title: [Familial primary pulmonary hypertension revealed by the association of bilateral chemosis, subacute myopia, and exophthalmos].
abstract: OBJECTIVE
  To report a rare case of primary pulmonary hypertension (PPH) revealed by a relapsing chemosis, exophthalmos, and subacute myopia.
  
  
  METHODS
  A 46-year-old woman presented with recurrent bouts of chemosis, exophthalmos, and subacute myopia. Her visual troubles were triggered by physical effort. She had a history of a longstanding high blood pressure that was noncompliant to all conventional hypotensive therapy. Automated refraction under cycloplegia showed recent myopia. At observation she had a bilateral, moderate chemosis. Hertel exophthalmometer measured a symmetric exophthalmos of 23 mm. Intraocular pressure was 20 mmHg OD and 24 mmHg OS. Eye fundus was strictly normal.
  
  
  RESULTS
  Laboratory analysis showed no thyroid or inflammatory disease. HIV serology was negative as were antinuclear antibodies. Orbital CT confirmed the axial, symmetric exophthalmos. Echocardiogram brought out the diagnosis of PPH. Genetic studies by PCR localized the BMPR2 mutation that is characteristic of the familial type of PPH. Symptoms decreased under treatment.
  
  
  CONCLUSIONS
  PPH is a rare disease caused by thickening of the arteriolar walls in the pulmonary bed, which results in a right-heart hypertension with venous stasis. Many authors have reported different posterior segment diseases secondary to PPH. However, anterior segment manifestations are extremely rare. These may be caused by a ciliary body congestion and retrobulbar vasodilatation.
  
  
  CONCLUSIONS
  The association of chemosis, exophthalmos, and subacute myopia is unusual in PPH. We recommend keeping in mind the PPH as a differential diagnosis in those cases, especially in patients who suffer from high blood pressure.
authors.affiliation: Service d'Ophtalmologie, Hôtel Dieu, Centre Hospitalier Universitaire, Nantes.
authors.forename: V
authors.initials: V
authors.lastname: Paire
authors.forename: M
authors.initials: M
authors.lastname: Weber
authors.forename: B
authors.initials: B
authors.lastname: Salama
authors.forename: E K
authors.initials: EK
authors.lastname: Agla
authors.forename: F
authors.initials: F
authors.lastname: Closs-Prophète
authors.forename: M
authors.initials: M
authors.lastname: Banchereau
mesh_terms: D003229:Conjunctival Diseases / Q000209:etiology*; D003937:Diagnosis, Differential; D005094:Exophthalmos / Q000209:etiology*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000235:genetics*; D008875:Middle Aged; D009216:Myopia / Q000209:etiology*; D012008:Recurrence