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PAPER Osler-Weber-

Rendu Syndrome
(Hereditary Hemorrhagic
Telangiectasia)

Supervisor:
dr. Amran Simanjuntak, Sp
THT-KL (K)
Presentator:
Nining Satriani
Department of Otorhinolaryngology
Novel Ramadhani RSU Haji Medan
Siti Rodiana Hasibuan FK UISU
INTRODUCTION
Osler-Weber-Rendu Syndrome or Hereditary
Haemoragic Telangiectasia (HHT) is an autosomal
dominant genetic disorder that leads to vascular
malformations in mucocutaneus tissues, visceral
organs, and the central nervous system.
Tahun Tahun Tahun
1896 1901 1907

Henri Jules Louis Frederick Parkes


Rendu William Osler Weber
EpidemiologY
• It is a rare but ubiquitous hereditary vascular disease
with incidence around 1/6000 births.
• Incidence in Europe and Japan at rates between
1:5000 and 1:8000.
• More Frequently occurs in whites
PATHOPHYSIOLOGY
Mechanism Of Telangiectasis and
AV Malformations
Focal dilatation of post capillary venules 
Surrounded by lymphocytic infiltrate 
Dilated arteries directly communicates with
venules
CLINICAL FEATURES

Frequency of abnormal HHT vessels: Compared to general population,


• >95% : Nose (Epistaxis) more frequent:
• >90% : skin telangiectasia • Iron deficiency anemia
• 50% : Lungs (Pulmonary AVMs) • Strokes (ischemic and
• 50% : Liver (Hepatic AVMs) haemorrhagic)
• 20% : Gastrointestinal tract • Brain (abscesses)
• 10% : Brain (Cerebral AVMs) • Migraines
• <2% : Spine (Spinal AVMs) • Pulmonary hypertension
Superfisial
Epistaksis Telangiektasis
Pulmonary AVMs GI Bleeding
Cerebral AVM
Diagnosis
Curacao’s criteria

1. Family History 3. Telangiectasia

2. Epistaxis 4. Visceral Lesions


MANAGEMENT

EPISTAXIS

Treatments are generally


directed to patients with
massive hemorrhages or
daily epistaxis Medicamentosa
SURGERY

Commonly used:
- KTP (532 nm)
- Yag (1060 nm)
- Argon (500 nm)
Using a
microbrider to
remove septal
mucosa with
telangiectasia

Fenestrated split-
thickness skin graft

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