Yokomori K, Kubo K, Kanamori Y, Takemura T, Yamamoto T
Department of Pediatric Surgery, Japanese Red Cross Medical Center, Tokyo.
J Pediatr Surg. 1990 Jul;25(7):762-5. doi: 10.1016/s0022-3468(05)80014-4.
Two brothers, 4 years and 6 years of age, presented with a swelling in the right side of the neck. Ultrasonography and venography confirmed a diagnosis of phlebectasia of the right internal jugular vein (IJV). In an attempt to elucidate the etiology of this rare lesion, venous pressures in both the dilated right IJV and in the left IJV were taken under general anesthesia with intratracheal intubation at the time of surgery in each patient. No significant difference in pressure elevation with increase of intrathoracic pressure by overinflating the breathing bag was observed between the right and left IJV, suggesting that there would be no mechanical obstructive process generated on exertion, in each case. Microscopic examinations of a dissected portion of the dilated IJV showed paucity of muscle layer of the vein wall in the younger patient, and absence of that in the elder. Therefore, we assume that congenital muscle defect of the right IJV wall, rather than mechanical obstruction in the lower neck or the mediastinum, might cause phlebectasia. To our knowledge, this is the first report of IJV phlebectasia in siblings.
两名兄弟，年龄分别为4岁和6岁，因右侧颈部肿胀前来就诊。超声检查和静脉造影确诊为右侧颈内静脉扩张症。为了阐明这种罕见病变的病因，在每位患者手术时，于气管插管全身麻醉下测量了扩张的右侧颈内静脉和左侧颈内静脉的静脉压。通过过度充气呼吸囊增加胸内压时，右侧和左侧颈内静脉的压力升高无显著差异，表明在每种情况下用力时不会产生机械性阻塞过程。对扩张的颈内静脉切除部分进行显微镜检查发现，年幼患者静脉壁肌肉层稀少，年长患者则无肌肉层。因此，我们认为右侧颈内静脉壁的先天性肌肉缺陷而非下颈部或纵隔的机械性阻塞可能导致颈内静脉扩张症。据我们所知，这是关于同胞兄弟患颈内静脉扩张症的首例报道。
