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Öğe The Association of Alagille Syndrome and Craniosynostosis(Elsevier Science Inc, 2013) Yilmaz, Sanem; Turhan, Tuncer; Mutluer, Saffet; Aydogdu, SemaAlagille syndrome is associated with various ocular abnormalities, including pseudopapilledema or optic disk edema due to increased intracranial pressure. Several mechanisms have been proposed to explain the mechanism of intracranial hypertension in Alagille syndrome. Craniosynostosis is an unusual but significant cause of increased intracranial hypertension in Alagille syndrome. It has recently been demonstrated in animal models that Jaggedl gene in which mutations are responsible for Alagille syndrome may also take part in cranial suture formation. We report a child with Alagille syndrome and craniosynostosis who presented with pruritus, elevated liver enzymes, and suspected increased intracranial pressure. (C) 2013 Elsevier Inc. All rights reserved.Öğe The Association of Alagille Syndrome and Craniosynostosis(Elsevier Science Inc, 2013) Yilmaz, Sanem; Turhan, Tuncer; Mutluer, Saffet; Aydogdu, SemaAlagille syndrome is associated with various ocular abnormalities, including pseudopapilledema or optic disk edema due to increased intracranial pressure. Several mechanisms have been proposed to explain the mechanism of intracranial hypertension in Alagille syndrome. Craniosynostosis is an unusual but significant cause of increased intracranial hypertension in Alagille syndrome. It has recently been demonstrated in animal models that Jaggedl gene in which mutations are responsible for Alagille syndrome may also take part in cranial suture formation. We report a child with Alagille syndrome and craniosynostosis who presented with pruritus, elevated liver enzymes, and suspected increased intracranial pressure. (C) 2013 Elsevier Inc. All rights reserved.Öğe Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases(Springer, 2009) Ertan, Yesim; Sezak, Murat; Turhan, Tuncer; Kantar, Mehmet; Ersahin, Yusuf; Mutluer, Saffet; Vergin, Canan; Oniz, Haldun; Akalin, TanerAtypical teratoid/rhabdoid tumor (AT/RT) is a rare aggressive infantile neoplasm of uncertain origin. This study was performed to assess the clinicopathologic and immunohistochemical features of four AT/RT cases. Two cases were male and two were female, and their ages ranged from 8 to 103 months. Tumors were located in the cerebellum (two cases), frontoparietal lobe (one case), and third ventricle (one case). Histopathologically, the tumors were composed of rhabdoid cells and undifferentiated small cells mixed with epithelial or mesenchymal components. However, one of the tumors was composed predominantly of a mesenchymal component mimicking a sarcoma. Immunohistochemically, vimentin (4/4), epithelial membrane antigen (4/4), cytokeratin (3/4), smooth muscle actin (4/4), glial fibrillary acidic protein (4/4), S-100 (4/4), and synaptophysin (1/4) were positive in varying proportions, while desmin and INI-1 were negative in all the cases. All of the patients died within a mean of 14 months due to tumor progression despite the chemotherapy. Only one of our patients lived for 40 months after the diagnosis. In conclusion, AT/RTs are aggressive tumors. They can occur in a variety of locations, such as the third ventricle. Morphologically, a large spectrum can be seen, like predominantly sarcoma in appearance, but immunohistochemistry is helpful in the correct diagnosis.Öğe Atypical teratoid/rhabdoid tumour of the central nervous system: Histopathologic and immunohistochemical features of 4 cases(Springer, 2007) Ertan, Yesim; Sezak, Murat; Ersahin, Yusuf; Mutluer, Saffet; Akalin, TanerÖğe Case report: Melanotic neuroectodermal tumor of infancy(Springer, 2007) Sezak, Murat; Doganavsargil, Basak; Ertan, Yesim; Mutluer, Saffet; Akalin, TanerÖğe Cerebro-Spinal Fluid Shunt Revisions, Importance of the Symptoms and Shunt Structure(Turkish Neurosurgical Soc, 2011) Turhan, Tuncer; Ersahin, Yusuf; Dinc, Muhammet; Mutluer, SaffetAIM: CSF shunt failure is still a frequent problem in children. This prospective study was designed for focusing symptoms and reasons of shunt failure. We also especially focused on the mechanical reasons of shunt failure. MATERIAL and METHODS: We focused on the causes of shunt failures, and the symptoms and signs in patients who were operated for shunt malfunction between January 1, 2001 and December 31, 2005 in the neurosurgery department. All examination and operative data were collected prospectively. Evaluation of these data was with the chi-square and Fisher exact tests. RESULTS: After the evaluation of data on 111 patients and 153 revision procedures, the major symptoms in this group were vomiting (62.16%), somnolence (59.45%) and headache (48.64%). In the majority of the shunt revisions (115 operations, 75.2% of the all 153 procedures), one or more mechanical problems of the shunt systems were identified in surgery. CONCLUSION: Shunt failures in children sometimes appear with very unusual symptoms. Also, probable structural problems of the shunt systems seem very important for shunt failure according to patient characteristics and etiology of the hydrocephalus. A systematic approach including CT, shunt series and abdominal ultrasound is needed to rule out shunt malfunction.Öğe Embriyonel kafa içi basınç artmasının arnold -chiari malformasyonu gelişmesine etkisi(Ege Üniversitesi, 1982) Mutluer, Saffet; Tunçbay, Erdem[Abstract Not Available]Öğe Fenestration methods for Sylvian arachnoid cysts-endoscopy or microsurgery(Springer, 2012) Turhan, Tuncer; Ersahin, Yusuf; Akinturk, Nevhis; Mutluer, SaffetIntroduction Two different approaches to fenestration, endoscopic and microsurgical, are in use for Sylvian arachnoid cysts (SACs), the most frequent among intracranial arachnoid cysts. We presented the clinical data and compared our results, with regard to technique and clinical success, with either microsurgical or neuroendoscopic fenestration of SACs. Materials and methods Twenty-nine patients who subjected to cysto-cisternostomy by the same team, using either of the two methods, were studied retrospectively. Results Thirteen patients underwent microsurgery, and 16 had endoscopic cysto-cisternostomy. No reoperation was needed in either of these groups, i.e., full clinical and radiological success was achieved in both. The complication rate was 23% in the microsurgery patients and 47% in the endoscopic surgery group, with no statistically significant difference. Conclusions No relevant differences between the two methods are seen either in the published data or in our results. Adding our results to the published data, both techniques were reviewed in a discussion of the one that allows a better controlled or safer fenestration. We conclude that endoscopic fenestration of SACs is not superior to microsurgical cyst fenestration, and the latter seems safer.Öğe The history of little things that changed our lives(Springer, 2011) Mutluer, SaffetÖğe Medulloblastoma: clinicopathologic evaluation of 42 pediatric cases(Springer, 2009) Ertan, Yesim; Sezak, Murat; Demirag, Bengu; Kantar, Mehmet; Cetinguel, Nazan; Turhan, Tuncer; Ersahin, Yusuf; Mutluer, Saffet; Anacak, Yavuz; Akalin, TanerThe aim of this study was to assess the prognostic value of MIB-1 and p53 in the pediatric medulloblastoma group. Forty-two pediatric medulloblastoma cases diagnosed in a single institution during the past 10 years were evaluated. Follow-up data were available for 35 patients. The immunoreactivity of MIB-1 ranged from 10% to 95%; p53 immunoreactivity was found in five cases. Of the 35 patients with follow-up, 34 patients received a combination of chemotherapy and radiotherapy, while one received chemotherapy alone. The follow-up period ranged from 5 to 64 months. Of the 35 patients, 21 were alive without any evidence of recurrent disease, three were alive with evidence of recurrent disease and 11 died of disease during follow-up. The mean survival for these 11 patients was 21.9 +/- 10.4 months. Of the 35 cases, 16 had MIB-1 value of 25% or lower and 19 had a value of 26% or more. Of the 16 cases with low MIB-1 value, six died of disease; of the 19 cases with high MIB-1 value, five died of disease. The statistical difference between MIB-1 and prognosis was not significant. Three of the 35 (8.5%) cases were found to be positive with p53 protein; no correlation was observed between p53 immunoreactivity and prognosis. It appears that the MIB-1 value and p53 immunoreactivity have no relation with prognosis in pediatric medulloblastomas. However, it is convenient to support these findings with large series.Öğe Medulloblastoma: Clinicopathologic evaluation of 51 cases.(Springer, 2007) Ertan, Yesim; Sezak, Murat; Demirag, Bengu; Kantar, Mehmet; Cetingul, Nazan; Ersahin, Yusuf; Mutluer, Saffet; Akalin, TanerÖğe Melanotic progonoma of the skull in infancy(Springer, 2008) Kantar, Mehmet; Sezak, Murat; Turhan, Tuncer; Kitis, Omer; Mutluer, Saffet; Demirag, Bengu K.; Aksoylar, Serap; Akalin, Taner; Ersahin, Yusuf; Cetingul, NazanIntroduction Melanotic progonoma or melanotic neuroectodermal tumor is a rare tumor in infancy. This lesion has to be considered in the differential diagnosis of benign or malignant lesions of calvarium. Case report The authors present a case of a 4-month-old infant with left retroauricular mass. The patient had a subcutaneous mass that is fixed to the underlying skull. CT and MRI scans showed left occipitotemporal expansile mass. The tumor was removed by surgery. A tumor, brownish-black in color, was diagnosed as melanotic progonoma. The patient remained symptom-free for the last 2 years after complete surgery. Discussion Extracranial subcutaneous masses involving the skull are uncommon in infants. Benign or malignant lesions may occur as lumps on calvarium. Physical examination and some laboratory findings are helpful in the assessment of patient. Benign or malignant lesions can be differentiated by craniography, CT, or MRI scans, but exact diagnosis of melanotic progonoma is made by histopathology and immunostaining, as was in the presented case. Cranial vault progonomas have a better outcome by complete surgery. The tumors usually do not recur in long-term period.Öğe Pediatrik Nöroşirürji Konsepti: Erişkin Nöroşirürjisinden Farkları. Dünyada ve Türkiye’de Pediatrik Nöroşirürjinin Gelişimi(2023) Mutluer, Saffet; Ünlü, AğahanÇocuk erişkinin küçük bir modeli değildir. Erişkinden anatomi, fizyoloji, psikoloji ve patolojiler açısından belirgin farklılıklar gösterir. Pediatrik nöroşirürji de genel nöroşirürjinin küçük bir modeli değildir. Pediatrik Nöroşirürjiyi erişkin nöroşirürjisinden farklı kılan birçok özellik vardır. Pediatrik nöroşirürji, “çocukların sinir sistemi hastalıklarının cerrahi tedavisinin teori ve sanatı” olarak tarif edilmiştir. Türk Nöroşirürji Derneği Pediatrik Nöroşirürji Öğretim ve Eğitim Grubu tüzüğünde, pediatrik nöroşirürjiyen tanımı, nöroşirürji aktivitesi içinde kendini tamamen veya büyük çoğunlukla çocuk hastaların nöroşirürjikal hastalıklarına vakfetmiş kişi olarak tanımlanmıştır. Bu yazıda, pediatrik nöroşirürjinin erişkin nöroşirürjisinden temel farklılıklarına değinilmiştir. Yazıda, pediatrik nöroşirürji konseptinin yanısıra, dünyada ve Türkiye’de pediatrik nöroşirürjinin tarihçesi ve gelişimi ile ilgili bilgiler de sunulmuştur.
