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Short case assessments: cardiac enlargement
PAEDIATRIC RESPIRATORY REVIEWS (2000) 1, 375–378 doi: 10.1054/prrv.2000.0084, available online at http://www.idealibrary.com on
SELF ASSESSMENT
Short case assessments CASE 1:ASSESSMENT Symptomatic asthma There are frequent asthma symptoms, despite adequate dose of inhaled steroids. Lung function is not very helpful: in fact, during the visit the boy was quite well. FE,NO is very high, which is unexpected when 800 µg of budesonide are taken daily. Steroids suppress NO production effectively, even at low doses that do not control symptoms. Your finding therefore strongly suggests that the boy has not taken his steroids, or has a deficient inhalation technique. You will focus on this possibility by asking about treatment compliance and by checking and instructing inhalation technique. You may wish to change the inhalation device. You should not increase the dose of budesonide but you do prescribe a long acting beta agonist for relief of symptoms, whilst waiting for a few weeks for the steroids to produce a better anti-inflammatory effect. You bring the boy back after 2 months, intending to stop the long-acting beta agonist if at that time FEV1 is normal. CASE SUBMITTED BY: J. C. de Jongste Department of Paediatrics/Respiratory Medicine, Room 5p 2465, Erasmus University and University Hospital/Sophia Children’s Hospital, PO BOX 2060, 3000 CB Rotterdam, The Netherlands doi: 10.1053/prrv.2000.0085, available online at http://www.idealibrary.com on
on the first postoperative night. There were no complications and his sleep study became normal; his secondary cardiovascular problems also regressed. Interestingly, this boy had a 5-year-old sister, who also snored; until her brother’s adenotonsillectomy, noone at home had been able to appreciate this. She too had obstructive sleep apnoea and underwent adenotonsillectomy.
KEY LEARNING POINTS ABOUT PULMONARY HYPERTENSION IN CHILDREN
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CASE 2:ASSESSMENT Cardiac enlargement 1. The heart is enlarged and there is diffuse pulmonary shadowing consistent with pulmonary oedema, infection, or any interstitial process. 2. Does he snore at night? 3. Polysomnography. 4. Adenotonsillectomy. This child had enormously enlarged adenoids and tonsils, and had profound hypoxaemia and hypercapnia during sleep. Note that this had progressed to the point of pulmonary hypertension and heart failure despite a previous ENT evaluation (this is a true story). He underwent emergency adenotonsillectomy and was nursed in PICU 1526–0550/00/040375 + 04 $35.00/0
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The commonest causes are secondary to endstage lung disease (which will be clinically obvious) and congenital heart disease (which may be occult). Pulmonary hypertension with a normal chest X-ray may be secondary to occult interstitial disease, or sleep disordered breathing (obstructive sleep apnoea, neuromuscular disease, kyphoscoliosis. The clinical findings and chest X-ray are often very non-specific, even in quite severe pulmonary hypertension. Pulmonary embolism (due to blood clot or another embolic material) is underdiagnosed in children; typically, thromboembolism occurs in the setting of a child with an intravascular line, immobility or low cardiac output state who deteriorates unexpectedly. Most children with pulmonary embolism have a serious underlying diagnosis, which should be sought. The other primary pulmonary vascular diseases (primary pulmonary hypertension, pulmonary veno-occlusive disease, invasive pulmonary capillary haemangiomatosis) are rare, and require open lung biopsy for full diagnosis. Secondary causes of primary pulmonary hypertension and pulmonary veno-occlusive disease should always be excluded.
© 2000 Harcourt Publishers Ltd
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 CASE 1:ASSESSMENT Symptomatic asthma There are frequent asthma symptoms, despite adequate dose of inhaled steroids. Lung function is not very helpful: in fact, during the visit the boy was quite well. FE,NO is very high, which is unexpected when 800 µg of budesonide are taken daily. Steroids suppress NO production effectively, even at low doses that do not control symptoms. Your finding therefore strongly suggests that the boy has not taken his steroids, or has a deficient inhalation technique. You will focus on this possibility by asking about treatment compliance and by checking and instructing inhalation technique. You may wish to change the inhalation device. You should not increase the dose of budesonide but you do prescribe a long acting beta agonist for relief of symptoms, whilst waiting for a few weeks for the steroids to produce a better anti-inflammatory effect. You bring the boy back after 2 months, intending to stop the long-acting beta agonist if at that time FEV1 is normal. CASE SUBMITTED BY: J. C. de Jongste Department of Paediatrics/Respiratory Medicine, Room 5p 2465, Erasmus University and University Hospital/Sophia Children’s Hospital, PO BOX 2060, 3000 CB Rotterdam, The Netherlands doi: 10.1053/prrv.2000.0085, available online at http://www.idealibrary.com on
on the first postoperative night. There were no complications and his sleep study became normal; his secondary cardiovascular problems also regressed. Interestingly, this boy had a 5-year-old sister, who also snored; until her brother’s adenotonsillectomy, noone at home had been able to appreciate this. She too had obstructive sleep apnoea and underwent adenotonsillectomy.
KEY LEARNING POINTS ABOUT PULMONARY HYPERTENSION IN CHILDREN
• •
• •
CASE 2:ASSESSMENT Cardiac enlargement 1. The heart is enlarged and there is diffuse pulmonary shadowing consistent with pulmonary oedema, infection, or any interstitial process. 2. Does he snore at night? 3. Polysomnography. 4. Adenotonsillectomy. This child had enormously enlarged adenoids and tonsils, and had profound hypoxaemia and hypercapnia during sleep. Note that this had progressed to the point of pulmonary hypertension and heart failure despite a previous ENT evaluation (this is a true story). He underwent emergency adenotonsillectomy and was nursed in PICU 1526–0550/00/040375 + 04 $35.00/0
• •
•
The commonest causes are secondary to endstage lung disease (which will be clinically obvious) and congenital heart disease (which may be occult). Pulmonary hypertension with a normal chest X-ray may be secondary to occult interstitial disease, or sleep disordered breathing (obstructive sleep apnoea, neuromuscular disease, kyphoscoliosis. The clinical findings and chest X-ray are often very non-specific, even in quite severe pulmonary hypertension. Pulmonary embolism (due to blood clot or another embolic material) is underdiagnosed in children; typically, thromboembolism occurs in the setting of a child with an intravascular line, immobility or low cardiac output state who deteriorates unexpectedly. Most children with pulmonary embolism have a serious underlying diagnosis, which should be sought. The other primary pulmonary vascular diseases (primary pulmonary hypertension, pulmonary veno-occlusive disease, invasive pulmonary capillary haemangiomatosis) are rare, and require open lung biopsy for full diagnosis. Secondary causes of primary pulmonary hypertension and pulmonary veno-occlusive disease should always be excluded.
© 2000 Harcourt Publishers Ltd
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