Blood within the bone: orbital intraosseous venous malformation.

PURPOSE: Description of clinical features, radiological characteristics, and management strategies in primary orbital intraosseous venous malformation (OIVM) with pertinent literature review. METHODS: A retrospective analysis including clinical, radiologic, operative, and histopathological data of six cases of histopathologically proven OIVM was done. A comprehensive literature review was conducted using online databases and augmented with manual search to identify reported cases of OIVM. RESULTS: Study data showed five females and one male in young to middle-age group, with an average age of 30 years (range: 20-48 years). Proptosis was noted in five cases (83.33%), and the duration of symptoms ranged from 6 months to 10 years. Frontal and zygomatic bones were most frequently affected and expansile bony lesion was the most common CT scan finding. Three patients underwent pre-operative embolization of feeders followed by en bloc excision of mass and surgical reconstruction (50%); one patient was managed with partial excision (16.66%) while two were regularly followed-up after incision biopsy (33.33%). Histopathology revealed vascular spaces with endothelial lining, separated by bony trabeculae in all patients. Follow-up periods ranged from 6 to 48 months and no recurrence or progression were noted. CONCLUSIONS: OIVM is an exceptionally rare disorder with a gradually progressive benign course. Ophthalmologists need to be mindful of this entity during patient evaluation as it has propensity for large volume blood loss intra-operatively, owing to its vascular nature. Complete excision with reconstruction of resultant defect is the preferred treatment strategy and without known recurrence.

Crouzon syndrome and the eye: An overview.

The current literature review aims to evaluate the ocular findings and associated ophthalmic features in Crouzon syndrome. Craniosynostoses are syndromes characterized by premature fusion of sutures of the skull and Crouzon syndrome is the most common of the craniosynostosis syndromes. Early fusion of sutures results in craniofacial anomalies, including abnormalities of the orbits. To prepare this review of the ophthalmic findings in this disorder, an organized search on online databases such as PubMed, Scopus, Cochrane Library, and Ovid was carried out. The key terms searched were "Crouzon", "craniosynostosis", "eye" and "ophthalmic", and 51 research items were found. A total of 17 articles were included after scrutiny of the databases and a further 25 articles were added after augmented search. A detailed review was performed from the final 42 articles. A comprehensive description of associated anomalies is given along with the author's own technique of surgical management in cases with Crouzon syndrome having bilateral luxation bulbi with exposure keratopathy. However, for optimum management of cranial and oculo-facial dysmorphisms, a multidisciplinary team of specialists is required.