Osler-Weber-Rendu disease--unexpected complication following excision of splenic aneurysm--a case report

Osler-Weber-Rendu disease is an autosomal dominant disease, sometimes known as hereditary hemorrhagic telangiectasia (HHT) with a family history. It is a rare disease and there are no records of families with inheritance lineage in Riyadh. We experienced an anesthetic management of a 28-yr-old woman with Osler-Weber-Rendu disease. She was diagnosed to have splenic aneurysm and was scheduled for excision of splenic aneurysm. A chest X-ray revealed pulmonary arterio-venous malformation all over the lungs. The anesthesia and surgery went deliberately smooth with selective period of Intensive Care admission. In the intermediate post operative period, however, she was diagnosed as having pleural effusion. A chest drain was inserted as an emergency, but the patient developed tension pneumothorax, which necessitated resuscitation and readmission to Intensive Care Unit. Lung atelactasis was diagnosed. Attendance by qualified surgical night staff, communication, vigilance and consultations are as important as the proper management of such rare cases.