the CPAP in these cases serves as a means of assisted ventilation, with the applied pressure aiding in inflating the lungs during inspiration, partially compensating for the lack of diaphragmatic contraction. Since the expiratory system is relatively intact, the applied pressure can be overcome by contraction of the expiratory muscles. Theoretically, constant negative pressure applied to the chest wall should be equally effective. Since the lungs of these babies may have relatively normal compliance, the pressure employed should be kept at the minimum effective in maintaining adequate blood gases. Nasal CPAP, utilizing a low pressure of only 5 em H 20, permitted adequate ventilation to be maintained until spontaneous improvement occurred. This therapy may reduce the need for more invasive techniques.
REFERENCES 1 Greene W, L'Heureux P, Hunt CE: Paralysis of the diaphragm. Am J Dis Child 129:1402-1405, 1975 2 Bucci G, Marzetti G, Picece-Bucci S, et al: Phrenic nerve palsy treated by continuous positive pressure breathing by nasal cannula. Arch Dis Child 49:230-232, 1974 3 Behrman RE, Mangurten H: Birth injuries. In Neonatology: Diseases of the Fetus and Infant (Behrman RE, ed). St. Louis, CV Mosby Co, 1973, pp 83-84 4 Richard J, Chevalier V, Capelle R, et al: La paralysie diaphragmatique obstaricale: A propos de dix cas. Arch Francaises de Pediatrie 14:563-598, 1957 3 Anagnostakis D, Economou-~avrou C, Moschos ~· et ~: Diaphragmatic paralysis in the newborn. Arch Dis Child 48:977-979, 1973 6 Adams FH, Gyepes MT: Diaphragmatic paralysis in the newborn infant simulating cyanotic heart disease. J Pediatr 78:119-121, 1971 . 7 Bingham JAW: Two cases of unilateral paralysis of the diaphragm in the newborn treated surgically. Thorax 9:248-252,1954 8 Erzsebet K, Tamas M, Tfbor V, et al: Fokozott 16g(J.ti nyomUsal tamogatott respiratio ( CPAP) Ujszulottelc rekesz-an6mali8ban. Orvosi Hetilap 116:679-680, 1975 9 Gregory GA, Kitterman JA, Phibbs RH, et al: Treatment of the idiopathic respiratory-distress syndrome with continuous positive airway pressure. N Eng} J Med 284:13331340,1971
A 50-year-old male elprette smoker witb a poRerlor parenchymal lmag mass developed a bloody, e:mdatlve pleunl eftusion. Patbologie examination of tbe resected 111111 eonirmed the preoperative diagnOiis of an Intralobar pulmonary sequestration. pulmonary sequestration is a congenital I ntralobar anomaly in which aberrant pulmonary tissue seis questered within a lower lobe of the lung, usually a posterior basal segment. It receives its blood supply from a systemic artery and has no normal communication with the bronchial tree or pulmonary arteries. 1 The anomaly is found either on routine chest roentgenogram or when some well-recognized complication occurs. The case reported here is that of a man with an intralobar sequestration associated with the rare complication of a bloody pleural effusion.
CASE REPoRT A 50-year-old man was referred for evaluation of an asymptomatic mass noted on routine chest roentgenogram. He had a 30 pack-year history of cigarette abuse, but no respiratory symptoms. Results of physical examination were normal. The chest roentgenogram showed a rounded opacity in the right lower lobe posteriorly with no evidence of pleural disease. Old films were not available. Sputum examination was negative for acid-fast bacilli, tumor cells, and fungi. Fluoroscopic examination demonstrated that the lesion was located within the lung parenchyma, did not change in size or shape with changes in transpulmonary pressure, and did not pulsate. Fiberoptic bronchoscopy was normal. A bronchogram showed no communication between the broncbial tree and the mass. A perfusion lung scan showed generalized hypoperfusion of the right lung but no segmental defects. An aortogram demonstrated two 2-mm vessels passing through the diaphragm into the mass. He was discharged with the diagnosis of probable intralobar sequestration and hypoplastic lung. He returned 15 months later with a one-month history of dull, aching, right-sided chest pain without hemoptysis or other symptoms. He denied any trauma. A chest roentgenogram revealed a right pleural effusion. The previously noted mass was unchanged in appearance (Fig 1). Tuberculin skin
Bloody Pleural EHusion Associated With an Intralobar Pulmonary Sequestration• Melofn It PrtlltM, M.D.;•• John T. Ktr8rnmiJrliJn, M.D.,1 and
AlUm D. Ericklon, M.D.:
•From the ~ of Medicine, Veterau Administration Hospital, and the Division of Biological and Medical Sciences, Brown University, Providence, RL • •Research Fellow. tAssociate Professor of Medicine. :Assistant Professor of Medicine.
394 PRAnD, IWMMERLEII, ERICKSON 394
F'JGtJRB 1. Right posterior mass density and pleural effusion are seen.
CHEST, 75: 3, MARCH, 1979
test ( PPD-S) induced no induration. Bleeding measurements, including platelet count, prothrombin time, and partial thromboplastin time, were all within normal limits. Thoracocentesis revealed grossly hemorrhagic fluid containing WBCs, 1,350/cu mm, and RBCs, 580,000/cu mm. The protein concentration was 4.9 gm/100 ml; glucose was 84 mg/100 ml; and lactic dehydrogenase, 965 IU/liter. Cope needle biopsy showed only chronic inflammation on histologic examination of the pleura. Stains for fungi and tubercle bacilli were negative. Cultures of the pleural fluid and the biopsy material were negative for aerobic and anaerobic bacteria, Mycobacterium tuben:uloris, and fungi. Cytologic examination findings were negative for malignant cells. The patient underwent an elective thoracotomy, and a 3cm 6rm mass was found within the right lower lobe. The surgeon noted &brous adhesions but no other speci&c pleural pathologic findings. On inspection, prior to lobectomy, the two feeder arteries were identi&ed and ligated. Pathologic examination showed the mass to be atelectatic parenchyma surrounded by a dense 6brous wall. No communication between the mass and the bronchial tree was demonstrable. The structure was not lined by bronchial epithelium. No bleeding site was found. DISCUSSION
Bloody pleural effusions are commonly associated with puhnonary infarction, malignant involvement of the pleura, and chest trauma. Although a bloody effusion may be associated with other diseases, eg, pancreatitis and uremia, its presence in a 50-year-old smoker with a mass lesion seen on chest roentgenogram almost always signifies malignancy. A careful review of the literature revealed only two previous reports of bloody effusions associated with intralobar sequestration. Each of those cases was characterized by the sudden development of a massive hemothorax, and immediate thoracotomy was required in both patients to effect hemostasis. 1 •8 This case is the first report of an association between an intralobar sequestration and a subacute bloody, exudative, pleural effusion. Since there was no clinical or pathologic evidence for any of the recognized causes of hemothorax, we can only assume its occurrence was related to the sequestration. It is attractive to speculate that clinically inapparent trauma caused one of these thin abnonnal vessels, which have been described as lacking the usual musculature of systemic arteries, to leak blood into the pleural space.' Intralobar puhnonary sequestration may be associated with other congenital abnonnalities, including situs inversus, the scimitar or venolobar syndrome, and diaphragmatic hernias. 5 In the absence of other anomalies, intralobar sequestration is discovered in the symptomatic patient almost exclusively because of complicating infection.8 However, when an undiagnosed roentgenographic • abnonnality is compatible with an intralobar sequesqation, the presence of a bloody pleural effusion should neither eliminate this benign process from consideration, nor deter one from obtaining the appropriate diagnostic studies.
fu:FmtENCJ!'S 1 Spencer H: Pathology of the Lung. Pbi1adelphia, WB Saunders, 1977 p 84
CHEST, 75: 3, MARCH, 1979
2 Zumbro GL, Green DC, Brott W, et al: Pulmonuy ~equestration with spontaueons intrapleural hemorrhage. J Thorac Cardiovasc Surg 68:673-674, 1974 3 Oxman LM: Intralobar sequestration causinl hemoptysis and hemot:hotu. NY State J Med 74:961-96i, 1974 4 Pryce DM: Lower accessory pulmonuy aartery with intra· lobar sequestration of lung: A report of seven cases. I Pathol Bact 58:457-467, 1946 5 Felson B: Chest Roentgenology. Philadelphia, WB Saunders,1973 6 Marks C, Wiener SN, Reydman IC: Pulmonuy sequestration. Chest 61:253-257, 1972
Spontaneous Closure of Atrial Septal Defect With Cardiac Failure in Infancy* Andrea C.V. Giardina. M.D.;•• Arthur S. .Raptoulil, M.D.;t Mary Allen Engle, M.D., F.C.C.P.;t and Aaron R. Leofn, M.D.§
Two serial eardiae catheterlzatlou before the ap of four months and another at sb: yean con&rmed I(IOntaneons dosnre of a laqe atrial septal defec:t wblda cansed eon.. gestlve heart fallnn in infaney. bile atrial septal defect (ASD) is one of the most W frequently encountered congenital malformations
of the heart, it is not commonly diagnosed in infancy, and it rarely causes problems early in life. Nonetheless, this usually benign lesion may produce congestive heart failure and even death during infancy and childhood.1 •1 Another uncommon aspect of the natural history of ASD is its spontaneous_, closure, and quite rare is spontaneous closure of an isolated secundum-type of ASD with heart failure in infancy. 8 • 8 This report presents serial cardiac catheterization data that demonstrated such an occurrence.
The patient seemed well for his &rst two months, then tachypnea with wheezing appeared. He was foUDd to be in cardiac failure with tachycardia, tachypnea, cardiomegaly, and hepatomegaly, but no cyanosis. A grade 2/6 systolic mur~ mur with midsystolic accentuation and early systolic ejection sound was heard at the second right and left int8rcoltal ~. in the suprasternal notch, and over both lung&elds posteriorly, •From the Divflion of Pediatric Cardiology. The New York Hospital-Cornell University Medical Centmf New York. ••Assistant AttendinJr in Pediatrics, Beth lsrae Medical Center. ~ent of Pediatrics, Beth Israel Medical Center, New YOrk. tAttending Ph~cian in Pediatric Cardiology, Orlando Regional MediCal Centm, the Depa$1ent of Pediatrics, Orlando Regional Medical Center, Florida. :tProfessor of Pediatrics, The New York Hospital-Cornell University Medical Center.
§Professor of Pediatrics, The New York Hospital-Cornell
University Medical Center. .Rsprint reqU6818: Dr. Engls, 'NBW Yorlc HOif'lltJZ, 'NBW York Cifr/100!1
SPONTANEOUS CLOSURE OF ASD -
REFERENCES 1 Greene W, L'Heureux P, Hunt CE: Paralysis of the diaphragm. Am J Dis Child 129:1402-1405, 1975 2 Bucci G, Marzetti G, Picece-Bucci S, et al: Phrenic nerve palsy treated by continuous positive pressure breathing by nasal cannula. Arch Dis Child 49:230-232, 1974 3 Behrman RE, Mangurten H: Birth injuries. In Neonatology: Diseases of the Fetus and Infant (Behrman RE, ed). St. Louis, CV Mosby Co, 1973, pp 83-84 4 Richard J, Chevalier V, Capelle R, et al: La paralysie diaphragmatique obstaricale: A propos de dix cas. Arch Francaises de Pediatrie 14:563-598, 1957 3 Anagnostakis D, Economou-~avrou C, Moschos ~· et ~: Diaphragmatic paralysis in the newborn. Arch Dis Child 48:977-979, 1973 6 Adams FH, Gyepes MT: Diaphragmatic paralysis in the newborn infant simulating cyanotic heart disease. J Pediatr 78:119-121, 1971 . 7 Bingham JAW: Two cases of unilateral paralysis of the diaphragm in the newborn treated surgically. Thorax 9:248-252,1954 8 Erzsebet K, Tamas M, Tfbor V, et al: Fokozott 16g(J.ti nyomUsal tamogatott respiratio ( CPAP) Ujszulottelc rekesz-an6mali8ban. Orvosi Hetilap 116:679-680, 1975 9 Gregory GA, Kitterman JA, Phibbs RH, et al: Treatment of the idiopathic respiratory-distress syndrome with continuous positive airway pressure. N Eng} J Med 284:13331340,1971
A 50-year-old male elprette smoker witb a poRerlor parenchymal lmag mass developed a bloody, e:mdatlve pleunl eftusion. Patbologie examination of tbe resected 111111 eonirmed the preoperative diagnOiis of an Intralobar pulmonary sequestration. pulmonary sequestration is a congenital I ntralobar anomaly in which aberrant pulmonary tissue seis questered within a lower lobe of the lung, usually a posterior basal segment. It receives its blood supply from a systemic artery and has no normal communication with the bronchial tree or pulmonary arteries. 1 The anomaly is found either on routine chest roentgenogram or when some well-recognized complication occurs. The case reported here is that of a man with an intralobar sequestration associated with the rare complication of a bloody pleural effusion.
CASE REPoRT A 50-year-old man was referred for evaluation of an asymptomatic mass noted on routine chest roentgenogram. He had a 30 pack-year history of cigarette abuse, but no respiratory symptoms. Results of physical examination were normal. The chest roentgenogram showed a rounded opacity in the right lower lobe posteriorly with no evidence of pleural disease. Old films were not available. Sputum examination was negative for acid-fast bacilli, tumor cells, and fungi. Fluoroscopic examination demonstrated that the lesion was located within the lung parenchyma, did not change in size or shape with changes in transpulmonary pressure, and did not pulsate. Fiberoptic bronchoscopy was normal. A bronchogram showed no communication between the broncbial tree and the mass. A perfusion lung scan showed generalized hypoperfusion of the right lung but no segmental defects. An aortogram demonstrated two 2-mm vessels passing through the diaphragm into the mass. He was discharged with the diagnosis of probable intralobar sequestration and hypoplastic lung. He returned 15 months later with a one-month history of dull, aching, right-sided chest pain without hemoptysis or other symptoms. He denied any trauma. A chest roentgenogram revealed a right pleural effusion. The previously noted mass was unchanged in appearance (Fig 1). Tuberculin skin
Bloody Pleural EHusion Associated With an Intralobar Pulmonary Sequestration• Melofn It PrtlltM, M.D.;•• John T. Ktr8rnmiJrliJn, M.D.,1 and
AlUm D. Ericklon, M.D.:
•From the ~ of Medicine, Veterau Administration Hospital, and the Division of Biological and Medical Sciences, Brown University, Providence, RL • •Research Fellow. tAssociate Professor of Medicine. :Assistant Professor of Medicine.
394 PRAnD, IWMMERLEII, ERICKSON 394
F'JGtJRB 1. Right posterior mass density and pleural effusion are seen.
CHEST, 75: 3, MARCH, 1979
test ( PPD-S) induced no induration. Bleeding measurements, including platelet count, prothrombin time, and partial thromboplastin time, were all within normal limits. Thoracocentesis revealed grossly hemorrhagic fluid containing WBCs, 1,350/cu mm, and RBCs, 580,000/cu mm. The protein concentration was 4.9 gm/100 ml; glucose was 84 mg/100 ml; and lactic dehydrogenase, 965 IU/liter. Cope needle biopsy showed only chronic inflammation on histologic examination of the pleura. Stains for fungi and tubercle bacilli were negative. Cultures of the pleural fluid and the biopsy material were negative for aerobic and anaerobic bacteria, Mycobacterium tuben:uloris, and fungi. Cytologic examination findings were negative for malignant cells. The patient underwent an elective thoracotomy, and a 3cm 6rm mass was found within the right lower lobe. The surgeon noted &brous adhesions but no other speci&c pleural pathologic findings. On inspection, prior to lobectomy, the two feeder arteries were identi&ed and ligated. Pathologic examination showed the mass to be atelectatic parenchyma surrounded by a dense 6brous wall. No communication between the mass and the bronchial tree was demonstrable. The structure was not lined by bronchial epithelium. No bleeding site was found. DISCUSSION
Bloody pleural effusions are commonly associated with puhnonary infarction, malignant involvement of the pleura, and chest trauma. Although a bloody effusion may be associated with other diseases, eg, pancreatitis and uremia, its presence in a 50-year-old smoker with a mass lesion seen on chest roentgenogram almost always signifies malignancy. A careful review of the literature revealed only two previous reports of bloody effusions associated with intralobar sequestration. Each of those cases was characterized by the sudden development of a massive hemothorax, and immediate thoracotomy was required in both patients to effect hemostasis. 1 •8 This case is the first report of an association between an intralobar sequestration and a subacute bloody, exudative, pleural effusion. Since there was no clinical or pathologic evidence for any of the recognized causes of hemothorax, we can only assume its occurrence was related to the sequestration. It is attractive to speculate that clinically inapparent trauma caused one of these thin abnonnal vessels, which have been described as lacking the usual musculature of systemic arteries, to leak blood into the pleural space.' Intralobar puhnonary sequestration may be associated with other congenital abnonnalities, including situs inversus, the scimitar or venolobar syndrome, and diaphragmatic hernias. 5 In the absence of other anomalies, intralobar sequestration is discovered in the symptomatic patient almost exclusively because of complicating infection.8 However, when an undiagnosed roentgenographic • abnonnality is compatible with an intralobar sequesqation, the presence of a bloody pleural effusion should neither eliminate this benign process from consideration, nor deter one from obtaining the appropriate diagnostic studies.
fu:FmtENCJ!'S 1 Spencer H: Pathology of the Lung. Pbi1adelphia, WB Saunders, 1977 p 84
CHEST, 75: 3, MARCH, 1979
2 Zumbro GL, Green DC, Brott W, et al: Pulmonuy ~equestration with spontaueons intrapleural hemorrhage. J Thorac Cardiovasc Surg 68:673-674, 1974 3 Oxman LM: Intralobar sequestration causinl hemoptysis and hemot:hotu. NY State J Med 74:961-96i, 1974 4 Pryce DM: Lower accessory pulmonuy aartery with intra· lobar sequestration of lung: A report of seven cases. I Pathol Bact 58:457-467, 1946 5 Felson B: Chest Roentgenology. Philadelphia, WB Saunders,1973 6 Marks C, Wiener SN, Reydman IC: Pulmonuy sequestration. Chest 61:253-257, 1972
Spontaneous Closure of Atrial Septal Defect With Cardiac Failure in Infancy* Andrea C.V. Giardina. M.D.;•• Arthur S. .Raptoulil, M.D.;t Mary Allen Engle, M.D., F.C.C.P.;t and Aaron R. Leofn, M.D.§
Two serial eardiae catheterlzatlou before the ap of four months and another at sb: yean con&rmed I(IOntaneons dosnre of a laqe atrial septal defec:t wblda cansed eon.. gestlve heart fallnn in infaney. bile atrial septal defect (ASD) is one of the most W frequently encountered congenital malformations
of the heart, it is not commonly diagnosed in infancy, and it rarely causes problems early in life. Nonetheless, this usually benign lesion may produce congestive heart failure and even death during infancy and childhood.1 •1 Another uncommon aspect of the natural history of ASD is its spontaneous_, closure, and quite rare is spontaneous closure of an isolated secundum-type of ASD with heart failure in infancy. 8 • 8 This report presents serial cardiac catheterization data that demonstrated such an occurrence.
The patient seemed well for his &rst two months, then tachypnea with wheezing appeared. He was foUDd to be in cardiac failure with tachycardia, tachypnea, cardiomegaly, and hepatomegaly, but no cyanosis. A grade 2/6 systolic mur~ mur with midsystolic accentuation and early systolic ejection sound was heard at the second right and left int8rcoltal ~. in the suprasternal notch, and over both lung&elds posteriorly, •From the Divflion of Pediatric Cardiology. The New York Hospital-Cornell University Medical Centmf New York. ••Assistant AttendinJr in Pediatrics, Beth lsrae Medical Center. ~ent of Pediatrics, Beth Israel Medical Center, New YOrk. tAttending Ph~cian in Pediatric Cardiology, Orlando Regional MediCal Centm, the Depa$1ent of Pediatrics, Orlando Regional Medical Center, Florida. :tProfessor of Pediatrics, The New York Hospital-Cornell University Medical Center.
§Professor of Pediatrics, The New York Hospital-Cornell
University Medical Center. .Rsprint reqU6818: Dr. Engls, 'NBW Yorlc HOif'lltJZ, 'NBW York Cifr/100!1
SPONTANEOUS CLOSURE OF ASD -
