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Dilated lymphatics in congenital lobar emphysema
Dilated lymphatics in congenital lobar emphysema Report of a second case David C. Hitch, M.D., George R. Minor, M.D., Alex R. Mitchell, M.D., and Theodore E. Keats, M.D., Charlottesville, Va.
COngenital lobar emphysema is life-threatening to the neonate. With prompt recognition and therapy, results have been gratifying. This report describes a rare variant of the disease. Although virtually unknown prior to 1930, congenital lobar emphysema is presently a well-recognized clinical and radiographic entity. In 1939, Overstreet' associated lobar emphysema in infants with a deficiency of cartilage in the lobar bronchus. It was not until 1945 that the first successful lobectomy was performed by Gross and Lewis- for the treatment of this condition. Since 1945, reports have become more abundant. Between 1935 and 1967, 113 cases of lobar emphysema were reported in the English and French literatures." In 1966, Allen and colleagues" reported the first case of lymphatic dilatation associated with congenital lobar emphysema. The etiology of lobar emphysema is not completely understood, although intrinsic bronchial obstruction has most often been cited. Gross and Lewis- first observed a deficiency in the bronchial cartilage. The involved lobar bronchi containing cartilage are said to expand during inspiration and collapse From the Departments of Surgery, Radiology, and Pathology, University of Virginia Medical Center, Charlottesville, Va. 22901. Received for publication April 27, 1973.
during expiration, thus causing obstruction. Internal obstruction may also be produced by redundant mucosal folds." Although it is uncommon, external compression of the bronchi by vascular structures or enlarged nodes has been cited by Raynor" as well as Leape and Longino. There is general agreement that the left upper lobe is most frequently affected, followed by the right middle and right upper lobes. The child with this condition is usually less than 6 months of age, more frequently male, and usually not premature." Cardiovascular abnormalities are the most frequently associated aberrations. Acute respiratory distress with dyspnea is the usual method of presentation. Infection of the respiratory tract is not associated with the symptoms. Raynor" states that, in addition to dyspnea, tachypnea, wheeze, cough, and cyanosis are frequently seen. Other clinical features include hyperresonance and decreased breath sounds, decreased respiratory excursion of the chest wall, and a shift of the apical impulse away from the emphysematous lobe. On radiographic examination, the affected lobe is more radiotransparent, with the mediastinum displaced toward the unaffected (or contralateral) side. The diaphragm is frequently depressed on the affected side, and there is atelectasis oj
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Fig . 1. Admission che st film showing the hone ycombed appear ance of the lung .
of the normal lobes . Fluoroscopy reveals little motion of the involved lobe. When the chest is opened, the involved lobe classically herniates through the incision. The texture is usually not remarkable, but the lobe generally docs not deflate on expiration.
Fig. 2. Excised left upper lobe showing the cystic areas on the surface of the lung and lung parenchyma, dotted with cystic spaces ranging from I to 5 mm . in diameter.
Case report A 19-day -old m ale infant was admitted to the University of Virginia Medical Center on Aug . 10, 197 I. The inf ant was the product of a 7 month pregnancy complicated by premature labor. Th e child's weight at birth wa s 998 grams. He cried spo ntaneously and had no app arent difficulty unt il the fifteenth day , when he became acutely cyanotic. A roentgenogram of the chest at that time revealed overexpansion of the left lung, with a shift of the he art and mediastinum to the right. as well as "cystic changes" of the left upper lung field. Oxygen a nd antibiotics (penicillin and kan amycin ) were instituted. Three da ys later, when the infant wa s 18 days old, oxygen was gradually discontinued ; however, he ate poorly, developed progressive abdominal distention, and had no spontaneous bow el movements. F ilms of th e abdomen and che st revealed generalized dil at ion of both the large and small bowel as well as the previously described pulmonary findings . The following day the infant was transferred to the University of Virginia Medic al Center. On admission, the infant weighed 1,160 grams. There was a n expiratory grunt with a respiratory rate of 38 bre aths per minute and a pulse rate of 152 beats per minute. His temperature was 36 ° C. Examination of the chest revealed mild overe xpansion with intercostal and suprasternal retrac-
tion s. Breath sounds were dimin ished on the right, and rale s were a udible on the left. There was egophony at the left ba se. The heart was shifted to the right. There were no audible or palp able murmurs. The abdomen was markedl y distended and ten se. The liver was palpable 2 em. below the right costal margin and the splenic tip 1 ern. below the left cost al margin. No bowel sounds were heard . The abdomen appeared to be tender to palp ation. Rectal examination revealed the anus to be patent. Laboratory dat a were unremarkable except for a hem atocrit value of 34 per cent a nd a white count of 13,800. A sample of art eri al blood showed a pH of 7.2 8, a Po, of 48 mm . Hg, and a Pee, of 44 mm. Hg, Rev iew of the pre vious films and those taken on admi ssion revealed genera lized overexpansion of the chest with emphysema of the left upper lobe , compensatory com pression of the remaining lung fields, and medi astinal shift to the right. There was a cystic or honeycombed appearance to the left upper lobe (Fig. I) . The rad iologic impression was that of a form of congenital cystic disease of the lung. Abdominal films showed dila tion of the bowel without evidence of mechanical ob struction. The infant was taken to the operating room on
Volume 66 Number 1 July, 1973
Congenital lobar emph ysema
12 9
Fig. 3. Lymphatic spaces extending to visceral pleura with foci of multinucleated giant cell reaction . (Hematoxylin and eosin; original magnification >'30. )
Fig. 4. Flat, single-layered endothelial cells contrasted to multinucleated giant cells. (Hematoxylin and eosin; or iginal magnification x215.)
the day of admission , at which time an aerated, hyperexpanded left upper lobe was found and removed . The left upper lobe was of normal color. The texture was somewhat coarser than the remaining normal lung. There were "cystic" areas on the surface and within the lung parenchyma (Fig. 2), which were assumed to be the result of rup ture of air into the peribronchial tissue s, although neither escape of air nor flow of lymph was noted postoperatively. After the operation, the infant continued to ha ve abdomin al distention and to be without bowel sounds or bowel movements. On the second postoperat ive day, free intraperitoneal air was seen on the roentgenogram. He was aga in taken to the operating room, at which time exploratory laparotomy revealed necrotizing entercolitis . A 23 em . length of necrotic small bowel was removed, a nd the continuity of the small bowel was restored by a single-layered, end -toend anastomosis. The infant did well after his second exploration. On his fifth postoperative day, he was taking oral alimentation. The diet was advanced easily. The infant was discharged home on the twentyfifth hospital day , weighing 2,280 grams. Followup at 7 months of age reve aled the chest to be clear to exam ination, the heart sounds to be normal, and the abdominal examination to be nor -
mal except for a right hydrocele. Radiographic examination of the chest showed the med iastinum to be in the normal position with the expected compensatory overexpansion of the remaining left lower lobe . Pathology. The left upper lobe measured 65 by 10 by 18 mm . On cut section, the parenchyma contained numerous cystic spaces varying in size from I to 5 mm. in diameter (Fig. 2). There was a mild emphysema present , characterized by distention of the terminal and respiratory bronchioles and alveoli. There were occa sional ruptured alveoli associated with a marked decrease in the elastic fibers that are seen in a similar-aged nor mal infant." The numerous cystic spaces observed in the gross specimen were not emphysematous changes but represented large cystic areas located in the interlobular and intralobular interstitium, often extending to the visceral pleura ( F ig. 3). These lymphatic spaces were lined by flat, singlelayered endothelial cells with interspersed multinucleated giant cells (Fig. 4). There was focal fibrosis in the lymphatic walls with focally intense, multinucleated giant cell reaction surrounded by nests of histiocytes, lymphocytes, and occa sional neutrophilic leukocytes. Several of the multinucleated giant cells were vacuolated. Red blood cells and pink -staining homogeneous rna-
The Journal of
130
Hitch et at.
Thoracic and Cardiovascular Surgery
terial were present in several of these lymphatic spaces.
Discussion Neonatal distress presents a challenge to the pediatrician and surgeon alike. When caused by lobar emphysema, an efficient solution can be offered, but recognition of this abnormality remains difficult. There has been reported only I other case of lobar emphysema with associated dilated lymphatics. These cases are similar in several ways: Both involved male infants; both infants developed respiratory distress; the microscopic appearance of the abnormal lobe was similar in the 2 cases; and both patients were successfully treated by lobectomy. The lymphatic dilatation occurred in the interstitial spaces, especially in the interlobular interstitium, and extended to the visceral pleura. The radiographic appearances were similar, with multiple linear densities giving. a honeycombed appearance to the lobe. These cystic areas appear to be homogeneous collections of lymph, surrounded by compressed lung parenchyma. The adjacent lobes were compressed, as is usually found in congenital lobar emphysema. The pathologic process appeared in each case to be confined to a single lobe and not to be a diffuse abnormality of lung parenchyma. The normal development of these 2 infants attests the isolated nature of the condition. Although no etiologic factor has been determined, developmental aberration of fetal lymphatics has been suggested and is supported by this case. Treatment of infants with this condition appears to be straightforward. As with congenital lobar emphysema, excision of the affected lung is curative. The
most common form of intervention is lobectomy. As with lobectomies for other conditions, the remaining lung appears to hyperexpand to fill the thoracic cavity. There likewise is no apparent immediate sequela of the condition once the abnormal lung has been excised. Summary A second case of lobar emphysema with associated lymphatic dilatation has been presented. In each infant, respiratory distress was the initial complaint; the radiographic appearance of the abnormal lung was of honeycombing; and, in each, marked dilatation of interstitial lymphatics was prominent. Successful treatment appears to be lobectomy. REFERENCES
2
3
4
5 6
7
8
9
Allen, R. P., Taylor, R. L., and Reiguam, C. W.: Congenital Lobar Emphysema With Dilated Septal Lymphatics, Radiology 86: 929, 1966. Gross, R. E., and Lewis, 1. E., Jf.: Defect of the Anterior Mediastinum, Surg. Gynecol. Obstet. 80: 549, 1945. Janovski, N. A., Balacki, J. A., and Keenan, F. D.: Congenital Segmental Lobar Emphysema, Med. Ann. D. C. 38: 249-254, 294, 1969. Leape, L. L., and Longino, L. A.: Infantile Lobar Emphysema, Pediatrics 34: 246, 1964. Murray, G. F.: Congenital Lobar Emphysema, Surg. Gynecol. Obstet. 124: 611, 1967. Nelson, T. T.: Tension Emphysema in Infants, Arch. Dis. Child. 32: 38, 1957. Overstreet, R. M.: Emphysema of a Portion of the Lung in the Early Months of Life, Am. J. Dis. Child. 57: 861, 1939. Raynor, A. C., Capp, M. P., and Sealy, W. C.: Lobar Emphysema of Infancy: Diagnosis, Treatment, and Etiologic Aspects, Ann. Thorac. Surg. 4: 374, 1967. Reyes, K.: Localized Hypertrophic Emphysema, Br. Med. J. 2: 659, 1938.
COngenital lobar emphysema is life-threatening to the neonate. With prompt recognition and therapy, results have been gratifying. This report describes a rare variant of the disease. Although virtually unknown prior to 1930, congenital lobar emphysema is presently a well-recognized clinical and radiographic entity. In 1939, Overstreet' associated lobar emphysema in infants with a deficiency of cartilage in the lobar bronchus. It was not until 1945 that the first successful lobectomy was performed by Gross and Lewis- for the treatment of this condition. Since 1945, reports have become more abundant. Between 1935 and 1967, 113 cases of lobar emphysema were reported in the English and French literatures." In 1966, Allen and colleagues" reported the first case of lymphatic dilatation associated with congenital lobar emphysema. The etiology of lobar emphysema is not completely understood, although intrinsic bronchial obstruction has most often been cited. Gross and Lewis- first observed a deficiency in the bronchial cartilage. The involved lobar bronchi containing cartilage are said to expand during inspiration and collapse From the Departments of Surgery, Radiology, and Pathology, University of Virginia Medical Center, Charlottesville, Va. 22901. Received for publication April 27, 1973.
during expiration, thus causing obstruction. Internal obstruction may also be produced by redundant mucosal folds." Although it is uncommon, external compression of the bronchi by vascular structures or enlarged nodes has been cited by Raynor" as well as Leape and Longino. There is general agreement that the left upper lobe is most frequently affected, followed by the right middle and right upper lobes. The child with this condition is usually less than 6 months of age, more frequently male, and usually not premature." Cardiovascular abnormalities are the most frequently associated aberrations. Acute respiratory distress with dyspnea is the usual method of presentation. Infection of the respiratory tract is not associated with the symptoms. Raynor" states that, in addition to dyspnea, tachypnea, wheeze, cough, and cyanosis are frequently seen. Other clinical features include hyperresonance and decreased breath sounds, decreased respiratory excursion of the chest wall, and a shift of the apical impulse away from the emphysematous lobe. On radiographic examination, the affected lobe is more radiotransparent, with the mediastinum displaced toward the unaffected (or contralateral) side. The diaphragm is frequently depressed on the affected side, and there is atelectasis oj
127
128
Hitch et al.
The Journal 01 Thora cic a nd Card iovascular Surgery
Fig . 1. Admission che st film showing the hone ycombed appear ance of the lung .
of the normal lobes . Fluoroscopy reveals little motion of the involved lobe. When the chest is opened, the involved lobe classically herniates through the incision. The texture is usually not remarkable, but the lobe generally docs not deflate on expiration.
Fig. 2. Excised left upper lobe showing the cystic areas on the surface of the lung and lung parenchyma, dotted with cystic spaces ranging from I to 5 mm . in diameter.
Case report A 19-day -old m ale infant was admitted to the University of Virginia Medical Center on Aug . 10, 197 I. The inf ant was the product of a 7 month pregnancy complicated by premature labor. Th e child's weight at birth wa s 998 grams. He cried spo ntaneously and had no app arent difficulty unt il the fifteenth day , when he became acutely cyanotic. A roentgenogram of the chest at that time revealed overexpansion of the left lung, with a shift of the he art and mediastinum to the right. as well as "cystic changes" of the left upper lung field. Oxygen a nd antibiotics (penicillin and kan amycin ) were instituted. Three da ys later, when the infant wa s 18 days old, oxygen was gradually discontinued ; however, he ate poorly, developed progressive abdominal distention, and had no spontaneous bow el movements. F ilms of th e abdomen and che st revealed generalized dil at ion of both the large and small bowel as well as the previously described pulmonary findings . The following day the infant was transferred to the University of Virginia Medic al Center. On admission, the infant weighed 1,160 grams. There was a n expiratory grunt with a respiratory rate of 38 bre aths per minute and a pulse rate of 152 beats per minute. His temperature was 36 ° C. Examination of the chest revealed mild overe xpansion with intercostal and suprasternal retrac-
tion s. Breath sounds were dimin ished on the right, and rale s were a udible on the left. There was egophony at the left ba se. The heart was shifted to the right. There were no audible or palp able murmurs. The abdomen was markedl y distended and ten se. The liver was palpable 2 em. below the right costal margin and the splenic tip 1 ern. below the left cost al margin. No bowel sounds were heard . The abdomen appeared to be tender to palp ation. Rectal examination revealed the anus to be patent. Laboratory dat a were unremarkable except for a hem atocrit value of 34 per cent a nd a white count of 13,800. A sample of art eri al blood showed a pH of 7.2 8, a Po, of 48 mm . Hg, and a Pee, of 44 mm. Hg, Rev iew of the pre vious films and those taken on admi ssion revealed genera lized overexpansion of the chest with emphysema of the left upper lobe , compensatory com pression of the remaining lung fields, and medi astinal shift to the right. There was a cystic or honeycombed appearance to the left upper lobe (Fig. I) . The rad iologic impression was that of a form of congenital cystic disease of the lung. Abdominal films showed dila tion of the bowel without evidence of mechanical ob struction. The infant was taken to the operating room on
Volume 66 Number 1 July, 1973
Congenital lobar emph ysema
12 9
Fig. 3. Lymphatic spaces extending to visceral pleura with foci of multinucleated giant cell reaction . (Hematoxylin and eosin; original magnification >'30. )
Fig. 4. Flat, single-layered endothelial cells contrasted to multinucleated giant cells. (Hematoxylin and eosin; or iginal magnification x215.)
the day of admission , at which time an aerated, hyperexpanded left upper lobe was found and removed . The left upper lobe was of normal color. The texture was somewhat coarser than the remaining normal lung. There were "cystic" areas on the surface and within the lung parenchyma (Fig. 2), which were assumed to be the result of rup ture of air into the peribronchial tissue s, although neither escape of air nor flow of lymph was noted postoperatively. After the operation, the infant continued to ha ve abdomin al distention and to be without bowel sounds or bowel movements. On the second postoperat ive day, free intraperitoneal air was seen on the roentgenogram. He was aga in taken to the operating room, at which time exploratory laparotomy revealed necrotizing entercolitis . A 23 em . length of necrotic small bowel was removed, a nd the continuity of the small bowel was restored by a single-layered, end -toend anastomosis. The infant did well after his second exploration. On his fifth postoperative day, he was taking oral alimentation. The diet was advanced easily. The infant was discharged home on the twentyfifth hospital day , weighing 2,280 grams. Followup at 7 months of age reve aled the chest to be clear to exam ination, the heart sounds to be normal, and the abdominal examination to be nor -
mal except for a right hydrocele. Radiographic examination of the chest showed the med iastinum to be in the normal position with the expected compensatory overexpansion of the remaining left lower lobe . Pathology. The left upper lobe measured 65 by 10 by 18 mm . On cut section, the parenchyma contained numerous cystic spaces varying in size from I to 5 mm. in diameter (Fig. 2). There was a mild emphysema present , characterized by distention of the terminal and respiratory bronchioles and alveoli. There were occa sional ruptured alveoli associated with a marked decrease in the elastic fibers that are seen in a similar-aged nor mal infant." The numerous cystic spaces observed in the gross specimen were not emphysematous changes but represented large cystic areas located in the interlobular and intralobular interstitium, often extending to the visceral pleura ( F ig. 3). These lymphatic spaces were lined by flat, singlelayered endothelial cells with interspersed multinucleated giant cells (Fig. 4). There was focal fibrosis in the lymphatic walls with focally intense, multinucleated giant cell reaction surrounded by nests of histiocytes, lymphocytes, and occa sional neutrophilic leukocytes. Several of the multinucleated giant cells were vacuolated. Red blood cells and pink -staining homogeneous rna-
The Journal of
130
Hitch et at.
Thoracic and Cardiovascular Surgery
terial were present in several of these lymphatic spaces.
Discussion Neonatal distress presents a challenge to the pediatrician and surgeon alike. When caused by lobar emphysema, an efficient solution can be offered, but recognition of this abnormality remains difficult. There has been reported only I other case of lobar emphysema with associated dilated lymphatics. These cases are similar in several ways: Both involved male infants; both infants developed respiratory distress; the microscopic appearance of the abnormal lobe was similar in the 2 cases; and both patients were successfully treated by lobectomy. The lymphatic dilatation occurred in the interstitial spaces, especially in the interlobular interstitium, and extended to the visceral pleura. The radiographic appearances were similar, with multiple linear densities giving. a honeycombed appearance to the lobe. These cystic areas appear to be homogeneous collections of lymph, surrounded by compressed lung parenchyma. The adjacent lobes were compressed, as is usually found in congenital lobar emphysema. The pathologic process appeared in each case to be confined to a single lobe and not to be a diffuse abnormality of lung parenchyma. The normal development of these 2 infants attests the isolated nature of the condition. Although no etiologic factor has been determined, developmental aberration of fetal lymphatics has been suggested and is supported by this case. Treatment of infants with this condition appears to be straightforward. As with congenital lobar emphysema, excision of the affected lung is curative. The
most common form of intervention is lobectomy. As with lobectomies for other conditions, the remaining lung appears to hyperexpand to fill the thoracic cavity. There likewise is no apparent immediate sequela of the condition once the abnormal lung has been excised. Summary A second case of lobar emphysema with associated lymphatic dilatation has been presented. In each infant, respiratory distress was the initial complaint; the radiographic appearance of the abnormal lung was of honeycombing; and, in each, marked dilatation of interstitial lymphatics was prominent. Successful treatment appears to be lobectomy. REFERENCES
2
3
4
5 6
7
8
9
Allen, R. P., Taylor, R. L., and Reiguam, C. W.: Congenital Lobar Emphysema With Dilated Septal Lymphatics, Radiology 86: 929, 1966. Gross, R. E., and Lewis, 1. E., Jf.: Defect of the Anterior Mediastinum, Surg. Gynecol. Obstet. 80: 549, 1945. Janovski, N. A., Balacki, J. A., and Keenan, F. D.: Congenital Segmental Lobar Emphysema, Med. Ann. D. C. 38: 249-254, 294, 1969. Leape, L. L., and Longino, L. A.: Infantile Lobar Emphysema, Pediatrics 34: 246, 1964. Murray, G. F.: Congenital Lobar Emphysema, Surg. Gynecol. Obstet. 124: 611, 1967. Nelson, T. T.: Tension Emphysema in Infants, Arch. Dis. Child. 32: 38, 1957. Overstreet, R. M.: Emphysema of a Portion of the Lung in the Early Months of Life, Am. J. Dis. Child. 57: 861, 1939. Raynor, A. C., Capp, M. P., and Sealy, W. C.: Lobar Emphysema of Infancy: Diagnosis, Treatment, and Etiologic Aspects, Ann. Thorac. Surg. 4: 374, 1967. Reyes, K.: Localized Hypertrophic Emphysema, Br. Med. J. 2: 659, 1938.