David D J, Cooter R D, Edwards T J
Adelaide Children's Hospital, Australia.
J Craniofac Surg. 1991 Jul;2(2):56-60; discussion 61. doi: 10.1097/00001665-199102020-00002.
We describe unique monozygotic twins with Crouzon's disease and cloverleaf-shaped skull deformities who have been closely followed since birth. Their abnormal skull shapes were identified during antenatal ultrasound examination. The twins had gross exophthalmos and hydrocephalus with papilledema, so early calvarial decompression surgery was required. Although born to healthy parents of normal appearance, a third cousin of the twins had a milder form of Crouzon's disease, and there was a family history of high-arched palate on the twins' paternal side. Because Crouzon's disease is an uncommon condition and the cloverleaf skull shape is unusual in patients with Crouzon's disease, identical twins with this constellation of deformities must be exceptionally rare.
我们描述了一对患有克鲁宗病和三叶草形颅骨畸形的独特同卵双胞胎,自出生起就受到密切跟踪。他们异常的颅骨形状在产前超声检查时被发现。这对双胞胎有明显的眼球突出和伴有视乳头水肿的脑积水,因此需要早期进行颅骨减压手术。尽管这对双胞胎的父母外表健康,但他们的一个远房表亲患有症状较轻的克鲁宗病,并且在双胞胎父亲一方有高拱腭的家族病史。由于克鲁宗病并不常见,且三叶草形颅骨在克鲁宗病患者中也不寻常,所以具有这种畸形组合的同卵双胞胎肯定极其罕见。