1975, British Journal of Radiology, 48, 494-496
Case reports Neonatal respiratory distress due to a bronchogenic cyst By D. Beatty Crawford, M.B., D.M.R.D., F.R.C.R.,* Kook Sang Oh, M.D., and D. W. Shermeta, M.D. Departments of Radiology and Pediatric Surgery, The Johns Hopkins Medical Institution, Baltimore, Maryland 21205, U.S.A. {Received August, 1974, and in revised form February, 1975) CASE REPORT
A three-month-old Iranian girl was referred to the Johns Hopkins Hospital (JHH) because of increasing respiratory distress. She was a product of a full-term spontaneous vaginal delivery. Antenatal history was normal. The parents were first cousins, as were the mother's parents. It was the third pregnancy; the first ended with spontaneous abortion at three months and the second resulted in an infant who died in the first 24 hours from severe respiratory distress syndrome. The infant did well until six weeks of age when she was noted to have respiratory distress. A chest radiograph at that time showed overinflation of the left lung with mediastinal shift towards the right. The right lung showed patchy radiodensities and loss of volume. She was treated at home with antibiotics and steroids and, although she improved slightly on initial therapy, she was never symptom free. Eventually the symptoms became worse, and she was admitted to a hospital in Iran. When there was no improvement in her condition, she was transferred to JHH with a diagnosis of congenital lobar emphysema. On arrival, her weight was 3,650 grams, pulse 120 per minute, and respiratory rate 60 per minute. The infant was in moderate respiratory distress with retractions and
was cyanotic. The volume of the thoracic cage was increased and the left side was tympanic and free of breath sounds. Rales were heard on the right side. Repeat chest radiographs (Fig. 1A and B) showed no change. We noted stretching of the left pulmonary vessels and saw no evidence of collapse of the left lower lobe. The right lung was quite
*Present address: University of Maryland Hospital, Baltimore, Maryland 21201.
FIG. 1. Chest radiographs (antero-posterior and lateral) demonstrate overinflation of the left lung with gross herniation across the anterior mediastinum to the right. The mediastinum is shifted towards the right and there are aeas of infiltrate in the right lung. There is marked stretching of the pulmonary vessels on the left lung without pulmonary vascular redistribution.
494
JUNE 1975
Case reports small with many scattered areas of increased density in the region of the right middle and upper lobes, consistent with atelectasis and/or pneumonia. The heart was normal. There was no evidence of abscess or pleural effusion. Chest fluoroscopy showed normal movement of the right hemidiaphragm, but the left hemidiaphragm was fixed and moved only with the ribs, giving the effect of paradoxical movement. The appearances were thought to indicate massive air trapping in the entire left lung and a tube oesophagram was advised. Bronchoscopy, the next procedure, demonstrated almost total extrinsic obstruction of the left main stem bronchus. The tube oesophagram followed. To avoid aspiration, a feeding tube was introduced into the stomach and then withdrawn under fluoroscopic control to slightly below the carina. A small quantity of barium was injected, which demonstrated a round soft tissue mass at the level of the carina, causing extrinsic impression on the anterior aspect of the oesophagus. There also appeared to be some compression of the left main stem bronchus (Fig. 2). The findings were most striking on the lateral view with the oesophagus almost empty. The next day the patient underwent thoracotomy as a semi-emergency procedure. When the hilum of the left lung was first visualized, no immediate cause of the bronchial obstruction was evident. After dividing the ligamentum
arteriosum, the descending aorta was retracted laterally and the hilum medially to display a bluish cystic structure between the oesophagus and the left main stem bronchus. It proved to contain mucus and to arise in the posterior wall of the bronchus. Once it had been excised, all that remained of the posterior wall of the left main stem bronchus was the intact mucosa. Following removal, the left lung inflated and deflated satisfactorily. The cyst measured 1*5 cm in diameter. The lining was composed of respiratory epithelial cells and smooth muscle that contained bronchial glands. There was no evidence of cartilage. The appearance was considered typical of a bronchogenic cyst. Post-operatively, the left lung gradually returned to normal size and the child returned home after two weeks in satisfactory condition. DISCUSSION
The diagnosis of congenital lobar emphysema with which the child was referred was unlikely since the emphysema involved the entire left lung rather than a single lobe (Raynor, Capp and Sealy, 1967). The even aeration of the overinflated left lung ruled out congenital cystic lung (Avery, 1968).
FIG. 2. Tube oesophagram. Three different views demonstrate that the most diagnostic is the lateral view with an almost empty oesophagus (middle). This shows an oval opacity lying between the bronchus and oesophagus just below the carina. This lesion is causing an extrinsic impression on the oesophagus. W7ith the oesophagus full (far left) the lesion is partly obscured. The antero-posterior view (far right) is also helpful and suggests an impression on the right side of the oesophagus.
495
1975, British Journal of Radiology, 48, 496-498 Case reports
The fluoroscopic findings were consistent with a check valve obstruction of the left main stem bronchus rather than atelectasis of the right lung. The possible causes included a bronchogenic cyst (Avery, 1968; Brown and Robbins, 1944; Erakilis, Griscom and McGovern, 1969; Opsahl and Berman, 1962), cystic hygroma (Feutz et al., 1973), vascular anomaly, such as a pulmonary sling (Berdon and Baker, 1972; Capitanio, Ramos and Kirkpatrick, 1971), or an aspirated foreign body. The latter was unlikely because of the very young age. A pulmonary sling is caused by an anomalous left pulmonary artery arising from the right main pulmonary artery. It goes over the right main stem bronchus and then usually passes behind the lower trachea (Capitanio et al., 1971). It usually causes compression of the right main stem bronchus with resulting emphysema of the right lung. Small cystic hygroma or oesophageal duplications are unlikely to occlude a bronchus (Feutz et al., 1973). However, a bronchogenic cyst with an intimate relationship to the posterior aspect of the airway can cause partial obstruction even though the cyst is small and invisible on plain chest radiography (Avery, 1968; Erakilis et al., 1969; Opsahl and Berman, 1962). Bronchoscopy, which in this case was performed prior to the tube oesophagram is potentially dangerous because an infant's respiratory reserve is small and anything that even partially occludes the airway could result in severe respiratory failure. The child became severely distressed during bronchoscopy and at one stage stopped breathing, but
was quickly resuscitated without difficulty. Bronchography is contraindicated in these cases. The tube oesophagram was the most helpful investigation. This has been emphasized by Erakilis et al. (1969). One of their illustrations of a proven bronchogenic cyst is almost identical to ours. In our case a small soft tissue mass causing extrinsic pressure on the anterior aspect of the oesophagus at the level of the carina was demonstrated. The mass was best seen on the lateral view with the oesophagus virtually empty, a most important technical point.
REFERENCES AVERY, M. E., 1968. The Lung and Its Disorders in the New-
born Infant, 3rd edn., pp. 100-107 (W. B. Saunders Co., London, Philadelphia). BERDON, W. E., and BAKER, D. H., 1972. Vascular anomalies
and the infant lung: Rings, slings and other things. Seminars in Roentgenology, 7, 39—64. BROWN, R. K., and ROBBINS, L. L., 1944. The diagnosis
and treatment of bronchogenic cysts of the mediastinum and lung. Journal of Thoracic Surgery, 13, 84-105. CAPITANIO, M. D., RAMOS, R., and KIRKPATRICK, J., 1971.
Pulmonary sling, roentgen observations. American Journal of Roentgenology, 112, 28-34. ERAKILIS, A. J., GRISCOM, N. T., and MCGOVERN, J. B.,
1969. Bronchogenic cyst of mediastinum in infancy. New England Journal of Medicine, 281, 1150-1155. OPSAHL, T., and BERMAN, E. J., 1962. Bronchogenic
mediastinal cysts in infants: Case report and review of literature. Pediatrics, 30, 372-377. FEUTZ, E. P., UNE, H. Y., MANDELBAUM, I., and BRASHERAR,
R. E., Intrathoracic cystic hygroma. Radiology, 108, 61-66. RAYNOR, A. C , CAPP, M. P., and SEALY, W. C , 1967.
Lobar emphysema of infancy. The Annals of Thoracic Surgery, 4, 378-384.
Polysplenia By A . L. Baert, and J . Myle Department of Radiology, University Hospital St. Rafaell, Capucijnenvoer 35, B-3000 Leuven, Belgium {Received July, 1974, and in revised form November, 1974) ABSTRACT
Polysplenia is a rare syndrome of visceral anomalies. The diagnosis in vivo may be difficult. Recently Vaughan, Hawkins and Elliott (1971) stressed the importance of visceral arteriography for a correct diagnosis. A patient with this condition was seen by us and the angiographic findings are reported hereby. Case history A male patient, 42 years old, was submitted to an IVP because of right renal colic with microscopic haematuria. On the urogram (Fig. 1) several oval opacities superimposed on the liver shadow were seen. Tomograms demonstrated that the opacities had sharp margins, were posterior and varied from 2 cm to 7 cm.
When the renal symptoms had subsided an abdominal aortagram was performed. It showed the common hepatic artery originating from the superior mesenteric artery in the form of a common trunk. A second artery was seen on the right side partially superimposed on the course of the common hepatic artery. An injection (Fig. 2) at the level of the coeliac trunk revealed filling of a tortuous artery coursing to the right hypochondrium; this artery supplied the structures under investigation; the vascular pattern within these was normal, without any evidence of a tumour. During the parenchymatous phase there was heavy accumulation of contrast medium in the lesions, 2-3 seconds later (Fig. 3) small veins began to fill; these assembled to form one common venous trunk. The same injection also filled the left gastric
496
Case reports Neonatal respiratory distress due to a bronchogenic cyst By D. Beatty Crawford, M.B., D.M.R.D., F.R.C.R.,* Kook Sang Oh, M.D., and D. W. Shermeta, M.D. Departments of Radiology and Pediatric Surgery, The Johns Hopkins Medical Institution, Baltimore, Maryland 21205, U.S.A. {Received August, 1974, and in revised form February, 1975) CASE REPORT
A three-month-old Iranian girl was referred to the Johns Hopkins Hospital (JHH) because of increasing respiratory distress. She was a product of a full-term spontaneous vaginal delivery. Antenatal history was normal. The parents were first cousins, as were the mother's parents. It was the third pregnancy; the first ended with spontaneous abortion at three months and the second resulted in an infant who died in the first 24 hours from severe respiratory distress syndrome. The infant did well until six weeks of age when she was noted to have respiratory distress. A chest radiograph at that time showed overinflation of the left lung with mediastinal shift towards the right. The right lung showed patchy radiodensities and loss of volume. She was treated at home with antibiotics and steroids and, although she improved slightly on initial therapy, she was never symptom free. Eventually the symptoms became worse, and she was admitted to a hospital in Iran. When there was no improvement in her condition, she was transferred to JHH with a diagnosis of congenital lobar emphysema. On arrival, her weight was 3,650 grams, pulse 120 per minute, and respiratory rate 60 per minute. The infant was in moderate respiratory distress with retractions and
was cyanotic. The volume of the thoracic cage was increased and the left side was tympanic and free of breath sounds. Rales were heard on the right side. Repeat chest radiographs (Fig. 1A and B) showed no change. We noted stretching of the left pulmonary vessels and saw no evidence of collapse of the left lower lobe. The right lung was quite
*Present address: University of Maryland Hospital, Baltimore, Maryland 21201.
FIG. 1. Chest radiographs (antero-posterior and lateral) demonstrate overinflation of the left lung with gross herniation across the anterior mediastinum to the right. The mediastinum is shifted towards the right and there are aeas of infiltrate in the right lung. There is marked stretching of the pulmonary vessels on the left lung without pulmonary vascular redistribution.
494
JUNE 1975
Case reports small with many scattered areas of increased density in the region of the right middle and upper lobes, consistent with atelectasis and/or pneumonia. The heart was normal. There was no evidence of abscess or pleural effusion. Chest fluoroscopy showed normal movement of the right hemidiaphragm, but the left hemidiaphragm was fixed and moved only with the ribs, giving the effect of paradoxical movement. The appearances were thought to indicate massive air trapping in the entire left lung and a tube oesophagram was advised. Bronchoscopy, the next procedure, demonstrated almost total extrinsic obstruction of the left main stem bronchus. The tube oesophagram followed. To avoid aspiration, a feeding tube was introduced into the stomach and then withdrawn under fluoroscopic control to slightly below the carina. A small quantity of barium was injected, which demonstrated a round soft tissue mass at the level of the carina, causing extrinsic impression on the anterior aspect of the oesophagus. There also appeared to be some compression of the left main stem bronchus (Fig. 2). The findings were most striking on the lateral view with the oesophagus almost empty. The next day the patient underwent thoracotomy as a semi-emergency procedure. When the hilum of the left lung was first visualized, no immediate cause of the bronchial obstruction was evident. After dividing the ligamentum
arteriosum, the descending aorta was retracted laterally and the hilum medially to display a bluish cystic structure between the oesophagus and the left main stem bronchus. It proved to contain mucus and to arise in the posterior wall of the bronchus. Once it had been excised, all that remained of the posterior wall of the left main stem bronchus was the intact mucosa. Following removal, the left lung inflated and deflated satisfactorily. The cyst measured 1*5 cm in diameter. The lining was composed of respiratory epithelial cells and smooth muscle that contained bronchial glands. There was no evidence of cartilage. The appearance was considered typical of a bronchogenic cyst. Post-operatively, the left lung gradually returned to normal size and the child returned home after two weeks in satisfactory condition. DISCUSSION
The diagnosis of congenital lobar emphysema with which the child was referred was unlikely since the emphysema involved the entire left lung rather than a single lobe (Raynor, Capp and Sealy, 1967). The even aeration of the overinflated left lung ruled out congenital cystic lung (Avery, 1968).
FIG. 2. Tube oesophagram. Three different views demonstrate that the most diagnostic is the lateral view with an almost empty oesophagus (middle). This shows an oval opacity lying between the bronchus and oesophagus just below the carina. This lesion is causing an extrinsic impression on the oesophagus. W7ith the oesophagus full (far left) the lesion is partly obscured. The antero-posterior view (far right) is also helpful and suggests an impression on the right side of the oesophagus.
495
1975, British Journal of Radiology, 48, 496-498 Case reports
The fluoroscopic findings were consistent with a check valve obstruction of the left main stem bronchus rather than atelectasis of the right lung. The possible causes included a bronchogenic cyst (Avery, 1968; Brown and Robbins, 1944; Erakilis, Griscom and McGovern, 1969; Opsahl and Berman, 1962), cystic hygroma (Feutz et al., 1973), vascular anomaly, such as a pulmonary sling (Berdon and Baker, 1972; Capitanio, Ramos and Kirkpatrick, 1971), or an aspirated foreign body. The latter was unlikely because of the very young age. A pulmonary sling is caused by an anomalous left pulmonary artery arising from the right main pulmonary artery. It goes over the right main stem bronchus and then usually passes behind the lower trachea (Capitanio et al., 1971). It usually causes compression of the right main stem bronchus with resulting emphysema of the right lung. Small cystic hygroma or oesophageal duplications are unlikely to occlude a bronchus (Feutz et al., 1973). However, a bronchogenic cyst with an intimate relationship to the posterior aspect of the airway can cause partial obstruction even though the cyst is small and invisible on plain chest radiography (Avery, 1968; Erakilis et al., 1969; Opsahl and Berman, 1962). Bronchoscopy, which in this case was performed prior to the tube oesophagram is potentially dangerous because an infant's respiratory reserve is small and anything that even partially occludes the airway could result in severe respiratory failure. The child became severely distressed during bronchoscopy and at one stage stopped breathing, but
was quickly resuscitated without difficulty. Bronchography is contraindicated in these cases. The tube oesophagram was the most helpful investigation. This has been emphasized by Erakilis et al. (1969). One of their illustrations of a proven bronchogenic cyst is almost identical to ours. In our case a small soft tissue mass causing extrinsic pressure on the anterior aspect of the oesophagus at the level of the carina was demonstrated. The mass was best seen on the lateral view with the oesophagus virtually empty, a most important technical point.
REFERENCES AVERY, M. E., 1968. The Lung and Its Disorders in the New-
born Infant, 3rd edn., pp. 100-107 (W. B. Saunders Co., London, Philadelphia). BERDON, W. E., and BAKER, D. H., 1972. Vascular anomalies
and the infant lung: Rings, slings and other things. Seminars in Roentgenology, 7, 39—64. BROWN, R. K., and ROBBINS, L. L., 1944. The diagnosis
and treatment of bronchogenic cysts of the mediastinum and lung. Journal of Thoracic Surgery, 13, 84-105. CAPITANIO, M. D., RAMOS, R., and KIRKPATRICK, J., 1971.
Pulmonary sling, roentgen observations. American Journal of Roentgenology, 112, 28-34. ERAKILIS, A. J., GRISCOM, N. T., and MCGOVERN, J. B.,
1969. Bronchogenic cyst of mediastinum in infancy. New England Journal of Medicine, 281, 1150-1155. OPSAHL, T., and BERMAN, E. J., 1962. Bronchogenic
mediastinal cysts in infants: Case report and review of literature. Pediatrics, 30, 372-377. FEUTZ, E. P., UNE, H. Y., MANDELBAUM, I., and BRASHERAR,
R. E., Intrathoracic cystic hygroma. Radiology, 108, 61-66. RAYNOR, A. C , CAPP, M. P., and SEALY, W. C , 1967.
Lobar emphysema of infancy. The Annals of Thoracic Surgery, 4, 378-384.
Polysplenia By A . L. Baert, and J . Myle Department of Radiology, University Hospital St. Rafaell, Capucijnenvoer 35, B-3000 Leuven, Belgium {Received July, 1974, and in revised form November, 1974) ABSTRACT
Polysplenia is a rare syndrome of visceral anomalies. The diagnosis in vivo may be difficult. Recently Vaughan, Hawkins and Elliott (1971) stressed the importance of visceral arteriography for a correct diagnosis. A patient with this condition was seen by us and the angiographic findings are reported hereby. Case history A male patient, 42 years old, was submitted to an IVP because of right renal colic with microscopic haematuria. On the urogram (Fig. 1) several oval opacities superimposed on the liver shadow were seen. Tomograms demonstrated that the opacities had sharp margins, were posterior and varied from 2 cm to 7 cm.
When the renal symptoms had subsided an abdominal aortagram was performed. It showed the common hepatic artery originating from the superior mesenteric artery in the form of a common trunk. A second artery was seen on the right side partially superimposed on the course of the common hepatic artery. An injection (Fig. 2) at the level of the coeliac trunk revealed filling of a tortuous artery coursing to the right hypochondrium; this artery supplied the structures under investigation; the vascular pattern within these was normal, without any evidence of a tumour. During the parenchymatous phase there was heavy accumulation of contrast medium in the lesions, 2-3 seconds later (Fig. 3) small veins began to fill; these assembled to form one common venous trunk. The same injection also filled the left gastric
496
