Orbital “shanking”: a unique prison injury

The purpose of this paper is to describe orbitocranial penetration due to “shanking”, a common mode of assault in prison facilities. We report the case of a prisoner who presented with orbital apex syndrome 5 days after an assault. He died 9 days after surgical removal of the “shank” due to a presumed ruptured traumatic aneurysm. Physicians who evaluate prisoners must maintain a high index of suspicion for penetrating injuries. The entrance site is often inconspicuous and the history may be limited in this unique population.     

Orbital infantile haemangioma: radiological features and treatment – case series and literature review

Aim: The aim of this study is to report a retrospective case series on orbital infantile haemangiomas (OIH). Radiological features and treatment with oral propranolol (OP) are illustrated along with an updated literature review. Methods: A retrospective chart review of six children, diagnosed with OIH from November 2015 to October 2016, was carried out. Only children with deep documented orbital involvement were included. All patients underwent magnetic resonance imaging (MRI) under general anaesthesia. OP was administered to the infants according to the Nottingham Children's Hospital guideline. As per the guideline, a preliminary paediatric assessment was performed and a 1 mg/kg test dose was administered, followed by definitive treatment at a dosage of 2 mg/kg in three divided doses.Results: Average age at presentation was within the first 3 weeks of life. T1 hypointensity, T2 hyperintensity, avid enhancement with contrast, and the presence of flow-voids appear a fixed pattern of OIH on MRI. Response to treatment was noticed within 4 weeks in all children, and two of them (33.3%) responded within the first 7 days. In two children (33.3%), the haemangioma became clinically undetectable by the seventh month of treatment, while the other four (66.6%) experienced an almost complete regression of the OIH by the last follow-up. No complications were found.Conclusions: Our series strengthens the understanding that MRI is the preferred imaging modality in the investigation of OIH, showing vascular features, detailed orbital extension, and possible associated malformations. OP is the treatment of choice for OIH, and our study confirms its safety and effectiveness.     

Is this really a capillary haemangioma?

Periocular tumours in newborn babies are not uncommon and their diagnosis and management can be challenging. Capillary haemangioma is the most common of them and superficial ones are easy to recognise. Deep-seated (orbital) lesions can mimic various other orbital mass lesions - dermoid cysts, cellulitis, lymphangioma and the more serious rhabdomyosarcoma and neuroblastoma. A careful elicitation of history, physical examination, and appropriate orbital imaging (ultrasound for superficial ones and magnetic resonance imaging/angiography for deep-seated lesions) helps in the diagnosis. This is a brief report of a very large vascular lesion involving the lower lid with very atypical clinical features. The approach to diagnosis and successful treatment and the histological features are discussed.     

3D Orbital Reconstruction in a Patient with Microphthalmos and a Large Orbital Cyst—A Case Report

Background: Orbital cysts are rare developmental anomalies that can occur in microphthalmic and anophthalmic patients. Such cysts can promote orbital growth and subsequently markedly increase the size of the orbit, which is commonly underdeveloped in these patients. Cyst removal is therefore generally dissuaded (at least) in the first 5 years.

Clinical case: A 6-year-old boy with a microphthalmos and a cyst developed protrusion of his prosthetic eye and a swelling of the lower eyelid. MRI showed a large cyst causing distortion of the right orbit. Due to the expansive orbital growth and subsequent misfitting of the prosthesis, cystectomy and orbital floor reconstruction was performed using 3D technology.

Conclusion: 3D imaging and printing enables exact delineation of orbital cysts and the adjacent bony structures. Furthermore it offers the possibility to plan an individual surgical approach and to design and fabricate a custom fit orbital floor implant.     

Rosai–Dorfman disease presenting as choroidal melanoma: a case report and review of the literature

Background

Rosai–Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-malignant proliferation of histiocytes of unknown aetiology. It was first recognised as a distinct clinicopathologic entity in 1969, and is classified as an idiopathic non-Langerhans cell histiocytosis. The disease process is usually self-limiting and often involves lymph nodes, but extranodal involvement is well-described and any anatomic site can be involved.

Methods

We describe a unique case of a 40-year-old male who presented with a fundus mass diagnosed clinically as choroidal melanoma. The tumour showed rapid growth. The patient developed a total retinal detachment and underwent enucleation. The globe contained a choroidal tumour with histologic and immunophenotypic features characteristic of RDD. The literature of ocular Rosai–Dorfman disease was reviewed.

Results

This is the first case in the English literature of intraocular choroidal RDD, mimicking choroidal melanoma.

Conclusions

Rosai–Dorfman disease can present as a mass-producing lesion in the choroid and may mimic other choroidal tumours. The case emphasises the need to consider diagnostic biopsy prior to definitive treatment of choroidal tumours.     

IgG4-related orbital disease masquerading as thyroid eye disease,vice versa,or both?

A 40 year-old male presented after one year of unilateral, progressive, steroid-responsive, orbital inflammatory disease causing proptosis, extraocular muscle (EOM) restriction, and compressive optic neuropathy. The development of anti-thyroidal antibodies prompted the diagnosis of thyroid eye disease (TED); however, the prolonged active phase, remarkable reversibility of ophthalmic features with high-dose corticosteroids, unilaterally of disease, uncharacteristic EOM involvement (including both obliques), and the absence of autoimmune thyroid disease provoked consideration of alternative diagnoses. Inferior oblique biopsy stained positive for IgG4 with histologic features atypical of TED. The patient received rituximab for presumed IgG4-related orbital disease (IgG4-ROD) with subsequent reversal of compressive optic neuropathy, near complete resolution of EOM restriction, and improved proptosis, the latter two of which are not routinely anticipated in advanced TED. The possible role for B-cell depletion in both TED and IgG4-ROD suggests a degree of overlap in the underlying immune-related pathophysiology that is yet to be defined.     

“Melanoma inhibitory activity” (MIA): a promising serological tumour marker in metastatic uveal melanoma

Purpose To assess the role of “melanoma inhibitory activity” (MIA) as a potential serum marker for screening and detection of metastatic uveal melanoma. Design Prospective, clinical study. Material and methods Serum samples of 305 patients with uveal melanoma were collected. Serum samples were analysed by a one-step enzyme-linked immunosorbent assay (ELISA) to quantify the MIA serum levels. All patients underwent a standardized echography of the globe to evaluate maximum tumour height and were checked for systemic metastasis of the tumour by liver enzyme tests and ultrasonography of the liver. Results Twenty patients (6.6%) had proven metastatic disease; eight of them developed it during follow-up. The mean serum concentration of MIA in the 285 patients without metastasis was 6.72 ng/ml, whereas the mean serum concentration of MIA in the 20 patients with metastasis was 13.03 ng/ml (P<0.001). The eight patients who developed metastatic disease during follow-up showed an MIA of 5.92 ng/ml before detection of metastasis and 12.21 ng/ml afterwards (P<0.001). MIA serum levels did neither correlate with the tumour height or to whether local therapy had been applied. Conclusion The elevation of MIA serum levels in patients with metastatic disease from melanoma supports its promising role as a serum marker for monitoring patients with uveal melanoma. The results from this study were presented at the Annual Meeting of the German Society of Ophthalmology (102. Tagung der DOG - Deutsche Ophthalmologische Gesellschaft)     

Immunoscintigraphy for ocular melanoma: a reliable diagnostic technique?

Background: Immunoscintigraphy (IS) has recently been used as a diagnostic tool for ocular melanoma. We wanted to re-evaluate published data in our own patients and to correlate immunoscintigraphic results with histologic findings and immunohistochemical characteristics of the tumour tissue. Methods: During a 4-year period, IS was performed on 35 patients (average age 64 years) with suspected ocular melanoma by i.v. injection of 225.28S, a monoclonal antibody against high-molecular-weight melanoma-associated antigen. Histology was available in 22 cases. Tumour tissue was evaluated for cell type, vascularization, necrosis, pigmentation, and lymphocytic infiltration, and immunohistochemistry was performed with 225.28S and antibodies against HMB-45, S-100 and vimentin. One hundred and two patients with metastasizing cutaneous melanoma served as controls. In these patients the identical immunoscintigraphic technique was applied. Results: IS yielded a positive result in about 50% of our patients with ocular melanoma, while in patients with cutaneous melanoma sensitivity was 89%. In five patients who turned out not to have melanoma, two false-positive results were obtained (one subretinal hemorrhage and one Wegener's granulomatosis). No correlation was found between any of the histological features or the immunoreactivity pattern and the immunoscintigraphic outcome. However, antigenic differences between ocular and cutaneous melanoma were evident. Conclusion: We conclude that IS, using the antibody applied in this study, is of only limited value in patients with ocular melanoma. Our results suggest that antigenic differences, rather than histological characteristics or technical problems, are responsible for the low sensitivity in ocular melanoma compared to cutaneous melanoma.Presented in part at the Annual Meeting of the Association for Research in Vision and Ophthalmology, Sarasota, Florida, 1–6 May, 1994     

Is every iris pigment lesion a melanoma? Case report

In this paper authors present a history and clinical findings of 78 years old man with suspicion of iris melanoma. Malignant melanoma accounts for 70% of cell malignant tumors of the eye. The paper also contains short review of the most important clinical characteristic, diagnostic and treatment of iris melanoma.