Oculoplastic and Orbital Service, Istituto G. Gaslini, Genova, Italy.
Ophthalmic Plast Reconstr Surg. 2009 Nov-Dec;25(6):434-6. doi: 10.1097/IOP.0b013e3181b80d2d.
To describe the clinical characteristics and ophthalmic management of 2 patients who developed gross enophthalmos after ventriculo-peritoneal shunting performed in their teenage years. A key radiologic feature is presented, and a conjectural mechanism is proposed for this disfiguring condition.
Retrospective case note review for 2 patients requiring ophthalmic care for gross enophthalmos after prior ventriculo-peritoneal shunting.
Two patients, aged 24 and 25 years, presented with severe bilateral enophthalmos, bridging of all the tarsal plates off the ocular surface with secondary upper eyelid entropion, and significant lagophthalmos, associated with diffuse keratopathy. Both patients were of normal body weight, and neither had a history of anorexia nervosa. CT of the orbit revealed gross enophthalmos, with air entrapment between the globe and upper eyelids, together with a marked upward bowing of the orbital roof in the anterior cranial fossa, a newly recorded sign in this condition. One patient underwent bilateral orbital roof implants, and the other had bilateral upper eyelid entropion repair.
Progressive, severe, bilateral, symmetrical enophthalmos with bridging of the eyelids across the ocular surface due to upward bowing of the orbital roof many years after venticulo-peritoneal shunt in the absence of symptomatic intracranial disease are pathognomonic features of the "silent brain syndrome." A common feature was shunting in the early teenage years; although the enophthalmos had been noted for several years before presentation, the corneal symptoms had only become troublesome enough to seek ophthalmic care in their third decade, and the speed of development for this condition remains unclear. The authors suggest that a sudden reduction of raised intracranial pressure causes an "implosion" of the only available thin cranial bone-namely, the frontal plate of the orbit. Such remodeling might be greater if the bone was still relatively unmineralized, because of youth or preceding hydrocephalus. The expansion of orbital volume is responsible for the characteristic clinical features and symptoms and can be treated with placement of appropriately sized orbital roof implants or, if this is not desired, by upper eyelid entropion repair.
描述 2 例青少年时期行脑室-腹腔分流术后出现明显眼球内陷的患者的临床特征和眼部处理。提出一个关键的放射学特征，并提出了一种用于这种毁容状况的推测机制。
回顾性病例记录，对 2 例因先前脑室-腹腔分流术后出现严重双侧眼球内陷而需要眼科治疗的患者进行分析。
2 例患者，年龄分别为 24 岁和 25 岁，表现为严重双侧眼球内陷，所有睑板都从眼球表面桥接，导致继发性上睑内翻，以及明显的睑裂闭合不全，伴有弥漫性角膜病变。两名患者的体重均正常，且均无神经性厌食症病史。眼眶 CT 显示明显眼球内陷，眼球与上睑之间有空气嵌顿，同时在前颅窝中眶顶呈明显向上弯曲，这是该疾病的一个新记录的征象。1 例患者行双侧眶顶植入物治疗，另 1 例患者行双侧上睑内翻修复术。
在脑室-腹腔分流术后多年，由于眶顶向上弯曲，导致双侧、对称、进行性严重的眼球内陷，眼睑横跨眼球表面，且无明显颅内疾病症状，这是“无声脑综合征”的特征性表现。一个共同的特征是青少年早期分流；尽管眼球内陷已经存在数年，但直到第三十年才因角膜症状变得足够麻烦而寻求眼科治疗，而且这种情况的发展速度尚不清楚。作者认为，颅内压的突然降低会导致唯一可用的薄颅骨（即眶顶的额骨板）“内爆”。如果骨骼由于年轻或先前的脑积水仍然相对未矿化，这种重塑可能更大。眼眶容积的扩大是导致特征性临床特征和症状的原因，可以通过放置适当大小的眶顶植入物进行治疗，或者如果不希望这样做，则通过上睑内翻修复进行治疗。
