Cranium metastasis of HPV positive oropharyngeal squamous cell carcinoma

Head and neck squamous cell carcinomas rarely cause cranial metastases, but HPV related subgroup is known for unusual metastasis sites on the body. Despite the good overall treatment responses in this group, intracranial metastases are always accepted as a sign of rapid deterioration. We present a case of cranium metastasis of HPV related oropharyngeal squamous cell carcinoma, which had been very well responded to the treatment for years but deteriorates in a short period after the cranium metastasis

Pituitary adenoma in monozigotic twins with Cri du Chat syndome: A rare case report

Pituitary adenomas are rare tumours of pediatric population. In etiology, genetic factors are more common than they are in adults. Because of the rarity of the cases, there are only a few large case studies in the literature. Pituitary tumours in children are often related with syndromes like MEN type 1, Carney Complex and Mc Cune Albright, but there is no case in the literature associated with Cri Du Chat syndrome. Statisticlly, it has been reported that, pediatric tumours occur more often in twins, in the pediatric population. Main treatment for prolactinomas is medical intervention with dopamine agonists, as in adults. Surgery is prefered when the tumour is resistant to medical treatment or shows mass effects around sellae. In that situation, as in adults, both transcranial and transsphenoidal approach is possible

Statistical analysis of associated vertebra and costal anomalies in spina bifida patients

Objective: Spina bifida is one of the most severe birth defects and can happen as a result of disrupted primary neurulation. Congenital vertebra and costa anomalies are more frequently seen with spina bifida, and associated anomalies significantly affect the prognosis of affected children. In this study, we aimed to determine the incidence of scoliosis, costal anomalies, and vertebral deformations seen at the time of diagnosis and to statistically evaluate their concomitancies. Methods: Gender and mean ages of the patients were determined. The spina bifida patients were examined for deformation anomalies, butterfly vertebra, hemivertebra, wedge vertebra, costal anomalies and scoliosis. The relationships between these anomalies were evaluated. Results: 94 patients with a mean age of 11,5 months examined. The incidence of scoliosis was 21.8% among female infants and 17.9% among males. Rates of scoliosis with vertebra anomalies (hemivertebra, wedge vertebra) and costal anomalies did not differ significantly (P > 0.05). Wedge vertebra were the most frequent vertebra anomaly type with 38.2% ratio. Costal anomalies were detected in 25.5% of females and 20.5% of male infants. Hemivertebra and wedge vertebra were seen significantly more frequently in this group. Gender distribution did not differ between with and without any vertebra types. Conclusion: Congenital vertebra and costa anomalies are more frequently seen with spina bifida. We believe that these anomalies and relationship with spina bifida may demonstrate differences among different ethnic groups or locations. More detailed multi-centered studies performed on this issue will aid in the determination of etiologies, genetics, and treatment principles of these congenital anomalies

Morphometric analysis of the arteries of Willis Polygon

Objective: Willis polygon forms the basis of the arterial circulation of the cerebrum. Willis polygon is a vascular structure whom variations are not rare. Knowledge of the anatomy and preservation of its integrity is crucial for performing neurovascular surgery and intracranial tumour surgery. Because of the important vascular and neurological structures, approaches to this region are considered extremely risky. One of the main variations in-person basis is the diameter differences of the arteries, which forms Willis polygon, between the left and right hemispheres. About structure and variations, studies of Rhoton and Yasargil had formed the touchstone.Our aim is to contribute to the literature and clinical studies, to be done in the future, by comparing our results with previous studies about variations and morphometric features of Willis polygon.Methods: Arteries of 30 fresh cadaver brains were examined during autopsies in T.C. Ministry of Justice Istanbul Forensic Science Institute. Bilaterally anterior cerebral artery A1 segment lengths, distance between anterior communicating artery-callosomarginal artery outputs, posterior cerebral artery P1 segment lengths were measured using a digital calliper. After dissections and measures, photos of the region were taken and vascular anatomy and variations noted. From every single cerebrum samples were obtained from bilaterally A1, A2, callosomarginal artery, middle cerebral artery, posterior communicant artery, P1 and basilar artery. Samples were fixed by using 10% buffered-formalin. Taken samples were transported to Marmara University Faculty of Medicine, Department of Anatomy Laboratory. Samples were examined and interior diameters were measured under the microscope.Results: Our results with artery diameters and lengths were similar with literature. Different from literature, in anterior cerebral artery A1 segment, posterior cerebral artery P1 segment and posterior communicant artery no aplasia were noted. In 50% of the samples, callosomarginal artery were originate from A2 segment. In one case, we observed left and right pericallosal arteries were joined together at the end of the A2 segment and continued as a single pericallosal artery. We could not find any information about this variation in the literature.Conclusion: Before surgical operations, detailed knowledge of Willis polygon and evaluation of the pre-op cerebral angiography considering possible variations, reduce mortality and morbidity ratios. In addition, because of the role of flow gradients of Willis polygon in aneurysm formation, and in terms of better understanding the collateral circulation which is important in vascular occlusive diseases and vascular surgery, we believe, more anatomic studies about this region needed

Hemimetameric shift in spina bifida: three case reports

WOS: 000398041500023PubMed ID: 27822764Purpose Hemivertebrae is the most frequent reason of congenital scoliosis, and hemimetameric shift is a finding, which is characterized by two hemivertebraes located contralateral, with at least one normal vertebra between them. Embryologically, hemivertebrae is caused by delay in somite movements and as a result, a total vertebral shift occurs because of mismatches in the following segments. Hemimetameric shift accompanying spina bifida is described as extremely rare. There are only two case series of hemimetameric shift in literature but no spina bifida patient was reported in these series. Methods We report three cases of hemimetameric shift with spina bifida with their detailed clinical and radiological evaluations. Results Case 1 is a 3-year-old congenital scoliosis patient with tethered cord. She has mild scoliosis with a very demonstrative hemimetameric shift. Case 3 is an infant with multiplelevel hemivertebrae anomalies and hemimetameric shift who had myelomeningocele closure and ventriculoperitoneal shunt installation neonatally. Case 3 is a 9-year-old male who had operated for myelomeningocele in the neonatal period. He had evident scoliosis with cervicothoracic hemimetameric shift and he is the only patient we operated for prominent scoliosis. Conclusions The present classification of hemimetameric shift was described by Kawakami et al. in 2009. It is based on development of anterior and posterior hemivertebrae segments but we think it is not suitable for patients with posterior fusion defects such as spina bifida patients. Spina bifida patients with scoliosis and hemimetameric shift are a rare but important patient group to evaluate in terms of understanding scoliosis patients with neural tube defects

Shunt revision rates in myelomeningocele patients in the first year of life: a retrospective study of 52 patients

WOS: 000429793400017PubMed ID: 29159427Shunt placement indications are stringent and require confirmation of clinical and radiological evidence of hydrocephalus (HC). The aim of this study was to determine the rate of shunting and discuss the outcome in the first year of life in patients with myelomeningocele (MMC) on the basis of review of the literature. All patients who underwent postnatal repair of MMC at our institution between March 2014 and March 2015 were evaluated. Patients were only included if they underwent both MMC repair and ventriculoperitoneal (VP) shunt insertion at our institution and were followed up for at least 12 months. The mean ages for repair of MMC, MMC levels, timing of VP shunt placement, shunt revisions, and causes of shunt revisions were documented. Fifty-two patients with MMC were included in this study. The average gestational age at birth was 38 weeks. The level of MMC was thoracolumbar in 13 cases, 11 times lumbar, 21 times lumbosacral, and 7 times sacral. Thirty-one patients (59.61%) suffered from hydrocephalus and required placement of a shunt. When we evaluate the lesion levels of patients who require shunting, 13 cases were thoracolumbar, 6 cases were lumbar, and 11 cases were lumbosacral. None of the sacral cases needed VP shunt. Seven patients (13.4%) had shunt revision within the first year of life. The cause of shunt revision was wound problem in one patient (1.9%), underdrainage in two patients (3.8%), infection in three patients (5.7%), and mechanical obstruction in another one patient (1.9%). MMC closure and management of the associated HC are one of the most basic, but never simple, legs of the pediatric neurosurgery around the world. As clinicians and neurosurgeons, we are obligated to analyze recent evidences and evaluate present approaches to achieve optimization in this subject until further technologies or approaches became more advantageous for our patients

Russell - Silver syndrome associated with low conus medullaris

WOS: 000395657900019PubMed ID: 28217167Russell - Silver syndrome is a rare heterogeneous disorder mainly characterized by intrauterine and postnatal growth retardation, craniofacial disproportion, clinodactyly, variation in urogenital development, and skeletal asymmetry. It is rare to come across tethered cord-associated Russell - Silver syndrome. We report a rare case of Russell-Silver syndrome associated with low conus medullaris in a 2-year-old patient with demonstrative phenotype. Magnetic resonance imaging indicated a low conus medullaris at the inferior border of the L3 vertebral body. Urodynamic study revealed detrusor-sphincter dyssynergia and detrusor overactivity. A decision to follow-up the patient was made because of the suspicion of tethered cord syndrome. Even though tethered cord syndrome is not a common finding in Russell-Silver syndrome, it is important to consider tethered cord syndrome to avoid scoliosis and other long-term complications

Foramina parietalia permagna: familial and radiological evaluation of two cases and review of literature

WOS: 000403477000023PubMed ID: 27975139Purpose Foramina parietalia permagna is a variable intramembranous ossification defect of the parietal bones. Foramina parietalia permagna have an autosomal dominant inheritance, and it is showed that mutations in chromosome 5 and 11 are causing this anomaly. Enlarged parietal foramina occurs extremely rare. They are usually asymptomatic, but occasional headache, vomiting, pain over unprotected cerebral cortex, and seizures may be experienced by the patients. In the literature, some associated congenital bony defects, soft tissue pathologies, underlying neuronal deficits, and vascular variations have been described. Methods We report two cases of foramina parietal permagna with their pedigrees and genetic analysis. Results In case 1, cytogenetic analysis revealed a mutation of the ALX4 gene and all of the members of the family diagnosed with FPP. MRI revealed inferior vermian cerebellar hypoplasia. Surgery was not considered. In case 2, cytogenetic analysis could not be obtained because of financial reasons. Cranial MRI revealed hypoplastic right transverse sinus and sigmoid sinus, with a persistent parafalcine sinus. Surgery was not considered. Conclusion Despite of its rarity, genetic background and some important associated anomalies make foramina parietalia permagna more than an uncommon insignificant genetic disorder