Eur J Cardiothorac Surg 2003;23:1065-1067
© 2003 Elsevier Science NL
Mediastinal haemangiomas in children
Carol Tan*,
Nelson Alphonso,
David Anderson,
Conal Austin
Cardiothoracic Department, Guy's Hospital, St Thomas Street, London SE1 9RT, UK
Received 9 November 2002;
received in revised form 19 February 2003;
accepted 27 February 2003.
* Corresponding author. 18a Fairholme Road, Harrow, Middlesex HA1 2TN, UK. Tel./fax: +44-20-8424-8821
e-mail: caroltan{at}bluecarrots.com
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Abstract
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A 5-month-old girl presented with upper airway obstruction and was found to have a mediastinal mass compressing the trachea. She successfully underwent resection of this mass, later confirmed to be a juvenile haemangioma. This case highlights mediastinal haemangiomas in the paediatric population as a diagnostic and therapeutic challenge, and the importance of a multidisciplinary approach in the management of such patients.
Key Words: Mediastinal haemangioma • Multidisciplinary approach • Magnetic resonance arteriography
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1. Case report
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A 5-month-old girl presented to her local hospital with a 6-week history of noisy breathing, cough and poor feeding. Physical examination revealed an irritable, well-perfused child with a tracheal tug and biphasic stridor.
Chest radiograph showed a widened superior mediastinum and overnight sleep studies revealed oxygen desaturation to 85%. Computed tomography (CT) of the chest showed a large soft tissue mass around the trachea, suggestive of an arterio-venous malformation. Her respiratory condition worsened necessitating intubation. Micro-laryngeal bronchoscopy showed a soft, compressible mid-tracheal lesion with contact bleeding three centimetres below the vocal cords. Intravenous dexamethasone was started and she was transferred to Guy's Hospital.
Despite steroids, repeated attempts at extubation were unsuccessful and she remained intubated 3 weeks later. Her case was reviewed jointly by paediatric intensive care, cardiology, cardiac surgery, otolaryngology, radiology, anaesthesia and oncology.
Echocardiography was normal and aortogram revealed normal mediastinal vasculature. Repeat micro-laryngeal bronchoscopy showed intact tracheal mucosa, making it more likely that the mass was extrinsic. Magnetic resonance imaging (MRI) and angiography confirmed the presence of a large vascular mass extending from below the level of the carina up to the root of the neck, and encasing the great vessels (Fig. 1)
. No feeding vessels were identified. It was deemed unsafe to attempt a trucut biopsy in view of the vascular nature of the lesion and her critical condition. It was therefore jointly decided to proceed with surgical resection without histological diagnosis.
The tumour was approached via median sternotomy. The tumour, which was distinct from the thymus, was firm, dense and loculated in areas but separated from the great vessels by well-developed tissue planes. It encircled the left common carotid artery, left vagus nerve and left subclavian artery completely and extended up along the left common carotid artery into the neck. On the right, it partially encircled the brachiocephalic and subclavian arteries. Both trachea and oesophagus were compressed from the left. Inferiorly, the mass extended extra-pericardially beneath the aortic arch into the subcarinal region. It was dissected out in two halves (Fig. 2)
. Unfortunately, dissection around the left vagus nerve was virtually impossible and it had to be sacrificed proximal to the origin of the recurrent laryngeal nerve.
Post-operatively, the patient was extubated within 24 h and discharged home 8 days later. She showed no evidence of vocal cord palsy. Histological examination classified the tumour as a juvenile haemangioma.
Our patient was followed up with biannual MRI and she remains well 1 year later.
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2. Discussion
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Vascular anomalies can be divided based on clinical and histological findings into either tumours (principally haemangiomas) or malformations. Haemangiomas are proliferative lesions characterized by increased endothelial cell turnover, usually appearing after birth, growing rapidly and then involuting over time. Vascular malformations are errors in morphogenesis with a stable, mature vascular endothelium, presenting at birth, growing commensurately with the child and not involuting [1].
In infancy and childhood, haemangiomas are the most common benign soft tissue tumours with an incidence of 0.8–12% [1,2]. They are found in subcutaneous tissue, liver, bone, central nervous system and spleen but are extremely rare in the mediastinum with a reported incidence of 0.05–1.7% [2,3]. This is likely to be underestimated because of their tendency to involute. They account for less than 0.5% of all mediastinal tumours [2–4].
Mediastinal haemangiomas can arise anywhere within the mediastinum. They tend to be well circumscribed but as in our case, may encroach and compromise vital structures [5]. At a microscopic level, cellular proliferation into adjacent connective and fatty tissue may give the impression of invasion but its biologic aggressiveness is often limited and spontaneous regression usually occurs [4,6]. Feeding vessels may have a role in the development of these tumours [3].
The majority of patients with mediastinal haemangiomas are asymptomatic but can present with chest pain, cough, dyspnoea, stridor or wheeze. Spontaneous haemothorax and facial telangectasis can also occur [2,6].
Investigations should include plain chest radiography, bronchoscopy, echocardiography, arteriography and either CT or MRI [4]. We found that MRI with angiography was most helpful. Other authors have also described its superiority with vascular tumours [7].
Various treatment options are available depending on the site and size of the lesion. As these lesions are known to regress, a conservative approach with regular follow-up should be employed in asymptomatic mediastinal haemangiomas. Involution may be induced by corticosteroid or interferon [2,3,5,8]. Other options include arterial ligation of major feeding vessels, laser therapy, cryotherapy and long-term intubation [3,5].
Given the natural history of haemangiomas, there are few indications for surgical resection: failure to involute; large tumours when diagnosis cannot be made despite extensive investigations; or continued life-threatening symptoms [2,3,6]. Our patient presented with significant tracheal compression necessitating intubation. Intravenous steroids proved unsuccessful in effecting any involution. There were also no feeding vessels to be ligated or embolized which may have resulted in shrinkage of the tumour and relief of the tracheal compression. At the multidisciplinary meeting it was agreed that any attempt at obtaining histological diagnosis was perilous given its vascularity and location. Surgery offered the patient the best chance of both relieving the tracheal compression and providing a possible cure.
The median sternotomy approach was chosen because the tumour extended widely on either side of the mid line. It was felt that this approach would facilitate vascular control and allow access to all extensions of the tumour. Although the left vagus nerve was sacrificed, our patient remained asymptomatic and we expect compensation by the contralateral vocal cord [9].
Recurrence of these tumours is rare. However, in view of our patient's presenting clinical condition, regular MRI scans were performed in the early follow-up period. She has shown no evidence of recurrence at 12 months, or any evidence of vocal cord palsy.
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3. Conclusion
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Mediastinal haemangiomas present both a diagnostic and therapeutic challenge especially in the paediatric population. We believe that a multidisciplinary approach facilitates decision-making and surgery when indicated can be undertaken safely with good results.
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References
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- Orchard P.J., Smith C.M., III, Woods W.G., Day D.L., Dehner L.P., Shapiro R. Treatment of haemangioendotheliomas with alpha interferon. Lancet 1989;2:565-567.[Medline]
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Abstract
1. Case report
