Güngör Şükrü, Öztürk Mehmet, Varol Fatma İlknur, Sığırcı Ahmet, Selimoğlu Mukadder Ayşe
Department of Pediatric Gastoenterology, İnönü University School of Medicine, Malatya, Turkey.
Turk J Gastroenterol. 2017 Jul;28(4):303-306. doi: 10.5152/tjg.2017.17035.
A wandering spleen is a rare condition characterized by the malposition of the spleen due to laxity or absence of its supporting ligaments. Although Gaucher disease generally presents with massive splenomegaly, which one of the predisposing causes of a wandering spleen, literature shows only one report of a wandering spleen in a child with Gaucher disease. In this case presentation, a 13-year-oldadolescent with Gaucher disease on enzyme replacement treatment was presented, who was detected having an abdominal mass on a routine visit and diagnosed with partial torsion of a wandering spleen associated with left lobe hypoplasia of the liver.
游走脾是一种罕见的病症,其特征是由于脾脏支持韧带松弛或缺失导致脾脏位置异常。尽管戈谢病通常表现为脾脏巨大,这也是游走脾的易感原因之一,但文献中仅报道过1例戈谢病患儿出现游走脾。在本病例报告中,介绍了一名13岁接受酶替代治疗的戈谢病青少年,其在常规就诊时被检测出腹部有肿块,诊断为游走脾部分扭转并伴有肝左叶发育不全。