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Question :
Posted On : 03/27/2018 00:00:08
a 5 year old girl, previously healthy, has been running fever for last 3 weeks. she was brought to emergency with progressively increasing respiratory distress. on exam, she is pale, with swelling of face, neck and arms. x ray chest shows normal heart size with wide mediastinum.
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Expert Answer :
History seems incomplete. However, this girl probably has superior vena cava syndrome. Malignancy is most common cause for SVC syndrome in children. Anemia suggests some degree of bone marrow involvement.
Most likely she has a non-hodgkin lymphoma with SVC syndrome. At this age it is also not uncommon to have superior mediastinal syndrome {compression of trachea} as trachea is pliable in this age.
This IS a medical emergency. Things to do on urgent basis
1} Manage with supplemental oxygen
2} Do not let her lie down completely, even for CT scan. This can be life threatening for her. Situation is dynamic and can change on minute to minute or howebsitey basis.
3} If not comfortable or facilities not availbale, refer to higher center.
4} Packed red cell {whole blood if Packed red cells not available} transfusion to increase oxygen carriage.
5} Accurate fluid balance {intake, output}
6} Try doing lymph node biopsy. I presume she must have other lymph nodes.
7} If condition deteriorating fast, then give steroids empirically. Downside is, it is likely to hamper correct diagnosis but I will say saving life is a priority given the clinical description and dynamic situation. Radiotherapy is another alternative but generally steroids work very well and radiotherapy is rarely required.
8} Manage according to diagnosis

I do not have all details to suggest more than this at this time point.
All the best.
Answer Discussion :
T
TAUQIR KHAN
SVS wth mediastinal mass, Lymphoma
8 months ago
E
Evelin Dafni
Lymphoma leading to superior venacaval syndrome
8 months ago
M
M Yousif
acute haemolytic anemia
8 months ago
R
rana sleiman
SVC sd,R/O Lymphoma
8 months ago
A
Anoop K
Superior venacaval syndrome due to mediastinal mass probably lymphoma
8 months ago
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