- Email: [email protected]
Q scan
376
SELF ASSESSMENT: SHORT CASE ASSESSMENTS
CASE SUBMITTED BY: A. Bush Department of Paediatric Respiratory Medicine, Royal Prompton Hospital, Sydney Street, London SW3 6NP, UK
2.
3. doi: 10.1053/prrv.2000.0086, available online at http://www.idealibrary.com on
tertiary hospital. Ann Allergy Asthma Immunol 2000; 84: 25–30. Vinke JG, KleinJan A, Severijnen LW, Hoeve LJ, Fokkens WJ. Differences in nasal cellular infiltrates between allergic children and age-matched controls. Eur Respir J 1999; 13: 797–803. Taccariello M, Parikh A. Darby Y, Scadding G. Nasal douching as a valuable adjunct in the management of chronic rhinosinusitis. Rhinology 1999; 37: 29–32.
CASE 3:ASSESSMENT A small girl with persistent purulent rhinorrhoea and coughing The most likely diagnosis is IgGA or IgGsubclass deficiency. If IgA and IgG subclasses are normal, the response to Pnuemovax can demonstrate a deficiency of specific anti-polysaccharide antibodies formation. In children in this age group the deficiencies are often transient.1 It is often very difficult or impossible to distinguish rhinorrhoea, nasal blockage and coughing due to adenoid hypertrophy, allergic rhinitis and/or immunoglobulin deficiencies. From a practical point of view, based on prevalence it seems plausible to do an adenoidectory before further analysis of the rhinorrhoea. Sinus X-ray does not help in the diagnosis of rhinosinusitis in small children. The volume of the sinuses are too small compared to the nose to make an extra contribution. All children with purulent rhinosinusitis (and many others) have opacification of the sinuses. In this girl there seems to be a combination of problems. She definitely has an allegic component her disease. Twenty to thirty percent of children under 4 years old presenting with rhinitis have allergic rhinitis.2 In patients with asthma this percentage is twice as high. However, these children usually react well to local corticosteroid treatment. Moreover, these children usually do not have this thick green nasal discharge. More serious diseases like immotile cilia syndrome or cystic fibrosis need to be excluded by the proper diagnostic tests.
TREATMENT If the diagnosis is IgG subclass deficiency and the symptomatology is severe enough to warrant further therapy, the treatment of choice should be prophylactic antibiotics, e.g. trimethoprim/sulfamethoxazol twice daily. Daily rinsing of the nose with NaCl reduces the amount of thick section and inflammation.3 Usually these children respond very well to prophylactic antibiotics for 6 months. In severe cases intravenous immunoglobulins may be necessary.
REFERENCES 1.
Javier FC III, Moore CM, Sorensen RU. Distribution of primary immunodeficiency diseases diagnosed in a pediatric
CASE SUBMITTED BY: W. J. Fokkens and R. A. Scheeren Department of Otorhinolaryngology, Grasmes University Medical Centre, Rotterdam, The Netherlands; Department of Otorhinolaryngology, Free University, Amsterdam, The Netherlands doi: 10.1053/prrv.2000.0087, available online at http://www.idealibrary.com on
CASE 4:ASSESSMENT Chest X-ray and V/Q scan The chest radiograph (Fig. 1, p. 372) shows hyperinflation of the left lung with reduced pulmonary vascular markings. The differential diagnosis for this would include causes of air trapping (i.e. an inhaled foreign body), mucus plugging or external causes of bronchial narrowing (ie. foregut duplication cysts, lymphadenopathy, etc.), and congenital lobar emphysema (CLE). On the V/Q (Fig. 2, p. 372) scan there is no perfusion and virtually no ventilation in the left lung, and the mediastinum is shifted towards the right. The differential diagnosis would include chronic airway narrowing, i.e. a foreign body, or extrinsic bronchial compression and CLE. In view of the symptoms related to eating and vomiting, a barium swallow was performed. This showed displacement of the oesophagus to the right at the subcarinal level. A contrast-enhanced CT of the thorax, bronchoscopy and angiography confirmed hyperinflation of the left lung and a low attenuation, non-enhancing ovoid mass behind and below the left hilum causing extrinsic compression and narrowing the left main bronchus and left main pulmonary artery. The findings in the left lung are due to compression of the main bronchus and pulmonary vessels by a mass in the middle mediastinum. The findings of a well-defined, thinwalled structure with contents of approximately water attenuation are in keeping with a bronchogenic cyst. At operation there was a bronchogenic cyst at the level of the carina, surrounding the left main bronchus and also compressing the left pulmonary artery and veins. This was excised and she made an uneventful recovery. The patient is now well, with a normal exercise tolerance and oxygen saturations of 100% in air. She is learning ballet. Bronchogenic cysts are usually solitary, assymptomatic mediastinal masses. They are congenital cysts derived from the embryological foregut which can present at any © 2000 Harcourt Publishers Ltd
SELF ASSESSMENT: SHORT CASE ASSESSMENTS
age. They are typically well defined, thin-walled, round or oval, unilocular cysts and usually contain thick mucoid material. They are mostly of water density but may be greater than soft tissue density when they are indistinguishable from a tumour. They are most commonly found in the middle mediastinum and originate from the trachea or any of the larger airways. Bronchogenic cysts may grow very large without causing symptoms; however, they can compromise surrounding structures and so become clinically evident. They may be life-threatening if there is infection or haemorrhage within them. On plain films, bronchogenic cysts appear as spherical or oval masses and can project from either side of the mediastinum. They push the carina anteriorly and the oesophagus posteriorly. If there is compression of adjacent structures, evidence of air trapping, consolidation or collapse may be seen.
REFERENCES 1. 2.
Merten DF. Diagnostic imaging of mediastinal masses in children. Am J Roentgenol 1992; 158: 825–832. Nakata H, Nakayama C, Kimoto T, Nakayama T, Tsukamoto Y, Nobe T, Suzuki H. Computed tomography of mediastinal bronchogenic cysts. J Comput Assist Tomogr 1982; 6: 733–738.
PRACTICE POINTS
• • •
Recurrent chest symptoms and a persistently abnormal chest X-ray, despite treatment, require further investigation to exclude an underlying cause. Bronchogenic cysts may be found in the middle or posterior mediastinum. Bronchogenic cysts compressing main bronchi or pulmonary vessels are a cause of recurrent wheeze and infections.
CASE SUBMITTED BY: S. Parsonage and K. Johnson Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK
377
The V/Q scan shows that there is no ventilation and no perfusion at all in the right hemithorax. Normal ventilation and perfusion are seen in the left lung. In combination, the findings are suggestive of right lung aplasia or agenesis. A CT scan of the thorax confirmed that there was no right main bronchus, and that the space in the right hemithorax was taken up by the heart and the liver, with a raised hemidiaphragm. An echocardiogram confirmed dextroposition of the heart with a left-sided aortic arch, and a patent foramen ovale. Pulmonary agenesis is defined as the total absence of lung parenchyma, its vasculature and its bronchus beyond the bifurcation, whereas in pulmonary aplasia there is a rudimentary blind-ending bronchus.1 Bronchography could be used to demonstrate this. It is a rare condition, with an estimated incidence of 1 in 10 000.2 There is an equal sex incidence and both lungs are equally affected. There are associated anomalies in 50% of cases. These include anomalies in the cardiovascular system, in the alimentary system (anal and oesophageal atresia, tracheo-oesophageal fistula), and renal agenesis. Skeletal anomalies involve the ribs and vertebra, and there is a particular association with ipsilateral hypoplasia of the thumb with metacarpal and radial abnormalities.3 In pulmonary aplasia/agenesis both ventilation and perfusion are totally absent on V/Q scanning, whereas in unilateral hypoplasia there is complete absence of perfusion but with ventilation images showing a small but well ventilated lung.4 Congenital unilateral pulmonary hypoplasia may be a primary embryological defect but may be secondary to space occupation in the hemithorax, for example from an antenatal pleural effusion, congenital diaphragmatic hernia, cystic hygroma or cystic adenomatoid malformation.5 Unilateral pulmonary hypoplasia is more common on the right. Bilateral pulmonary hypoplasia may be secondary to oligohydramnios, renal failure (Potter syndrome), or neuromuscular disorders. The differential diagnosis for absent ventilation and perfusion in one side of the hemithorax on V/Q scanning includes central bronchial obstruction (tumour, extrinsic compression, inhaled foreign body) and pneumonectomy.
REFERENCES
doi: 10.1053/prrv.2000.0088, available online at http://www.idealibrary.com on
1.
CASE 5:ASSESSMENT
2.
Chest X-ray and V/Q scan
3.
The chest radiograph shows complete opacification of the right hemithorax with shift of the mediastinum to the right. Gas in the stomach indicates that there is a distal tracheo-oesophageal fistula in association with the oesophageal atresia. © 2000 Harcourt Publishers Ltd
4.
5.
Kuhn JP, Slovis TL, Silverman FN, Kuhn LR. Normal lung and anomalies. In: Silverman FN, Kuhn JP (eds). Caffey’s Pediatric X-ray Diagnosis, 9th ed. St. Louis: Mosby 1993; 448–452. Dahnert W. Radiology Review Manual, 3rd ed. Baltimore: Williams and Wilkins, 1996; 365–366. Osborne J, Masel J, McCreadie J. A spectrum of skeletal abnormalities associated with pulmonary agenesis: possible neural crest injuries. Pediatr Radiol 1989; 19: 425–432. Gainey MA. Ventilation and perfusion studies of the lung. In: Miller JH, Gelfand MJ (eds). Pediatric Nuclear Imaging. Philadelphia: W.B. Saunders Company 1994; 68–71. Shaw P. The chest. In: Carty H et al. (eds). Imaging Children. Edinburgh: Churchill Livingstone 1994; 39–42.
SELF ASSESSMENT: SHORT CASE ASSESSMENTS
CASE SUBMITTED BY: A. Bush Department of Paediatric Respiratory Medicine, Royal Prompton Hospital, Sydney Street, London SW3 6NP, UK
2.
3. doi: 10.1053/prrv.2000.0086, available online at http://www.idealibrary.com on
tertiary hospital. Ann Allergy Asthma Immunol 2000; 84: 25–30. Vinke JG, KleinJan A, Severijnen LW, Hoeve LJ, Fokkens WJ. Differences in nasal cellular infiltrates between allergic children and age-matched controls. Eur Respir J 1999; 13: 797–803. Taccariello M, Parikh A. Darby Y, Scadding G. Nasal douching as a valuable adjunct in the management of chronic rhinosinusitis. Rhinology 1999; 37: 29–32.
CASE 3:ASSESSMENT A small girl with persistent purulent rhinorrhoea and coughing The most likely diagnosis is IgGA or IgGsubclass deficiency. If IgA and IgG subclasses are normal, the response to Pnuemovax can demonstrate a deficiency of specific anti-polysaccharide antibodies formation. In children in this age group the deficiencies are often transient.1 It is often very difficult or impossible to distinguish rhinorrhoea, nasal blockage and coughing due to adenoid hypertrophy, allergic rhinitis and/or immunoglobulin deficiencies. From a practical point of view, based on prevalence it seems plausible to do an adenoidectory before further analysis of the rhinorrhoea. Sinus X-ray does not help in the diagnosis of rhinosinusitis in small children. The volume of the sinuses are too small compared to the nose to make an extra contribution. All children with purulent rhinosinusitis (and many others) have opacification of the sinuses. In this girl there seems to be a combination of problems. She definitely has an allegic component her disease. Twenty to thirty percent of children under 4 years old presenting with rhinitis have allergic rhinitis.2 In patients with asthma this percentage is twice as high. However, these children usually react well to local corticosteroid treatment. Moreover, these children usually do not have this thick green nasal discharge. More serious diseases like immotile cilia syndrome or cystic fibrosis need to be excluded by the proper diagnostic tests.
TREATMENT If the diagnosis is IgG subclass deficiency and the symptomatology is severe enough to warrant further therapy, the treatment of choice should be prophylactic antibiotics, e.g. trimethoprim/sulfamethoxazol twice daily. Daily rinsing of the nose with NaCl reduces the amount of thick section and inflammation.3 Usually these children respond very well to prophylactic antibiotics for 6 months. In severe cases intravenous immunoglobulins may be necessary.
REFERENCES 1.
Javier FC III, Moore CM, Sorensen RU. Distribution of primary immunodeficiency diseases diagnosed in a pediatric
CASE SUBMITTED BY: W. J. Fokkens and R. A. Scheeren Department of Otorhinolaryngology, Grasmes University Medical Centre, Rotterdam, The Netherlands; Department of Otorhinolaryngology, Free University, Amsterdam, The Netherlands doi: 10.1053/prrv.2000.0087, available online at http://www.idealibrary.com on
CASE 4:ASSESSMENT Chest X-ray and V/Q scan The chest radiograph (Fig. 1, p. 372) shows hyperinflation of the left lung with reduced pulmonary vascular markings. The differential diagnosis for this would include causes of air trapping (i.e. an inhaled foreign body), mucus plugging or external causes of bronchial narrowing (ie. foregut duplication cysts, lymphadenopathy, etc.), and congenital lobar emphysema (CLE). On the V/Q (Fig. 2, p. 372) scan there is no perfusion and virtually no ventilation in the left lung, and the mediastinum is shifted towards the right. The differential diagnosis would include chronic airway narrowing, i.e. a foreign body, or extrinsic bronchial compression and CLE. In view of the symptoms related to eating and vomiting, a barium swallow was performed. This showed displacement of the oesophagus to the right at the subcarinal level. A contrast-enhanced CT of the thorax, bronchoscopy and angiography confirmed hyperinflation of the left lung and a low attenuation, non-enhancing ovoid mass behind and below the left hilum causing extrinsic compression and narrowing the left main bronchus and left main pulmonary artery. The findings in the left lung are due to compression of the main bronchus and pulmonary vessels by a mass in the middle mediastinum. The findings of a well-defined, thinwalled structure with contents of approximately water attenuation are in keeping with a bronchogenic cyst. At operation there was a bronchogenic cyst at the level of the carina, surrounding the left main bronchus and also compressing the left pulmonary artery and veins. This was excised and she made an uneventful recovery. The patient is now well, with a normal exercise tolerance and oxygen saturations of 100% in air. She is learning ballet. Bronchogenic cysts are usually solitary, assymptomatic mediastinal masses. They are congenital cysts derived from the embryological foregut which can present at any © 2000 Harcourt Publishers Ltd
SELF ASSESSMENT: SHORT CASE ASSESSMENTS
age. They are typically well defined, thin-walled, round or oval, unilocular cysts and usually contain thick mucoid material. They are mostly of water density but may be greater than soft tissue density when they are indistinguishable from a tumour. They are most commonly found in the middle mediastinum and originate from the trachea or any of the larger airways. Bronchogenic cysts may grow very large without causing symptoms; however, they can compromise surrounding structures and so become clinically evident. They may be life-threatening if there is infection or haemorrhage within them. On plain films, bronchogenic cysts appear as spherical or oval masses and can project from either side of the mediastinum. They push the carina anteriorly and the oesophagus posteriorly. If there is compression of adjacent structures, evidence of air trapping, consolidation or collapse may be seen.
REFERENCES 1. 2.
Merten DF. Diagnostic imaging of mediastinal masses in children. Am J Roentgenol 1992; 158: 825–832. Nakata H, Nakayama C, Kimoto T, Nakayama T, Tsukamoto Y, Nobe T, Suzuki H. Computed tomography of mediastinal bronchogenic cysts. J Comput Assist Tomogr 1982; 6: 733–738.
PRACTICE POINTS
• • •
Recurrent chest symptoms and a persistently abnormal chest X-ray, despite treatment, require further investigation to exclude an underlying cause. Bronchogenic cysts may be found in the middle or posterior mediastinum. Bronchogenic cysts compressing main bronchi or pulmonary vessels are a cause of recurrent wheeze and infections.
CASE SUBMITTED BY: S. Parsonage and K. Johnson Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK
377
The V/Q scan shows that there is no ventilation and no perfusion at all in the right hemithorax. Normal ventilation and perfusion are seen in the left lung. In combination, the findings are suggestive of right lung aplasia or agenesis. A CT scan of the thorax confirmed that there was no right main bronchus, and that the space in the right hemithorax was taken up by the heart and the liver, with a raised hemidiaphragm. An echocardiogram confirmed dextroposition of the heart with a left-sided aortic arch, and a patent foramen ovale. Pulmonary agenesis is defined as the total absence of lung parenchyma, its vasculature and its bronchus beyond the bifurcation, whereas in pulmonary aplasia there is a rudimentary blind-ending bronchus.1 Bronchography could be used to demonstrate this. It is a rare condition, with an estimated incidence of 1 in 10 000.2 There is an equal sex incidence and both lungs are equally affected. There are associated anomalies in 50% of cases. These include anomalies in the cardiovascular system, in the alimentary system (anal and oesophageal atresia, tracheo-oesophageal fistula), and renal agenesis. Skeletal anomalies involve the ribs and vertebra, and there is a particular association with ipsilateral hypoplasia of the thumb with metacarpal and radial abnormalities.3 In pulmonary aplasia/agenesis both ventilation and perfusion are totally absent on V/Q scanning, whereas in unilateral hypoplasia there is complete absence of perfusion but with ventilation images showing a small but well ventilated lung.4 Congenital unilateral pulmonary hypoplasia may be a primary embryological defect but may be secondary to space occupation in the hemithorax, for example from an antenatal pleural effusion, congenital diaphragmatic hernia, cystic hygroma or cystic adenomatoid malformation.5 Unilateral pulmonary hypoplasia is more common on the right. Bilateral pulmonary hypoplasia may be secondary to oligohydramnios, renal failure (Potter syndrome), or neuromuscular disorders. The differential diagnosis for absent ventilation and perfusion in one side of the hemithorax on V/Q scanning includes central bronchial obstruction (tumour, extrinsic compression, inhaled foreign body) and pneumonectomy.
REFERENCES
doi: 10.1053/prrv.2000.0088, available online at http://www.idealibrary.com on
1.
CASE 5:ASSESSMENT
2.
Chest X-ray and V/Q scan
3.
The chest radiograph shows complete opacification of the right hemithorax with shift of the mediastinum to the right. Gas in the stomach indicates that there is a distal tracheo-oesophageal fistula in association with the oesophageal atresia. © 2000 Harcourt Publishers Ltd
4.
5.
Kuhn JP, Slovis TL, Silverman FN, Kuhn LR. Normal lung and anomalies. In: Silverman FN, Kuhn JP (eds). Caffey’s Pediatric X-ray Diagnosis, 9th ed. St. Louis: Mosby 1993; 448–452. Dahnert W. Radiology Review Manual, 3rd ed. Baltimore: Williams and Wilkins, 1996; 365–366. Osborne J, Masel J, McCreadie J. A spectrum of skeletal abnormalities associated with pulmonary agenesis: possible neural crest injuries. Pediatr Radiol 1989; 19: 425–432. Gainey MA. Ventilation and perfusion studies of the lung. In: Miller JH, Gelfand MJ (eds). Pediatric Nuclear Imaging. Philadelphia: W.B. Saunders Company 1994; 68–71. Shaw P. The chest. In: Carty H et al. (eds). Imaging Children. Edinburgh: Churchill Livingstone 1994; 39–42.