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Pulmonary sequestration with bronchoesophageal fistula
Pulmonary Sequestration with Bronchoesophageal
Fistula
By J. EUGENELEWIS ANDROBERT E. MURRAY
M
IJLTIPLE
CASES
of intralobar and extralobar pulmonary sequestration have been reported. 1,2 However, total pulmonary sequestration with esophageal communication is a rare anomaly.3,4 Recently a 4-month-old infant with this anomaly was successfully managed. The diagnosis and surgical treatment of this patient warrants a detailed report. CASE REPORT A 4-month-old white female weighing 10 pounds was transferred with a diagnosis of unresolved pneumonia involving the right lung. Allegedly
the infant
to
our
institution
developed
a cough and rhinorrhea at 2 months of age. No improveand antibiotics. The infant failed to gain weight and developed a persistent fever. Hospitalization and evaluation revealed opacihcation of the right lung with a shift of the mediastinum. After antibiotics, expectorants, and moist air failed to improve the pulmonary symptoms, the infant was transferred. On admission the patient was pale with a rectal temperature of 100” F and labored respirations. The right lung was dull to percussion. Breath sounds were absent over the right lung, there was decreased motion to the right chest, and the trachea was shifted to the right. The left lung was clear to percussion and auscultation. There were no other significant physical findings. Hemoglobin was 7.2 Gm. and the white blood count, 10,100. A chest radiograph revealed atelectasis of the right lung with a shift of the mediastinum to the right and hyperaeration of the left lung. There were areas in the right lung suggestive of cystic changes (Fig. IA). A diagnosis of staphylococcus pneumonia was suspected. Bronchoscopy was done and no right main stem bronchus could be visualized. The absence of a right main stem bronchus was confirmed by a bronchogram. (Fig. 1B). In addition it was noted that there appeared to be improved aeration of the right lung. These observations led to a diagnosis of a bronchoesophageal communication. This was confirmed by an esophagram which demonstrated a direct communication between the lower esophagus and the right lung (Fig. 1C).
ment was noted from expectorants
After preparation with whole blood and antibiotics, operation was performed through a right posterolateral thoracotomy. The pleural cavity entered through the sixth intercostal space (Fig 2A). Exploration revealed a collapsed lung of rubbery consistency covered with :r fibrinous membrane. The lung failed to expand with inspiration. On dissection and mobilization there was a systemic blood supply and a tubular structure connecting the lung to the distal one third of the esophagus. There were cartilage rings in the tubular structure adjacent to the lung (Fig 2B). The systemic blood supply was ligated and divide& From the Surgical Service of Cardinal Glennon Memorial Hospital for Children, Saint Louis, Missouri and the Department of Surgery Saint Louis Uniuersity School of Medicine, Saint Louis, Missouri. J. EUGENE LEWIS, JR., M.D.: Clinical Professor of Pediatric Surgery, Saint Louis University School of Medicine; Chief of Pediatric Surgery, Cardinal Glennon Memorial Hospital for Children, Saint Louis, Missouri. ROBERT E. MURRAY, M.D.: Resident in Surgery, Saint Lotris IJniver.rit!/ School of hledicine, NOW serrkg itr the Arm!/ .\fedical C01.p.S..
Fig. 1.-A, Admission radiograph revealing apacification of right lung, shift of mediastinum, and cystic areas in the right lung. B, Bronchogram revealing absent right bronchus. Note increase in air of right lung. C, Esophagram with esophagobronchial communication.
PULMONARY
SEQUESTRATION
Fig. 2.-A, SUpply and esophagus.
Posterolateral incision through sixth interspace. B, fist&. C, Fistula clamped. D. Division of fistola.
Systemic E,
blood
Closrrrc
of
So pulmonary blood supply was seen. A clamp was placed across the fistula just below the cartilage rings and two silk sutures were used for traction and identification of the casophagus (Fig. Y.2). The muscular wall of the fistula was circumferentially incised and allowed to retract. ‘i‘his exposed the mucosa which was divided and sutured with interrupted 5-O silk sutures. The muscle layer was closed with interrupted 5-O silk sutures (Fig. 2D,E). The pleura \xas closed over the repaired esophagus. A polyethylene catheter was placed in the pleural cxvity through a separate stab wound and the thoracotomy wound The tube was used to assist in stabilizing the mediastinum postoperativeIy
closed iu layers. and was removed
in 72 hours. The postoperative course was uneventful. The infant was discharged 3 weeks .rfter surgery when there was evidence of progressive weight gain and a stabilized pulmonary
status.
On pathological examination there were rings of cartilage in the f&da tn~rn aqunmous epithehum to ciliated columnar epithelium. The lung
with a transition was a (lark rpd
57s
LEWIS AND MURRAY
with multiple abscesses. There was microscopic bronchiectasis, and multiple abscesses.
evidence
of atelectasis,
bronchopneumonia,
DISCUSSION
Since Huber in 1777596 first described an anomalous artery arising from the aorta and supplying the lung, numerous terms have been applied to this clinical entity, including accessory lung, pulmonary sequestration, and anomalous lung. r** We have preferred the term sequestration because it seems to agree with the embryologic aberrations. The lung bud first appears in the third week of embryonic life9 as an outpouching from the primitive foregut and initially shares the vascular connections of its parent structure. Thus it is supplied by branches of the splanchnic plexus, which derive from the dorsal aortae, and its veins drain into the into the cardinal venous plexus. As the lung grows, connections are gradually established with branches of the sixth embryonic arches. These are destined to become the pulmonary arteries. Connections are also gradually established with the veins leading directly to the heart. Interference with the development of the lung bud or the transition from one circulation to the other results in various malformations of the lung. It is obvious that this malformation is a primitive form of congenital anomaly. There was interference with the normal development of the right bronchus, tthe systemic circulation prevailed, and the primitive communication with the esophagus persisted. One significant clinical observation proved important in suggesting the diagnosis in this patient: the observation that the opacilied lung appeared to aerate when the infant strained or cried suggested communication with the esophagus or stomach. SUMMARY
A 4-month-old white female with total right pulmonary sequestration communicating with the esophagus was successfully treated surgically. The esophogeal communication was suggested by aeration of the opacified lung when the infant cried. SUMMARIO IN INTERLINGUA Un puera de racia blanc de quatro menses de etate con total sequestration dextero-pulmonar communicante con le esophago esseva tractate successosemente per resection de1 sequestrate pulmon, sequite de clausura de1 fistula broncho-esophagee. Suspicion de1 fistula resultava de1 observation que le opacificate pulmon se aerava quando le patiente critava. REFERENCES 1. Harris, H. A., and Lewis, I.: Anomalies of the lungs with special reference to the danger of abnormal vessels in lobectomy. J. Thorac. Surg. 9:666-671, 1940. 2. Bruwer, A., Clagett, 0. T., and McDonald, J. R.: Anomalous arteries to the lung associated with congenital pulmonary abnormality. J. Thorac. Surg. 19:957-972, 1950.
3. Gans, S. L., and Potts, W. J.: Anomalous lobe of lung arising from the esophagus. J. Thorac. Surg. 21:313, 1951. 4. Case Records of The Massachusetts General Hospital, New Eng. J. Med. 236: 6721947. 5. Huber, J. J.: Quoted by McCotter. 6. McCotter, R. E.: On the occurrence of pulmonary arteries arising from the thoracic
PULMONARY
SEQUESTRATION
aorta. Anat. Rec. 4:291-297, 1910. 7. DeBakey, M. Arey, J. B., and Brunazzi, R.: Successful removal of lower accessory lung. J. Thorac. Surg. 19:304, 1950. 8. Pryce, D. M.: Lower accessory pulmonary artery with intralobar sequestration of lung: A report of seven cases. J. Path.
579 and Bact. 58457, 1946. 9. Lindskog, G. E., and Liebow, A. A.: Embryology and developmental abnormalities of the lung. In: Thoracic Surgery and Related Pathology. New York, Appleton, 1953.
Fistula
By J. EUGENELEWIS ANDROBERT E. MURRAY
M
IJLTIPLE
CASES
of intralobar and extralobar pulmonary sequestration have been reported. 1,2 However, total pulmonary sequestration with esophageal communication is a rare anomaly.3,4 Recently a 4-month-old infant with this anomaly was successfully managed. The diagnosis and surgical treatment of this patient warrants a detailed report. CASE REPORT A 4-month-old white female weighing 10 pounds was transferred with a diagnosis of unresolved pneumonia involving the right lung. Allegedly
the infant
to
our
institution
developed
a cough and rhinorrhea at 2 months of age. No improveand antibiotics. The infant failed to gain weight and developed a persistent fever. Hospitalization and evaluation revealed opacihcation of the right lung with a shift of the mediastinum. After antibiotics, expectorants, and moist air failed to improve the pulmonary symptoms, the infant was transferred. On admission the patient was pale with a rectal temperature of 100” F and labored respirations. The right lung was dull to percussion. Breath sounds were absent over the right lung, there was decreased motion to the right chest, and the trachea was shifted to the right. The left lung was clear to percussion and auscultation. There were no other significant physical findings. Hemoglobin was 7.2 Gm. and the white blood count, 10,100. A chest radiograph revealed atelectasis of the right lung with a shift of the mediastinum to the right and hyperaeration of the left lung. There were areas in the right lung suggestive of cystic changes (Fig. IA). A diagnosis of staphylococcus pneumonia was suspected. Bronchoscopy was done and no right main stem bronchus could be visualized. The absence of a right main stem bronchus was confirmed by a bronchogram. (Fig. 1B). In addition it was noted that there appeared to be improved aeration of the right lung. These observations led to a diagnosis of a bronchoesophageal communication. This was confirmed by an esophagram which demonstrated a direct communication between the lower esophagus and the right lung (Fig. 1C).
ment was noted from expectorants
After preparation with whole blood and antibiotics, operation was performed through a right posterolateral thoracotomy. The pleural cavity entered through the sixth intercostal space (Fig 2A). Exploration revealed a collapsed lung of rubbery consistency covered with :r fibrinous membrane. The lung failed to expand with inspiration. On dissection and mobilization there was a systemic blood supply and a tubular structure connecting the lung to the distal one third of the esophagus. There were cartilage rings in the tubular structure adjacent to the lung (Fig 2B). The systemic blood supply was ligated and divide& From the Surgical Service of Cardinal Glennon Memorial Hospital for Children, Saint Louis, Missouri and the Department of Surgery Saint Louis Uniuersity School of Medicine, Saint Louis, Missouri. J. EUGENE LEWIS, JR., M.D.: Clinical Professor of Pediatric Surgery, Saint Louis University School of Medicine; Chief of Pediatric Surgery, Cardinal Glennon Memorial Hospital for Children, Saint Louis, Missouri. ROBERT E. MURRAY, M.D.: Resident in Surgery, Saint Lotris IJniver.rit!/ School of hledicine, NOW serrkg itr the Arm!/ .\fedical C01.p.S..
Fig. 1.-A, Admission radiograph revealing apacification of right lung, shift of mediastinum, and cystic areas in the right lung. B, Bronchogram revealing absent right bronchus. Note increase in air of right lung. C, Esophagram with esophagobronchial communication.
PULMONARY
SEQUESTRATION
Fig. 2.-A, SUpply and esophagus.
Posterolateral incision through sixth interspace. B, fist&. C, Fistula clamped. D. Division of fistola.
Systemic E,
blood
Closrrrc
of
So pulmonary blood supply was seen. A clamp was placed across the fistula just below the cartilage rings and two silk sutures were used for traction and identification of the casophagus (Fig. Y.2). The muscular wall of the fistula was circumferentially incised and allowed to retract. ‘i‘his exposed the mucosa which was divided and sutured with interrupted 5-O silk sutures. The muscle layer was closed with interrupted 5-O silk sutures (Fig. 2D,E). The pleura \xas closed over the repaired esophagus. A polyethylene catheter was placed in the pleural cxvity through a separate stab wound and the thoracotomy wound The tube was used to assist in stabilizing the mediastinum postoperativeIy
closed iu layers. and was removed
in 72 hours. The postoperative course was uneventful. The infant was discharged 3 weeks .rfter surgery when there was evidence of progressive weight gain and a stabilized pulmonary
status.
On pathological examination there were rings of cartilage in the f&da tn~rn aqunmous epithehum to ciliated columnar epithelium. The lung
with a transition was a (lark rpd
57s
LEWIS AND MURRAY
with multiple abscesses. There was microscopic bronchiectasis, and multiple abscesses.
evidence
of atelectasis,
bronchopneumonia,
DISCUSSION
Since Huber in 1777596 first described an anomalous artery arising from the aorta and supplying the lung, numerous terms have been applied to this clinical entity, including accessory lung, pulmonary sequestration, and anomalous lung. r** We have preferred the term sequestration because it seems to agree with the embryologic aberrations. The lung bud first appears in the third week of embryonic life9 as an outpouching from the primitive foregut and initially shares the vascular connections of its parent structure. Thus it is supplied by branches of the splanchnic plexus, which derive from the dorsal aortae, and its veins drain into the into the cardinal venous plexus. As the lung grows, connections are gradually established with branches of the sixth embryonic arches. These are destined to become the pulmonary arteries. Connections are also gradually established with the veins leading directly to the heart. Interference with the development of the lung bud or the transition from one circulation to the other results in various malformations of the lung. It is obvious that this malformation is a primitive form of congenital anomaly. There was interference with the normal development of the right bronchus, tthe systemic circulation prevailed, and the primitive communication with the esophagus persisted. One significant clinical observation proved important in suggesting the diagnosis in this patient: the observation that the opacilied lung appeared to aerate when the infant strained or cried suggested communication with the esophagus or stomach. SUMMARY
A 4-month-old white female with total right pulmonary sequestration communicating with the esophagus was successfully treated surgically. The esophogeal communication was suggested by aeration of the opacified lung when the infant cried. SUMMARIO IN INTERLINGUA Un puera de racia blanc de quatro menses de etate con total sequestration dextero-pulmonar communicante con le esophago esseva tractate successosemente per resection de1 sequestrate pulmon, sequite de clausura de1 fistula broncho-esophagee. Suspicion de1 fistula resultava de1 observation que le opacificate pulmon se aerava quando le patiente critava. REFERENCES 1. Harris, H. A., and Lewis, I.: Anomalies of the lungs with special reference to the danger of abnormal vessels in lobectomy. J. Thorac. Surg. 9:666-671, 1940. 2. Bruwer, A., Clagett, 0. T., and McDonald, J. R.: Anomalous arteries to the lung associated with congenital pulmonary abnormality. J. Thorac. Surg. 19:957-972, 1950.
3. Gans, S. L., and Potts, W. J.: Anomalous lobe of lung arising from the esophagus. J. Thorac. Surg. 21:313, 1951. 4. Case Records of The Massachusetts General Hospital, New Eng. J. Med. 236: 6721947. 5. Huber, J. J.: Quoted by McCotter. 6. McCotter, R. E.: On the occurrence of pulmonary arteries arising from the thoracic
PULMONARY
SEQUESTRATION
aorta. Anat. Rec. 4:291-297, 1910. 7. DeBakey, M. Arey, J. B., and Brunazzi, R.: Successful removal of lower accessory lung. J. Thorac. Surg. 19:304, 1950. 8. Pryce, D. M.: Lower accessory pulmonary artery with intralobar sequestration of lung: A report of seven cases. J. Path.
579 and Bact. 58457, 1946. 9. Lindskog, G. E., and Liebow, A. A.: Embryology and developmental abnormalities of the lung. In: Thoracic Surgery and Related Pathology. New York, Appleton, 1953.