Solitary Orbital Myofibroma: Clinical,Radiographic, and Histopathologic Findings. A Report of Two Cases

This describes a non-interventional case series of 2 patients, aged 7 and 9 years referred to Oculoplastic Unit, both for evaluation of a gradually enlarging, painless, mass of the cheek. CT scan of the first case revealed left orbital floor destruction from a well-defined intraosseous mass. The second was a round circumscribed orbital floor tumor without bone destruction. Histological diagnosis of myofibroma was rendered in both cases. Solitary myofibromas are rare in the orbit. Their rapid growth and bony destruction can mimic malignant tumors. Complete excision with close follow-up is the preferred treatment. Solitary myofibroma should be considered in the differential diagnoses of fibrous tumors with bone destruction in the orbit.     

Solitary orbital myofibroma: clinical, radiographic, and histopathologic findings. A report of two cases

This describes a non-interventional case series of 2 patients, aged 7 and 9 years referred to Oculoplastic Unit, both for evaluation of a gradually enlarging, painless, mass of the cheek. CT scan of the first case revealed left orbital floor destruction from a well-defined intraosseous mass. The second was a round circumscribed orbital floor tumor without bone destruction. Histological diagnosis of myofibroma was rendered in both cases. Solitary myofibromas are rare in the orbit. Their rapid growth and bony destruction can mimic malignant tumors. Complete excision with close follow-up is the preferred treatment. Solitary myofibroma should be considered in the differential diagnoses of fibrous tumors with bone destruction in the orbit.     

Differentiating intradiploic orbital dermoid and epidermoid cysts utilizing clinical features and machine learning

Purpose:The purpose of this study was to characterize intradiploic dermoid and epidermoid orbital cysts to determine any differences in clinical, radiographic, or surgical features.Methods:A retrospective review was performed of patients presenting with intradiplopic dermoid or epidermoid cysts. Additionally, a complete review of the literature was performed to identify cases of intradiplopic orbital dermoid and epidermoid cysts. Data collected included age, sex, presenting symptoms, location of intradiplopic cyst, ophthalmic findings, treatment, and follow-up. Clinical features of dermoid versus epidermoid cyst were compared. Additionally, machine-learning algorithms were developed to predict histopathology based on clinical features.Results:There were 55 cases of orbital intradiploic cysts, 49 from literature review and six from our cohort. Approximately 31% had dermoid and 69% had epidermoid histopathology. Average age of patients with dermoid cysts was significantly lesser than that of patients with epidermoid cysts (23 vs. 35 years, respectively; P = 0.048). There was no difference between sex predilection, presenting symptoms, radiographic findings, or surgical treatment of dermoids and epidermoids. The majority of patients (64%) underwent craniotomy for surgical removal. Machine-learning algorithms KStar and Neural Network were able to distinguish dermoid from epidermoid with accuracies of 76.3% and 69%, respectively.Conclusion:Orbital intradiploic cysts are more commonly epidermoid in origin. Dermoid cysts presented in younger patients; however, there were no other significant differences in features including ophthalmic or radiographic findings. Despite similar features, machine learning was able to identify dermoid versus epidermoid with good accuracy. Future studies may examine the role of machine learning for clinical guidance as well as new surgical options for intervention.     

Ectopic orbital meningioma: report of two cases and literature review

Ectopic orbital meningioma is a rare tumor usually affecting the medial orbit. We present two cases that occurred in a 56-year-old woman and a 28-year-old man. The tumors in both patients were subtotally excised via orbitotomy surgery and were located in the superior quadrant in one of our patients and in the temporal quadrant in the other. Following histopathologic diagnosis, external beam radiotherapy (EBRT) was administered to one patient and intensity modulated radiotherapy to the other. We identified 12 other well-documented cases of ectopic orbital meningioma previously reported. Ectopic meningioma should be considered in the differential diagnosis of medial as well as lateral and superior orbital tumors. The tumor is usually well circumscribed but can be ill defined in imaging studies. There are intralesional calcifications and sclerosis of adjacent bone in some cases. Ectopic orbital meningioma can recur after incomplete excision. Based on the efficacy of EBRT in optic nerve sheath meningioma, we used this treatment to decrease the risk of recurrence in our two patients and found no tumor recurrence at follow-ups of 24 and 74 months, but one patient had severe vision loss from radiation retinopathy.     

Is primary optic nerve sheath schwannoma a misnomer? Report of two cases and literature review

Aims: To report clinicopathological characteristics of two patients with optic nerve sheath schwannoma (ONSS) and review the literature.

Method: The first patient (22-year-old man) presented with left eye proptosis and decreased vision in 2012 whose orbital imaging showed a large cystic lesion around the optic nerve. The second patient (52-year-old man) presented with decreased vision in the left eye (without proptosis) in the 2006. His imaging showed a small orbital apex lesion between the medial rectus and optic nerve. Both lesions were histopathologically consistent with ONSS. ONSS has previously been reported in 12 patients.

Results: Orbital biopsy and subsequently external beam radiotherapy were performed for the first patient who showed a temporary improvement of vision. However, his proptosis progressed and vision decreased to light perception (LP) a few months after radiotherapy when the lesion was resected in April 2013. Second patient declined any procedure until his vision gradually decreased to LP in October 2007. It was then removed through a combined medial and lateral orbitotomy procedure. Both patients ended up with visual acuity of no LP and no recurrence in their last follow up visits in April 2018. No histopathological evidence was found to show that optic nerve sheath could be the origin for presenting and previously reported cases in the literature.

Conclusion: Presenting cases and literature review imply that ONSS is a misnomer and all the lesions are different types of orbital schwannomas with optic nerve sheath attachment.     

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.     

A Review of 244 Orbital Tumors in Japanese Patients During a 21-Year Period: Origins and Locations

Purpose To review 244 cases of orbital tumors to determine the distribution of patient age, pathology, and tumor origin and location.Methods We reviewed cases of histopathologically or radiologically verified orbital tumors at our institution from 1981 through 2002, and studied the distribution of patient age, pathology, origin of tumors, and tumor location in the orbit in 244 consecutive patients (aged from 0 to 90 years, mean, 48.7 years; 114 men, 130 women).Results Among the 244 cases, 213 (89%) were primary orbital tumors, 23 (9%) were secondary tumors originating in contiguous spaces, and 8 (2%) were metastatic tumors. Regarding the location in the orbit, 122 tumors (50%) were extraconal, 36 (15%) were intraconal, and 86 (35%) were in the lacrimal gland area. The common tumors were reactive lymphoid hyperplasia (22%) and malignant lymphoma (20%) in the extraconal area; cavernous hemangioma (25%), optic nerve glioma (14%), and optic nerve sheath meningioma (14%) in the intraconal area; and malignant lymphoma (40%) and pleomorphic adenoma (24%) in the lacrimal gland area. The age distribution of the 244 patients exhibited two peaks, at 0 to 9 years and 60 to 69 years. In the 0- to 9-year-old patients, the most common tumors were dermoid cyst (26%), optic nerve glioma (11%), capillary hemangioma (11%), and hemorrhagic lymphangioma (11%). On the other hand, in patients over 40 years of age, the common tumors were malignant lymphoma (31%), orbital pseudotumor (24%), pleomorphic adenoma (10%), and cavernous hemangioma (9%).Conclusions The pathologic profiles of orbital tumors were characterized according to patient age and the location of the tumor in the orbit. The age of onset, tumor location, and radiological findings provide us with important information for the diagnosis of a tumor prior to biopsy or tumor resection and for the determination of the treatment strategy. Jpn J Ophthalmol 2005;49:49–55 © Japanese Ophthalmological Society 2005     

Functional Magnetic Resonance Imaging (MRI) and MRI Tractography in Progressive Supranuclear Palsy-Like Syndrome

ABSTRACT

An 18-year-old woman underwent an uneventful ascending aortic aneurysm repair then developed progressive supranuclear palsy-like syndrome. Extensive neuroimaging including contrasted fat-suppressed cranial and orbital magnetic resonance imaging (MRI), MRI tractography, and functional MRI (fMRI) revealed no clear radiographic involvement except for a single tiny hypoechoic midbrain dot on the T2*-weighted gradient-echo imaging, which is not considered sufficient to account for the patient’s deficits. This case attests to the occult nature of this rare and devastating syndrome.     

Metástasis orbitaria pulsátil como signo de presentación de carcinoma renal de células claras metastásico

Case reportWe present the case of a 56 year-old male with a pulsating lesion in the temporal region of the left orbit as presenting sign of a renal cell carcinoma. A review is also presented of all cases of orbital metastases diagnosed in our hospital between 1957 and 2012.DiscussionCarcinoma is the most common malignancy involving the kidney but, only rarely does it metastasize to the orbit. As these tumors can be confused with other amelanotic or vascular tumors, a high index of suspicion is required for early detection and management of the primary tumor.     

Primitive neuroectodermal tumor of the orbit in an adult: A case report and literature review

ObjectiveThe authors describe the salient clinical, radiologic, and histopathologic features of an orbital primitive neuroectodermal tumor in a 28-year-old man. This is an extremely rare tumor of the orbit, previously reported exclusively in children.DesignCase report.InterventionExcisional biopsy of the tumor en bloc was performed.Main outcome measuresHistopathologic examination was performed by standard techniques and immunohistochemical stains on formaldehyde-fixed, paraffin-embedded tumor tissues.ResultsHistologic examination of the sections of the tumor showed small, blue, round cells with occasional Homer-Wright rosette formations. The tumor cells stained positively with neuron-specific enolase and vimentin.ConclusionsThis newly recognized, highly unusual peripheral primitive neuroectodermal tumor should be considered in the differential diagnosis of hypercellular, small, round cell tumor of the orbit in adults.     

Multiple intraorbital neurofibromas: a rare cause of proptosis

BACKGROUND: Orbital neurofibromas are rare, accounting for 0.5 to 2.4 % of all orbital tumors. Generally, they manifest as slowly progressive proptosis, in a young adult or middle-aged person, and are usually solitary lesions. Sometimes, they can be associated with type 1 neurofibromatosis. We present a case of proptosis related to multiple intraorbital neurofibromas in an 82-year-old woman without type 1 neurofibromatosis. HISTORY AND SIGNS: An 82-year-old woman was referred for slowly progressive left proptosis associated with an ocular burning sensation. Neuro-ophthalmic examination revealed 9.5 mm of left exophthalmos, signs of minimal left optic neuropathy but normal extraocular movements. Magnetic resonance imaging revealed the presence of 4 intraorbital lesions. THERAPY AND OUTCOME: The two most anterior tumors were removed. Pathological studies showed these tumors to be neurofibromas. Post-operative evolution was favorable with reduction of left proptosis to 7 mm and disappearance of the burning sensation of the left eye. No other signs of neurofibromatosis were found. CONCLUSIONS: Multiple circumscribed intraorbital tumors are rare. Slowly progressive proptosis with radiological imaging of multiple round lesions should evoke the diagnosis of orbital neurofibromas, even in patients outside the typical age range or without neurofibromatosis.     

Photodynamic therapy in the management of circumscribed choroidal hemangioma

ObjectiveTo evaluate the efficacy of photodynamic therapy (PDT) for symptomatic circumscribed choroidal hemangioma (CCH).DesignA retrospective case series analysis through chart review of patients with CCH treated by means of PDT.ParticipantsThirteen patients with circumscribed choroidal hemangioma.MethodsThrough retrospective charts review, the patients' demographics, clinical features, management, and follow-up data were obtained and analyzed. Outcome measures were change in visual acuity, required sessions, and adjunctive treatments.ResultsMedian follow-up was 26 months. Seven patients (54%) underwent only 1 session, 5 patients (38%) 2 sessions, and 1 received 5 sessions and was finally treated with external beam radiotherapy (EBRT). Visual acuity improved by at least 1 line (1-5) in 11 patients and remained stable in 2. Exudative subretinal fluid was completely resolved in 92% of the cases at the last visit. No major local side effects were found.ConclusionsPDT of CCH can effectively induce tumor regression and resolution of exudative subretinal fluid, improving, or stabilizing vision.     

Orbital Retinoblastoma in an Adult

ABSTRACT

Purpose: Retinoblastoma is usually seen in children before 5 years of age. We report an unusual case of retinoblastoma in an adult who presented to us with an orbital mass.

Methods: A 24 year-old-male presented to our centre with a history of protrusion of the right eye for 6 months, and associated loss of vision. Ultrasonography B-scan revealed an intraocular mass with calcification and MRI of the orbits showed extra-ocular spread. An incisional biopsy was taken from the orbital mass.

Results: On biopsy, histopathologic features and immunohistochemical stains were consistent with retinoblastoma.

Conclusion: To our knowledge, this is the first report of retinoblastoma presenting as an orbital mass in adulthood and highlights the importance of considering this tumour in the differential diagnosis of an intraocular mass with orbital extension in an adult patient.     

Conjunctival plasmacytoma

BackgroundPlasmacytomas are plasma cell tumors that may be a primary or secondary tumor focus, the latter of which are associated with multiple myeloma. We present a rare case of a solitary extramedullary plasmacytoma involving the conjunctiva.Case ReportA 33-year-old white man presented with the initial complaint of redness in both eyes, more in his right than left eye. A vascularized conjunctival lesion was noted in his right eye. The patient underwent excisional biopsy, which found a conjunctival plasmacytoma.ConclusionAlthough plasmacytomas of the eye and orbit are rare, it is important to be familiar with these tumors that may be associated with multiple myeloma. Definitive diagnosis is made by biopsy and histopathologic examination of the tissue. Plasmacytomas may be treated with external beam radiation, local excision, or radiotherapy after surgical excision. Long-term follow-up, including periodic systemic evaluation, is required to establish that orbital involvement is not an early manifestation of multiple myeloma.     

Retinoblastoma: postenucleation orbital recurrence

Objective: The objective of this study was (i) to identify clinicopathological variables that significantly influence the frequency of postenucleation orbital recurrence and (ii) to create an awareness regarding follow-up, to pre-empt orbital recurrence.Design: Retrospective, case-controlled, longitudinal study.Participants: A total of 176 children with retinoblastoma who were admitted and treated at Khyber Teaching Hospital between August 1, 1978, and July 31, 1998.Methods: This study was undertaken by reviewing the appropriate charts. The data of children with primary enucleation were analyzed to extract cases of orbital recurrence.Results: The mean age was 3.8 years (range 1–10 years). Of 176 patients, 83 (47.3%) presented with proptosis and fungating mass. Of 136 patients who were enucleated, 80 (58.82%) presented with proptosis and fungating mass. The 31 patients who had been diagnosed as having had orbital recurrence had a mean age of 4.09 years. The duration between enucleation and orbital recurrence was 1–12 months in 28 patients (90.4%). Histopathology reports of only 8 patients (26%) could be traced. Follow-up was recorded in only 9.6% of cases.Conclusions: The failure to identify clinical and pathological parameters in the course of the disease in retinoblastoma children results in human misery caused by poor follow-up and gross negligence.     

A comparative analysis of piezosurgery and oscillating saw for balanced orbital decompression

Introduction: Different minimally invasive surgical approaches to the orbit allow individualized bone resection to reduce proptosis and decompress the optic nerve in patients with Graves’ orbitopathy (GO). This study aims to compare piezosurgery to an oscillating saw used to resect bone from the lateral orbital wall.

Methods: In a retrospective study, we analyzed balanced orbital decompressions performed on 174 patients (318 cases) with GO. An oscillating saw was used in 165 cases (saw group) and piezosurgery in 153 cases (piezo group). Peri- and postoperative complications, reduction of proptosis, new onset of diplopia and improvement of visual acuity in cases of pre-operative optic nerve compression were analyzed.

Results: We observed no significant differences in the surgical outcome between the two groups. Proptosis reduction was 4.6 mm in the saw group (p < 0.01) and 5.3 mm in the piezo group (p < 0.01). Intraoperative handling of the piezosurgery device was judged superior to the oscillating saw, due to soft tissue conservation and favourable cutting properties. Duration of the surgery did not differ between the groups. No serious adverse events were recorded in both groups.

Conclusion: The application of piezosurgery in orbital decompression is more suitable than an oscillation saw due to superior cutting properties such as less damage to surrounding soft tissue or a thinner cutting grove.     

Orbital Metastases

BackgroundOrbital metastasis, although uncommon, is a condition optometrists should consider in a patient presenting with proptosis, ptosis, diplopia, or a lid mass with a history of cancer. However, in as many as 19% of cases, patients have no prior or concurrent history of systemic cancer when presenting with ophthalmic symptoms. If suspecting an orbital metastasis, neuroimaging is important, as well as a referral to the patient's primary care provider, oncologist, and ophthalmologist.Case ReportsThree patients with orbital metastasis are discussed. The first was a 55-year-old white man who initially presented with a left ptosis of unclear etiology. Magnetic resonance imaging of his orbits and an orbital biopsy found metastatic esophageal adenocarcinoma. Radiotherapy and chemotherapy were initiated, but the patient died shortly afterward. The second patient was a 49-year-old black man who also presented with a ptosis of the right upper eyelid. An area of the retina appeared elevated; ophthalmic B-scan and computed tomography of the orbits confirmed the presence of a mass, determined to be metastatic lung carcinoma to the right orbit. A course of radiotherapy was initiated, but the patient died 3 days after completing therapy. The last case was a 77-year-old white man with a history of metastasis to the left orbit from non-Hodgkin's lymphoma. On examination, he had proptosis of the left eye, initially thought to be caused by a recurrence of the metastasis. However, a computed tomography scan showed a new meningioma in the same orbit, and treatment was started. The proptosis improved, and the patient continues to be followed up regularly.ConclusionsAny patient with proptosis and/or ptosis with a history of cancer should be evaluated for orbital metastasis. Optometrists should keep in mind that an orbital metastasis may represent the initial manifestation of undiagnosed systemic cancer. Prognosis can be poor, and thus treatment is sometimes palliative in nature, intending to slow the progression of the disease instead of providing a cure.     

Orbital Adherence with Titanium Mesh Floor Implants: A Review of 10 Cases

Abstract

Background: Multiple materials have been used in the repair of orbital floor fractures. We report 10 cases of complications relating to the use of titanium mesh orbital floor implants.

Method: A retrospective review of 10 cases in 2 centres in New Zealand. Patients presented with diplopia or eyelid retraction following repair of an orbital floor fracture with titanium mesh implants.

Results: Ten patients (7 male, 3 female) aged between 15–78 years old (mean 39 years) presented with significant restriction of eye movement and/or eyelid retraction following repair of an orbital floor fracture with a titanium mesh implant. Seven patients presented with restriction of eye movement alone. Three patients had lower lid retraction in addition to restriction of eye movement. One patient presented with epiphora following erosion of the implant through the nasolacrimal duct. Seven patients underwent surgical removal of the implant with all patients showing improvement of extraocular movement post-operatively. Three cases did not undergo implant removal with one case showing mild improvement over 9 months, and 2 cases showing no improvement. The mean interval between the initial surgery and removal of the implant was 7.1 months.

Discussion: In our series, 7 cases required explantation of the original titanium implant. In these cases a vigorous fibrotic reaction had taken place between the orbital contents and the titanium mesh implant. We postulate that the fibrous reaction between the implant and the orbital contents caused the eye movement restriction and the lid retraction. Implant materials used in orbital floor fracture surgery should be inert with a flat profile rather than a mesh to prevent adhesions through the mesh that may cause cicatricial eye movement restriction and eyelid retraction.     

Orbital pseudotumor after an upper respiratory infection: a comprehensive review

BackgroundIdiopathic orbital inflammation (IOI), also known as orbital pseudotumor, is a nonspecific orbital inflammation of unknown etiology. IOI can clinically mimic many other orbital pathologies, some of which can be life-threatening, as in the case of orbital cellulitis. Thus, it is imperative for the clinician to emergently arrive at the correct diagnosis. In many cases, however, IOI presents as a clinical and therapeutic challenge, and conclusive diagnosis is only confirmed after all other etiologies have been ruled out.Case ReportA 63-year-old man presented urgently with a red, proptotic, painful eye. After history, blood tests, radiologic testing, and ruling out other emergent etiologies, such as orbital cellulitis, the patient was placed on oral steroids. He responded immediately to the steroid treatment. However, the patient did have a recurrence. With the second IOI episode, the patient noted, as previously, a preceding upper respiratory infection. This case may possibly show an association between an infectious trigger leading to a nonspecific aberrant immune response in the orbit.ConclusionIOI is a difficult condition to diagnose and treat. After ruling out other orbital pathologies, it is appropriate to begin treatment with oral steroids. In this case report, the patient noted an upper respiratory infection before each episode of IOI. Although there is no proof of cause, there is a strong case for the consideration of a viral respiratory infection leading to IOI.     

Orbital optic nerve gliomas in children with neurofibromatosis type 1

PURPOSE: To describe the clinical course and treatment of symptomatic orbital optic nerve gliomas in children with neurofibromatosis type-1 (NF-1). METHODS: A retrospective review of the records of patients with NF-1 and symptomatic orbital optic nerve gliomas seen in a large multidisciplinary NF-1 clinic of a tertiary care children's hospital. The main outcome measures included presenting symptoms and signs, ophthalmologic examination at diagnosis, the presence of progressive disease following diagnosis, type of therapy, and the reasons therapy was instituted. RESULTS: Twelve patients with symptomatic orbital optic nerve gliomas, all of which led to proptosis (eight girls, four boys), were identified. The mean age of diagnosis of NF-1 was 20 months; the mean age of diagnosis of the orbital optic nerve glioma was 26 months. At the time of diagnosis of the tumor, 10 of 12 patients (83%) had decreased visual acuity in the affected eye. Three patients underwent optic nerve resection; eight received chemotherapy, and one was observed without therapy. Of the eight children who received chemotherapy, progressive disease prior to treatment could be documented in only three; none of these eight children had a reproducible improvement in vision following chemotherapy. There was no demonstrable improvement in vision in any treated patient with NF-1-associated orbital optic nerve gliomas. CONCLUSIONS: Although not definitively proven, our data and previous studies suggest that NF-1-associated orbital optic nerve gliomas should not be treated unless there is clear evidence of either ophthalmologic or radiographic progression. Surgical excision of tumors which have led to proptotic eyes without functional vision should be reserved for cosmetic purposes or to treat complications of exposed globes.