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Anesthetic Management for Cesarean Delivery in a Parturient with Hereditary Hemorrhagic Telanglectasia (Osler-Weber-Rendu Syndrome) and Pulmonary Embolism: A Case Report
Abstract Number: F 42
Abstract Type: Case Report/Case Series
INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder of autosomal dominant pattern. It is manifested by recurrent epistaxis in early age, but arteriovenous malformation (AVM) in solid organs may remain silent until later. We present a case of anesthetic management for CD in a parturient with HHT. Her first pregnancy had been complicated by splenic AVM rupture and the second by pulmonary embolism (PE).
CASE: A 34 y/o, G3P1 at 34 wks of gestation (GA), was referred for anesthesia consult. Her HHT was suspected at age 16 with recurrent epistaxis and family history. At age 25, she had a stroke from cerebral AVM rupture. Her first pregnancy at age 32 was complicated with ruptured splenic AVM at 31 wks GA, required emergency CD and splenectomy. She had chronic anemia, asthma, depression, ADDH, and smoking. Current pregnancy has been uneventful and repeat CD was planned at 39 wks GA. Vitals were normal and SpO2 100% in both sitting and supine. Physical exam was unremarkable with no telangiectasia on lips or buccal mucosa. MRI was negative for spinal AVM. At 38 wks GA, she was admitted with 3 wks history of sudden onset and worsening SOB, audible wheezing, and hemoptysis . While exacerbation of asthma or new onset pulmonary AVM was suspected, V/Q scan was ordered to rule out PE. Surprisingly, it showed high probability of PE without any evidence of pulmonary shunt. Anticoagulation with heparin was added to prednisone and bronchodilator with clinical improvement. She was taken to OR 5 days later for elective repeat CD under CSE anesthesia. Preop PTT was normal, and Hgb and platelets 8.9 gm/dL and 191K, respectively. She remained stable intraoperatively with EBL 1.3 L. Epidural was removed at the end. Postop analgesia was provided by bilateral TAP block. PRBC 2 u were given for postop Hgb 6.8 gm/dL. Heparin, resumed 5 hrs after removal of epidural, bridged to enoxaparin 100 mg daily. She made uneventful recovery and discharged home on POD #3.
DISCUSSION: Clinical diagnosis of HHT requires 3 or more features among the Curacao criteria, ie., recurrent epistasis, telangiectases, visceral lesions such as GI telangiectasia or AVMs, and family history.1 Pulmonary AVM is the most common, up to 48%. Due to late-onset penetrance, clinically silent patients may present with sudden, catastrophic complications, such as hemorrhagic stroke, hemothorax, or high output heart failure, from rupture of AVM. Therefore, in the suspected, it is important to screen for AVMs, pulmonary in particular, which could be safely treated with embolization but otherwise potentially life-threatening. The surveillance in parturient with HHT is even more important since the physiologic changes during pregnancy may cause disease progression and severe complications.2 Spinal AVM occurs in 1 % on patients with HHT and should be ruled out before regional anesthesia is considered.
REFERENCES 1. Am J Med Genet 2009;91:66-7. 2. Can J Anesth 2009;56:374-8