Department of Clinic Neuroscience, Section of Otorhinolaryngology, University of Palermo, Via Autonomia Siciliana no. 70, Palermo, Italy.
Acta Otorhinolaryngol Ital. 2009 Oct;29(5):274-8.
Arteriovenous malformation of the head and neck is a rare vascular anomaly but when present is persistent and progressive in nature and can represent a lethal benign disease. An unusual case is presented of an arteriovenous malformation of the base of tongue in a 32-year-old primigravida at 23.2 weeks of gestation with a history of haemoptysis. The patient was admitted to hospital with 10.7 g/dl of haemoglobin and 32.1% of haematocrit but due to recurrent massive haemoptysis, in the next few days, dropped to 6.7 g/dl of haemoglobin and 20.2% of haematocrit which required immediate blood transfusions. To maintain the upper airways patent the patient underwent tracheostomy; during angiography, showing an arteriovenous malformation with its feeding arteries (lingual artery, internal maxillary artery, and maxillary artery) embolization was made without a significant blood flow reduction. After surgical ligation of the external carotid artery, on the right side, the patient was readmitted for further angiographic evaluation, which confirmed complete occlusion of the carotid artery but, at the same time, revealed the integrity of the arteriovenous malformation perfusion on account of a new feeding artery (left lingual artery). A new superselective catheterization of the lingual artery was performed but due to the effect of progesterone, which causes smooth muscle relaxation and leads to arteriovenous malformation dilatation and rupture, the primigravida again presented haemoptysis. In agreement with the gynaecologists, the patient was given betamethasone to induce foetal lung maturation, and induction of labour was planned at 26 weeks, and a healthy baby was delivered naturally. Over the following days, the patient had no further haemoptysis and so far clinical examination showed no evidence of the original mass (slight haemorrhagic suffusion of the right anterior amygdala region).
头颈部动静脉畸形是一种罕见的血管畸形，但存在时具有持续性和进行性，可能表现为致命的良性疾病。本文报道了一例不常见的妊娠 23.2 周的 32 岁初产妇舌根动静脉畸形病例，该患者有咯血病史。入院时血红蛋白为 10.7g/dl，血细胞比容为 32.1%，但由于反复大量咯血，在接下来的几天内降至 6.7g/dl 的血红蛋白和 20.2%的血细胞比容，需要立即输血。为了保持上呼吸道通畅，患者接受了气管切开术；血管造影显示动静脉畸形及其供血动脉（舌动脉、上颌内动脉和上颌动脉）栓塞，无明显血流减少。右侧颈外动脉结扎后，患者再次入院进行进一步的血管造影评估，证实颈总动脉完全闭塞，但同时发现新的供血动脉（左侧舌动脉）使动静脉畸形的灌注完整。再次对舌动脉进行超选择性导管插入术，但由于孕激素的作用，导致平滑肌松弛，引起动静脉畸形扩张和破裂，初产妇再次出现咯血。根据妇科医生的建议，给予倍他米松诱导胎儿肺成熟，并计划在 26 周时引产，然后自然分娩出一个健康的婴儿。在接下来的几天里，患者没有再次咯血，目前临床检查没有发现原发病灶的证据（右侧前杏仁核区域轻微出血性瘀斑）。
