Persistent respiratory symptom can be a manifestation of neurofibromatosis-1
Agni S Saha1, Anil Tuladhar, Leslie Mitchell
1Department of Paediatrics, Queen Elizabeth Hospital, Gateshead, United Kingdom. agnisekhar@doctors.org.uk
Abstract:
A 3-year old boy with neurofibromatosis-1 presented with persistent respiratory symptoms and X-ray changes. High resolution computerised tomography (CT), bronchoscopy and spiral CT showed a mediastinal mass encasing and compressing the left main stem bronchus. Surgical removal and histology of the mass revealed a neurofibroma. Repeat scan six months post-surgery showed some persistence of the mass. Symptomatic, mediastinal neurofibroma in the paediatric age group is very rare. We could not find any previous report of a neurofibroma encasing the mediastinal structures. This case also demonstrates that persistent respiratory symptom in a patient with neurofibromatosis-1 warrants a search for intra-thoracic neurofibroma.
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