Verhelst Helene, Van Coster Rudy
Department of Pediatrics, Division of Pediatric Neurology, Ghent University Hospital, Belgium.
J Child Neurol. 2005 Nov;20(11):911-3. doi: 10.1177/08830738050200110901.
The patient presented here has extensive hemangiomata plana, especially on the right forehead, right upper eyelid, and right leg, as well as right leg hypertrophy and macrocephaly. Cerebral magnetic resonance imaging (MRI) showed abnormalities in only one occipital lobe consisting of focal cortical atrophy, leptomeningeal enhancement, and ipsilateral choroid plexus enlargement. Mental and motor development is normal, and he has no seizures. The parents are consanguineous. Leg hypertrophy associated with ipsilateral cutaneous vascular malformations is suggestive of Klippel-Trenaunay syndrome. The patient's central nervous system abnormalities on MRI and the hemangiomata plana on the ipsilateral upper eyelid and forehead point to Sturge-Weber syndrome. We conclude that the patient has an overlap syndrome between Klippel-Trenaunay syndrome and Sturge-Weber syndrome.
此处介绍的患者有广泛的扁平血管瘤,尤其是在右前额、右上眼睑和右腿,同时伴有右腿肥大和巨头畸形。脑部磁共振成像(MRI)显示仅一个枕叶存在异常,包括局灶性皮质萎缩、软脑膜强化和同侧脉络丛增大。智力和运动发育正常,且无癫痫发作。父母为近亲结婚。与同侧皮肤血管畸形相关的腿部肥大提示为克-特综合征。患者MRI上的中枢神经系统异常以及同侧上眼睑和前额的扁平血管瘤指向斯特奇-韦伯综合征。我们得出结论,该患者患有克-特综合征和斯特奇-韦伯综合征的重叠综合征。