Left eye enucleation caused by multi-systemic Klebsiella pneumoniae invasive syndrome

Klebsiella pneumoniae is generally considered the most common pathogenic bacterium causing community-acquired pneumonia. In recent years, cases of liver abscess caused by the bacterium and its spread have been reported in Asia and other parts of the world. This clinical symptom of liver abscess caused by hypervirulent K. pneumoniae and its migrating infection is also called invasive K. pneumoniae liver abscess syndrome (IKPLAS). This study explored the clinical characteristics, diagnosis, and treatment of an elderly patient with IKPLAS who experienced multi-organ failure caused by the infection. The treatment of the patient was difficult, and despite our efforts, the invasive infection led to eye enucleation. This paper is expected to improve our understanding and awareness of this disease in the clinic.

Liver Resection for Metastasis of Adenoid Cystic Carcinoma of the Lacrymal Gland : A Case Report and Literature Review

Adenoid cystic carcinoma (ACC) is a rare malignant tumor. The first description of the ACC dates back to 1856 by Billroth. The ACC of the lacrimal glands are very rare. They are known for their locally aggressive character. Liver metastases are almost exceptional. We report the case of a patient with hepatic metastasis ACC. After eye enucleation of the primitive lacrimal gland ACC, the patient received external adjuvant radiotherapy. Three years later, she presented an upper abdominal pain. CT scan and MRI showed a liver tumor and the diagnosis of intrahepatic cholangiocarcinoma was made regarding imaging features. A left hepatectomy was performed; histology concluded to a liver metastasis of ACC. The immediate post-operative course was uneventful and no adjuvant treatment was decided. Thirty four months later, the patient presented with lung metastasis. Liver metastasis of ACC are very rare. Extended liver surgery is recommended especially for isolated liver metastasis with a long free gap between primary tumor and the liver metastasis. Keywords: Adenoid cystic carcinoma, Hepatic metastasis, Lacrymal gland

Retinal Ependymoma: A Rare Extra-Axial Presentation

Introduction/Objective Ependymomas are well-demarcated and slow-growing neuroepithelial neoplasms that comprise 3–9% of primary CNS tumors. The vast majority of ependymomas arise either intracranially, mostly in children, or in the spinal cord and are associated with ependymal lining. Histologic hallmarks are perivascular pseudorosettes, ependymal rosettes and alternating zones of nuclear crowding and nuclear free zones composed of coarse cell processes. In high grade ependymomas increased mitoses, necrosis and nuclear pleomorphism may be seen. Methods We present the case of a 63-year-old woman in with a past medical history of retinopathy of prematurity, glaucoma and right eye enucleation. She presented with a painful blind left eye refractory to medical treatment and subsequently underwent left eye enucleation. Results On histologic examination, an incidental retinal ependymoma was identified. The neoplastic cells were fusiform and had long coarsely fibrillar cell processes. Characteristic periodicity of nuclear crowding and scarcity was observed. In places, neoplastic cell processes extended radially to delicate and sometimes hyalinized blood vessels, forming so-called perivascular pseudorosettes. Definite ependymal rosettes were not recognized in the examined sections. Stigmata of chronicity were found, such as ischemic type necrosis, blood vessels with dystrophic calcifications and foci of ossification replete with fibroadipose tissue in the marrow spaces. The neoplastic cells labeled with antibodies against glial fibrillary acidic protein (GFAP), thus confirming glial lineage in the neoplastic cell. Additionally, there was scattered intracytoplasmic expression of epithelial membrane antigen (EMA) in a dot-like or circular pattern. The latter is well described in ependymomas and often used to support the diagnosis. Conclusion Ependymomas rarely occur at extracranial sites, such as the chest, abdomen and pelvis. We are presenting the fourth case of retinal ependymoma reported in literature. The tumor had classical immunomorphologic findings.

Cause and Treatment of Exophthalmos in Aged Cotton Rats (Sigmodon hispidus)

Aged cotton rats (Sigmodon hispidus) from an established breeding colony displayed signs of spontaneous exophthalmos. Of a total of 118 colony animals that were older than 6 mo of age, 37 (31%) displayed signs of exophthalmos. These rats were clinically healthy and had no other signs of disease. Ophthalmic exams, molecular and microbiologic testing, and histopa- thology were performed to determine the cause of the exophthalmos and to provide appropriate treatment. Environmental monitoring records were also reviewed for vivarium rooms in which the cotton rats were housed. Histopathology findings supported that the exophthalmos in these cotton rats was secondary to retro-orbital thrombosis associated with cardiomyopathy. The exophthalmic eyes were treated by either removal of the affected eye (enucleation) or surgical closure of the eyelids (temporary tarsorraphy). Enucleation of the exophthalmic eye was the best intervention for these aged cotton rats. These findings demonstrate the potential for a high incidence of ocular problems occurring secondary to cardiomyopathy in aged cotton rats. Enucleation as a therapeutic intervention for exophthalmic eyes in aged cotton rats prolongs the morbidity-free time span during which these aged animals can be used experimentally.

Abstract WP20: Intra-Arterial Chemotherapy for Retinoblastoma in Infants<10 kg: 74 Treated Eyes With 222 IAC Sessions

Background: Intra-arterial chemotherapy (IAC) for retinoblastoma (Rb) has dramatically altered the natural history of the disease. The spectacular outcomes associated with a high safety profile have pushed the envelope to offer treatment for patients < 10 kg of weight. Objective: We aim to share our findings and experience in a large series of IAC infusions performed in infants < 10 kg. Methods: A retrospective chart review was performed for patients diagnosed with retinoblastoma and managed with IAC as either primary or secondary treatment. Results: There were 207 Rb tumors of 207 eyes in 197 consecutive patients. Of these, patient weight was 10 kg in 69 (35.7%) and >10 kg in 133 (67.5%) patients. Of the 69 patients weighing < 10 kg, 5 patients had bilateral Rb. Thus, a total of 74 eyes of 69 patients weighing < 10 kg were compared to 133 eyes of 133 patients weighing > 10 kg. Comparison ( < 10 kg vs. >10 kg) revealed the total number of IAC cycles was 222 vs. 436. Periprocedural complications occurred in 2 (0.9%) vs. 2 (0.45%) infusions ( p =0.49). Aborted procedure occurred in 12 (5.4%) vs. 7 (1.6%) infusions ( p =0.005). Enucleation following IAC was required in 14 (18.9) vs. 44 (33%) eyes ( p =0.029). On multivariate analysis, weight < 10 kg was not an independent predictor of complications, procedure failure, or eye enucleation. Conclusions: Our results speak for the safety and efficacy of IAC in patients < 10 kg.

BT-05 A CASE OF GLIOMATOSIS CEREBRI WITH TOTAL SPINAL CORD EXTENSION

INTRODUCTION Although gliomatosis cerebri is no longer a pathological diagnostic name, it is still an important disease state as a gliomatosis cerebri growth pattern. Here we report a case of gliomatosis cerebri originating from the left cerebral hemisphere that developed whole spinal cord. CASE A 10-year-old boy. He has a history of 13q-syndrome and left retinoblastoma. Left eye enucleation and chemotherapy (modified 98A1) have been performed. MRI in July 2017 showed no abnormalities, but in September 2018, he developed epilepsy. MRI revealed a gliomatosis cerebri that spreads extensively in the left cerebral hemisphere. Biopsy revealed anaplastic astrocytoma (MIB-1 LI; 22%, IDH1 / 2; WT, TERT C228T mutation positive) and IMRT (59.4Gy) and temozolomide (Stupp regimen) were performed in December 2018. In June 2019, neck pain developed. Head and neck MRI revealed that the tumor in the head increased lightly, and there was no suspicion of tumor growth in the brainstem, but the tumor progressed to the entire spinal cord. Therefore, radiation therapy was started from the lower brainstem that had not been irradiated last time to the entire spinal cord, and administration of bevacizumab was started. DISCUSSION Based on the single cell origin theory, the left hemisphere tumor and spinal cord tumor should be continuous. Since gliomatosis cerebri is visualized on MRI only after the tumor volume has increased and edema has occurred, it may appear as if there is no tumor in between. The spinal cord MRI was not taken, so it is only speculation, but it seems that tumor cells had probably infiltrated the spinal cord from the beginning, and it seems that it gradually increased because it was not irradiated. Considering the possibility of remote invasion as in this case, it is necessary to consider taking MRI of spinal cord at the first occurrence.

Enucleation Induces Parvalbumin and Glial Fibrillary Acidic Protein, but Not Calbindin D28k Protein Expression in Superior Colliculus of Wistar Rats

Background: It is known that eye enucleation causes various morphological and functional alterations in the central nervous system (CNS). The purpose of this study was to examine the sub-chronic effects of monocular enucleation on the distribution of the calcium binding proteins calbindin D28k (CB) and parvalbumin (PV) as well as the glial fibrillary acidic protein (GFAP) immunoreactivity in the superior colliculus (SC) of Wistar rats.Materials and Methods: Thirty young adult (8 weeks) male Wistar rats from SLC (Shizuoka, Japan), weighing 200-250 grams, were housed in separate cages under controlled conditions with a constant temperature kept in 12:12 light/dark cycle and ad libitum water and food. In this study the rats were divided into two groups, a control and an enucleated groups. The experimental group received unilateral eye enucleation and was allowed 1, 4 or 12 weeks recovery before sacrificed.Results: Unilateral enucleation over a period of 1 week or more caused a decrease in the number CB-immunoreactive (CBIR) neurons. This loss was associated with an increase in GFAP-IR astrocytes in the superficial gray layer and the optic layer of the SC with contralateral side predominance. In addition, the CB-IR neurons illustrated a smaller soma and poor dendritic arborization. Conversely, the GFAP-IR astrocytes were hypertrophied with longer foot processes on the contralateral side of enucleation. Interestingly, the number of PV-IR neurons was elevated for up to 4 weeks in enucleated rats versus shamoperated rats.Conclusion: This study demonstrates the importance of calcium-binding protein homeostasis and reversible glial response for maintaining variability of neuronal function in sub-cortical visual centers following optic nerve deafferentation.Keywords: enucleation, superior colliculus, calbindin D28k, parvalbumin, glial fibrillary acidic protein