Cystic degeneration of the fibrous dysplasia presenting with loss of vision.

Cystic degeneration of the fibrous dysplasia is a very rare clinical condition and may present with loss of vision when it involved the skull base. A 12-year-old female child presented with an enlargement of the skull. She was diagnosed as large skull base and skull vault tumor. She underwent partial removal of the tumor, and custom-made titanium implant was inserted. The diagnosis was fibrous dysplasia. Two years after the initial diagnosis, she presented with total loss of vision at her right eye. Radiological imaging confirmed the cystic degeneration within the tumor. She re-operated and the cyst fluid was evacuated in association with the removal of cyst wall. The diagnosis was the cystic degeneration of the fibrous dysplasia. Her vision was improved a few days after the surgery. Fibrous dysplasia of the skull base should be closely followed-up in order to prevent severe visual complications.

Unveiling the etiological impact of GST-M1, GST-T1, and P53 genotypic variations on brain carcinogenesis.

BACKGROUND: Functional variants of glutathione-S-transferase (GST)-M1, GST-T1, p53 might modulate brain cancer risk by altering the rate of metabolism and clearance of carcinogens from the brain tissue. In this study, the role of GST-M1, GST-T1, p53 polymorphisms on brain tumor was investigated. METHODS AND RESULTS: Brain tumor tissues of 143 patients were obtained from the Gulhane Training and Research Hospital, Department of Neurosurgery between 2019 and 2020. In the xenobiotic mechanism, the null allele frequency in the GST-T1, GST-M1 gene regions of Phase II enzymes by qPCR method were investigated. Single nucleotide polymorphism encoding Arg/Pro conversion in the p53 gene region was analyzed in 120 cases by sequence analysis method. The data were analyzed statistically with patient's demographic and clinical data. GST-M1, GST-T1, p53 genotypes of the patient group were determined. The most frequent genotype was null genotype (0/0) for GST-M1 (χ2 = 39.756, p < 0.001). GST-M1 genotype frequencies were 30.8%, 23.1%, 44.3% for 1/1, 1/0, 0/0, respectively. The most frequent genotype was GST-T1 1/1 following by GST-T1 1/0 (χ2 = 0.335, p = 0.846). GST-T1 genotype frequencies were 64.3%, 30.8%, 4.9% for 1/1, 1/0, 0/0, respectively. GST-M1 null genotype might be associated with the development of brain tumors. Genotype distribution obtained in p53 exon 4 codon 72; Arg/Arg was determined as 31 (25.8%), Arg/Pro 70 (58.3%), and Pro/Pro 19 (15.8%) in the case group, while there were 18 (38.3%), 23 (48.9%), and 6 (12.8%) respectively in the control group. However, the genotype distribution of p53 exon 4 codon 72 among tumorous tissue did not significantly vary from healthy control tissues (χ²=2.536, p = 0.281). CONCLUSION: The null allele frequency encountered in the GST-M1, GST-T1 gene regions is consistent with the rates in the gene pool called Caucasian in the literature. GST-M1 gene polymorphism may play a crucial role in brain carcinogenesis in Turkish patients. This study based on clinical data is thought to help to understand the important epidemiological features of brain tumors.

Endoscopic endonasal approach to the cavernous sinus Epstein-Barr virus-positive B cell non-Hodgkin lymphoma in a child: case report.

Cavernous sinus (CS) lymphoma without paranasal sinuses involvement is extremely rare in pediatric population and remains a diagnostic challenge due to its similarity to other tumors located in this area. An 8-year-old boy presented with a 6-day history of gradually developing ptosis in the right eyelid. After admission, his symptoms progressed within 24 h to include right-sided ophthalmoplegia consisting of oculomotor and abducens nerve palsies. Endoscopic endonasal approach (EEA) was performed urgently to decompress the CS and to obtain a diagnosis. The postoperative course was uneventful, and there was no complication related to the surgical approach. No immunodeficiency was identified. The histopathological diagnosis was an Epstein-Barr virus (EBV)-positive high-grade mature B cell non-Hodgkin lymphoma. He was initiated chemotherapy according to COG ANHL01P1 protocol. Two months after surgery, the third and sixth nerve palsies had resolved completely. Currently, he is well and has no clinical or radiological recurrence. This is the first pediatric case with EBV-positive CS lymphoma that underwent EEA for the diagnosis and decompression. In the pediatric population, EEA enables minimally invasive access to the CS and can play an alternative role in the management of CS lesions, either through biopsy or debulking.

Direct Stimulation of Neural Placode and Nerve Roots in Open Myelomeningocele: The Efficacy of Neural Tube Reconstruction.

INTRODUCTION: Intraoperative electrophysiological studies are increasingly used in spinal surgery. However, its use in myelomeningocele (MMC) surgery is still not widespread. The aim of this study was to present our experience in neural placode (NP) and nerve root stimulations in newborns with open MMC. METHODS: Eight newborns underwent surgical treatment for thoracolumbar and lumbosacral MMCs. Intraoperative neuromonitoring including free-running electromyography and stimulation of NP, nerve roots, and spinal cord were performed in all cases. Stimulation sites and intensities and distal response's amplitudes and latencies were recorded. RESULTS: Five patients had thoracolumbar and 3 patients had lumbosacral MMC. Two patients had no movements at the lower extremities while the other had some movements. No response on the lower extremities was obtained in only 1 patient. Responses from the nerve root stimulations were more robust and significant than the placode stimulations. CONCLUSIONS: It is clear that the NP and nerve roots originating from the placode are mostly functional and should be preserved during the surgery. Intraoperative neuromonitoring and direct stimulation should be performed during the MMC repair in order to obtain a better neurological outcome.

Port-a-cath and ventriculoatrial shunt at the same atrium: technical note.

Hydrocephalus is a rare complication of brain involving acute lymphoblastic leukemia (ALL). The standard treatment is ventriculoperitoneal (VP) shunting, while ventriculoatrial (VA) shunting is the second option in a case of VP shunt failure in young children. But the presence of port catheter at the right atrium restricts and makes a VA shunt difficult to place in the same atrium. We presented a 4-year-old boy who had the diagnoses of ALL and underwent chemotherapy through a port-a-cath. He also had hydrocephalus due to the brain invasion of the ALL. He firstly underwent VP shunting for the treatment of hydrocephalus, but it failed due to an intraabdominal cyst. Then, he underwent VA shunting through the left internal jugular vein. This is the first case in the literature showing both catheters in the right atrium.

Intracranial infections: lessons learned from 52 surgically treated cases.

OBJECTIVE: Intracranial infections are serious and life-threatening health problems. They may present as subdural empyemas or intracerebral abscesses. Surgical drainage and subsequent antibiotic treatment is the main technique for a satisfactory clinical outcome. The aims of this study were to present a 10-year intracranial infection series and discuss the surgical characteristics in the light of literature. METHODS: Fifty-two patients with intracranial infection underwent surgical treatment between 2008 and 2018. Eleven patients were female and 41 patients were male. The mean age was 40.46 years (range 10-75 years). Eighteen patients had intracerebral abscesses, and 34 had subdural empyemas. All patients underwent surgical treatment as well as an antibiotic regimen. RESULTS: No etiological agent was isolated in 29 (56%) cases. Bacterial agents were detected in 20 cases, while fungi were observed in 3 cases. Staphylococci species were the most common agents and were isolated in 8 (15%) cases. Endoscopic aspiration was performed in 3 cases, while surgical drainage and capsule resection via craniotomy was performed in 49 cases. An associated intracranial tumor was diagnosed in 2 patients with brain abscesses. Four (8%) patients died despite surgical and medical treatments. CONCLUSIONS: Surgical treatment via craniotomy is an older method, but it is still the best to treat the intracranial infections not only for decompression of the brain but also to attain an accurate diagnosis. The abscess wall should always be histologically examined after surgery to rule out any intracranial tumor.

Glutathione S-Transferases and Cytochrome P450 Enzyme Expression in Patients with Intracranial Tumors: Preliminary Report of 55 Patients.

OBJECTIVE: Intracranial tumors are one of the most frightening and difficult-to-treat tumor types. In addition to surgery, protocols such as chemotherapy and radiotherapy also take place in the treatment. Glutathione S-transferase (GST) and cytochrome P450 (CYP) enzymes are prominent drug-metabolizing enzymes in the human body. The aim of this study is to show the expression of GSTP1, GSTM1, CYP1A1, and CYP1B1 in different types of brain tumors and compare our results with those in the literature. SUBJECTS AND METHODS: The expression of GSTP1, GSTM1, CYP1A1, and CYP1B1 was analyzed using immunostaining in 55 patients with intracranial tumors in 2016-2017. For GST and CYP expression in normal brain tissue, samples of a portion of surrounding normal brain tissue as well as a matched far neighbor of tumor tissue were used. The demographic features of the patients were documented and the expression results compared. RESULTS: The mean age of the patients was 46.72 years; 29 patients were female and 26 were male. Fifty-seven specimens were obtained from 55 patients. Among them, meningioma was diagnosed in 12, metastases in 12, glioblastoma in 9, and pituitary adenoma in 5. The highest GSTP1, GSTM1, and CYP-1A1 expressions were observed in pituitary adenomas. The lowest GSTP1 expression was detected in glioblastomas and the lowest CYP1B1 expression in pituitary adenomas. CONCLUSION: GSTP1 and CYP expression is increased in intracranial tumors. These results should be confirmed with a larger series and different enzyme subtypes.

Limited Dorsal Myeloschisis with and without Type I Split Cord Malformation: Report of 3 Cases and Surgical Nuances.

Limited dorsal myeloschisis (LDM) is a rare form of spina bifida which is characterized by a fibroneural stalk between the inner part of the skin and the spinal cord. It may be associated with split cord malformation (SCM). Diagnosis and management of this complex malformation is challenging. We presented 3 different cases of LDM. Two of them were associated with Type I SCM and the other had no associated malformation. All of them were evaluated radiologically just after the birth and underwent surgical treatment under intraoperative neuromonitoring. They discharged without any complication. Newborns with spinal cystic lesions should be carefully evaluated for spinal malformations after the birth and treated surgically as soon as possible in order to prevent neurological and urological complications secondary to tethered cord syndrome. Surgical technique in LDM-SCM patients is quite different than the patients with solitary LDM.

Expression of Sigma-Class Glutathione-S-Transferase in Fetal and Pediatric Filum Terminale Samples: A Comparative Study.

Background and objectives: The pathophysiology of tethered cord syndrome (TCS) in children is not well elucidated. An inelastic filum terminale (FT) is the main factor underlying the stretching of the spinal cord in TCS. Our study aimed to investigate the expression of glutathione-S-transferase (GST) in children and fetal FT samples in order to understand the relationship between this enzyme expression and the development of TCS. Materials and Methods: FT samples were obtained from ten children with TCS (Group 1) and histological and immunohistochemical examinations were performed. For comparison, FT samples from fifteen normal human fetuses (Group 2) were also analyzed using the same techniques. Statistical comparison was made using a Chi-square test. Results: Positive GST-sigma expression was detected in eight (80%) of 10 samples in Group 1. The positive GST-sigma expression was less frequent in nine (60%) of 15 samples from Group 2. No statistically significant difference was detected between the two groups (p = 0.197). Conclusions: Decreased FT elasticity in TCS may be associated with increased GST expression in FT. More prospective studies are needed to clarify the mechanism of the GST-TCS relationship in children.

Relationship between individual payload weight and spondylolysis incidence in Turkish land forces.

OBJECTIVEThe aim of this study was to investigate the relationship between lumbar spondylolysis and payload weight between different combat units of Turkish land forces (TLF).METHODThe authors reviewed clinical and radiological data of the military personnel with low-back pain (LBP) admitted to their clinic between July 2017 and July 2018. Age, BMI, average payload weight, and military service unit were recorded. CT scans were evaluated for pars interarticularis fractures and spondylolisthesis, whereas MRI studies were evaluated for spondylolisthesis, Modic-type endplate changes, or signal loss on T2-weighted images compatible with disc degeneration.RESULTFollowing exclusion, a total of 642 all-male military personnel were included. Of these personnel, 122 were commandos, 435 were infantry, and 85 were serving in the artillery units. Bilateral pars interarticularis fracture was noted in 42 commandos (34.42%) and 2 infantrymen (0.45%). There was no spondylolysis in the artillery units. There was no multiple-level spondylolysis and the most common level of spondylolysis was L5. Commandos had a significantly higher incidence of spondylolysis and more average payload weight (p < 0.001). Twelve patients (27.2%) with spondylolysis had accompanying MRI pathologies at the same level, whereas 32 patients (72.7%) had no accompanying MRI pathologies.CONCLUSIONSIncreased payload weight in military personnel is associated with spondylolysis, and commandos in the TLF have significantly heavier payloads, which causes an increased rate of spondylolysis compared to other units. Additionally, spondylolysis without adjacent-level changes on MRI could be undiagnosed. LBP in active military personnel who have a history of carrying heavy payloads should be evaluated extensively with both MRI and CT scans.