Surgical Considerations in Addressing Cholesterol Granuloma of the Petrous Apex through an Infracochlear Approach.

Cholesterol granuloma of the petrous bone is a foreign body giant cell reaction to cholesterol deposits with symptoms including hearing loss, vestibular dysfunction, and cranial nerve deficit as a result of cystic mass compression. Surgical treatment is often difficult to plan due to limited access to the lesion and possible injury to surrounding structures. We report on a case of petrous apex cholesterol granuloma drainage through an infracochlear approach. A 27-year-old female patient presented with acute diplopia due to left-sided abducens paralysis. Multislice computed tomography (MSCT) and magnetic resonance (MR) imaging described a 3.5-cm well-marginated lesion in petrous bone apex, compressing the left abducens nerve at the point of entry into the cavernous sinus, corresponding to cholesterol granuloma. The patients was surgically treated through a transcanal infracochlear approach, since preserving the external and middle ear conduction mechanisms was paramount for the patient. The patient was discharged on the second postoperative day and diplopia resolved within 5 days postoperatively. Six months after the surgery, her hearing on the left side is normal, and she remains symptom-free. This case underpins the value of preoperative planning when approaching the petrous apex, an anatomically complex area due to abundance of important neurovascular structures crowded in a narrow and confined region.

Pineal germinoma in a young adult: A case report.

BACKGROUND: Intracranial germinomas (GN) are rare cancers that primarily affect children, making them rarer still in adults. Standard treatment for this neoplasm includes neoadjuvant chemotherapy (NC) followed by radiotherapy (RT) or RT at a higher dose and larger field. These recommendations are based on studies focused mostly on children; it is currently unclear whether this treatment is applicable to adults. CASE: We present a case of a 23-year-old adult male with no underlying pathologies, drug allergies, or family history of cancer, who presented for medical evaluation with blurred vision, diplopia, forgetfulness, and weight loss starting 3-4 months before the evaluation. Clinical examination indicated Parinaud's Syndrome. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a pineal tumor with ependymal dissemination in both lateral ventricles, which was causing obstructive hydrocephalus. The patient had surgery consisting of ventriculostomy, Holter shunt insertion, cisternal ventricular intubation, and cisterna magna anastomosis to improve ventricular drainage. Pathology confirmed pineal germinoma. Cerebrospinal fluid cytology and MRI of the axis were negative. Four cycles of NC were given to the patient (carboplatin, etoposide, and ifosfamide), with reduced dosage. Once a partial volumetric response was confirmed, whole-ventricular radiotherapy (WVR) was initiated with a total tumor bed dose of 45 Gy over 25 sessions in 5 weeks. Optimum clinical results were observed, and no short-term (<90 day) radiation toxicity was observed. The patient was able to resume his normal activities soon after treatment. Follow-ups over 2 years post-surgery indicated continued control of the lesion and absence of symptoms except for mild diplopia. CONCLUSION: Although this is a case report, these data suggest that a reduced NC course and WVR may effectively treat adult GN. This protocol likely decreases the risk of undesirable NC and RT secondary effects, while providing excellent local control; however, using a narrower RT field is not recommended.

Population pharmacokinetic model development and its relationship with adverse events of oxcarbazepine in adult patients with epilepsy.

This study aimed to develop a pharmacokinetic (PK) model of oxcarbazepine (OXC) and analyse the relationship between monohydroxylated derivative (MHD), an active metabolite of OXC, and the adverse events of OXC. We obtained 711 OXC samples from 618 patients with epilepsy who were enrolled in the Epilepsy Registry Cohort of Seoul National University Hospital from February 2011 to January 2014. The plasma PK model was developed using a nonlinear mixed-effect modelling method with NONMEM (ver 7.3). A one-compartment model with a first-order absorption model and proportional residual error adequately described the MHD concentration-time profiles. The only covariate incorporated for CL/F and V/F was body weight. Of the 447 patients analysed, 28 (6.26%) had dose-related adverse events (DRAEs), which were dizziness, somnolence, headache, and diplopia. For DRAE occurrence, the cut-off values of the MHD trough and AUC were 12.27 mg/L (specificity 0.570, sensitivity 0.643) and 698.5 mg h/L (specificity, sensitivity 0.571), respectively. Multivariate analysis showed the sole dizziness symptom was significantly associated with both the MHD trough and the AUC (p = 0.013, p = 0.038, respectively). We newly developed a population PK model using sparse sampling data from patients with epilepsy, and the model better reflects the actual clinical situation.

Intravenous Glucocorticoid Treatment for Korean Graves' Ophthalmopathy Patients.

BACKGROUND: High-dose intravenous steroids are the first-line treatment for patients with moderate-to-severe and active Graves' ophthalmopathy (GO). We aimed to investigate the response rate of methylprednisolone (MPD) treatment among Korean patients with active moderate-to-severe GO and to identify predictive factors of treatment response. METHODS: This is a retrospective observational study. We included 54 active moderate-to-severe GO patients treated with 4.5 g intravenous MPD over 12 weeks between November 2011 and November 2018. Response was defined as an improvement in at least two of five indicators (clinical activity score [CAS], soft-tissue involvement, exophthalmos, diplopia, and visual acuity) at immediate and 3 months after treatment completion. We examined predictive factors for response using logistic regression analysis. RESULTS: Twenty-four (44.4%) and 22 (40.7%) patients showed response at immediate and 3 months after intravenous (IV) steroid treatment. Of the five ophthalmic parameters, all patients in the responsive group (100.0%) showed a decrease in CAS and 90.9% showed less soft tissue involvement after IV steroid treatment. Among variables, the sum of extraocular muscle width was positively (odds ratio [OR], 1.163; 95% confidence interval [CI], 0.973-1.389; P = 0.096) associated with treatment response. While, the OR of age was 0.918 (95% CI, 0.856-0.985; P = 0.017) and thyrotropin binding inhibitory immunoglobulin (TBII) was 0.921 (95% CI, 0.864-0.982; P = 0.012). CONCLUSION: In Korean active moderate-to-severe GO patients, intravenous steroid treatment is not as effective as previously reported. Parameters associated with CAS and soft-tissue involvement were found to be influenced by IV MPD treatment. Extraocular muscle enlargement, younger age and lower TBII are predictive factors for a good steroid treatment response.

Herpes simplex virus type 2 meningitis as a manifestation of Good's syndrome.

Good's syndrome is a primary immunodeficiency phenocopy characterized for thymoma and immunodeficiency. The most frequent clinical presentation is recurrent or opportunistic infections, hematological alterations, and chronic diarrhea. We treated a 66-year-old man who consulted for 5 days of headache and diplopia with right sixth cranial nerve palsy at examination. Patient reported chronic diarrhea and prolonged febrile syndrome accompanied by weight loss of 23 kg in the last year. Exhaustive evaluation revealed Herpes simplex virus (HSV) type 2 meningitis, eosinophilic colitis, and type A thymoma. Severe antibody deficiency (hypogammaglobulinemia) associated with thymoma confirmed the diagnosis of Good's syndrome.

Diplopia: a unique presentation of recurrence of a salivary gland carcinoma.

A 79-year-old man with a history of radical excision of a left submandibular gland carcinoma ex-pleomorphic adenoma presented with a new 2 cm lump in his left submandibular region which proved to be recurrence on surgical excision. During work up for revision surgery he developed a right VI cranial nerve palsy, which was attributed to his microvascular status having had a history of three previous transient ischemic attacks (TIAs). 6 months later, his palsy had not resolved. MRI revealed new soft tissue by the cavernous segment of the internal carotid artery. The exact source of this was unclear as there was no evidence of local recurrence or nodal disease. A repeat MRI scan 16 months later revealed further growth of abnormal tissue in the cavernous sinus and the primary submandibular location, now involving multiple nerves including branches of cranial nerves IV, V, VI, VII and XII making surgical excision impossible.

Metastasis of advanced gastric cancer to the extraocular muscle: a case report.

BACKGROUND: Metastatic tumors in the orbit, especially from gastric cancer, are rare. We present a rare case of extraocular muscle metastasis from gastric cancer and raise consideration of metastasis to extraocular muscle as a differential diagnosis of proptosis/lid swelling in a patient with history of malignancy. CASE PRESENTATION: A 54-year-old Japanese woman presented with proptosis, lid swelling, diplopia, and retro-orbital pain in her left eye, which she had been experiencing for 1 day. She had a medical history of poorly differentiated adenocarcinoma of the stomach, which had metastasized to several organs. A computed tomography scan showed enlargement of the medial rectus muscle in her left eye. She was diagnosed as having gastric cancer metastasis to the medial rectus muscle of her left eye, and received a total of 20 Gy radiation therapy to the orbit, which resulted in resolution of her ocular symptoms. She died 3 months after her initial visit to our ophthalmic department. CONCLUSIONS: Metastasis from malignancy should be considered in the differential diagnosis of a patient presenting with proptosis or lid swelling who has a history of gastric cancer. Radiation therapy of metastases in the orbit may be an effective treatment in such cases.

Endoscopic resection of a huge orbital ethmoidal mucocele masquerading as dacryocystocele.

Paranasal mucoceles are cystic masses lined with epithelium thought to result from chronic obstruction of an impaired sinus ostia. If sufficiency large, they can cause ophthalmological sequelae including diplopia, visual acuity, globe displacement as well as the rhinological symptoms of facial pain and headache. We present the case of a 57-year-old man who presented with a 1-year history of epiphora and right globe prominence with associated diplopia. Imaging demonstrated a mass located within the medial aspect of the orbit, closely associated to the lamina papyracea and nasolacrimal duct consistent with a dacryocystocele. An alternate diagnosis of an ethmoidal mucocele was considered preoperatively following rhinologist opinion. Complete endoscopic resection of the cyst was undertaken. Histopathology confirmed diagnosis of an ethmoidal mucocele. Our report highlights mucocele should be considered in patients with chronic symptoms secondary to a mass situated in the nasolacrimal duct without radiological orbital bone destruction.

Diplopia With Dural Fibrotic Thickening.

Idiopathic hypertrophic pachymeningitis (HP) is a rare disorder of diffuse thickening of the cranial or spinal dura mater without an identifiable cause. Most common in adult males, idiopathic HP typically presents with headache with or without varied associated focal neurologic deficits and findings of dural enhancement on magnetic resonance imaging in a linear, nodular, or combined pattern. As it is felt to be an autoimmune disorder, treatment with high-dose corticosteroids is typically recommended, and without intervention, the course is usually progressive. The disease can commonly progress with a relapsing remitting course requiring other immune modulators such as methotrexate, azathioprine, or cyclophosphamide for control. Here, we describe a unique case of idiopathic HP as it presented in a pediatric patient and resolved without immunomodulatory therapy.

Measurement of Medial Wall Bowing and Clinical Associations in Thyroid Eye Disease.

PURPOSE: To propose and validate a measure of medial wall bowing in thyroid eye disease (TED) and to assess the clinical correlates of bowing in TED. METHODS: In this cross-sectional cohort study, all patients affected with TED seen by a single specialist over a 2-year period were screened for study entry. Eligible participants were adults with clinical evidence of TED and either CT or MRI of the orbits. Exclusion criteria included prior history of decompression surgery and/or medical or other ophthalmic conditions that could alter the orbital anatomy. The primary outcome measure was prevalence of medial wall bowing. Secondary outcomes included the associations between medial wall bowing and exophthalmometry, diplopia, rectus muscle restriction, dysthyroid optic neuropathy (DON), strabismus, and quality of life. Medial wall bowing was defined as medial divergence of the medial wall from a straight line drawn between the anterior lacrimal crest and the middle of the optic canal and measured radiographically using axial images of the orbits. Volumetric analysis of bowing was performed on a sample of orbits with and without bowing. Volumetric and axial single slice measurements were compared. Bivariate statistics were performed. RESULTS: The final sample included 112 orbital images from 56 patients with TED. Medial wall bowing was found to be evident in 11.6% (n = 13/112) of orbits. Medial wall bowing was significantly associated with greater Hertel measurements, horizontal and vertical muscle restriction, ocular surface symptoms and Clinical Activity Score, as well as lower quality of life scores. Patients with demonstrable medial wall bowing were more likely to be affected by optic neuropathy, diplopia (Gorman score ≥ 1), strabismus, or horizontal muscle restriction. Analysis of the volume for medial wall bowing demonstrated that the height of the arc at the peak of bowing on an axial image of a 2-dimensional CT scan correlated highly with the total volume of bowing (r = 0.90, p < 0.001). CONCLUSIONS: The identification of medial wall bowing on CT or MRI is associated with clinical measures of disease severity, including diplopia and DON.

Preoperative evaluation and surgical technique of functional and cosmetic aspects in zygomatic complex fracture patients.

The zygomatico-maxillary complex functions as the principle buttress of the face and is the cornerstone to an individual’s aesthetic appearance. Its fracture not only creates cosmetic deformities owing to its position and facial contour, but can also cause disruption of ocular and mandibular functions. The aim of this study was to evaluate the quality, efficacy and impact of internal fixation of zygomatic complex fractures on functional and cosmetic outcomes. A prospective study was carried out on 100 patients who were divided according to the classification and the severity of injury. Subjective evaluation was submitted based on the patient’s perception of signs and symptoms in the preoperative and postoperative periods. Intraoperative and postoperative assessment of bone reduction quality was made according to the type of the fracture and related difficulties; also, the difference between these groups was observed as functional and esthetic outcome. To optimize the treatment of zygomatic bone fractures, a pre-designed questionnaire was used for subjective evaluation of symptoms and treatment outcome. In 70% of cases, ophthalmologic consultation was taken and was most common in type VII fractures (100% cases). Neurosensory disturbance was the most common finding (60%), followed by diplopia (56R%), pain upon mouth opening (54%) and malar depression (50%). Out of all possible 400 fracture sites in 100 patients of zygomatic complex fractures, 266 (66.5%) fractures were detected by clinical examination, in contrast to 330 (82.5%) on radiological examination, which were highest at zygomatic-maxillary buttress (93%) followed by infraorbital rim (91%) and almost equal among fronto-zygomatic site (72%) and zygomatic arch (74%). The scores from the questionnaire for annoyance were significantly higher for paraesthesia (23%) than for trismus (10%), pain (8.5%), or deformity (8.25%). Residual deformity and pain significantly influenced the total satisfaction. Conclusively, there are many treatment modalities available for zygomatic complex fractures, and the preferred methods should be selected on the basis of fracture type, fracture severity, pre-operative signs and symptoms. Regarding the requirements of fracture site exposure and actual fixation, one priority should be to minimize postoperative complications, morbidity and residual deformities.

Pediatric orbital wall fractures: Prognostic factors of diplopia and ocular motility limitation.

OBJECTIVES: To investigate the factors affecting recovery of diplopia and limited ocular motility in pediatric patients who underwent surgery for orbital wall fracture. DESIGN: Retrospective observational case series. METHODS: In this retrospective observational case series, 150 pediatric patients (1-18 years old) who were diagnosed with orbital medial wall or floor fracture and underwent corrective surgery between 2004 and 2016 at Seoul National University Bundang Hospital were included. The medical records of patients with orbital medial wall or floor fracture were reviewed, including sex, age, diplopia, ocular motility, preoperative computed tomographic finding, and surgical outcomes. Factors affecting recovery of diplopia and ocular motility limitation were analyzed. RESULTS: Of the 150 patients (134 boys; mean age, 14.4 years) who underwent corrective surgery for orbital wall fracture, preoperative binocular diplopia was found in 76 (50.7%) patients and limited ocular motility in 81 (54.0%). Presence of muscle incarceration or severe supraduction limitation delayed the recovery of diplopia. In case of ocular motility limitation, presence of muscle incarceration and retrobulbar hemorrhage were related with the delayed resolution. Multivariate analysis revealed supraduction limitation (Hazard ratio [HR] = 1.74, 95% confidence interval [CI] = 1.19-2.55), larger horizontal orbital floor defects (HR = 1.22, 95% CI = 1.07-1.38), and shorter time interval to first visit (HR = 0.73) as negative prognostic factors for the recovery of diplopia. In addition, muscle incarceration (HR = 3.53, 95% CI = 1.54-8.07) and retrobulbar hemorrhage (HR = 3.77, 95% CI = 1.45-9.82) were found as negative prognostic factors for the recovery of motility limitation. CONCLUSIONS: Presence of muscle incarceration and retrobulbar hemorrhage, horizontal length of floor fracture, supraduction limitation, and time interval from trauma to first visit were correlated with the surgical outcomes in pediatric orbital wall fracture patients. These results strengthen that the soft tissue damage associated with bony fracture affects the orbital functional unit. When managing children with orbital wall fracture, meticulous physical examination and thorough preoperative computed tomography based evaluation will help physicians to identify damage of orbital functional unit.

Diplopia as isolated presentation of varicella zoster central nervous system reactivation.

Here, we report a patient who developed diplopia secondary to a right cranial nerve III and IV palsy, as well as fever and headache. Cerebrospinal fluid analysis (CSF) showed high varicella-zoster virus (VZV)-DNA viral load (>300,000,000 copies/ml). VZV antibodies in CSF was ≥1:16. Diagnosis of neurological reactivation of VZV infection was made without the presence of characteristic vesicular rash. Quantitative real-time PCR for VZV and intrathecal dosage of VZV IgM and IgG should be performed in cases suspected for viral encephalitis and also in all patients with not otherwise attributable cranial nerve lesions.

Accommodative spasm might influence surgical planning and outcomes in acute acquired distance esotropia in myopia.

INTRODUCTION: Acute acquired distance esotropia (AADE) is a poorly understood moderate-angle strabismus, affecting young adult myopes and determining bothersome diplopia. Symptoms can be intermittent in the early stages, becoming constant in long-lasting disease. Symptomatic therapy includes prism correction, while surgery is the only curative treatment. However, the latter is affected by high rate of symptoms recurrence with the frequent need for reoperation. HYPOTHESIS: We hypothesize that AADE could be caused by the increase of the accommodative demand, often secondary to a myopic overcorrection. This condition could determine an increase in induced hyperopia at near, dominated by an excess of accommodation and therefore of convergence. The latter cannot be relaxed at distance and diplopia develops. We speculate that early-stage AADE could be successfully treated by cycloplegic eye drops slowly tapered within three months. On the other hand, surgery remains the only option in long-lasting AADE. In these cases, we propose a new pre-operative assessment of esotropia by asking the patient to fix alternatively a stimulus at near and at distance in order to stimulate the accommodative convergence. This technique allows to unmask the total amount of the angle of deviation and to plan a wider bilateral medial rectus muscle recession avoiding long-term residual esotropia. DISCUSSION: Currently, AADE curative therapy is surgical regardless of onset time but it is usually affected by poor outcomes. If our hypothesis was to be confirmed, pharmacological treatment could solve early-stage AADE, avoiding any surgery. Furthermore, a wide bilateral medial rectus muscle recession, quantified on the basis of the above mentioned test for measuring the total amount of the strabismus angle, could improve outcomes eliminating the need for reoperation in long-lasting AADE.

Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression.

Progressive multifocal leukoencephalopathy (PML) is a viral demyelinating disease due to the reactivation of the JC virus (JCV), which usually occurs in the context of immunosuppression in HIV infection, malignancy, or in patients on disease modifying therapy for autoimmune diseases, such as multiple sclerosis (MS) and Crohn's disease. Notably, there is growing recognition that PML can occur in patients with transient immune dysfunction. Here, we present a case of a 55-year-old man without history of immunosuppression or evidence of ICL who was diagnosed with PML on brain biopsy. We will discuss the potential etiologies of mild and transient immunosuppression that can lead to PML with non-apparent immunosuppression.