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Unilateral emphysema in total anomalous pulmonary venous return
Volume 87 Number 3
4. 5. 6. 7. 8.
9. 10.
11.
12.
Brief clinical and laboratory observations
levels after childhood splenectomy, Arch Dis Child 45:114, 1970. Carlisle HN, and Saslaw S: Properdin levels in splenectomized persons, Proc Soc Exp Biol Med 102:150, 1959. Winkelstein JA: Opsonins: Their function, identity, and clinical significance, J PEDIATR82:747, 1973. Ellis EF, and Smith R_T: The role of the spleen in immunity, Pediatrics 37:111, 1966. Robinson MG, and Watson R J: Pneumococcal meningitis in sickle-cell anemia, N Engl J Med 274:1006, 1966. Pearson HA, Spencer RP, and Cornelius EA: Functional asplenia in sickle~cell anemia, N Engl J Meal 281:923, 1969. Diggs LW: Siderofibrosis of the spleen in sickle-cell anemia, JAMA 104:538, 1935. Winkelstein JA, and Drachman RH: Deficiency of pneumococcal serum opsonizing activity in sickle-cell disease, N EngI J Med 279:459, I968. Shin HS, and Mayer MM: The third component of the guinea pig complement system, Biochemistry 7:2991, 1968. Shin HS, Pickering RJ, and Mayer MM: The fifth component of the guinea pig complement system, J Immunol 106:473, 1971.
433
13. Shin HS, Smith MR, and Wood WB Jr: Heat labile opsonins to pneumococcus. II. Involvement of C3 and C5, J Exp Meal 130:1229, 1969. 14. Winkelstein JA, Shin HS, and Wood WB Jr: Heat labile opsonins to pneumococcus. III. Involvement of immunoglobulin and alternate pathway of C3 activation, J Immunol 108:1960, 1972. 15. Rowley DA: The formation of circulating antibody in the splenectomized human being following intravenous injection of heterologous erythrocytes, J Immunol 65:515, 1950. 16. Constantopoulos A, Najjar VA, Wish JB, Necheles TH, and Stolbach LL: Defective phagocytosis due to tuftsin deficiency in splenectomized subjects, Am J Dis Child 125:663, 1973. 17. Schulkind ML, Ellis EF, and Smith RT: Effect of antibody upon clearance of P~qabelled pneumococci by the spleen and liver, Pediatr Res 1:178, 1967. 18. Johnston RB Jr, Newman SL, and Struth AG: An abnormality of the alternate pathway of complement activation in sickle-cell disease, N Engl J Med 288:803, 1973.
Unilateral emphysema in total anomalous pulmonary venous return Rabi Sulayman, M.D., Otto Thilenius, M.D., Ph.D., Robert
Replogle, M.D., and Ren6 A. Arcilla, M.D.,*
Chicago, Ill:
A NEWLY RECOGNIZED ETIOLOGY for unilateral emphysema o f the left lung is described here. A n infant with total anomalous pulmonary venous return presented with unilateral emphysema due to a b n o r m a l course o f the c o m m o n vertical vein behind the left bronchus. Openheart surgery under deep hypothermia corrected the lesion. From the departments of Pediatrics and Surgery, The University of Chicago Pritzker School of Medicine. Supported in part by a grant (RR-305) from the General Clinieal Research Centers Program of the Division of Research Resources, National Institutes of Health. *Reprint address: 950 E. 59th St., Chicago, IlL 6063Z
Fig. 1. Frontal chest roentgeflogram before surgery, showing mediastinal shift, flattened left diaphragm, and pulmonary hypervascularity.
C A S E REPORT Patient L. B., a 3~k-month-old female, had had tachypnea since birth. She was admitted to another hospital because of severe respiratory distress and was transferred to the University of Chicago Wyler Children's Hospital. On admission, she had
434
Brief clinical and laboratory observations
The Journal of Pediatrics September 1975
p-r
Fig. 2. Cine-frame in frontal projection during pulmonary venous opacification following injection of contrast into pulmonary trunk. Note kinking of vertical vein at site of left bronchus (arrow). Circular shadow on right upper corner was from a monitoring stethoscope. VV, Vertical vein; SVC, superior vena cava.
Fig. 3. Composite tracings of the lateral cineangiocardiograms during opacification of the pulmonary trunk and, later, of the pulmonary veins to demonstrate their positional relations with the left main bronchus. PT, Pulmonary trunk; RPA, right main pulmonary artery; LPA, left main pulmonary artery; TR, trachea; L Br, left bronchus; VV, vertical vein.
respiratory distress a n d cyanosis; the respiratory rate was 70/ min; pulse rate, 180/min. There were coarse rfiles over each lung, and wheezing, more prominent on the left. Cardiac examination revealed a prominent right ventricular impulse, split and accentuated S~, and a faint ejection systolic murmur at the midprecordial area. The liver edge was palpable 3 cm below the right costal margin. There was no peripheral edema. Results of a blood count and urinalysis were not abnormal. The electrocardiogram showed right atrial and right ventricular hypertrophy. Serial chest roentgenograms demonstrated persistent cardiomegaly, hypervascularity, and progressive emphysema of the left lung with shift of the mediastinal structures to the right (Fig. 1). A barium esophagram disclosed no abnormality. The baby was digitalized and given oxygen as well as antibiotics. Two days after admission, positive pressure ventilation was started and continued for 16 days, up to the time of surgery. Serial arterial blood gas values were: Po2, 38 to 67 mm Hg; Pco.,, 17 to 48 mm Hg; pH, 7.17 to 7.44. An abbreviated cardiac catheterization was performed while the infant received positive pressure ventilation with 60% oxygen. Total anomalous pulmonary venous drainage into the left innominate vein was demonstrated. The right ventricular and pulmonary arterial pressures were 50/7 and 40/22 mm Hg, respectively. Injection of contrast material into the pulmonary trunk showed kinking of the vertical vein at the level of the left main bronchus (Fig. 2). No vascular ring was noted, nor was there abnormal origin or course
of the right or left pulmonary arteries. Bronchoscopy revealed localized narrowing of the left main bronchus, presumed to be due to extraluminal compression. Open-heart surgery was performed using profound hypothermia at 18~ and total circulatory arrest for 44 minutes. The common vertical vein was dilated; it coursed abnormally cephalad behind the left main bronchus. The latter appeared compressed by the vertical vein posteroinferiorly, and by the left pulmonary artery anteriorly. The transverse pulmonary vein was anastomosed to the left atrium, and the left atrial cavity was enlarged with a Dacron patch; the vertical vein was then ligated and cut. The postoperative course was uneventful; she was discharged 2 weeks later and has remained asymptomatic. At clinic follow-up 6 weeks after surgery, she had gained 6 pounds in weight. There was borderline evidence of right ventricular hypertrophy in the electrocardiogram, and the chest was radiologically clean. DISCUSSION The usual d r a i n a g e site in total a n o m a l o u s p u l m o n a r y venous return is into the left i n n o m i n a t e vein. T h e p u l m o n a r y veins from the right l u n g converge into a transverse c h a n n e l p r o c e e d i n g leftward a n d posterior to the left atrium; t h e left p u l m o n a r y veins j o i n this transverse vein to form a c o m m o n vertical vein w h i c h goes
Volume 87 Number 3
cephalad to open into the left innominate vein. In most instances, the vertical vein lies anterior to the left pulmonary artery; occasionally, it courses behind the latter but in front of the left main bronchus. The "sandwiched" vein is then often obstructed, resulting in severe pulmonary hypertension.1-5 In each instance the left bronchus lies posterior to the vessels and remains unobstructed. The vertical vein in our patient was most unusual, since it coursed behind the left bronchus. The latter was situated between the vertical vein (posteroinferiorly) and the left pulmonary artery (anteriorly) (Fig. 3). This accounted for the unilateral emphysema of the left lung. A similar complication in anomalous pulmonary venous return has not been reported previously. Unilateral emphysema in children is generally due to causes other than vascular compression of a bronchus. The usual types of vascular rings produce narrowing of the lower trachea but not of either bronchus. The only vascular anomaly presently known that can do this is the vascular sling syndrome6 in which the left pulmonary artery courses abnormally to the right of and then behind the right main bronchus before proceeding to the left lung. This can result in obstructive emphysema or even atelectasis of the right lung.
T lymphocyte differentiation in vitro in ataxia telangiectasia associated with lymphosarcoma
B r i e f clinical a n d laboratory observations
435
Our case demonstrates that unilateral left emphysema can result from abnormal course of the common vertical vein in anomalous pulmonary venous return. The anomaly is extremely rare; it can be corrected by openheart surgery.
REFERENCES 1. Mustard WT, Keon WJ, and Trusler GA: Transposition of the lesser veins (total anomalous pulmonary venous drainage), Progr Cardiovasc Dis 11:145, 1968. 2. Gathman GE, and Nadas AS: Total anomalous pulmonary venous connection; clinical and physiologic observations in 75 pediatric patients, Circulation 42:143, 1970. 3. Behrendt MD, Aberdeen E, Waterson JD, and Carter REB: Total anomalous pulmonary venous drainage in infants: I. Clinical and hemodynamic findings, methods and results of operation in 37 cases, Circulation 46:347, 1972. 4. Hastreiter AR, Paul MH, Molthan ME, and Miller RA: Total anomalous pulmonary venous connection with severe pulmonary venous obstruction: A clinical entity, Circulation 25:916, 1%2. 5. Nakib A, Moller JH, Kanjuh VI, and Edwards JE: Anomalies of the pulmonary veins, Am. J Cardiol 20:77, 1967. 6. Jue KL, Raghib G, Amplatz K, Adams P Jr, and Edwards JE: Anomalous origin of the left pulmonary artery from the right pulmonary artery; report of 2 cases and review of the literature, Am J Roentgenol 95:598, 1965.
TELANGIECTASIA is a poorly understood muttisystem disease? Recently, awareness of immunologic abnormalities has appeared, and humoral and cellular immunodeficiencies have been defined. 2 A striking feature is the frequent occurrence of malignancies, particularly reticulum cell sarcomas, lymphomas, ATAXIA
From the Memorial S.loan-Kettering Cancer Center.
Abbreviations used: BSA: bovine serum albumin PBL: peripheral blood lymphocytes NRS: normal rabbit serum HTLA: human T-lymphocyte antigen NBT: nitro blue tetrazolium ATCS: anti-human-T-cell serum SRBC: sheep red blood cells PHA: phytohemagglutinin Con A: concanavalin A PPD: purified protein derivative
Supported by grants from the National Cancer Institute, CA 08748-09 and CA 05826-13, and the National Foundation. A. Bernard is supported by a Fellowshipfrom DGRST, France. *Reprint address: Memorial Sloan Kettering Cancer Center, New York, N. Y. 10021
and lymphosarcomas (hematosarcomas). We report here a case of ataxia telangiectasia with a lymphosarcoma, in which we investigated the capacity of marrow and blood cells to develop T-cell markers under influence of extracts obtained from normal human thymus.
L. Boumsell, M.D., G. S. Incefy, Ph.D.,* A. Bernard, M.D., S. Schwartz, M.D., E. Smithwick, M.D., and R. A. Good, Ph.D., M.D., N e w York, N. Y.
4. 5. 6. 7. 8.
9. 10.
11.
12.
Brief clinical and laboratory observations
levels after childhood splenectomy, Arch Dis Child 45:114, 1970. Carlisle HN, and Saslaw S: Properdin levels in splenectomized persons, Proc Soc Exp Biol Med 102:150, 1959. Winkelstein JA: Opsonins: Their function, identity, and clinical significance, J PEDIATR82:747, 1973. Ellis EF, and Smith R_T: The role of the spleen in immunity, Pediatrics 37:111, 1966. Robinson MG, and Watson R J: Pneumococcal meningitis in sickle-cell anemia, N Engl J Med 274:1006, 1966. Pearson HA, Spencer RP, and Cornelius EA: Functional asplenia in sickle~cell anemia, N Engl J Meal 281:923, 1969. Diggs LW: Siderofibrosis of the spleen in sickle-cell anemia, JAMA 104:538, 1935. Winkelstein JA, and Drachman RH: Deficiency of pneumococcal serum opsonizing activity in sickle-cell disease, N EngI J Med 279:459, I968. Shin HS, and Mayer MM: The third component of the guinea pig complement system, Biochemistry 7:2991, 1968. Shin HS, Pickering RJ, and Mayer MM: The fifth component of the guinea pig complement system, J Immunol 106:473, 1971.
433
13. Shin HS, Smith MR, and Wood WB Jr: Heat labile opsonins to pneumococcus. II. Involvement of C3 and C5, J Exp Meal 130:1229, 1969. 14. Winkelstein JA, Shin HS, and Wood WB Jr: Heat labile opsonins to pneumococcus. III. Involvement of immunoglobulin and alternate pathway of C3 activation, J Immunol 108:1960, 1972. 15. Rowley DA: The formation of circulating antibody in the splenectomized human being following intravenous injection of heterologous erythrocytes, J Immunol 65:515, 1950. 16. Constantopoulos A, Najjar VA, Wish JB, Necheles TH, and Stolbach LL: Defective phagocytosis due to tuftsin deficiency in splenectomized subjects, Am J Dis Child 125:663, 1973. 17. Schulkind ML, Ellis EF, and Smith RT: Effect of antibody upon clearance of P~qabelled pneumococci by the spleen and liver, Pediatr Res 1:178, 1967. 18. Johnston RB Jr, Newman SL, and Struth AG: An abnormality of the alternate pathway of complement activation in sickle-cell disease, N Engl J Med 288:803, 1973.
Unilateral emphysema in total anomalous pulmonary venous return Rabi Sulayman, M.D., Otto Thilenius, M.D., Ph.D., Robert
Replogle, M.D., and Ren6 A. Arcilla, M.D.,*
Chicago, Ill:
A NEWLY RECOGNIZED ETIOLOGY for unilateral emphysema o f the left lung is described here. A n infant with total anomalous pulmonary venous return presented with unilateral emphysema due to a b n o r m a l course o f the c o m m o n vertical vein behind the left bronchus. Openheart surgery under deep hypothermia corrected the lesion. From the departments of Pediatrics and Surgery, The University of Chicago Pritzker School of Medicine. Supported in part by a grant (RR-305) from the General Clinieal Research Centers Program of the Division of Research Resources, National Institutes of Health. *Reprint address: 950 E. 59th St., Chicago, IlL 6063Z
Fig. 1. Frontal chest roentgeflogram before surgery, showing mediastinal shift, flattened left diaphragm, and pulmonary hypervascularity.
C A S E REPORT Patient L. B., a 3~k-month-old female, had had tachypnea since birth. She was admitted to another hospital because of severe respiratory distress and was transferred to the University of Chicago Wyler Children's Hospital. On admission, she had
434
Brief clinical and laboratory observations
The Journal of Pediatrics September 1975
p-r
Fig. 2. Cine-frame in frontal projection during pulmonary venous opacification following injection of contrast into pulmonary trunk. Note kinking of vertical vein at site of left bronchus (arrow). Circular shadow on right upper corner was from a monitoring stethoscope. VV, Vertical vein; SVC, superior vena cava.
Fig. 3. Composite tracings of the lateral cineangiocardiograms during opacification of the pulmonary trunk and, later, of the pulmonary veins to demonstrate their positional relations with the left main bronchus. PT, Pulmonary trunk; RPA, right main pulmonary artery; LPA, left main pulmonary artery; TR, trachea; L Br, left bronchus; VV, vertical vein.
respiratory distress a n d cyanosis; the respiratory rate was 70/ min; pulse rate, 180/min. There were coarse rfiles over each lung, and wheezing, more prominent on the left. Cardiac examination revealed a prominent right ventricular impulse, split and accentuated S~, and a faint ejection systolic murmur at the midprecordial area. The liver edge was palpable 3 cm below the right costal margin. There was no peripheral edema. Results of a blood count and urinalysis were not abnormal. The electrocardiogram showed right atrial and right ventricular hypertrophy. Serial chest roentgenograms demonstrated persistent cardiomegaly, hypervascularity, and progressive emphysema of the left lung with shift of the mediastinal structures to the right (Fig. 1). A barium esophagram disclosed no abnormality. The baby was digitalized and given oxygen as well as antibiotics. Two days after admission, positive pressure ventilation was started and continued for 16 days, up to the time of surgery. Serial arterial blood gas values were: Po2, 38 to 67 mm Hg; Pco.,, 17 to 48 mm Hg; pH, 7.17 to 7.44. An abbreviated cardiac catheterization was performed while the infant received positive pressure ventilation with 60% oxygen. Total anomalous pulmonary venous drainage into the left innominate vein was demonstrated. The right ventricular and pulmonary arterial pressures were 50/7 and 40/22 mm Hg, respectively. Injection of contrast material into the pulmonary trunk showed kinking of the vertical vein at the level of the left main bronchus (Fig. 2). No vascular ring was noted, nor was there abnormal origin or course
of the right or left pulmonary arteries. Bronchoscopy revealed localized narrowing of the left main bronchus, presumed to be due to extraluminal compression. Open-heart surgery was performed using profound hypothermia at 18~ and total circulatory arrest for 44 minutes. The common vertical vein was dilated; it coursed abnormally cephalad behind the left main bronchus. The latter appeared compressed by the vertical vein posteroinferiorly, and by the left pulmonary artery anteriorly. The transverse pulmonary vein was anastomosed to the left atrium, and the left atrial cavity was enlarged with a Dacron patch; the vertical vein was then ligated and cut. The postoperative course was uneventful; she was discharged 2 weeks later and has remained asymptomatic. At clinic follow-up 6 weeks after surgery, she had gained 6 pounds in weight. There was borderline evidence of right ventricular hypertrophy in the electrocardiogram, and the chest was radiologically clean. DISCUSSION The usual d r a i n a g e site in total a n o m a l o u s p u l m o n a r y venous return is into the left i n n o m i n a t e vein. T h e p u l m o n a r y veins from the right l u n g converge into a transverse c h a n n e l p r o c e e d i n g leftward a n d posterior to the left atrium; t h e left p u l m o n a r y veins j o i n this transverse vein to form a c o m m o n vertical vein w h i c h goes
Volume 87 Number 3
cephalad to open into the left innominate vein. In most instances, the vertical vein lies anterior to the left pulmonary artery; occasionally, it courses behind the latter but in front of the left main bronchus. The "sandwiched" vein is then often obstructed, resulting in severe pulmonary hypertension.1-5 In each instance the left bronchus lies posterior to the vessels and remains unobstructed. The vertical vein in our patient was most unusual, since it coursed behind the left bronchus. The latter was situated between the vertical vein (posteroinferiorly) and the left pulmonary artery (anteriorly) (Fig. 3). This accounted for the unilateral emphysema of the left lung. A similar complication in anomalous pulmonary venous return has not been reported previously. Unilateral emphysema in children is generally due to causes other than vascular compression of a bronchus. The usual types of vascular rings produce narrowing of the lower trachea but not of either bronchus. The only vascular anomaly presently known that can do this is the vascular sling syndrome6 in which the left pulmonary artery courses abnormally to the right of and then behind the right main bronchus before proceeding to the left lung. This can result in obstructive emphysema or even atelectasis of the right lung.
T lymphocyte differentiation in vitro in ataxia telangiectasia associated with lymphosarcoma
B r i e f clinical a n d laboratory observations
435
Our case demonstrates that unilateral left emphysema can result from abnormal course of the common vertical vein in anomalous pulmonary venous return. The anomaly is extremely rare; it can be corrected by openheart surgery.
REFERENCES 1. Mustard WT, Keon WJ, and Trusler GA: Transposition of the lesser veins (total anomalous pulmonary venous drainage), Progr Cardiovasc Dis 11:145, 1968. 2. Gathman GE, and Nadas AS: Total anomalous pulmonary venous connection; clinical and physiologic observations in 75 pediatric patients, Circulation 42:143, 1970. 3. Behrendt MD, Aberdeen E, Waterson JD, and Carter REB: Total anomalous pulmonary venous drainage in infants: I. Clinical and hemodynamic findings, methods and results of operation in 37 cases, Circulation 46:347, 1972. 4. Hastreiter AR, Paul MH, Molthan ME, and Miller RA: Total anomalous pulmonary venous connection with severe pulmonary venous obstruction: A clinical entity, Circulation 25:916, 1%2. 5. Nakib A, Moller JH, Kanjuh VI, and Edwards JE: Anomalies of the pulmonary veins, Am. J Cardiol 20:77, 1967. 6. Jue KL, Raghib G, Amplatz K, Adams P Jr, and Edwards JE: Anomalous origin of the left pulmonary artery from the right pulmonary artery; report of 2 cases and review of the literature, Am J Roentgenol 95:598, 1965.
TELANGIECTASIA is a poorly understood muttisystem disease? Recently, awareness of immunologic abnormalities has appeared, and humoral and cellular immunodeficiencies have been defined. 2 A striking feature is the frequent occurrence of malignancies, particularly reticulum cell sarcomas, lymphomas, ATAXIA
From the Memorial S.loan-Kettering Cancer Center.
Abbreviations used: BSA: bovine serum albumin PBL: peripheral blood lymphocytes NRS: normal rabbit serum HTLA: human T-lymphocyte antigen NBT: nitro blue tetrazolium ATCS: anti-human-T-cell serum SRBC: sheep red blood cells PHA: phytohemagglutinin Con A: concanavalin A PPD: purified protein derivative
Supported by grants from the National Cancer Institute, CA 08748-09 and CA 05826-13, and the National Foundation. A. Bernard is supported by a Fellowshipfrom DGRST, France. *Reprint address: Memorial Sloan Kettering Cancer Center, New York, N. Y. 10021
and lymphosarcomas (hematosarcomas). We report here a case of ataxia telangiectasia with a lymphosarcoma, in which we investigated the capacity of marrow and blood cells to develop T-cell markers under influence of extracts obtained from normal human thymus.
L. Boumsell, M.D., G. S. Incefy, Ph.D.,* A. Bernard, M.D., S. Schwartz, M.D., E. Smithwick, M.D., and R. A. Good, Ph.D., M.D., N e w York, N. Y.