Long-term results of dermatoplasty in the treatment of hereditary haemorrhagic telangiectasia

Ulsø, Claus; Vase, P.; Stoksted, Poul

doi:10.1017/s0022215100094044

Cited by 28 publications

(17 citation statements)

References 5 publications

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“…The technique has been modified by several authors, it seems to control epistaxis bleeding effectively, depending on type and size of grafts. Further interventions are normally required [34]. In patients with previous septal perforation the performance of septectomy followed by graft implants seem to improve the results [35].…”

Section: A) Septodermoplastymentioning

confidence: 99%

A Review on Clinical Management and Pharmacological Therapy on Hereditary Haemorrhagic Telangiectasia (HHT)

Zarrabeitia¹,

Albiñana²,

Salcedo³

et al. 2010

CVP

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Hereditary Haemorrhagic Telangiectasia (HHT) or Rendu-Osler-Weber syndrome, is an autosomal dominant rare disease characterized by localized angiodysplasia. This is manifested as epistaxis, mucocutaneous and gastrointestinal telangiectases, and arteriovenous malformations in the pulmonary, cerebral or hepatic circulation. The prevalence is between 1 in 5,000 to 8,000, although it is higher in some regions. The most frequent clinical manifestation of HHT is epistaxis, normally from light to moderate from the 4(th) decade of life. However, many patients show severe epistaxis which may interfere with their quality of life. The epistaxis is due to telangiectasia on the nasal mucosa. These are focally dilated postcapilar venules, which in advanced phases show many layers of smooth muscle cells without elastic fibers, and very frequently directly connect with dilated arterioles. As a consequence of these vascular alterations, telangiectases are very sensitive to slight trauma and even to the friction with the air when breathing, which gives rise to nose bleeds. Unfortunately, there is no optimal pharmacological treatment for the epistaxis in HHT. The use of antifibrinolytic agents for the treatment of HHT has been studied recently by our group as an effective relief for nasal and gastric haemorrhages. This work represents a systematic review and the beginning of a systematic laboratory work we are now conducting in our lab to screen for "orphan drugs" as therapeutic agents in HHT. In this context, the use of hormones, immunosuppresants and anti-angiogenic agents are under preclinical study in our laboratory.

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Section: A) Septodermoplastymentioning

confidence: 99%

A Review on Clinical Management and Pharmacological Therapy on Hereditary Haemorrhagic Telangiectasia (HHT)

Zarrabeitia¹,

Albiñana²,

Salcedo³

et al. 2010

CVP

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“…O uso de membrana amniótica na cirurgia tem mostrado algum sucesso. 1,2,11,12 A técnica de Young modificada tem se mostrado efetiva. Consiste no fechamento uni ou bilateral da cavidade nasal, evitando a turbulência e o ressecamento causado pelo constante fluxo de ar através das teleangiectasias.…”

Section: Discussionunclassified

Síndrome de Rendu-Osler-Weber: tratamento clínico e cirúrgico

Garcia

Cecatto

Costa

et al. 2003

Rev. Bras. Otorrinolaringol.

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Tel eangiectasia Hemorrágica Hereditária (Síndrome deRendu-Osler-Weber) consiste de uma rara displasia fibrovascular sistêmica, de transmissão autossômica dominante, com incidência de 1-2/100000, afetando vasos sangüíneos da pele, mucosas, pulmões e trato gastrointestinal. É reconhecida pela tríade clássica de teleangiectasias em face, mãos e cavidade oral, epistaxes recorrentes e histórico familiar. Em 90% dos casos, a epistaxe recorrente é o principal sintoma, porém a doença pode afetar qualquer parte do organismo. O tratamento é paliativo, sendo ainda controverso. Neste estudo relatamos três pacientes portadores da doença, enfocando aspectos clínicos e tratamento.

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“…According to Ulsø et al [52], repetitions of the operations were necessary in 8 of 14 patients over a period of 5 to 18 years. Siegel and colleagues evaluated the mean interval from septodermoplasty to the next inpatient recovery or surgical intervention [14]; the mean length of this period when using normal skin grafts was 12.3 months (7 treatments), and 24.4 months for buccal mucosa (5 treatments).…”

Section: Septodermoplasty (Saunders)mentioning

confidence: 99%

Treatment of Recurrent Epistaxis in HHT

Geisthoff¹,

Fiorella²,

Fiorella³

2006

CPD

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No optimal treatment modality is currently available for the treatment of recurrent epistaxis in HHT. In this review, different therapeutic concepts are discussed together with their pathophysiologic background. Patients often profess a preventive effect for nasal ointments and use packings which can be self-administered in the case of bleeding. An effective first-line treatment for physicians is the endonasal laser coagulation or argon plasma coagulation. A second line surgical procedure is septodermoplasty according to Saunders which can provide long-lasting relief if performed correctly. There have been reports on antifibrinolytic agents and hormones, but their efficacy has yet to be determined.

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Long-term results of dermatoplasty in the treatment of hereditary haemorrhagic telangiectasia

Cited by 28 publications

References 5 publications

A Review on Clinical Management and Pharmacological Therapy on Hereditary Haemorrhagic Telangiectasia (HHT)

A Review on Clinical Management and Pharmacological Therapy on Hereditary Haemorrhagic Telangiectasia (HHT)

Síndrome de Rendu-Osler-Weber: tratamento clínico e cirúrgico

Treatment of Recurrent Epistaxis in HHT

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