation of the milky fluid.
1 Sors C, Debesse B, Heintz C, et al: Metastatic pulmonary echinococcosis of hepatic origin. Presse Moo 79 :2521-2525, 1971 2 Sarris MC: Secondary echinococcosis of the pleura following ruptured hydatid cyst of the lung: Report of a case. Dis Chest 43: 106-108, 1963 3 Lopez-Majano V: Biological diagnosis of pulmonary hydatidosis. Respiration 28:472-479, 1971 4 Jesiotr M, Romanoff H: Pneumothorax following rupture of a primary pleural hydatid cyst. J Thorac Cardiovasc Surg 63:594-596, 1972 5 COO JE, Aikawa JK: Cholesterol pleural effusion. Arch Intern Med 108:163-174, 1961 6 Klepser RG, Berry JF: The diagnosis and surgical management of chylothorax with the aid of lipophilic dyes. Dis Chest 25:409-426, 1954 7 Bower GC: Chylothorax: Observations in 20 cases. Dis Chest 46:464-468, 1964 8 Bower GC: Chyliform pleural effusion in rheumatoid arthritis. Am Rev Resp Dis 97 :455-459, 1968
CHEST, 68: 4, OCTOBER, 1975
A patient with superior vena cava syndrome due to 811 infected benign mediastinal bronchogenic cyst is reported. Although bronchogenic cyst is a rare cause of the superior vena cava syndrome, it should be considered because it is a potentially treatable lesion. Patients with bronchogenic cysts should have elective surgery before major compHcations develop. cysts rarely cause the superior vena cava Bronchogenic syndrome. We recently treated a patient who de-
veloped superior vena cava obstruction due to a large benign bronchogenic cyst arising from the anterior mediastinum. A mediastinal mass was first detected 33 years ago. We report this case to illustrate that bronchogenic cysts may cause the superior vena cava syndrome, that not all cases of superior vena cava syndrome are due to malignant disease, and that elective surgery should be performed on bronchogenic cysts before serious complications develop. CASE REPORT
A 47-year-old woman was admitted to the Mount Sinai Hospital Services-City Hospital Center at Elmhurst on October 25, 1972, complaining of right pleuritic chest pain, shortness of breath, and nonproductive cough. She was known to have a right anterior mediastinal mass since the age of 14 years. A diagnosis of bronchogenic cyst was made at our institution four years previously when she was admitted for treatment of nonspecific chest pain. She refused surgery at that time and was followed-up in the chest clinic. During this period, the size of the lesion decreased and the patient remained asymptomatic until 17 days prior to admission. Physical examination on admission to the hospital revealed an orthopneic, tachypneic, and anxious-looking woman with marked jugular neck vein distension. Prominent superficial veins were present on the upper chest. Her blood pressure was 140/80 mm Hg in both upper arms, temperature 37.5°C, pulse rate 110 per minute, and respiratory rate 30 per minute. The central venous pressure was 40 em H20. Breath sounds were absent at the base of the right lung. The rest of the physical examination was unremarkable. Admission chest xray film (Fig 1) revealed a right anterior mediastinal mass measuring 10 em in diameter. This mass was larger than in the previous examination. A small "right pleural effusion was also present. Electrocardiogram showed sinus rhythm and incomplete right bundle branch block. The hematocrit was 30 percent and white blood cell count 8200 per cu mm with 68 percent polymorphonuclears, 25 percent lymphocytes, and 7 percent monocytes. Urinalysis revealed 2+ albumin. Because °From the Departments of Medicine and Surgery, Mount Sinai Hospital Services-City Hospital Center at Elmhurst, Elmhurst, New York, and The Mount Sinai School of Medicine of the City University of New York, New York City. Reprint requests: Dr. Berger, Mount Sinai Hospital Seroices, City Hospital Center at_Elmhurst, Elmhurst, New York 11373
SUPERIOR VENA CAVA SYNDROME 599
FIGURE 1. PA view of chest showing mass in superior mediastinum and small right pleural effusion.
of the sudden increase in the size of the mass and the evidence of superior vena cava obstruction, right thoracotomy was performed on Octoher 31, 1972. At surgery, a large fluid-filled cyst approximately 12 em in diameter was found adjacent to the right upper lobe. The medial wall of the cyst WlL~ adherent to the ascending aorta, right main hronchus, and the pulmonary artery . The superior vena cava and the azygos vein were dilated and measured about 5 em and 2.5 em across respectively. Complete removal of the cyst WlL~ not possihle due to adhesions. Aspiration of the cyst WlL~ performed with a Garlock trochar and 200 ml of cloudy, nonfoul smelling fluid was evacuated. Immediately after aspiration there was marked reduction in the size of the superior vena cava and azygos vein and both returned to normal size. Most of the lateral, anterior, inferior, and superior margins of the cyst were excised. However, the posterior wall of the cyst WlL~ left intact and the edge cauterized . The remaining medial wall was irrigated with hydrogen peroxide solution. The straw colored pleural fluid and the cloudy fluid from the cyst grew Staphylococcus aureus on culture . The pathologic diagnosis was bronchogenic cyst. The postoperative course was uneventful. On follow-up examination April 17, 1974, the patient WlL~ asymptomatic, without any evidence of superior vena cava syndrome. DISCUSSION
Bronchogenic cysts are congenital, usually benign lesions which arise in the lung parenchyma or mediastinum. The cysts result from an abnormal budding or branching of the tracheobronchial tree and are lined by ciliated columnar epithelium, the wall containing mucous glands, cartilage, elastic tissue, and smooth muscle. The pulmonary parenchyma is the most common location and there is a predilection for the lower lobes. Rogers and Osmer- reported 46 pathologically proved cases seen over a period of 15 years : 32 were within the pulmonary parenchyma and 14 in the mediastinum. Mediastinal bronchogenic cysts may be arbitrarily divided into the following groups: (I) paratracheal, (2) carinal, (3) hilar, (4) paraesophageal, and (5) miscellaneous; the hilar group is the most common.s The clinical course depends upon the size and location of the cysts. Symptoms may be produced by pressure on adjacent structures such as tracheobronchial tree,
600 RAMMOHAN ET AL
esophagus, or blood vessels. Many patients are asymptomatic and the bronchogenic cysts are found on routine chest roentgenograms. Pressure on the trachea or large bronchi may lead to chronic infection of the respiratory passages. The majority of pulmonary bronchogenic cysts have a patent bronchial communication. Usually this communication is small, creating a stagnant pool of secretions behind it; therefore, secondary suppurative infections within these cysts are frequent and lead to cough, chest pain, and fever.' The mediastinal variety rarely communicates with the tracheobronchial tree; it is always solitary, but may be multiloculated. The cyst may increase in size rapidly due to hemorrhage or infection. Because of these potential dangers, a bronchogenic cyst should be removed when first diagnosed . Superior vena cava syndrome due to benign bronchogenic cyst is very uncommon. In the report by Dabbs and colleagues," of two cases of intrapericardial bronchogenic cysts, one patient had evidence of superior vena cava obstruction which was relieved after removal of the cyst. . Mclntire and Sykes' collected 502 cases of superior vena cava syndrome in 1949 and found that the three most common causes were malignant intrathoracic tumor, aortic aneurysm, and mediastinal fibrosis . A more recent review indicated that superior vena cava obstruction was secondary to primary intrathoracic malignancy in 76 percent of cases, 65 percent of which were bronchogenic carcinornas.s Obstruction of the superior vena cava gives rise to edema of the head, neck, and upper extremities, cyanosis, prominent superficial veins of the chest wall, and an increased venous pressure in the upper extremities. Benign intrathoracic lesions rarely cause this syndrome, but mediastinal fibrosis, dermoid cyst," intrathoracic goiter, 7 aortic aneurysm, and mediastinal cystic lymphangioma" have been reported. One should be aware of these benign causes since symptomatic venous obstruction is commonly a sign of inoperable malignancy. When the cyst cannot be completely resected, partial excision with destruction of the lining mucosa of the remaining portion gives good results." In our patient, the cyst might have been resected completely if surgery had been performed when the initial diagnosis was made four years previously. In our patient, the superior vena cava syndrome was due to a longstanding benign bronchogenic cyst which suddenly increased in size due to infection and produced superior vena cava obstruction. We are not aware of a similar report in the literature. REFERENCES
Rogers LF, Osmer JC: Bronchogenic cyst. A review of 46 cases. Am J Roentg 91 :273-283, 1964 2 Maier HC : Bronchogenic cysts of the mediastinum . Ann Surg 127:476-502, 1948 3 Dabbs CH, Berg R j-, Peirce EC II : Intrapericardial bronchogenic cysts. Report of two cases and probable embryologic explanation. J Thorac Surg 34:718-735, 1957 4 Mcintire FT, Sykes EM Jr : Obstruction of the superior
CHEST, 68: 4, OCTOBER, 1975
5
6 7 8
9
vena cava : A review of the literature and report of two personal cases. Ann Intern Med 30 :925-960, 1949 Banker VP, Maddison FE : Superior vena cava syndrome secondary to aortic dis ease. Report of two cases and review of th e literature. Che st 51 :656-662, 1967 Case records Mass. C en Hosp : N Eng J Med 224 :207-210, 1941 Silverst ein GE , Burke G, Goldberg D, et al : Superior ven a caval system obstruction caused by benign endothoracic goit er. Ch est .'56 :519-523, 1969 Daniel TM , Staub EW, Clark DE : Symptomatic venous compression from a mediastinal cystic lymphangioma. Ch est 63 :834-835, 1973 Adam s WE, Thornton TF : Bronchogenic cysts of the mediastinum with a report of three cases. J Thorac Surg 12:503-516, 1943
Right Cervical Aortic Arch Associated with a Ventricular Septal Defect* Margo M. Schleman, M.D .;oO Leslie A. Koru, M.D .;t Norman Gootman, M.D .;: and Dan iel Silbert , M.D.§
A patient with a right cervical aortic arch and ventricular septal defect is presented. This is the second reported case to have this rare combination of defects and the first to have survived corrective surgery. The x-ray film findings are discussed, and the similarity to the right posterior aortic arch is stressed. °From the Departments of Pedi atrics and Radiology, Long Island Jewish-Hillside Medical Center, New Hyde Park, NY, and the School of Medicine, Health Sciences Center, State University of New York at Stony Brook. ° ° Instructor in Pedi atri cs. tAssociate Prof essor of Radiology. : Associate Prof essor of Ped iatrics. §Assistant Profes sor of Pediatrics. Reprint requ ests: Dr. Schleman, Long Island Jewish Medi cal Cent er, New Hyde Park, New York 11040
T
h is case report is of a child with a ventricular septal d efect a nd a right cervical aortic arch. This is the 16th known case of a cervical arch but only the second to have an as sociated intracardiac lesion. The purpose of this report is to familiarize the reader with the clinical features of the cervical arch syndrome and to pl ace the cervical arch into an accepted aortic arch clas sifica tion . CASE REPORT
After a normal pregnancy and delivery, the child was noted at birth to have a systolic cardiac murmur, Catheterization at two week s of age revealed a large ventricular septal defect, severe pulmonary hypertension, and increas ed pulmonary vascular resistance. The aortic angiogram showed the aortic arc h to be tortuous and to the right of th e trach ea. At six months of age, pulmonary artery banding was perform ed because of continued high pulmonary vascular resistance and poor weight gain . Cardiac catheterization was repeated at three years of age. The ventricular septal defect was aga in found to be larg e. The righ t ventricular pre ssur es were at systemic levels, but th e pulmonary pres sures distal to the band were normal, An aortic angiogra m demonst rated the arch to be quite unusual. It ascend ed on the right to T-I , passed behind the trachea and esophagus at T -3, and descended to the left of the vertebral column. The gre at vessels came off in the following sequence : left common carotid , right external carotid, right internal carotid, and right subclavian (Fig 1 and 2) . The left subclavi an came off last from a diverticulum of the descending aorta. No compression of the trachea was noted. A year later , the child retu rned to the hospital for successful correc tion of her ventricular sep tal defect and removal of the pulmonary art ery band. Postoperatively, the child was noted to have a right sup raclavicular bruit and thrill wh ich had not been appreciated preoperati vely. Th is finding was interpreted as transmission of the cervical aortic puls e. DISCUSSION
Cervical aortic arch is a rare anatomic finding that characteristically reaches in to the upper thorax or cer-
FIGURE lA (le ft ). Aort ogr am . Posteroanterior view shows high cervical position of arch and successive takeoff of left carotids, right external, and internal carotids. FIGURE IB (right) . Labeled drawing of Figure lA .
CHEST, 68: 4, OCTOBER, 1975
RIGHT CERVICAL AORTIC ARCH 601
