Orbital Vascular Anomalies Research Articles

Prevalence and clinical features of orbital vascular anomalies in children

ABSTRACT Purpose To report the prevalence, clinical characteristics, and natural history of orbital vascular anomalies diagnosed among children over a 50-year period. Methods The medical records of all patients <19 years diagnosed with any form of an orbital vascular anomaly (OVA) at Mayo Clinic, Rochester, Minnesota from January 1 1966, through December 31 2015, were retrospectively reviewed. Results A total of 109 children were diagnosed with OVA during the 50-year period, of which 24 were from Olmsted County, MN, yielding a birth prevalence of 1 in 4,305 live births. The median age at diagnosis for the 109 patients was 1.2 years (range, 0–17.9 years) and 67 (61.5%) were female. Common presenting symptoms included proptosis in 80 (73.4%) patients, abnormalities in skin color in 45 (41.3%) patients, and pain in 18 (16.5%) patients. There were 55 (50.5%) vascular malformations [50 (91%) low-flow lymphatic malformations, 3 (5.5%) high-flow arteriovenous malformations, and 2 (3.5%) low-flow venous malformations] and 54 (49.5%) vascular tumors [53 (98%) capillary hemangiomas and 1 (2%) kaposiform hemangioendothelioma]. During a mean follow-up of 5.95 years (range 0–27.7 years), amblyopia and/or strabismus were diagnosed in 46 (43.4%) patients. Conclusions Capillary hemangiomas and low-flow lymphatic malformations comprise most of the orbital vascular anomalies in this cohort of children. Amblyopia and strabismus are common sequelae, highlighting the importance of early diagnosis and appropriate management.

Update on orbital vascular anomalies in pediatrics: imaging studies and management.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Orbital Vascular Anomalies: A Nomenclatorial, Etiological, and Nosologic Conundrum.

Vascular anomalies are a heterogeneous group of disorders that frequently present in the periorbital region. They encompass 2 broad entities: vascular tumors, which possess a proliferative endothelium, and vascular malformations, which are basically localized defects of vascular morphogenesis. The primary goal of this review was to address inaccurate or controversial terminology in the oculoplastic literature concerning orbital and periorbital vascular anomalies and to categorize these lesions in an abridged and simplified hierarchical list that adheres as much as possible to the most recent (2018) iteration for the classification of vascular lesions proposed by the International Society for the Study of Vascular Anomalies (ISSVA). The secondary goal of this review was to review and update information regarding the genetic underpinnings of vascular anomalies and the downstream signaling pathways that are subsequently affected as a result of these genetic errors. A literature review was conducted in PubMed, MEDLINE, PubMed Central, National Center for Biotechnology Information Bookshelf, and Embase for several related keywords including "vascular anomalies, vascular malformations, vascular tumors, and cavernous venous malformation," both with and without adding the keywords "eyelid," "orbital," and "periorbital." In addition, a detailed search was conducted for controversial or obsolete keywords like "cavernous hemangioma," "lymphangioma," and "varices," again in their systemic and orbital/periorbital context. Crucial issues in the 2018 ISSVA classification regarding the proper categorization of orbital vascular anomalies, particularly venous lesions, were critically evaluated and revised, and a regional, simplified, and abridged modification of the ISSVA 2018 classification was proposed. Interdisciplinary and intradisciplinary dialogue concerning orbital vascular anomalies is seriously compromised due to the lack of a unanimous agreement on terminology and the absence of a unified classification concept system. The authors recommend that oculoplastic surgeons adopt ISSVA terminology whenever technically possible and scientifically sound. However, they also propose modifying the ISSVA 2018 classification specifically to adapt to the peculiarities of vascular anomalies in the periorbital region. At present, the simplified classification proposed here is a preliminary first step towards managing patients with orbital vascular anomalies with greater diagnostic and therapeutic precision, until such time in the future when the entire genetic makeup of orbital vascular anomalies is more completely elucidated. Optimistically, this could pave the way for a more robust classification and the ultimate therapeutic cure.

Prevalence and clinical features of orbital vascular anomalies in children

While vascular anomalies have been reported to occur in up to 1% of children, there are no population-based studies describing the prevalence of orbital vascular anomalies. The purpose of this study was to report the birth prevalence, demographics, and ocular sequelae of orbital vascular anomalies diagnosed in children over a 50-year period.

Spontaneous Retrobulbar Haematoma.

Background. Spontaneous orbital haemorrhage is a very rare condition and vision-threatening event. It may occur due to trauma, orbital surgery/injections, orbital vascular anomalies, and a variety of systemic predisposing factors. Signs of retrobulbar hemorrhage include proptosis, ophthalmoplegia, increased intraocular pressure, loss of pupillary reflexes, and optic disc or retinal pallor. Both Computed Tomography scan and Magnetic Resonance Imaging may be performed in the diagnosis. Case Report. A 31-year-old woman was referred to our hospital with a complaint of headache and blurred vision following a strong sneeze. Ophthalmological examination revealed mild Relative Afferent Pupillary Defect in left eye. Computed Tomography revealed left hyperdense retrobulbar mass and displaced optic nerve. T1 weighted hypointense, T2 weighted hyperintense and non-enhanced round shape, sharply demarcated lesion measuring 18 × 15 × 14 × mm in diameter compatible with haematoma was detected by MRI. Surgically Caldwell-Luc procedure was performed. Histological examination confirmed haematoma. Follow-up Magnetic Resonance Imaging revealed a small reduction in the size of lesion but not complete resolution. The patient's complaint was regressed. She is now free of symptoms and is still under surveillance. To our knowledge, this is the first reported case of retrobulbar haematoma caused by sneeze.

The Role of Surgical Treatment in Orbital Vascular Anomalies

The details of the surgical treatment of orbital vascular anomalies (OVAs) are not fully defined in the literature. Two hundred forty-nine patients who underwent surgical treatment of OVAs at our institution between January 1995 and December 2005 were included in a consecutive-series study. Each patient's clinical data, technique details, and clinical outcomes were prospectively collected. The aesthetic results were analyzed according to clinical features and treatment by using the χ (2) association test or Fisher's exact test. Thirty-seven percent of the patients had OVAs involving the upper eyelid, 23% the lower lid, and 40% both eyelids. A complete resection was performed in 65.9% of the patients. A total of 379 surgeries were performed to achieve the final result. The majority of patients (98.3%) had no complaints about surgical scars. The majority of patients had no clinical treatment prior to the surgery. One or more clinical deformities were identified in association with the OVA. Those deformities included ptosis, scar sequelae due to ulceration/infection, eyelash or tarsal/ligament distortions, fibroadipose tissue residues, proptosis, or strabism. Bone deformities were present in 11.3% of the patients. A complete functional and aesthetic restoration was achieved in 66.6% of the patients. Outcomes with a significant relationship to not obtain complete functional and aesthetic restoration included involvement of both eyelids, upper-eyelid involvement, older age, bilateral involvement, diffuse facial pattern OVAs, and OVAs with dimensions greater than one third of the orbit dimension.

Orbital and periorbital vascular anomalies – an approach to diagnosis and therapeutic concepts

Conclusion: A correct and universally accepted terminology based on the biologic behavior of orbital vascular anomalies is essential to avoid misleading diagnoses, to choose an adequate therapy regimen, and to compare different therapeutic approaches. The management of these diseases is multi-modal and an interdisciplinary challenge. Background: There is persisting terminological and clinical confusion about vascular anomalies involving the orbit. Review of the literature reveals a diverse or misleading terminology and a lack of consensus for the treatment of vascular malformations or vascular tumors of the orbit. Methods: This study comprised a detailed analysis of cases presenting with orbital vascular anomalies followed by an extensive review of the literature. Results: Thirty-six patients were analyzed, among them 19 patients with peri- and/or intraorbital hemangiomas, 3 with intraorbital and 2 with periorbital venous malformations, 2 with orbital involvement of complex vascular malformations, and 10 with lymphatic malformations involving the orbit.

Cosmetic orbital surgery

Current indications for orbital surgery primarily aimed at improving cosmesis are considered in the context of subspecialist orbital practice by an ophthalmologist. Thyroid eye disease, orbital vascular anomalies, and dermolipomas are common orbital diseases in which the symptoms can be purely cosmetic. Accurate anatomical awareness, preoperative scanning, control of medical factors including smoking and thyroid status, and endoscopic techniques have all contributed to the aesthetic outcome of orbital surgery. The threshold for performing reconstructive orbital surgery has also been lowered by public demand. Orbital surgeons can therefore offer the familiar techniques, such as orbital decompression, for pure cosmesis. Sensitive history taking and awareness of the psychological element are of paramount importance for the orbital surgeon who develops a cosmetic practice.

Non-traumatic orbital haemorrhage.

To establish the incidence of underlying orbital vascular anomalies, the presence of systemic associations and predisposing factors, the natural history and appropriate management of patients with non-traumatic orbital haemorrhage presenting in an orbital clinic. The records of 115 patients with a diagnosis of non-traumatic orbital haemorrhage were reviewed with regard to clinical findings, investigations, management and outcome. Associated orbital vascular malformations were present in 104 patients (90%). Thirteen (11%) had additional or other predisposing factors (childbirth, prolonged headstands, hypertension or coagulopathies). Six patients (5%) had no predisposing factor. Acute onset painful proptosis, associated with lid swelling or a mass, was the most common presentation. Visual acuity was reduced in 37 patients (32%) at presentation. Excluding eight patients (7%) who underwent surgery for optic nerve compression, spontaneous resolution of the haemorrhage was complete in 62%, partial in 27%, while 4% had no resolution. Final visual acuity was reduced in 23 patients (20%). The majority of bleeds are associated with some form of orbital vascular anomaly. Where no such anomaly can be demonstrated a search for an underlying systemic cause should be performed. Haemorrhages in the young were usually localized whereas those in older patients were diffuse. Orbital imaging, with a combination of computed tomography and magnetic resonance imaging, was helpful in the assessment of these lesions. Most bleeds are venous and self-limiting. Surgical intervention was rarely necessary and should be confined to those with optic nerve compromise or a localized lesion which persists.

Color Doppler imaging of the eye and orbit. A synopsis of a 400 case experience.

Color Doppler imaging (CDI) is a recent advance in ultrasonography that allows simultaneous two-dimensional imaging of structure and blood flow. Doppler information is superimposed in color over a conventional gray-scale ultrasound image. Using this technique we have examined 400 eyes. The central retinal artery, posterior ciliary arteries, ophthalmic artery, the central retinal vein and the vortex veins could be located in all normal eyes. Using the color image as a guide, Doppler spectral analysis is used for quantitative assessment of blood flow velocity in these vessels. We also studied patients with intraocular tumors, arterial and venous retinal occlusions, orbital vascular anomalies and tumors. Color Doppler imaging is a new and promising modality for the study of ocular and orbital hemodynamics.