Lorenz Birgit, Elflein Heike M, Hofstädter Ferdinand
Department of Pediatric Ophthalmology and Ophthalmic Genetics, Klinikum, University of Regensburg, Regensburg, Germany.
Ophthalmic Genet. 2006 Dec;27(4):165-8. doi: 10.1080/13816810600977085.
To report the nature of the contents of a cyst associated with microphthalmia and optic nerve coloboma in a male infant.
Clinicopathologic report.
A male infant in good general health presented with a massively swollen, protruded left lower eyelid since birth. The globe was not identifiable. MRI disclosed a microphthalmic globe in the upper nasal orbit and an orbital cystic mass consisting of various compartments. Examination of the fellow eye showed a large optic nerve coloboma. As the protrusion of the left lower eyelid was progressive, aspiration of the cyst was performed twice. Finally, the cyst was removed surgically due to repeated filling.
Examination of the aspirated fluid showed resemblance to cerebrospinal fluid (CSF). During surgery, the cyst was not found to communicate with the cerebrospinal fluid system. However, tissue resembling choroidal plexus lined the wall of the cyst that was adherent to the optic nerve. Histological evaluation of the tissue revealed ependymal cells.
The presence of CSF in microphthalmic cysts does not necessarily imply a connection to the CNS, but may be due to local CSF production by ectopic ependymal cells.
报告一名男婴中与小眼症和视神经缺损相关的囊肿内容物的性质。
临床病理报告。
一名总体健康状况良好的男婴自出生以来左眼下睑出现大量肿胀和突出。眼球无法辨认。磁共振成像显示上鼻侧眼眶内有一个小眼眼球以及一个由多个腔室组成的眼眶囊性肿块。对另一只眼的检查显示有一个大的视神经缺损。由于左眼下睑的突出不断进展,对囊肿进行了两次穿刺抽吸。最后,由于囊肿反复充盈,遂进行了手术切除。
对抽吸液的检查显示其与脑脊液(CSF)相似。手术过程中,未发现囊肿与脑脊液系统相通。然而,在附着于视神经的囊肿壁上衬有类似脉络丛的组织。对该组织的组织学评估显示有室管膜细胞。
小眼囊肿中存在脑脊液不一定意味着与中枢神经系统有连接,而可能是由于异位室管膜细胞产生局部脑脊液所致。