Persistent respiratory symptom can be a manifestation of neurofibromatosis-1.
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.
Saha
Agni S
AS
Department of Paediatrics, Queen Elizabeth Hospital, Gateshead, United Kingdom. agnisekhar@doctors.org.uk
Tuladhar
Anil
A
Mitchell
Leslie
L
Spencer
David
D
D006801:Humans; D007223:Infant; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*; D012140:Respiratory Tract Diseases / Q000209:etiology*