Katz S E, Rootman J, Vangveeravong S, Graeb D
Department of Ophthalmology, Vancouver Hospital & Health Sciences Centre, University of British Columbia, Canada.
Ophthalmology. 1998 Jan;105(1):176-84. doi: 10.1016/s0161-6420(98)92058-9.
The authors present seven cases of orbital combined venous lymphatic vascular malformations (CVLVM) (lymphangioma) with evidence of noncontiguous intracranial vascular anomalies.
The study design was a review.
Radiologic studies of 25 patients with combined venous lymphatic vascular malformations were evaluated for noncontiguous intracranial vascular anomalies. Features of the intracranial anomalies and orbital lesions, their clinical presentation, and prognosis are described.
Seven patients (28%) had associated noncontiguous intracranial vascular anomalies. Intracranial hemorrhage occurred in one of these patients. The intracranial anomalies had radiologic characteristics of developmental venous anomalies (DVAs). Diffuse orbital lesions with superficial and deep components (7/7), orbital bony expansion (7/7), and intraconal and extraconal components (4/7) were most common. They involved the inferior orbital fissure and extended into the pterygopalatine fossa in five patients. Involvement of the superior orbital fissure was noted in all seven patients with extension into the middle cranial fossa in three patients. At birth, these patients generally had a visible superficial component and then had episodes of sudden proptosis associated with deep orbital hemorrhages. Visual outcome was poor (20/200 or less) in four (57%) of seven cases. Anterior extension into soft tissues of the face and forehead and other associated vascular lesions, such as palatal involvement, were relatively common. In contrast, CVLVMs (lymphangiomas) without noncontiguous intracranial vascular anomalies were more anterior, less diffuse, less likely to extend into the soft tissues of the face, have associated vascular lesions, or have a poor visual outcome.
Orbital CVLVMs (lymphangiomas) may be associated with noncontiguous intracranial vascular anomalies that may bleed. This association with intracranial DVAs has not been reported previously. The intracranial vasculature should be evaluated prospectively in these lesions, especially if they are diffuse.
作者报告7例眼眶静脉淋巴混合性血管畸形（CVLVM）（淋巴管瘤）伴有非连续性颅内血管异常的病例。
本研究为一项回顾性研究。
对25例静脉淋巴混合性血管畸形患者的影像学检查进行评估，以确定是否存在非连续性颅内血管异常。描述颅内异常和眼眶病变的特征、临床表现及预后。
7例患者（28%）伴有非连续性颅内血管异常。其中1例发生颅内出血。颅内异常具有发育性静脉异常（DVA）的影像学特征。弥漫性眼眶病变伴浅表和深部成分（7/7）、眼眶骨质扩张（7/7）以及肌锥内和肌锥外成分（4/7）最为常见。5例累及眶下裂并延伸至翼腭窝。7例患者均累及眶上裂，其中3例延伸至中颅窝。出生时，这些患者通常有可见的浅表成分，随后会出现与深部眼眶出血相关的突发性眼球突出。7例中有4例（57%）视力预后较差（20/200或更低）。向前延伸至面部和前额软组织以及其他相关血管病变，如腭部受累，较为常见。相比之下，不伴有非连续性颅内血管异常的CVLVM（淋巴管瘤）位置更靠前，范围更局限，延伸至面部软组织、伴有相关血管病变或视力预后差的可能性更小。
眼眶CVLVM（淋巴管瘤）可能与非连续性颅内血管异常有关，后者可能出血。这种与颅内DVA的关联此前未见报道。对于这些病变，尤其是弥漫性病变，应前瞻性地评估颅内血管系统。
