C. Pagano Boza, R. Vigo, J. E. Premoli, J. Croxatto & J. Gonzalez Barlatay
Introduction: Primary Apocrine adenocarcinomas (PAA) are very infrequent tumors that are often confused initially with benign lesions. Little is known about this disease and there is still much to be clarified. We present a case of PAA on the eyelid successfully treated with surgery alone and a literature review regarding what is currently described about this disease.
Methods: Noncomparative, retrospective case report of a patient with PAA on the eyelid succesfully treated with surgery alone and a literautre review.
Results: A 91-year-old man with a 2 months lesion on the upper left eyelid was treated with surgery alone with oncological margins of 5mm. The Histopathology diagnosis was a PAA of the eyelid and free margins were obtained. After 12 months of follow-up, the patient does not show any signs of local recurrence or distant metastasis. A review of the literature suggests these tumors are located more frequently in the axilla (50%) and secondly in the head and neck (35%), with similar distribution in the upper (41%) and lower eyelid (45%). The most commonly used treatment is surgical excision, but radiotherapy and chemotherapy have also been used with variable results.
Conclusions: PAA is a very rare and aggressive tumor. Because it is so infrequent, treatments are based on the sporadic cases encountered in the literature. As more cases are reported, more can be elucidated about the characteristics of this tumor, its behavior and best treatment choice and this may allow progress in the understanding and management of this disease.
Rehan Rajput, Amun Sachdev, Nizar Din, Erika Marie Damato & Aidan Murray
Methods: We present a rare case with atypical presenting features of unilateral CPEO with a false positive Acetylcholine Receptor Antibody (AchRA) test resulting in diagnostic delay. We illustrate the unilateral nature of this case and demonstrate the caveats of performing myogenic ptosis correction in such patients. We also discuss the differential diagnosis of false positive AchRA, a test commonly performed in the investigation of ptosis.
Results: A 34-year old female presented with a more than 3-year history of slowly-progressive, unilateral, right-sided restriction in eye movements and ptosis. Clinical examination showed EOM were grossly restricted in the right eye with a ptosis and normal in the left eye. Serum AchRA was positive on serum enzyme-linked immunosorbent assay (ELISA) however, following two months of oral pyridostigmine therapy there were no signs of clinical improvement. The initial serum sample sent was retested for AchRA by radio-immunoassay (RIA) which came back negative. Subsequently a muscle biopsy was requested which showed the presence of ragged red fibres.
Conclusion: Unilateral ptosis and ophthalmoplegia is an unusual presentation for CPEO which characteristically produces bilateral symmetrical motility defects. In addition to Myasthenia Gravis elevated AchRA levels have been reported in other autoimmune conditions such as Primary biliary cirrhosis, Eaton Lambert syndrome and Graves’s ophthalmopathy. We also highlight the superiority of RIA versus ELISA in the detection of AchRA and illustrate the diagnostic challenge of investigating and managing myogenic ptosis in this complex cohort of patients.
Reem Alnabulsi, Ahsen Hussain & Dan DeAngelis
Ipilimumab and Nivolumab are novel monoclonal antibodies that have recently been used successfully for treatment of metastatic melanoma. Ipilimumab is a human monoclonal antibody against Cytotoxic T Lymphocyte Antigen 4 (CTLA4) receptor, which suppresses T-cell proliferation and stimulates an inflammatory response against cancer cells. Nivolumab is an IgG4 monoclonal antibody against the cytotoxic T lymphocyte associated programmed death 1 receptor (PD-1). Ipilimumab and Nivolumab combination treatment has been shown to induce remission and prolong survival in patients with metastatic melanoma. The side effect profile of these medications has not been well studied. One entity of the side effects reported in the literature is immune-related adverse events (irAEs). There have been few case reports where these events were serious and irreversible. In this case report, we describe a fatal and severe diffuse panmyositis that involved the cardiac, respiratory, and extraocular muscles in a patient with metastatic melanoma secondary to combination treatment with Ipilimumab/Nivolumab.
Andrew J. Rong, Jan P. Ulloa-Padilla, Nathan W. Blessing, David T. Tse, Sander R. Dubovy & Catherine J. Choi
Fibromas are benign, well-circumscribed tumors that are characterized as spindle-cell lesions with interlacing fibrous stroma. Here, we describe the clinical presentation, management, and outcome of a patient with an orbital fibroma. To our knowledge, this is the second case report of a biopsy-proven subperiosteal orbital fibroma to date.
Siwei Zhou, Katherine Duncan & S. Tonya Stefko
Silent sinus syndrome was first described as spontaneous enophthalmos and hypoglobus associated with subclinical maxillary sinusitis without prior trauma or surgery. This clinical entity has later been described after trauma in which damage to the ostiomeatal complex leads to atelectasis of the maxillary sinus. We report a case of a 14-year-old boy who presented 4 years after sustaining a non-operative orbital floor fracture with enophthalmos and transient diplopia. Computed tomography (CT) demonstrated enlargement in size of the original orbital floor fracture and bilateral maxillary sinus disease. Bilateral chronic sinusitis suggested an anatomical predisposition to sinusitis unrelated to the prior trauma. The authors propose that, in this case, negative pressure in the maxillary sinus and chronic inflammation led to bone resorption and failure of the orbital fracture to heal. This differs from prior reports of silent sinus syndrome in that there was complete resorption of bone of the orbital floor and no decrease in volume of the maxillary sinus given the open communication of the sinus and the orbit, making this a unique presentation of pseudo-silent sinus syndrome in a pediatric patient.
Kaveh Vahdani, Katherine McVeigh, Rhys Harrison, Mandy Williams & Helen Garrott
Intracranial hypotension (ICH) is characterized by low cerebrospinal fluid pressure, postural headaches, and diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI). A variety of ophthalmoparetic manifestations have been reported in the context of the ICH. The authors describe an unusual case of a 64-year-old woman who presented with rapid onset of headaches, bilateral upper-lid ptosis, and blurring of vision within 4 days after sustaining a trivial head injury. She was noted to have bilateral symmetrical ophthalmoplegia and ptosis-simulating chronic progressive external ophthalmoplegia. MRI revealed characteristic features of ICH. Subsequent autologous epidural patch therapy led to resolution of the headache and imaging findings; however, her ptosis and motility disorder persisted. Despite existing therapeutic measures for ICH, irreversible cranial nerve damage may ensue due to significant cerebral decent or ischemic injury.
Sana Ali Bautista, Edward J. Wladis, Martha Farber, Naomi S. Falk & Paul O. Phelps
Purpose: To report a case in of intravitreal silicone oil migration into the inferior orbit. Silicone oil tamponade is commonly used in retinal detachment repair and extrusion into the orbital tissues is very rare.
Methods: A 70-year-old male with a remote history of repair of a right ruptured globe and retinal detachment surgery presented with progressive right lower eyelid edema. There was a known history of intravitreal silicone oil instillation and removal. An elective right inferior orbitotomy with excisional biopsy was performed.
Results: Histopathology confirmed the presence of silicone oil intermixed with necrotic fat. The patient had excellent cosmetic and functional outcome.
Conclusion: The presence of silicone oil within the orbital fat may be a result of increased intraocular pressure and extrusion through presumably water-tight sclerotomy sites.
Stacy Scofield-Kaplan, James Todaro & Bryan J. Winn
The management of cicatricial ectropion resulting from epidermal growth factor receptor (EGFR) inhibitors is unclear. We describe two cases of bilateral cicatricial ectropion following the use of an EGFR inhibitor who were treated with oral doxycycline, topical ophthalmic steroid and antibiotic ointment to the eyelids, and topical facial steroid cream with lubrication. The first case resolved with discontinuation of panitumumab infusions along with institution of the aforementioned regimen. However, it is unclear whether the resolution was from discontinuation of the infusions or from the instituted regimen. The second case resolved without a dose adjustment of cituximab. This case may provide support for the use of this regimen prior to discontinuation of the offending agent, as there was a successful outcome without alteration of the infusions. Additional cases are necessary to determine if this is a successful means of treating bilateral lower-lid cicatricial ectropion from EGFR inhibitors.
Sonia Huang, Michelle T. Sun, Garry Davis, Jude Fitzgerald, Dinesh Selva & Tim Henderson
A 39-year-old male developed bilateral periorbital oedema and tense orbits in keeping with orbital compartment syndrome (OCS) shortly after presenting to the emergency department for uncontrollable epistaxis. Bilateral lateral canthotomy and inferior cantholysis was performed within 30 minutes of onset, with the left side further decompressed via superior cantholysis. Computed tomography demonstrated bilateral proptosis and optic nerve stretch, but no intraorbital haemorrhage or haematoma. Laboratory findings were consistent with disseminated intravascular coagulation (DIC) and sepsis of unknown origin. The right visual acuity recovered to 6/6 -2 from counting fingers, but the left eye failed to improve beyond light perception. This unique case of OCS is the first associated with DIC which had no evidence of intraorbital haemorrhage.
Rashed N. Alhabshan & Tamer N. Mansour
It is well established and documented that fluoroquinolone use is associated with the development of tendinopathy. However, little is known about the possible effects of this class of antibiotics on the orbit. We present a case of lateral canthal tendon rupture that presented with an acute right lower eyelid ectropion in a young, renal compromised patient in the setting of recent fluoroquinolone use for pneumonia. Eye care clinicians need to be aware of the possible effects of fluoroquinolones on the eyelids.