Life-Threatening Respiratory Distress from Mediastinal Masses in Infants J. Alex Haller, Jr., M.D., Dennis W. Shermeta, M.D., James S. Donahoo, M.D., and John J. White, M.D. ABSTRACT From 1972 through 1974,5 infants with severe respiratory distress secondary to mediastinal tumors were treated in the Division of Pediatric Surgery of The Johns Hopkins Hospital. The mediastinal masses included 2 intramural bronchial cysts, a giant esophageal duplication, a benign teratoma, and a highly malignant neuroblastoma. Individualized management was carried out successfully in all: total resection of the bronchial cysts and teratoma; partial resection of the duplication cyst with stripping of the remaining mucosa from the contiguous esophageal wall, thereby preserving esophageal integrity; and excision of involved chest wall and tumor combined with radiotherapy and chemotherapy for the neuroblastoma. This experience emphasizes the potential for lethal respiratory distress from mediastinal tumors in infants and supports the experience reported by others of serious consequences if resection is not performed. Primary physicians and thoracic surgeons must be aware of the lethal potential of such mediastinal tumors among the many other surgical causes of respiratory distress in neonates and infants. R espiratory difficulty in newborn infants is due most frequently to the respiratory distress syndrome (RDS) and in particular to hyaline membrane disease. A number of other conditions in neonates that cause severe respiratory distress and require immediate operative therapy are congenital and include the familiar esophageal atresia with distal tracheoesophageal fistula, vascular ring, diaphragmatic hernia, and heart abnormalities. Lifethreatening respiratory distress may also be caused by airway obstruction due to mediastinal masses, an entity rarely reported in the past. From 1972 through 1974, 5 infants (1 day to 3 months of age) with severe respiratory distress secondary to tumors in the superior mediastinum were managed successfully in the Division of Pediatric Surgery of The Johns Hopkins Hospital. The masses were two bronchogenic cysts, an esophageal duplication cyst, a malignant neuroblastoma, and a benign teratoma. The emergency nature of the effects of these masses has not been adequately emphasized in most reports of large series. Rapid diagnosis, which is essential for survival, depends primarily on recognition of those causes of respiratory distress From the Division of Pediatric Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md. Presented at the Twenty-first Annual Meeting of the Southern Thoracic Surgical Association, Williamsburg, Va., Nov. 7-9, 1974. The authors wish to express their appreciation to The Division of Pediatric Radiology at The Johns Hopkins Hospital for their outstanding diagnostic skills and especially to Dr. Richard Heller for his advice and guidance in the evaluation of these infants. Address reprint requests to Dr. Haller, Division of Pediatric Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md. 21205. 364 THE ANNALS OF THORACIC SURGERY
Respirato?y Distress from Mediastinal Masses in Infants which can be relieved by prompt operative treatment. A high mortality rate has been reported when such lesions are not recognized [4]. The purpose of this paper is to call attention to this important syndrome and to discuss essential elements of operative management. Clinical Material PATIENT 1 A black girl weighing 3.6 kg was born to an 18-year-old mother following an uncomplicated pregnancy, labor, and delivery. Although the initial Apgar score was 10-10, at 6 hours of age the infant was noted to have intercostal retractions, nasal flaring, and diminished breath sounds over the right hemithorax. Chest roentgenograms (Fig. la, B) demonstrated a large mass in the right posterosuperior mediastinum which distorted and narrowed the trachea. Barium swallow showed displacement of the esophagus to the right (Fig. lc, D). At right posterolateral thoracotomy an esophageal duplication cyst was found to have caused the esophagus to be splayed anteriorly to the right with direct compression of the posterior wall of the trachea. Seventy ml of thick, mucoid substance was aspirated from the cyst. After excision of most of the cyst the epithelial lining was removed from the remaining cyst wall, which was contiguous with the esophagus. The cyst was lined with nonstratified squamous epithelium typical of an esophageal duplication. Recovery was uneventful, and she was discharged 15 days after operation. She has remained well up to this time. PATIENT 2 A white boy weighing 4.1 kg was delivered by cesarean section to a 2 1-year-old mother; the indication for section was prolonged labor due to cephalopelvic disproportion. The infant was sluggish and cyanotic at birth and on the fourth and fifth days of life experienced mild respiratory distress that was treated by oxygen administration. A chest roentgenogram (Fig. 2) 6 days after birth demonstrated a mass in the right superior mediastinum, and he was transferred to the Children s Medical and Surgical Center. Fluoroscopic examination of the chest demonstrated a solid mass in the right posterosuperior mediastinum which contained a radiopaque area with the appearance of a canine tooth (Fig. 2A). The mass caused marked deviation of the esophagus and trachea to the left. A well-encapsulated benign teratoma was excised through a right posterolatera1 thoracotomy. His postoperative course was complicated by Horner s syndrome on the right side. He was discharged 11 days after operation and has remained well. PATIENT 3 An Iranian girl who weighed 2,850 gm at birth was admitted to the Children s Medical and Surgical Center at 3 months of age. The mother and father were second cousins; two previous pregnancies had been unsuccessful, the first fetus aborting at 3 months and the second baby dying 24 hours after birth from respiratory insufficiency. When she was 6 weeks old the patient developed tachypnea and cyanosis. Chest roentgenograms demonstrated hyperinflation of VOL. 19, NO. 4, APRIL, 1975 365
HALLER ET AL. the left lung with atelectasis of the right upper and middle lobes (Fig. 3A). She was treated with antibiotics and steroids for 20 days, during which time her respiratory status worsened. Congenital lobar emphysema was considered, and operation was recommended. The patient was then flown to The Johns Hopkins Hospital. Physical examination showed this small infant to have absent breath sounds over the left hemithorax and a respiratory rate of 60 to 80 breaths per minute. Fluoroscopic examination demonstrated paradoxical movement of the left diaphragm, and a barium swallow showed a small, oval, radiolucent area causing extrinsic compression of the anterior surface of the esophagus at the level of the carina as well as severe compression of the left main bronchus (Fig. 3B). An intramural bronchogenic cyst was excised from the left main bronchus through a left posterolateral thoracotomy. Her postoperative course was uneventful, with gradual resolution of the overexpansion and atelectasis. FIG. 1. Frontal (A) and lateral (B) chest roentgenograms show a mms displacing the trochea anteriorly and narrowing the tracheal air shadow. The mays is superimposed on the right heart border. Barium injected into the esophagus through a tube shows (C) displacement of tliu ~soplingiis to the child's right by the mass and (0) anterior displacement of the esoplmgm 366 THE ANNALS OF THORACIC SURGERY
Respiratory Distress from Mediastinal Masses in Infants FIG. 2. (A) Frontal roentgenogram shows large right superior intrathoracic mass with radiopaque area characteristic of a canine tooth (arrow). (B) In the lateral roentgenogram the mass is localized an the right superior mediastinum with the tracheal air column deviating anteriorly. PATIENT 4 A black girl weighing 3.0 kg, product of a normal pregnancy, labor, and delivery, was admitted to the Children s Medical and Surgical Center at 9 weeks of age. Two days prior to admission she had an acute onset of tachypnea, wheezing, and posttussic vomiting. A chest roentgenogram showed hyperexpansion of both lower lobes, and a diagnosis of bronchiolitis was considered. After 24 hours of bronchodilator therapy she was returned to the outpatient clinic with increasing respiratory distress. On admission her respiratory rate was 40 to 50 breaths per minute. Chest examination revealed bilateral, high-pitched inspiratory wheezing with diminished expiratory sounds. A chest roentgenogram showed extrinsic compression and narrowing of both the right and left main bronchi (Fig. 4A). A barium swallow demonstrated a mass compressing the anterior surface of the esophagus and posterior surface of the carina and right main bronchus (Fig. 4B). A 4 cm bronchogenic cyst arising from the posterior surface of the carina was excised through a right thoracotomy. Her postoperative course was uneventful, and she was discharged after 7 days with a normal chest roentgenogram. She has remained asymptomatic. PATIENT 5 A 4-week-old white girl weighing 2.8 kg, the product of an uncomplicated 41-week gestation with normal labor and delivery, suddenly vomited and became cyanotic. She was admitted to the Children s Medical and Surgical Center with increasing respiratory distress, a respiratory rate of 60 to 70 breaths per minute, and moderate intercostal retraction. Breath sounds were absent on the upper VOL. 19, NO. 4, APRIL, 1975 367
HALLER ET AL. right side, and the mediastinum was shifted to the left. A chest tube was inserted into the right side of the chest in the emergency room, and 50 mlof serosanguineous fluid was promptly drained; she then had a cardiopulmonary arrest from which she was resuscitated. A chest roentgenogram (Fig. 5) demonstrated a large mass in the right posterosuperior mediastinum with spreading of the upper ribs and erosion of the first rib; the trachea was deviated significantly to the left. A vanillylmandelic acid spot test was positive. Because of her overwhelming respiratory distress, an ideal treatment regimen of preoperative combined chemotherapy and radiotherapy could not be utilized. A right posterolateral thoracotomy was performed, and a large, solid extrapleural mass was found to arise from the sympathetic chain along the first, second, and third intercostal spaces. The mass was excised, along with the first and second ribs, from midaxilla to the vertebral bodies. The pathological diagnosis was highly malignant neuroblastoma. She continued to have poor respiratory function postoperatively, and was ventilated through a tracheostomy for several days. She subsequently improved and was discharged but died at home 3 months later from widely disseminated neuroblastoma. Comment Mediastinal masses rarely cause acute respiratory distress; indeed, this symptom seldom was noted in our previous survey of mediastinal tumors in children at FIG. 3. (A) Frontal roentgenogram shows marked hyperinfation of the lgt lung with displacement of the mediastinum to the right and compression of the right lung. (B) A tube-esophagogram demonstrates the presence of a probable mass (2 cm in diameter) between the trachea and esophagus at the level of the carina and left main bronchus. 368 THE ANNALS OF THORACIC SURGERY
Respiratory Distress from Mediustinal Masses in Infants FIG. 4. (A) A frontal chestfilm shows hyperexpansion of the left lung, widening of the tracheal bijiurcation, elevation and narrowing of the l&t and right main bronchi, and partial loss of volume of the right upper lobe with compensatory hyperexpansion of the right lower lobe. (B) Although a mass inferior to the carina is suggested, the barium studies clearly demonstrate that the mass ZS displacing the anterior wall of the esophagus posteriorly. The Johns Hopkins Hospital [2]. Lesions in a critical position in the diminutive superior mediastinum of a newborn baby or infant, however, may have a devastating effect on respiratory function. Heimburger and Battersby [3] reported mediastinal masses in 40 children of whom 21 had respiratory symptoms, including tachypnea, dyspnea, cough, and stridor. Eraklis, Griscom, and McGovern 111 reviewed 10 infants with bronchogenic cysts, of whom 9 were first seen with severe respiratory distress. Seven of 8 patients were cured by resection. Opsahl and Berman [4] discussed 31 infants with mediastinal cysts from their review of the literature; two-thirds of these cysts were diagnosed at postmortem examination! Twenty-five of these patients manifested acute respiratory distress typified by periodic episodes of dyspnea, wheezing, stridor, suprasternal and intercostal retractions, and cyanosis. Symptoms were aggravated by crying and eating and were partially relieved by the administration of oxygen. Mediastinal masses were found in 14 infants on routine chest roentgenograms, and 12 were treated successfully by operation. Of the remaining 11 symptomatic patients, 6 had no roentgenographic evidence of a mediastinal lesion; all 1 1 died from their obstructive respiratory distress. The majority of masses producing airway obstruction in neonates and infants are nonmalignant. They include bronchogenic cysts, teratomas, and duplication cysts. Teratomas and bronchogenic cysts should be excised completely. However, the part of a duplication cyst which has a common wall with the esophagus or VOL. 19, NO. 4, APRIL, 1975 369
HALLER ET AL. FIG. 5. In supine chest film mediastinal structures are shifted to the left b~ a large right mediastinal mass; at least part of the mass is posterior since the upper right posterior ribs are splayed and eroded. Compression atelectasis of the right middle and lower lobe with air bronchogram is also seen, as well as compensatory hyperinflation of the left lung. bronchus can be retained; excision of the mucosa from the retained portion will maintain organ integrity and avoid secretory cyst formation. Malignant tumors, such as the neuroblastoma in Patient 5, must also be included in a differential diagnosis. Any mediastinal mass in an infant with respiratory distress warrants an urgent diagnostic workup. Studies should include anteroposterior and lateral chest roentgenograms and a barium swallow. Because of the small space in the superior mediastinum, a lesion large enough to cause respiratory obstruction will cause deviation of the esophagus. Once this confirmatory information is obtained, thoracotomy is the procedure of choice. Bronchoscopy is seldom diagnostically helpful and may subject an infant to significant morbidity from ventilatory and airway complications. With significant extrinsic compression already present, bronchoscopy may fatally impair an already borderline respiratory status. Bronchography may be heipful when a mass is not obvious, but exploratory thoracotomy remains the mainstay of definitive diagnosis and treatment. Ref rences 1. 2. 3. 4. Eraklis, A. J., Griscom, N., and McGovern, J. B. Bronchogenic cysts of the mediastinum in infancy. N Engl J Med 281: 1150, 1969. Haller, J, A,, Jr., Mazur, D. O., and Morgan, W. W., Jr. Diagnosis and management of mediastinal masses in children. J Thorac Cardiouasc Surg 58~385, 1969. Heimburger, I., and Battersby, J. S. Primary mediastinal tumors of childhood. J Thorac Cardiovasc Surg 50:92, 1965. Opsahl, T., and Berrnan, E. J. Bronchogenic mediastinal cysts in infants: Case report and review of the literature. Pediatrics 30:372, 1962. 370 THE ANNALS OF THORACIC SURGERY