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INTRODUCTION
The various hemangiomatous syndromes in otolaryngology
include (1) Klippel Trenaunay -Weber syndrome (2)
Maffucci syndrome, (3) Sturge Weber syndrome, (4)
Tuberous sclerosis, (5) Epidermal nervus syndrome, (6)
Beckwith Wiedmann syndrome, (7) C-syndrome, (8) Hemi
hyper plasia, (9) Mulibrey nanism, (10) Pallister Hall
syndrome, (11) Proteus syndrome, (12) Trisomy - 13syndrome, (13) Bannaynan-Riley-Ruvalcaba syndrome.
Klippel Trenaunay Weber syndrome occurs mostly
sporadically and over 1000 cases have been reported till
date. The present case was an adult male who attended
the Department of Otolaryngology, G. M. and associated
Hospital, Lucknow, India.
CLINICAL PRESENTATION
This patient a 35 years old male was admitted in the
Department of ENT at G. M. and Associated Hospital,
Lucknow with the chief complains of change in voice
from 4 months along with the sensation of fullness in
throat. He took treatment by some local doctor who could
not pinpoint the disease and instead put him on
symptomatic treatment. About 1 & l/2months later he
started having pain in the throat along with difficulty in
swallowing. This throat pain was mild to moderate in
intensity throughout the day but further increased in
intensity during swallowing. Moreover there was no
history of oral bleeding.
On gereral examination the patient was essentially normal
except left sided jugulodigastric lymphadenopathy which
was non tender but freely mobile. The stystemic
examination did not reveal any organomegaly or other
abnormality. The s k in e x am in a t io n revealed a hemangiomatous lesion on the right forearm, which was
about 2 x 3 cm. in size, irregular, soft & nontender. The
Fig. I :
Department of Ot or hi nol ar yngol ogy, K i n g George's M e d i c a l College & G .M.associated Hospital, Shah muna Road,
Lucknow 226 003
49
s h o w i n g p r o l i f e r a t i n g s i n g l e la y e r of e n d o t hel ial
cells sig nifying capillary haemangioma.
DISCUSSION
Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 54 No. I, January - March 2002
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Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 54 No. I, January - March 2002