British Journal of Radiology (2008) 81, 261-263
© 2008 British Institute of Radiology
doi: 10.1259/bjr/60270972
Acute chest pain in a young woman with a chronic illness
D S KUMAR, FRCR, MS, DNB
R P YADAVALI, MRCS
L A CONCEPCION, MD, PhD
and
H ANIQ, FRCR
Department of Radiology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
Correspondence: Dr Dalavaye Suresh Kumar, FRCR, MS, DNB, Radiology, Royal Liverpool University Hospital, 47 California Close, Great Sankey, Warrington WA5 8WU, UK. E-mail: dalavaye{at}gmail.com
 |
Case history
|
|---|
A 39-year-old woman with a known chronic illness presented to the emergency department with a 2-day history of chest pain and shortness of breath. On clinical examination she was hypoxic with reduced air entry in the left lung base. Baseline cardiac investigations to rule out ischaemic heart disease were normal. Chest radiography showed a left basal consolidation with pleural effusion. Contrast-enhanced multislice CT of the chest was performed (Figures 1
–3).
What is the primary diagnosis? What are the thoracic manifestations of this condition?
|
Diagnosis
|
|---|
Figure 1
shows an embolus in the bifurcation of the right pulmonary artery (arrow). A pre-vertebral soft tissue mass is noted which is caused by extramedullary haematopoiesis. Consolidation and pleural effusion are seen on the left side.
Figure 2 shows H-shaped vertebral bodies due to end-plate necrosis at multiple levels in the mid-thoracic region.
Figure 3 shows H-shaped vertebral bodies, left shoulder hemi-arthroplasty and left basal consolidation. This patient underwent hemi-arthroplasty secondary to previous avascular necrosis of the left humeral head (images not shown).
The patient is known to have homozygous sickle cell anaemia (SCA). The causes of acute chest symptoms in this condition are due to pulmonary embolism, consolidation and pleural effusion. Extramedullary haematopoiesis is an unusual feature in homozygous subjects and is more common in sickle cell variants.
The thoracic manifestations of SCA are reviewed below.
|
Discussion
|
|---|
SCA is one of the most prevalent genetic disorders [1]. This haemolytic anaemia is characterized by abnormal sickle-shaped red blood cells (RBCs), which are removed from the circulation and destroyed by the reticuloendothelial system, leading to anaemia. The underlying abnormality in the RBCs of SCA is the presence of abnormal sickle cell haemoglobin (HbS). Upon exposure to low oxygen tension, the HbS becomes less soluble and aggregates into large polymers. This results in a distorted erythrocyte with a marked decrease in its deformability, which contributes to the vaso-occlusive and haemolytic aspects of the disease. Patients with SCA are at an increased risk of infection because of abnormalities in host defences including impairment of the complement system and functional asplenia [2].
SCA affects multiple organs. Acute chest syndrome (ACS) is the most common cause of death and the second most common cause of hospitalization of adults with SCA (second only to vaso-occlusive crisis) [2, 3]. Thoracic manifestations of sickle cell anaemia can be classified as cardiopulmonary and skeletal changes.
The lung is a common site of involvement in SCA [2]. Fatal pulmonary complications are seen in more than 20% of adults with SCA [3]. The pulmonary manifestations of SCA are both acute and chronic. ACS is defined as a new pulmonary infiltrate on chest radiography accompanied by fever, chest pain and a variety of respiratory symptoms including coughing, wheezing and tachypnoea [2, 3]. Multiple factors may contribute to the respiratory distress associated with ACS. They include pneumonia, pulmonary fat embolism from infarcting bone, hypoxaemia and atelectasis secondary to splinting from painful rib and sternal infarctions, and pulmonary vascular obstruction due to sickling and endothelial adherence of erythrocytes resulting in infarction of the pulmonary parenchyma [4]. Recurrent episodes of ACS and pneumonia lead to chronic lung disease characterized by pulmonary fibrosis, pleural scarring and pulmonary arteriolar intimal hyperplasia, with resultant pulmonary hypertension [2].
Cardiomegaly is often noted in SCA, caused either by the hyperdynamic circulatory state secondary to anaemia or by cor pulmonale secondary to pulmonary venous hypertension [2, 5]. Fibrosis of the electrical conducting pathway can lead to sudden cardiac death. Myocardial infarction often occurs in association with hypoxia, cor pulmonale, anaemia, sepsis, acidosis and renal failure [6].
The fundamental skeletal abnormalities consist of hyperplasia of marrow due to anaemia and areas of bone necrosis due to vascular occlusion. A further complication is the development of infection. Extramedullary haematopoiesis represents extruded hyperplastic bone marrow from the adjacent ribs or vertebrae. It is occasionally found in SCA and is more commonly found in sickle cell variants such as HbS-thal [2]. It appears as a unilateral or bilateral, smooth and sharply marginated paraspinal and paracostal mass without erosion of the adjacent vertebral bodies. The lack of symptoms and classic location in this clinical setting should discourage any invasive diagnostic procedures [5].
Bone infarcts can occur in any marrow-containing bone and are thought to be the underlying cause of most pain crises in SCA. They can also result in fat emboli and ACS [2]. Infarcts of the central part of the vertebral end plate are a characteristic feature of SCA. The central part of the end plate has a single vein and artery, whereas the periphery of the plate receives multiple vessels. Infarction weakens the central part of the plate, producing a stepped depression [7]. Unlike the nucleus pulposus impression on an osteoporotic bone, which is round and concave, the end plate infarcts have a central flat depression with oblique side slopes [8]. This H-shaped appearance is almost pathognomonic of sickle cell disease but has been found rarely in Gaucher's disease [7, 8]. Osteomyelitis occurs in the infarcted bone and may be difficult to differentiate from infarction, even with clinical, laboratory and radiological information [7]. The most commonly encountered organism is Salmonella followed by Staphylococcus aureus [2].
|
Conclusions
|
|---|
Thoracic manifestations of SCA are variable. In patients with SCA presenting with acute chest symptoms, both acute and chronic changes often coexist. Identifying the cause for the symptoms is quite challenging in this situation, and radiologists play a crucial role in the diagnosis and further management. They should therefore familiarize themselves with the various imaging patterns and complications of SCA and its variants.
Received for publication May 26, 2006.
Revision received July 12, 2006.
Accepted for publication July 31, 2006.
|
References
|
|---|
- Siddiqui AK, Ahmed S. Pulmonary manifestations of sickle cell disease. Postgrad Med J 2003;79:384–90.[Abstract/Free Full Text]
- Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics 2001;21:971–94.[Abstract/Free Full Text]
- Stuart MJ, Setty BN. Acute chest syndrome of sickle cell disease: new light on an old problem. Curr Opin Hematol 2001;8:111–22.[CrossRef][Medline]
- Minter KR, Gladwin MT. Pulmonary complications of sickle cell anemia. A need for increased recognition, treatment, and research. Am J Respir Crit Care Med 2001;164:2016–19.[Free Full Text]
- Leong CS, Stark P. Thoracic manifestations of sickle cell disease. J Thorac Imaging 1998;13:128–34.[Medline]
- Mansi IA, Rosner F. Myocardial infarction in sickle cell disease. J Natl Med Assoc 2002;94:448–52.[Medline]
- Stoker DJ, Saifuddin A. Myeloproliferative and similar disorders. In: Grainger RG, Allison DJ, Adam A, Dixon AK, editors. Grainger & Allison's diagnostic radiology. Edinburgh, UK: Churchill Livingstone, 2004: 1904–7.
- Cobby M, Watt I. Tumours and tumour-like conditions of bone. In: Sutton D, editor. Textbook of radiology and imaging. Edinburgh, UK: Churchill Livingstone, 2003: 1287–1320.
Top
Case history
Diagnosis
