Congenital Pulmonary Cysts in Infants and Children

Transcription

1 Congenital Pulmonary Cysts in Infants and Children * John C. Jones, M.D., Carl H. Almond, M.D., H. Martin Snyder, M.D., and Bert W. Meyer, M.D. T e earliest recorded congenital cystic disease of the lung was reported in 1687 by Bartholinus [3]. The first report of congenital pulmonary cysts in the American literature was by Koontz [141 in 1925, who reported the course of a 12-day-old infant with cysts of the lung first presenting respiratory distress and followed shortly by death. One hundred and eight cases from the European literature were reviewed and the pathological findings were studied and discussed in his paper. Nelson [20] described the clinical picture and reported additional examples in The successful management of a 71/2-week-old infant with respiratory distress and cysts in the right lung was reported in 1928 by Swanson, Platou, and Sadler [24]. The infant was treated by chest aspiration followed by tube thoracostomy with injection of Dakin s solution and formaldehyde through the tube. There was gradual reexpansion of the functional lung and recovery of the patient. Poor response to management by aspiration was observed and recorded in 1933 by Anspach and Wolman [Z]. Thoracentesis, which had been utilized in 2 patients who died, was thought to have aggravated each patient s condition. Since that report, the inadvisability of aspiration and of tube drainage has been mentioned repeatedly [7, 11, 211. It is thought that Sauerbruch [22] was the first to resect the lesion in the management of this disease in Four cases successfully managed by resection, including the first case in the United States (in 1941), were reported in 1943 by Tyson [25]. Fischer, Tropea, and Bailey [9] reported a successful lobectomy for congenital pulmonary cyst that same year. Gross [12] performed a pneumonectomy in a 3-week-old infant with congenital cysts in By this time, resectional therapy was well established. Moersch and Clagett [191 reported 44 resections From the Department of Surgery, University of Southern California School of Medicine, and the Childrens Hospital of Los Angeles, Los Angeles, Calif. Presented at the Third Annual Meeting of The Society of Thoracic Surgeons, Kansas City, Mo., Jan , Present address: Department of Thoracic Surgery, University of Missouri School of Medicine, Columbia, Mo. Requests for reprints should be sent to Dr. Jones, 1136 W. 6th St., Los Angeles, Calif. VOL. 3, NO. 4, APRIL,

2 JONES, ALMOND, SNYDER, AND MEYER for pulmonary cysts in A portion of these were congenital cysts. The age spread in this group was 7 to 58 years. Jones [ls] reported 9 infants and children treated surgically by resection. The feasibility of resectional management in all instances of this disease, including the very young [1, 4, 7, 8, 11, 17, 23, 261 and even newborn patients [18], became more evident with successive reports [l, 6, 7, 10, 15, 161. CLINICAL EXPERIENCE Of 52 cases of congenital ulmonary cysts in infants and children seen at the Childrens Hospital of Los flngeles, 46 were treated surgically with resection of the involved lung, and 6 patients were treated conservatively by aspiration or by closed thoracostomy (Table 1). There were no deaths in the surgically treated patients and 4 deaths (66%) in the medically treated patients. The ages of the patients varied from 2 days to 14 years; the average age was 4 years. In the resected group there were 24 males and 22 females. There were 5 newborn infants who were admitted in acute respiratory distress which necessitated resection of the involved lung to correct the respiratory problem. There were 6 cases of tension pneumothorax, 3 of which were spontaneous and 3 of which were caused by needle aspiration. These were also surgical emergencies. Twelve of the patients had infected cysts; of these, 3 had anatomically demonstrable bronchial communications and four had been infected by needle aspiration or chest tube drainage. The infected cases presented with chills, fever, and respiratory distress. The remainder of the patients presented symptoms of respiratory distress of longer duration. DIAGNOSIS The diagnosis of congenital pulmonary cyst should always be considered in any infant or child with respiratory distress. However, some of the children with cysts may not present symptoms for long periods of time, especially those with noncommunicating bronchogenic cysts. Due to late complications of mediastinal displacement associated with respiratory distress, bronchial obstruction associated with pulmonary infection, or rupture of cyst with consequent pneumothorax, we believe that all pulmonary cysts should be resected. The diagnosis is established from clinical signs and symptoms and verified by chest x-rays. Bronchoscopy and bronchography are not necessary and may be dangerous during the period of respiratory distress. A review of the symptomatology of these patients produced the following general classification (Table l), defined in detail below. TABLE 1. CLINICAL DISTRIBUTION OF RESECTED CYSTS No. of Pts. Newborn infants in acute respiratory distress 5 Tension pneumothorax 6 Spontaneous Induced 3 3 Infected cysts 12 Patent bronchus 3 Needle aspiration 4 Pneumonia (?) 5 Uncomplicated pulmonary cysts THE ANNALS OF THORACIC SURGERY

3 Congenital Pulmonary Cysts FIG. 1. Chest x-ray of a newborn infant with acute respiratory distress, showing a large cyst of the left upper lobe, compression of the left lower lobe, shift of the mediastinum, and partial compression of the right lung. 1. The Newborn Infant in Acute Respiratory Distress. The pulmonary cysts are of one or more lobes and are space-occupying, producing atelectasis of the adjacent lobe and shift of the mediastinum to the opposite side. These infants present with rapid respiration, intercostal and subcostal retraction, and flaring of the nares; often cyanosis and death result if the cysts are not resected. Occasionally the first symptom will be persistent vomiting; this is especially true in newborns. There will be hyperresonance and decreased breath sounds over the cystic area. The chest x-ray will substantiate the clinical impression and verify the diagnosis, showing the cystic area without lung markings, atelectasis of adjacent lobes, and a shift of the mediastinurn. Correct diagnosis and resection of the involved lobe or segment will cure the patient (Fig. 1). 2. Tension Pneumothorax. This second clinical category is also a respiratory emergency. It occurs with sudden onset of acute respiratory distress when the cyst has either spontaneously ruptured or has been perforated by needle aspiration, producing collapse of the lung, shift of the mediastinum, and increasing intrathoracic tension with progressive dyspnea. There is hyperresonance with decreased breath sounds and shift of the mediastinum revealed by physical examination. The chest x-ray shows shift of the mediastinum to the contralateral side and compression of the functional lung on the affected side. This is an acute FIG. 2. Chest x-ray of a child with tension pneumothorax and acute respiratory distress following needle perforation of a cyst of the left lower lobe. There is shift of the mediastinum to the right, medinstinal herniation, and partial compression of the right lung. VOL. 3, NO. 4, APRIL,

4 JONES, ALMOND, SNYDER, AND MEYER FIG. 3. Preoperative chest x-ray of a patient with an infected cyst of the right upper lobe showing the infiltl-ation and consolidation in the right upper lobe. surgical emergency and may necessitate aspiration of air and insertion of an intrathoracic tube with underwater seal suction followed by immediate thoracotomy, cystectomy, lobectomy, or pneumonectomy (Fig. 2). 3. Znfected Cysts. Patients with infected cysts may have symptoms of respiratory distress but almost always present symptoms of pulmonary infection with chills, fever, and chest pain; often there is also productive cough. There is dullness and decreased breath sounds over the area. Chest x-rays (Fig. 3) show areas of consolidation with or without fluid levels. Some patients with infected cysts have bronchial communication. Of the 12 cases of infected cysts we have had, 3 had communicating bronchi and 4 had been infected by needle aspiration or insertion of an intrathoracic tube. 4. Uncomplicated Pulmonary Cysts. The other cases occurring at any age during infancy or childhood present with persistent, intermittent respiratory symptoms of various degrees. The symptoms either are those of intrathoracic space-occupying lesions, i.e., cough, dyspnea, and tachypnea; or are those denoting bronchial obstruction and pulmonary infection, i.e., cough, sputum, chills. fever, and chest pain. A diagnostic chest x-ray will show the pulmonary cyst and the occasional associated pneumonia. Once recognized, surgical resection of the involved portion of lung is necessary for cure (Fig. 4). Among the patients we operated upon, 4 were asymptomatic. The most common symptoms were dyspnea (15) and cough (14), followed by cyanosis (12), fever (ll), tachypnea (9), pain (4), vomiting (3), and hemoptysis (2). The most frequently observed signs were decreased breath sounds (26), hyperresonance (12), mediastinal shift (lo), substernal retraction (8), rales (7), and dullness. The most common x-ray findings recognized and reported were cyst, mediastinal shift, consolidation, and pneumothorax. The average time from admission to operation in this group of patients was 4.6 days. However, the acute respiratory distress of 2 of the newborns necessitated operation on the day of admission. The right lower lobe was the most commonly involved lobe (18). This is compatible with reports in the literature. The anatomical distribution of the rest of the cysts was as follows: left lower lobe, 9; left upper lobe, 7; right upper lobe, 5; right middle lobe, 2. The cyst was located in the lingula in 2 cases and was peritracheal, pericardial, or mediastinal in 1 case each. 300 THE ANNALS OF THORACIC SURGERY

5 Congenital Pulmonary Cysts A B FIG. 4. (A) Chest x-rays of a child with a cyst in the anterior segment of the right upper lobe showing a fluid level. (B) Chest x-rays of a child with a pulmonay cyst adjacent to the pericardium which is visible only in the oblique view. TREATMENT The operations were patterned to correct the pathological process, and lobectomy (25), cystectomy (14), lobectomy and segmentectomy (3), segmentectomy (3), and pneumonectomy (1) were utilized. Misdiagnosis led to abdominal exploration for diaphragmatic hernia in a 1-month-old male. The infant was admitted in respiratory distress which had been present since birth, with tachypnea, intercostal and subcostal retraction, and vomiting and cyanosis with crying. There was mediastinal shift to the left and overexpansion of the right thorax which was thought to be compatible with a diaphragmatic hernia (Fig. 5). Laparotomy demonstrated no hernia; unfortunately, the cyst was perforated by transdiaphragmatic needle aspiration which caused a right pneumothorax. Thoracotomy then revealed a huge cyst of the right upper lobe with atelectasis of the right middle and lower lobes. The patient was relieved of his acute respiratory distress immediately upon opening the chest. Right upper lobectomy allowed expansion of VOL. 3, NO. 4, APRIL,

6 JONES, ALMOND, SNYDER, AND MEYER FIG. 5. (Left) Chest x-ray of an infant with a cyst of the right upper lobe. (Right) Postlaparotomy x-ray with transdiaphragmatic needle perforation of the cyst causing pneumothorax. the right middle and lower lobes with relief of symptoms. The infant did well postoperatively and was discharged in fourteen days. RESULTS In this group of 46 patients treated surgically, there were no deaths. Five postoperative complications were bronchopleural fistula, empyema, pneumothorax, pneumonia, and the later development of a pulmonary cyst. The bronchopleural fistula occurred following the resection of the right middle and lower lobes for infected cysts. The pneumothorax occurred four years postoperatively on the left side in a patient who had undergone cystectomy on the right with the cyst attached to the right lower lobe. The patient had had a pneumothorax at the time of the first operation. The left pneumothorax responded promptly to intrathoracic tube drainage. One child who had had a cyst removed from the left upper lobe three years previously returned with cystic areas in the left upper lung field. Of the 46 patients undergoing surgical resection, 39 of the cystic specimens contained respiratory epithelium; the remainder had only fibrous linings (5) or the lining was pathologically undeterminable (2). Ten of the 12 infected cysts were lined,by respiratory epithelium. Fourteen of the specimens contained other respiratory elements as well. The description of the respiratory epithelium varied among columnar, transitional, and stratified types; however, the most common type was columnar. DISCUSSION This study reports the congenital pulmonary parenchymal cysts and all intrathoracic cysts containing respiratory elements resected surgically in infants and children at the Childrens Hospital of Los Angeles from 1942 to An additional 6 cases of congenital pulmonary cysts were managed conservatively and diagnosed clinically or at autopsy. There is no effort to classify bronchogenic cysts separately even though they are not within the lung parenchyma, because we believe that this diagnosis also necessitates surgical intervention. We 302 THE ANNALS OF THORACIC SURGERY

7 Congenital Pulmonary Cysts have not included acquired cysts (pneumatoceles of staphylococcal pneumonia, bronchiectatic saccular cysts, and ECHO virus cysts [5]) which are not uncommon and must be differentiated from congenital pulmonary cysts and from certain congenital lesions such as diaphragmatic hernia and pneumothorax. Other congenital lesions, such as lobar emphysema in infancy, empyema, sequestrated lobes, and mediastinal tumors and cysts, must be differentiated even though the treatment is similar with surgical resection of the involved lung. The etiology of this group of cases is not a great problem since they are congenital. However, there is considerable discussion in the literature concerning etiology of pulmonary cysts which primarily centers around acquired lesions. We believe that infection in the congenital cysts is acquired and not a cause of the cyst. A patent bronchial opening to the cyst could well explain the infection. In our group of 12 cases of infected cysts, only 3 had patent bronchial openings to the cysts, whereas 4 of the 12 cysts were infected by needle aspiration. Two of the noninfected cysts were demonstrated pathologically to have patent bronchi to the cysts. The cause of the infection in the other 5 infected cases was not determined, but they could well have had bronchial patency that was not demonstrated or could have been infected secondarily to adjacent pneumonia or even by the bloodstream. Sequestration of the lung is sometimes confused with congenital lung cysts, especially if adjacent atelectasis or infection is present. It is interesting that there were 2 cases with anomalous vessels to the cysts, one from the mediastinum and one from the diaphragm. The symptoms and signs of respiratory distress or pulmonary infection should cause one to suspect a pulmonary cyst in infants or children. The chest x-ray is most helpful in the diagnosis and should clarify the clinical impression. Once the diagnosis is established, we believe that the diseased portion of the lung containing the cyst should be resected. This may necessitate cystectomy, segmentectomy, lobectomy, or occasionally pneumonectomy. There have been questions raised concerning surgical management of these lesions. We would like to stress that these are congenital cysts occupying space needed for normal respiratory tissue; they may cause acute respiratory distress or pulmonary infection at any time, either by infection of the cyst itself or by obstruction and secondary pneumonitis. These cysts will not spontaneously disappear. Aspiration is hazardous because of possible tension pneumothorax resulting from rupture of the cyst and because infection of the cyst may complicate the clinical course and make resection more difficult. In cases of tension pneumothorax, it may be lifesaving to institute intercostal drainage until resection can be done. However, intercostal tube drainage is not acceptable long-range therapy. VOL. 3, NO. 4, APRIL,

8 JONES, ALMOND, SNYDER, AND MEYER SUMMARY AND CONCLUSIONS Fifty-two cases of congenital pulmonary cysts of the lung in infants and children are reported. Forty-six of these were treated by resection of the involved lung without mortality. Six were treated conservatively with the death of 4 patients. The ages of the patients varied from 2 days to 14 years. In any infant or child with symptoms of respiratory distress or pneumonia, the diagnosis of pulmonary cyst should be considered. The chest x-ray will verify the clinical impression of pulmonary cystic disease. The treatment is resection of the diseased lung containing the cyst because of the threat of late complications of space-occupying lesions: pulmonary displacement, bronchial obstruction with secondary infection, or possible rupture and pneumothorax. The cysts will not spontaneously disappear, and infected cysts will not respond to intrathoracic tube drainage. REFERENCES Albert, H. M., and Potts, W. J. Congenital lung cysts in infants. Pediatrics 12:283, Anspach, W. E., and Wolman, I. J. Large pulmonary air cysts of infancy with special reference to pathogenesis and diagnosis. Surg. Gynec. Obstet. 56:634, Bartholinus, T. In Malpighi, M., Opera Omnia. London: Littlebury, P Burnett, W. E., and Caswell, H. T. Lobectomy for ulmonary cysts in a fifteen-day-old infant with recovery. Surgery 23:84, 194l Butterfield, J., Moscovici, C., Berry, C., and Kempe, C. H. Cystic emphysema in premature infants: A report of an outbreak with the isolation of type 19 ECHO virus in one case. New Eng. J. Med. 268:18, Cooke, F. N., and Blades, B. Cystic disease of the lungs. J. Thorac. Surg. 23:546, Crossett, E. S., and Shaw, R. R. Pulmonary resection in the first year of life. Surg. Gynec. Obstet. 97:417, Crowe, G. G. Congenital cystic lung: Successful pneumonectomy in a 3- week-old baby. J. Thorac. Surg. 27:399, Fischer, C. C., Tropea, F., Jr., and Bailey, C. P. Congenital pulmonary cysts: Report of an infant treated by lobectomy with recovery. J. Pediat. 23:219, Gilbert, J. W., Myers, R. T., and Bradshaw, H. H. Pulmonary cysts: Report of twenty-one cases. J.A.M.A. 151:1075, Gilbert, J. W., Jr., and Myers, R. T. Intrathoracic tension phenomena in the neonatal period and infancy. Arch. Surg. (Chicago) 76:402, Gross, R. E. Congenital cystic lung: Successful pneumonectomy in a threeweek-old baby. Ann. Surg. 123:229, Jones, J. C. Indications for pulmonary resection in infants and children. Calif. Med. 72:352, Koontz, A. R. Congenital cysts of the lung. Bull. Hopkins Hosp. 37:340, Lichtenstein, H. Congenital multiple cysts of the lung. Dis. Chest 24:646, THE ANNALS OF THORACIC SURGERY

9 Congenital Pulmonary Cysts Lucido, J. L., Murphy, P., and Sweet, H. C. Resection for localized air trap ing pulmonary disease: Preoperative and postoperative function studies. J. Ti OTUC. Cardiov. Surg. 45: 112, McEachern, C. G., McCoy, R. R., and Arata, J. E. Lobectomy for congenital cystic disease of the lung. J.A.M.A. 151:992, Minnis, J. F., Jr. Congenital cystic disease of the lung in infancy: Successful lobectomy in a one-day-old infant. J. Thorac. Cardiov. Surg. 43:262, Moersch, H. J., and Clagett, 0. T. Pulmonary cysts. J. Thorac. Surg. 16: 179, Nelson, R. L. Congenital cystic disease of the lung. J. Pediat. 1:233, Ravitch, M. M., and Hardy, J. B. Congenital cystic disease of the lung in infants and in children. Arch. Surg. (Chicago) 59:1, Sauerbruch, F. Die operative Behandlung der kongenitalen Bronchiektasen. Arch. Klin. Chir. 180:312, Swan, H., and Aragon, G. E. Surgical treatment of pulmonary cysts in infancy: Report of three cases. Pediatrics 14:651, Swanson, W. W., Platou, E. S., and Sadler, W. Congenital cyst of the lung. Amer. J. Dis. Child..35:1024, Tyson, M. D. The surgical management of solitary cysts, or cyst-like structures, of pulmonary origin. Ann. Surg. 118:50, Whitesell, F. B., Jr., and White, W. J. Congenital cystic disease of the lung in the newborn: Report of a successful lelt lower lobectomy in a seven-dayold infant. Ann. Surg. 136:299, DISCUSSION DR. ROBERT W. RIEMER (Providence, R.I.): I wish to congratulate the authors on such an extensive and comprehensive review. It is pioneers like Dr. Jones who have made such great contributions to the advancement of thoracic surgery. When one talks about cystic disease in children, I think we should also pay our respects to another great pioneer in thoracic surgery, Dr. Potts, who had a classic article on the differentiation between acquired and congenital bronchogenic cysts in children in the Archives of Surgery (61:684, 1950). The important point in distinguishing between an acquired and a congenital bronchogenic cyst is that the acquired one rarely needs surgery. The only surgery necelsary in an ac uired bronchogenic cyst (pneumatocele), such as those that you see after a Stap R ylococcus pneumonia, is in treating the complications such as pyopneumothorax or tension pneumothorax. I wish to present two cases to emphasize two important features of pulmonary cystic disease in children: first, the importance of differentiating between congenital bronchogenic cysts and acquired cystic disease in children, and second, the difficulty in making the diagnosis. The first case was an 18-month-old child who was treated with antibiotics by her pediatrician one month prior to admission to the hospital. When she presented at the hospital, she was in acute respiratory distress with a high fever and appeared severely ill. X-rays showed a large, air-fluid cavity on the right side. A diagnosis of tension pneumatocele was made. It was felt that this child had Staphylococcus pneumonia. We elected to wait and treat this child with intensive antibiotic therapy consisting of Staphcillin. Because the child did not show improvement in 24 hours, it was decided to catheterize the air-filled cavity. This was done, and when the catheter was placed into the cavity, fluid gushed out under tremendous pressure. The child was immediately relieved of its respiratory distress. The problem then arose as to whether this represented a pneumatocele in the Staphylococcus pneumonia or whether it represented an infected congenital cyst. X-rays after catheterization showed clear lung fields in the periphery, making US think that the cavity was not a complication of Staphylococcus pneu- VOL. 3, NO. 4, APRIL,

10 JONES, ALMOND, SNYDER, AND MEYER monia. This was an important point of differentiation, in that most tension pneumatoceles are associated with extensive pneumonia processes in the surrounding lung. On shutting off the catheter, this fairly thick-walled cystic area filled up with fluid, giving us the impression that there was internal secretion in this cyst. Dr. Potts has discussed the possibility of placing a thoracoscope in these cysts in order to view the inner wall in helping to differentiate them from acquired cysts. Our diagnosis was of an infected congenital cyst. The child was operated upon, and a large, thick-walled cyst occupying almost the entire right upper lobe was found. A right upper lobectomy was performed. Pathologically the cyst was shown to contain columnar epithelium, and several bronchial glands were visualized microscopically. The second patient illustrates the difficulty in making the diagnosis. This was a 3-year-old child who was admitted to the pediatric service with respiratory distress and abdominal pain. There was a question of some surgical condition in the abdomen. A chest x-ray was taken, and a large, air-filled cavity was present in the right chest. It was felt that this child had Staphylococcus pneumonia with a pyopneumothorax. Catheterization of the chest was carried out. This was difficult, because in attempting to get the catheter into the chest, there seemed to be some obstruction after passing through the chest wall. X-rays showed the catheter to lie in an upward direction. The child was immediately improved but in 24 hours deteriorated again. It was thought that perhaps the catheter was not placed properly, and another attempt was made to catheterize the air-filled cavity in the right chest. This was quite difficult, and because of the child s continuing deterioration, it was decided that the right chest should be explored surgically. Before exploration, we elected to study the upper gastrointestinal tract. A Levin tube was passed, and an x-ray was taken with the injection of Lipiodal. Chest x-rays showed the Levin tube curled up inside of a cystic-like structure (stomach) with Lipiodal in the stomach in the right pleural cavity. At surgery this child was found to have a stomach strangulated through a Bochdalek foramen in the right diaphragm and a perforation in the stomach with associated mediastinitis, pleuritis, and peritonitis. Much difficulty was encountered in getting the stomach through the Bochdalek foramen, but this was accomplished and the child went on to make an uneventful postoperative recovery. There was a gastric stasis for a period of approximately three months which did not interfere with alimentation. I feel it is quite important to emphasize in children with respiratory distress that the gastrointestinal tract should be ruled out as a factor. This can be done simply by adding a barium swallow to the chest x-ray studies. This is particularly important in cases of vascular ring and esophageal stenosis and atresia. DR. JONES: I just want to rise to thank Dr. Riemer for pointing out the pitfalls in some of these patients. I also want to say that we did not treat the 6 patients that were treated conservatively, and finally, I just want to emphasize again what Dr. Riemer said about being sure that you are not dealing with a stomach instead of a lung cyst. Furthermore, have a correct diagnosis before you operate on these patients; also, have a correct diagnosis before starting to put tubes and needles into these cavities. 306 THE ANNALS OF THORACIC SURGERY