[abstract] NEUROLOGIC DECOMPRESSION ILLNESS IN A SCUBA DIVER WITH HEREDITARY HEMORRHAGIC TELANGIECTASIA.

Abstract

INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disease characterized by disordered vascular development. Early in life patients have recurrent epistaxis and mucocutaneous telangiectasias. After the 3rd or 4th decades of life, symptoms relate more to visceral arterio-venous malformations affecting the pulmonary, cerebral and hepatic systems. The frequency of pulmonary AVM varies from 36 to 100 % of patients. We report a sport SCUBA diver who presented with serious neurologic decompression illness and HHT. MATERIALS: A 54 year old male completed an uneventful SCUBA dive and shortly upon surfacing developed chest pain, shortness of breath, hypotension and altered mental status and transient loss of consciousness. He was placed on 100% oxygen and transported to a nearby hyperbaric medical facility. He received hyperbaric oxygen therapy which included two table VI and 2 table V treatments. He had near complete resolution of symptoms and neurologic findings. Subsequent evaluation disclosed that he had multiple pulmonary AVMs and a right to left shunt demonstrated on bubble echocardiography. The patient underwent embolization to the pulmonary AVMs and repeat bubble study showed a marked diminution of the previously demonstrated intra-cardiac shunt. CONCLUSIONS: HHT is associated with multiple organ AVMs and may predispose the patient to serious decompression sickness. A review of the literature disclosed only one other case report by Hsu et al in a 15 year old scuba diver whose initial presentation of HHT was neurologic DCS.