Pediatric Radiology
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Delayed Radiographic Presentation of Congenital Right Diaphragmatic Hernia 1
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Sandra G. Kirchner, M.D., Henry Burko, M.D., James A. O'Neill, Jr., M.D., and Mildred Stahlman, M.D. The diagnosis of right-sided Bochdalek hernias is difficult. Problems encountered in diagnosis include delayed radiographic manifestations and stimulation of inflammatory disease. The case is presented of a neonate in whom a right-sided diaphragmatic hernia masqueraded as inflammatory disease of the chest. INDEX TERMS: Hernia, Bochdalek • Hernia, diaphragmatic
Radiology 115:155-156, April 1975
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ECAUSE the mortality of congenital diaphragmatic hernia without operation approaches 100 %. prompt diagnosis of this entity is vital. Approximately 80% of congenital Bochdalek hernias occur on the left (1), and the radiographic diagnosis is usually obvious, especially when gas rather than fluid· filled loops of small bowel are located in the left thorax. The diagnosis of right Bochdalek diaphragmatic hernias, on the other hand, may be extremely difficult, both clinically and radiographically (2). The following case report illustrates the difficulty of diagnosing this abnormality which may masquerade as inflammatory disease of the chest (3). This 5 lb. 7 oz. (2.5 kg) female infant was the product of an uncomplicated pregnancy and delivery. At approximately 15 hours of age the infant was noted to be lethargic and dusky with a heart rate of 200. Arterial blood gases with the patient breathing room air were documentedas p02 of 53 and a pC0 2 of 33 with a pH of 7.2. About 5 hours later the pC02 was elevated to 61. At 28 hours of age the patient was transferred to Vanderbilt University Hospital. On admission the infant was in moderate respiratory distress. The chest roentgenogram accompanying the patient from the referring hospital was interpreted by all observers to demonstrate mild, bilaterallower lobe infiltrates. Respiratorydistress was felt most likely to be secondary to pneumonia, and the patient was treated with Ampicillin and Gentamicin. During the next few days the patient appeared to improve, requiring a decrease in oxygen concentration. Betahemolytic Streptococcus non-group A or D was recovered from blood culture. Repeat chest roentgenograms revealed some increase in bilateral lower lobe infiltrates with normal position of the diaphragm (Fig. 1). On the sixth hospital day respiratory decompensation ensued and controlled ventilation was required. Subsequent chest roentgenogram (Fig. 2) showed a marked increase in infiltrates on the right associated with right pleural effusion and apparent elevation of the right hemidiaphragm, signifying a significant loss of lung volume. The mediastinum, however, was displaced slightly to the left, contradicting this explanation. The roentgen findings were thought to be secondary to inflammatory disease. The possibility of congenital diaphragmatic hernia was discounted because of the normal position of the diaphragm on the initial chest examination. Because the infant's condition continued to deteriorate and because a right diaphragmatic hernia was suspected, an upper gastrointestinal examination was performed on the thirteenth hospital day (Fig. 3). This study showed elevation of the right hemidiaphragm with the bowel being located high and posterior in the right upper quadrant. Pleural effusion was thought to be present on the right with further deviation of the mediastinum to the left. Diffuse irregular infiltrates were present in the left lung which was overexpanded. A rounded central lucency with air-fluid level on a cross-table lateral
B
chest examination was interpreted as loculated empyema secondary to a bronchopleural fistula. A right thoracotomy tube was inserted, resulting in drainage of 40-50 ml of sterile, serous sanguinous fluid. Following initial improvement. subsequent radiographs demonstrated resolution of the intrathoracic air-flUid level. Improvementdid not continue, and on the fifteenth hospital day bronchoscopy was performed. Small amounts of yellowishpus were seen in the right main stem bronchus, and the carina was deviated in a manner suggesting medial and anterior displacement of the right lung. On the nineteenth hospital day the decision for surgical exploration was made in view of the patient's deterioratingcondition and the bronchoscopic and radiographic findings which indicated the possibility of a congenital malformation of the right diaphragm or of the parenchymaof the right lung. At thoraco-abdominal exploration, herniation of the liver through a central defect in the right hemidiaphragm was found. The patient improved considerably following surgery and was discharged on the eighth postoperativeday. Congenital diaphragmatic hernias may be of three typeshiatal hernia, Morgagni hernia, and Bochdalek or pleuroperitonea/ hernia. The latter hernias result from a failure of the septum transversum to fuse with the pleuroperitoneaI membrane. The size and location of congenital diaphragmatic defects may vary from a small posterior defect in the area of the foramen of Bochdalek to a larger posterolateral defect in the dia'phragm to almost a complete absence of the diaphragm (4). Left-sided Bochdalek hernias are considerably more frequent than right-sided hernias, presenting primarily with respiratory distress and diagnostic roentgenograms (5). Radiographic diagnosis of pleuroperitoneaI hernias of the right diaphragm pose a more difficult diagnostic problem. As in this infant, a normal chest examination may be associated with respiratory distress in the neonate. A period of relative clearing of respiratory distress may be followed days or even months later by a sudden increase in respiratory problems. Radiographic findings may then simulate inflammatory disease of the chest (2, 3), with pleural effusion and pneumonic infiltrates obscuring the diaphragm (5). Often, only the liver herniates into the chest so that the classical signs of intrathoracic intestinal gas are absent. Important indications for the proper diagnosis are the paradoxical findings of pleural effusion, displacement of the mediastinum to the left, and the presence of bowel gas high in the right upper quadrant (2, 3). Additional radiographic studies may confirm the diagnosis, but none are invariably reliable. Upper gastrointestinal series will show the position of the bowel loops, and liver scanning or umbilical venography (6) may document the position of the liver. Diagnostic pneumoperitoneum may be misleading if air is loculated beneath a persistent anterior leaf of the diaphragm (2). The diagnosis of this abnormality is of crucial importance because the mortality of congenital diaphragmatic hernias of the Bochdalek type approaches 100% without surgery. In some instances, an initial normal chest roentgenogram may be followed days, weeks, or months later by subtle radiographic manifestations of hernia. Pleural effusion, possibly complicated by pneumonic infiltrates with shift of the mediastinum to the left, and paradoxical findings of gas high in the right upper quadrant should alert the examiner to the possibility of a right-sided Bochdalek hernia.
1 From the Departments of Radiology (S. G. K., H. B.), Pediatric Surgery (J. A. 0.), and Pediatrics (M. S.), Vanderbilt University Hospital, Nashville, Tenn. Accepted for publication in September 1974. dk
155
156
SANDRA
G. KIRCHNER AND OTHERS
April 1975
Fig. 1. Chest radiograph, fourth hospital day. Bilateral infiltrates have increased since initial roentgenograms, but hemidiaphragms remain in normal position. Fig. 2. Chest radiograph, sixth hospital day. The right hemidiaphragm is no longer clearly visible and bowel gas is seen slightly higher in the right upper quadrant. Infiltrates are markedly increased on the right and are associated with pleural effusion. Although the left lung is overexpanded, the mediastinum is shifted to the left.
Fig. 3. A and B. Anteroposterior and cross-table lateral chest radiographs, thirteenth hospital day. The right hemidiaphragm remains obscured by infiltrate. Barium in the small bowel emphasizes its abnormal position high and posterior in the right upper quadrant. The mediastinum is markedly shifted to the left, and infiltrates are increased on the left. The circular lucency (arrows) with air-fluid level on the cross-table lateral radiograph was felt most likely to represent loculated empyema with bronchopleural fistula. REFERENCES 1. Scheer CW, Linville JL: Congenital diaphragmatic hernia through the foramen of Bochdalek. Arch Surg 91:823-828, Nov 1965 2. McSweeney WJ: Radiologic evaluation of the newborn with respiratory distress. Semin RoentgenoI7:65-83, Jan 1972 3. Canino CWo Eichman J, Rominger CJ, Ryan JJ: Congenital
right diaphragmatic hernia. Radiology 82:249-253, Feb 1964 4. Snyder WH Jr, Greaney EM Jr: Congenital diaphragmatic hernia; 77 consecutive cases. Surgery 57:576-588, Apr 1965 5. Reed JO, Lang EF: Diaphragmatic hernia in infancy. Am J RoentgenoI82:437-449, Sep 1959 6. Sagel SS, Ablow RC: The use of umbilical venography for the diagnosis of congenital right-sided diaphragmatic hernia. Radiology 91:797-798, Oct 1968
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Delayed Radiographic Presentation of Congenital Right Diaphragmatic Hernia 1
•
Sandra G. Kirchner, M.D., Henry Burko, M.D., James A. O'Neill, Jr., M.D., and Mildred Stahlman, M.D. The diagnosis of right-sided Bochdalek hernias is difficult. Problems encountered in diagnosis include delayed radiographic manifestations and stimulation of inflammatory disease. The case is presented of a neonate in whom a right-sided diaphragmatic hernia masqueraded as inflammatory disease of the chest. INDEX TERMS: Hernia, Bochdalek • Hernia, diaphragmatic
Radiology 115:155-156, April 1975
•
•
ECAUSE the mortality of congenital diaphragmatic hernia without operation approaches 100 %. prompt diagnosis of this entity is vital. Approximately 80% of congenital Bochdalek hernias occur on the left (1), and the radiographic diagnosis is usually obvious, especially when gas rather than fluid· filled loops of small bowel are located in the left thorax. The diagnosis of right Bochdalek diaphragmatic hernias, on the other hand, may be extremely difficult, both clinically and radiographically (2). The following case report illustrates the difficulty of diagnosing this abnormality which may masquerade as inflammatory disease of the chest (3). This 5 lb. 7 oz. (2.5 kg) female infant was the product of an uncomplicated pregnancy and delivery. At approximately 15 hours of age the infant was noted to be lethargic and dusky with a heart rate of 200. Arterial blood gases with the patient breathing room air were documentedas p02 of 53 and a pC0 2 of 33 with a pH of 7.2. About 5 hours later the pC02 was elevated to 61. At 28 hours of age the patient was transferred to Vanderbilt University Hospital. On admission the infant was in moderate respiratory distress. The chest roentgenogram accompanying the patient from the referring hospital was interpreted by all observers to demonstrate mild, bilaterallower lobe infiltrates. Respiratorydistress was felt most likely to be secondary to pneumonia, and the patient was treated with Ampicillin and Gentamicin. During the next few days the patient appeared to improve, requiring a decrease in oxygen concentration. Betahemolytic Streptococcus non-group A or D was recovered from blood culture. Repeat chest roentgenograms revealed some increase in bilateral lower lobe infiltrates with normal position of the diaphragm (Fig. 1). On the sixth hospital day respiratory decompensation ensued and controlled ventilation was required. Subsequent chest roentgenogram (Fig. 2) showed a marked increase in infiltrates on the right associated with right pleural effusion and apparent elevation of the right hemidiaphragm, signifying a significant loss of lung volume. The mediastinum, however, was displaced slightly to the left, contradicting this explanation. The roentgen findings were thought to be secondary to inflammatory disease. The possibility of congenital diaphragmatic hernia was discounted because of the normal position of the diaphragm on the initial chest examination. Because the infant's condition continued to deteriorate and because a right diaphragmatic hernia was suspected, an upper gastrointestinal examination was performed on the thirteenth hospital day (Fig. 3). This study showed elevation of the right hemidiaphragm with the bowel being located high and posterior in the right upper quadrant. Pleural effusion was thought to be present on the right with further deviation of the mediastinum to the left. Diffuse irregular infiltrates were present in the left lung which was overexpanded. A rounded central lucency with air-fluid level on a cross-table lateral
B
chest examination was interpreted as loculated empyema secondary to a bronchopleural fistula. A right thoracotomy tube was inserted, resulting in drainage of 40-50 ml of sterile, serous sanguinous fluid. Following initial improvement. subsequent radiographs demonstrated resolution of the intrathoracic air-flUid level. Improvementdid not continue, and on the fifteenth hospital day bronchoscopy was performed. Small amounts of yellowishpus were seen in the right main stem bronchus, and the carina was deviated in a manner suggesting medial and anterior displacement of the right lung. On the nineteenth hospital day the decision for surgical exploration was made in view of the patient's deterioratingcondition and the bronchoscopic and radiographic findings which indicated the possibility of a congenital malformation of the right diaphragm or of the parenchymaof the right lung. At thoraco-abdominal exploration, herniation of the liver through a central defect in the right hemidiaphragm was found. The patient improved considerably following surgery and was discharged on the eighth postoperativeday. Congenital diaphragmatic hernias may be of three typeshiatal hernia, Morgagni hernia, and Bochdalek or pleuroperitonea/ hernia. The latter hernias result from a failure of the septum transversum to fuse with the pleuroperitoneaI membrane. The size and location of congenital diaphragmatic defects may vary from a small posterior defect in the area of the foramen of Bochdalek to a larger posterolateral defect in the dia'phragm to almost a complete absence of the diaphragm (4). Left-sided Bochdalek hernias are considerably more frequent than right-sided hernias, presenting primarily with respiratory distress and diagnostic roentgenograms (5). Radiographic diagnosis of pleuroperitoneaI hernias of the right diaphragm pose a more difficult diagnostic problem. As in this infant, a normal chest examination may be associated with respiratory distress in the neonate. A period of relative clearing of respiratory distress may be followed days or even months later by a sudden increase in respiratory problems. Radiographic findings may then simulate inflammatory disease of the chest (2, 3), with pleural effusion and pneumonic infiltrates obscuring the diaphragm (5). Often, only the liver herniates into the chest so that the classical signs of intrathoracic intestinal gas are absent. Important indications for the proper diagnosis are the paradoxical findings of pleural effusion, displacement of the mediastinum to the left, and the presence of bowel gas high in the right upper quadrant (2, 3). Additional radiographic studies may confirm the diagnosis, but none are invariably reliable. Upper gastrointestinal series will show the position of the bowel loops, and liver scanning or umbilical venography (6) may document the position of the liver. Diagnostic pneumoperitoneum may be misleading if air is loculated beneath a persistent anterior leaf of the diaphragm (2). The diagnosis of this abnormality is of crucial importance because the mortality of congenital diaphragmatic hernias of the Bochdalek type approaches 100% without surgery. In some instances, an initial normal chest roentgenogram may be followed days, weeks, or months later by subtle radiographic manifestations of hernia. Pleural effusion, possibly complicated by pneumonic infiltrates with shift of the mediastinum to the left, and paradoxical findings of gas high in the right upper quadrant should alert the examiner to the possibility of a right-sided Bochdalek hernia.
1 From the Departments of Radiology (S. G. K., H. B.), Pediatric Surgery (J. A. 0.), and Pediatrics (M. S.), Vanderbilt University Hospital, Nashville, Tenn. Accepted for publication in September 1974. dk
155
156
SANDRA
G. KIRCHNER AND OTHERS
April 1975
Fig. 1. Chest radiograph, fourth hospital day. Bilateral infiltrates have increased since initial roentgenograms, but hemidiaphragms remain in normal position. Fig. 2. Chest radiograph, sixth hospital day. The right hemidiaphragm is no longer clearly visible and bowel gas is seen slightly higher in the right upper quadrant. Infiltrates are markedly increased on the right and are associated with pleural effusion. Although the left lung is overexpanded, the mediastinum is shifted to the left.
Fig. 3. A and B. Anteroposterior and cross-table lateral chest radiographs, thirteenth hospital day. The right hemidiaphragm remains obscured by infiltrate. Barium in the small bowel emphasizes its abnormal position high and posterior in the right upper quadrant. The mediastinum is markedly shifted to the left, and infiltrates are increased on the left. The circular lucency (arrows) with air-fluid level on the cross-table lateral radiograph was felt most likely to represent loculated empyema with bronchopleural fistula. REFERENCES 1. Scheer CW, Linville JL: Congenital diaphragmatic hernia through the foramen of Bochdalek. Arch Surg 91:823-828, Nov 1965 2. McSweeney WJ: Radiologic evaluation of the newborn with respiratory distress. Semin RoentgenoI7:65-83, Jan 1972 3. Canino CWo Eichman J, Rominger CJ, Ryan JJ: Congenital
right diaphragmatic hernia. Radiology 82:249-253, Feb 1964 4. Snyder WH Jr, Greaney EM Jr: Congenital diaphragmatic hernia; 77 consecutive cases. Surgery 57:576-588, Apr 1965 5. Reed JO, Lang EF: Diaphragmatic hernia in infancy. Am J RoentgenoI82:437-449, Sep 1959 6. Sagel SS, Ablow RC: The use of umbilical venography for the diagnosis of congenital right-sided diaphragmatic hernia. Radiology 91:797-798, Oct 1968
