M. Kemali Baykaner

Intracranial Meningiomas of Childhood and Adolescence

Meningiomas are rare intracranial neoplasms in childhood and adolescence, representing 0.4–4.1% of the pediatric-age tumors and 1.5–1.8% of all intracranial meningiomas. The goal of this study was to determine epidemiology, clinical and radiological features, and long-term outcome of childhood and adolescence meningiomas. Patients operated for intracranial meningiomas of childhood and adolescence between 1983 and 2003 at Gazi University School of Medicine, Department of Neurosurgery, were evaluated retrospectively. This study presents 11 cases (6 male, 5 female), ranging in age from 14 months to 17 years. Age and sex distribution, presenting symptoms, neurological examination results, location of meningiomas, radiological and histopathological findings, and prognosis were reviewed. The results were compared with those reported in the existing literature. Atypical and malignant meningiomas seem to be more common in childhood and adolescence with respect to adult meningiomas. Tumor location, completeness of tumor removal, and pathological grade are the most important prognostic factors.

A comparison of the local effectiveness of mitomycin C, aprotinin, and Adcon-L in experimental peridural fibrosis

BACKGROUND Peridural fibrosis and leptomeningeal adhesion formation are among the common causes of FBSS. Various materials have been used to prevent the compressive effect of postoperative PF on neural structures. We investigated and compared the effects of 3 agents--aprotinin, mitomycin C, and Adcon-L--to PF after lumbar laminectomy in rabbits. METHODS Four groups each including 8 rabbits were formed: Adcon-L, aprotinin, mitomycin C, and control groups. L3 laminectomy was performed on each animal. One of the 3 agents was administered locally to laminectomy areas in each group. All the animals were killed 4 weeks after the surgery. Peridural fibrosis, arachnoidal fibrosis, and dural adhesions were evaluated histologically and graded. The results were compared statistically by using a standard chi2 test. RESULTS There were significant differences in the PF grades among the experimental groups and the control group (P < .05). When the fibroblast density and the inflammatory cell density were evaluated, the grades of the experimental groups were better compared with the grades of the control group, but the difference was not statistically significant (P > .05). CONCLUSION Various materials have been used to prevent the compressive effect of postoperative PF on the neural structures. Aprotinin, mitomycin C, and Adcon-L are effective in preventing PF and dural adhesions in postlaminectomy areas. However, mitomycin C and Adcon-L were more effective than aprotinin in preventing peridural scarring.

Malignant intracerebral giant nerve sheath tumor in a 14-month-old girl with neurofibromatosis type 1: a case report

IntroductionMalignant intracerebral nerve sheath tumor (MINST) is extremely rare and the origin is still unclear. The authors present the clinical, radiological, and pathological features of a malignant intracerebral giant nerve sheath tumor.Case reportA giant tumor in the right frontotemporoparietal lobes causing a midline shift was detected in a 14-month-old girl who presented with developmental delay, vomiting, and lethargy. The physical examination was consistent with neurofibromatosis type 1 (NF-1). Subtotal resection was performed and the histopathological examination revealed the diagnosis of MINST.DiscussionThere are only six cases of malignant intracerebral nerve sheath tumor in the literature. The presented case is the youngest and the occurrence of MINST in a 14-month-old girl may support the hypothesis of multipotent mesenchymal stem cell origin; however, the tumors which arise from multipotent mesenchymal stem cells may be seen in later stages of life. Another important feature of the presented case is the occurrence of MINST in NF-1.ConclusionMINSTs are extremely rare tumors with unknown origin. The location, the degree, and the size of the tumor and the general condition of the patient are prognostic factors in MINSTs, like in other malignant tumors.

Cervical tuberculosis in early childhood

Case historyWe present a case of spinal tuberculosis in a 4-year-old girl that grounds severe vertebral destruction involving four cervical vertebrae and a large abscess with retropharyngeal expansion. Presenting symptoms were neck and right arm pain, torticollis and weakness of the right arm. We drained the abscess and removed the infected bone and reconstructed the cervical spine with a fibular allograft. The neurological deficits were recovered and the torticollis was straightened up.Conclusion We emphasize the value of early diagnosis of spinal tuberculosis and early radical surgery with spinal reconstruction.

Retropharyngeal hematoma secondary to whiplash injury in childhood: a case report

Whiplash Associated Disorders (WAD) has been reported as an adult phenomenon. Whiplash injury has classically been described as a cervical soft tissue hyperextension- flexion injury after a trauma such as a rear end impact car crash, contact sport injuries, blows to the head from a falling object or a punch and shaken baby syndrome and is mostly seen in adults . It is important as it may cause severe disability due to spinal cord injury, decrease work productivity and even retropharyngeal hematoma resulting airway obstruction and mortality due to bleeding amongst deep cervical fascias. We describe a case of retropharyngeal hematoma after whiplash injury in a childhood.

Seven Distinct Coexistent Cranial and Spinal Anomalies

Existence of multiple cranial and spinal anomalies in spina bifida is well known; however, coexistence of seven different severe anomalies is extremely rare. The location of the anomalies, the patient’s age and presentation are other interesting aspects of the presented case. Case Report: A 1-year-old girl with an enlarged head and big, infected lumbosacral myelomeningocele (MMC) was admitted to the emergency department in a comatose state. Further investigations revealed hydrocephalus, Chiari malformation, syringohydromyelia, split cord malformation, dermal sinus tract, lumbosacral MMC, and tethered cord. Unfortunately, the patient died 3 days after admission because of meningitis and sepsis. Discussion: Coexistence of seven different craniospinal anomalies is extremely unusual. Further investigations with magnetic resonance imaging for associated anomalies, early shunt placement and MMC repair are required to prevent this life-threatening condition in spina bifida.

Split cord malformation: experience from a tertiary referral center.

Aim: To present clinical, radiological, and follow-up features of 34 cases with spinal split cord malformation (SCM) treated in a tertiary referral center between April 2000 and March 2012. Methods: A total of 59 patients were treated due to SCM between April 2000 and March 2012 at the Gazi University Faculty of Medicine, Division of Pediatric Neurosurgery. Data for each patient were evaluated retrospectively, and age, sex, clinical findings, radiological findings, complications, and surgical results were recorded. Patients with a follow-up period of less than 6 months, patients harboring an open spinal dysraphism, and patients who had had their index surgery in another clinic were excluded, thus leaving a total of 34 patients for further analysis. Results: There were 19 females and 15 males ranging in age from 2 months to 15 years (mean 5.04 years). In this cohort, skin stigma was the most common reason (76.5%) to seek medical care. Of the cases, 22 (64.7%) had lumbar SCM and 12 (35.3%) had thoracic SCM. There were no cervical SCM. Twenty-one (61.8%) of the cases had type 1 SCM and 13 (38.2%) had Type 2 SCM. Of the patients, 21 (61.8%) had a detectable neurological deficit at initial evaluation. There were no differences between patients with and without a neurological deficit regarding age, sex, type, and level of SCM. Overall evaluation of patients regarding their final neurological status revealed that 16 (47.1%) patients improved, 4 (11.8%) deteriorated, and 14 (41.2%) remained stable. Conclusions: In our opinion, all patients diagnosed with either type of SCM should be surgically treated to prevent further neurological deterioration. The results of this study, together with previously published data, confirm the effectiveness and safety of surgical intervention in SCM.

Effectiveness of skull models and surgical simulation: comparison of outcome between different surgical techniques in patients with isolated brachycephaly

PurposeThe aim of this study was to emphasize the importance of preoperative surgical planning using 3D skull models in craniosynostosis surgery.MethodsBy using 3D polymethyl methacrylate skull models manufactured using 3D tomography images, the authors previously showed that after fronto-parietal osteotomy, instead of fixing the fronto-parietal bone flap without rotation, angled advancement with horizontal osteotomy provides maximum increase in intracranial volume, in a bilateral coronal craniosynostosis model. After changing the operation technique using data gathered from previous studies, we reviewed two bilateral craniosynostosis patients operated with the new technique and compared it with two patients that were operated with the old technique.ResultsComparing cranial indexes (CI), significant improvement was detected in both groups. The decrease in CI in the second group was slightly better than the first group. In the comparison of intracranial volume (ICV), there was an increase in ICV values in both groups. The percentage of increase between two groups was similar. The morphological outcome was satisfactory in all patients. There were no major or minor complications and morbidity.ConclusionsCurrent multislice tomography technology and stereolithographic procedures provide an excellent surgical simulation model to find new techniques and predict the outcome. These models should be used in all complex and syndromic craniosynostosis for both better results and reducing the operative time and associated blood loss.

A mature cystic teratoma in pineal region mimicking parietal encephalocele in a newborn

ObjectiveTeratoma is the most frequently encountered intracranial tumor at birth and constitutes 18–20% of all germ cell tumors. They are usually located in pineal and suprasellar regions. The authors aim to report an extremely unusual presentation, location, and appearance of a teratoma in a newborn.Case reportA soft tissue swelling in the vertex was detected in a 1-month-old girl. Neurological examination was normal. A big, cystic–solid lesion beginning from pineal region and extending to the scalp was detected in magnetic resonance imaging. It is interesting to note that cerebral venous angiography showed that the superior sagittal sinus (SSS) was divided into three branches at the level of the lesion and they joined together distally. The tumor was excised totally. Histopathological examination revealed the diagnosis of a mature cystic teratoma.ConclusionA mature cystic teratoma mimicking parietal encephalocele is extremely rare. Germ cell tumors should be kept in mind in the differential diagnosis of all midline lesions with unusual radiographic appearance. Cerebral venous angiography or MR angiography must be performed for the diagnosis and the surgical planning in lesions located near SSS.

Intracranial undifferentiated malign neuroglial tumor in Smith-Lemli-Opitz syndrome: A theory of a possible predisposing factor for primary brain tumors via a case report

BackgroundSmith-Lemli-Opitz Syndrome (SLOS) is a rare hereditary autosomal recessive disorder with broken cholesterol synthesis causing by 7-dehydrocholesterol reductase deficiency. Although the clinical features and pathogenesis is well-defined, it is unknown whether there is a relationship between SLOS and neoplastic processes, especially brain neoplasms.PurposeWe aimed to attract the attentions to any possibility of relation between SLOS and intracranial tumor development via a pediatric case with both intracranial high-grade neuroglial tumor and SLOS, and thus to contribute an additional data to the literature on togetherness of these two clinical conditions.MethodIn our clinic, we experienced an interesting case of a 10-year-old child with both SLOS and huge brain tumor as rarely seen. Here, we reviewed the features and pathophysiology of SLOS and brain tumors via this case.ResultsThe patient was operated in our clinic, after, his brain tumor had been diagnosed, and his histopathology was resulted in undifferentiated malignant neuroglial WHO grade 4 tumor.ConclusionAccording to current literature, our case is the first report on coexisting of SLOS and intracranial undifferentiated malignant neuroglial tumor. Common pathways like impaired sonic hedgehog (Shh) signaling pathway may be considered for pathogenesis of a probable link between SLOS and brain tumors in further studies.