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:
• >95% : Nose (Epistaxis)
• >90% : skin telangiectasia
• 50% : Lungs (Pulmonary AVMs)
• 50% : Liver (Hepatic AVMs)
• 20% : Gastrointestinal tract
• 10% : Brain (Cerebral AVMs)
• <2% : Spine (Spinal AVMs)
Compared to general population,
more frequent:
• Iron deficiency anemia
• Strokes (ischemic and
haemorrhagic)
• Brain (abscesses)
• Migraines
• 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