Your Federal Quarterly Tax Payments are due April 15th Get Help Now >>

273 RCN Hereditary hemorrhagi by niusheng


									Rev Bras Otorrinolaringol
                                                                                 CASE REPORT

                                                                                                        Hereditary hemorrhagic
                                                                                                   telangiectasia- a new surgycal
        Diego Rosado de Miranda1, Márcio Meira Lima2,
   André Luiz Monteiro Cavalcante3, Elias Bezerra Leite4,                                              Keywords: epistaxis, rendu-osler-weber, telangiectasia.
Sebastião Diógenes Pinheiro5, Marcos Rabelo de Freitas6

               INTRODUCTION                                   was carried out on the skin-mucous joint                         efficient in controlling recurrent epistaxis.
                                                              (Figure 1), preserving the vibrissae on the                      Our patient did not have any epistaxis
         Hereditary Hemorrhagic telangiec-                    side of the nostril, and suturing up in two                      episode after two years and four months
tasia (HHT), or Rendu-Osler-Weber dise-                       planes, a more internal mucous layer and                         of surgery, and such time is in agreement
ase, is a vascular anomaly characterized                      another external, skin layer. After two years                    with data presented in the literature.
by multiple dilations of skin and mucosa                      and three months of follow up there was
capillaries and venules. It is an autosomal                                                                                    FINAL REMARKS
                                                              no other epistaxis episode and he also
dominant disease, equally distributed
                                                                                                                           Young’s procedure proved to be
between both genders and its incidence
                                                                                                                   efficient in controlling recurrent epistaxis
is of 1-2/100.000 inhabitants.1
                                                                                                                   secondary to HHT. We did not observe
         Bleeding may occur in numerous
                                                                                                                   any respiratory alteration, and nasal
places; however, epistaxis is the most
                                                                                                                   occlusion was well tolerated. Although
common, present in 90% of the cases .    2
                                                                                                                   the number of patients submitted to this
The basic lesion is found on the vessels’
                                                                                                                   technique is still small, we may state that
walls, with defects in the elastic and mus-
                                                                                                                   these procedures may be included in the
cular layers that make them more prone
                                                                                                                   therapeutic weaponry of our specialty
to bleeding .1,3
                                                                                                                   in an attempt to minimize the symptoms
         Many treatment modalities have
                                                                                                                   of these patients, and thus improve their
been used to control epistaxis; none of
                                                                                                                   life quality.
them have rendered entirely satisfactory
result4. Options are nasal packing, hormo-                                                                                        REFERENCES
ne-therapy, vascular embolizations, fas-
                                                                                                                   1. Pau H, Carney AS, Murty GE. Hereditary
                                                   1. skin-mucosa joint in
cioplasty and septodermoplasty. Young’s Figuresuture planes - Young’sthe nasal cavity: incision site and closure
                                            in two                         approach.                                  haemorrhagic telangiectasia (Osler-Weber-
surgery is based on nasal occlusion, thus                                                                             Rendu syndrome): otorhinolaryngological
preventing the friction between air and          had hematologic normalization. There was                             manifestations. Clin Otolaringol 2001;26:93-
the telangiectasia, precluding epistaxis no respiratory disorder and the cosmetic                                     8.
episodes.6                                       aspect was very satisfactory.                                   2.Byahatti SV, Rebeiz EE, Shapshay SM. Heredi-
                                                                                                                                   tary Haemorrhagia Telangiectasia: What the
                 CASE REPORT                                                      DISCUSSION                                       otolaryngologist should know. Am J Rhinol
        A.B.L., 56 years old, history of re-                                                                                       1997;11:55-62.
                                                                      Repetition epistaxis is the most                         3.Gluckman JL, Portugal LG. Modified Youngs
current epistaxis, mainly affecting the left                  common HHT symptom, present in 90%
side, since he was 15 years of age. He had                                                                                         procedure for refractory epistaxis due to
                                                              of the cases2. This was the only symptom                             hereditary hemorrhagic telangiectasia. La-
been submitted to many blood transfusions,                    our patient had. The condition started on                            ringoscope 1994;104:1174-7.
as well as cauterizations and fascioplasty,                   his second decade of life, becoming incre-                       4.Jahnke V. Ultrastructure of Hereditary Hemor-
with less than satisfactory results. At hos-                  asingly more intense, in agreement with                              rhagic Telangiectasia. Arch Otololaryngol
pital admittance he had lost much weight                      data from the literature.                                            1970;91:262-5.
and had excessively pale skin (+++/4+).                               The effectiveness of Young’s tech-                       5.Hashimoto K and Pritzker MS. Hereditary
Multiple telangiectasia lesions were seen on                  nique in controlling epistaxis in patients                           hemorrhagic telangiectasia.: An Eletron mi-
the upper lip, nasal tip and nasal cavities.                  with HHT is due to terminating with the air                          croscopic study. Oral surg 1972;34:751-7.
His hematocrit count was 24%. The patient                                                                                      6.Young A. Closure of the Nostrils in Atrophic
                                                              turbulence in a frail mucosa bearing dise-                           Rhinitis. J Laryngol Otol 1997, 81:515-524.
was then submitted to Young’s procedure                       ased vassels6. This procedure is not much
on the left side, since this one was the                      reported in the literature, and it is extremely
most symptomatic one. A circular incision

                                                                       Graduated from the UFRN - 2000. ENT Resident-UFC.
                                                                          MD. Graduated from UFC. ENT Resident -UFC.
                                          ENT - UFC, Otorhinolaryngologist – Department of otorhinolaryngology – Federal University of Ceará (UFC).
                                              ENT - UFC. Otorhinolaryngologist – Department of otorhinolaryngology – Federal University of Ceará.
                                          PhD in Medicine – University of São Paulo. Adjunct Professor of Otorhinolaryngologist – UFC Medical School.
                                                     M.S. in Medicine - USP/ Ribeirão Preto. Assistant Professor of Otorhinolaryngology UFC.
                               Universidade Federal do Ceará - Faculdade de Medicina - Hospital Universitário Walter Cantídio, Serviço de Otorrinolaringologia.
               Mailing Address: R. Prof. Costa Mendes 1608 Rodolfo Teófilo 60430-140 - Dep. de Cirurgia da Faculdade de Medicina da Universidade Federal do Ceará 3º andar.
        Paper submitted to the RBORL - SGP (Publications Management System – Brazilian Journal of Otorhinolaryngology) on April 27, 2005, and approved on May 1st, 2006. cod. 273.

                                                BRAZILIAN JOURNAL OF OTORHINOLARYNGOLOGY 73 (1) JANUARY/FEBRUARY 2007
                                             / e-mail:

To top