背景:肝外门静脉阻塞是儿童门静脉高压症的最常见原因。然而,它的患病率很低。儿童门静脉高压导致的食管静脉曲张可导致上消化道出血反复发作,如果不及时诊断和治疗,可能会有一个险恶的结果。
方法:一名7岁男性儿童出现上消化道出血反复发作3年。临床检查显示苍白和脾肿大。实验室检查显示脾功能亢进伴贫血的体征,白细胞减少症和血小板减少症,多普勒超声和CT腹部和骨盆显示脾静脉血栓形成伴脾肿大和门静脉海绵样变。该患者接受了脾切除术,脾肾分流术和食管胃静脉曲张断流术。
结论:肝外门静脉阻塞是儿童非肝硬化门静脉高压症的最常见原因。它在儿科人群中的发生非常罕见。门静脉高压可导致静脉曲张破裂出血和脾肿大,这会对儿童的长期健康产生重大影响。由于其阴险的性质,它的诊断需要细致的检查,在儿科人群中治疗是困难的,缺乏专门针对儿科人群的适当治疗指南。
结论:肝外门静脉梗阻在诊断和治疗困难的儿童中很少见。尽管有这些障碍,及时的干预可以减轻幼儿对其不利结局的负担,并大大提高这些患者的生活质量.
BACKGROUND: Extrahepatic Portal Vein Obstruction is the most common cause of portal hypertension in children. However, it has a very low prevalence. Esophageal varices due to portal hypertension in children can lead to recurrent episodes of upper gastrointestinal bleeding, which can have a sinister outcome if timely diagnosis and treatment are not initiated.
METHODS: A 7-year-old male child presents with recurrent episodes of upper gastrointestinal bleeding for 3 years. Clinical examination reveals pallor and splenomegaly. Laboratory investigations revealed signs of hypersplenism with anemia, leucopenia and thrombocytopenia, and Doppler ultrasonography and CT abdomen and pelvis revealed splenic vein thrombosis with splenomegaly and cavernous transformation of the portal vein. The patient was managed operatively with splenectomy with splenorenal shunting and devascularization of esophagogastric varices.
CONCLUSIONS: Extrahepatic Portal Vein obstruction is the most common cause of noncirrhotic portal hypertension in children. Its occurrence in the pediatric population is very rare. Portal hypertension can lead to variceal bleeding and splenomegaly, which can have a significant impact on a child\'s long-term health. Because of its insidious nature, a meticulous workup is required for its diagnosis, and treatment in the pediatric population is difficult, and appropriate guidelines for its management specifically targeting the pediatric population are lacking.
CONCLUSIONS: Extrahepatic Portal Vein obstruction is rare in children with a difficult diagnosis and management. Despite these hindrances, timely intervention can lift a significant burden of its detrimental outcome off the young children and drastically uplift the quality of life of these patients.