Challenges in the Management of a 7-Year-Old Child with Thyrotropin-Secreting Pituitary Adenoma and the Review of the Literature.

INTRODUCTION: Thyrotropin-producing pituitary adenoma (TSHoma) is a very rare disease, representing less than 1% of the pituitary tumours and presenting with elevated thyroid hormones and normal/high TSH concentrations. CASE PRESENTATION: A 7-year-old boy with nervousness was referred by his psychiatrist for elevated free T4, T3, and TSH levels. Initial evaluation revealed an elevated α-subunit. Pituitary magnetic resonance imaging (MRI) demonstrated a macroadenoma. The patient underwent a trans-sphenoidal tumour resection (TSS) which showed positive immunohistochemical staining for TSH, growth hormone, and prolactin in tumoral tissue. Euthyroidism was achieved for 1 year after TSS, then recurrence of tumour with elevated TSH and thyroid hormone levels necessitated a re-operation with TSS followed by gamma-knife radiosurgery. The euthyroid state was achieved and lasted for 2.5 years this time, but due to the recurrence, medical treatment had been commenced with cabergoline and octreotide. Euthyroidism was maintained for the last 4 years on monthly octreotide treatment. A repeat MRI demonstrated no pituitary mass, but a mass in the sphenoidal sinus had been detected. Removal of this mass by surgery did not achieve euthyroidism. 68Ga-DOTA-TATE positron emission tomography/computed tomography showed residual tissue extending from the pituitary region to the sphenoid sinus. The patient's bone age was advanced by 2 years at diagnosis which became 4 years in 1 year after the diagnosis and remained so throughout follow-up, leading to a final height of -3.3 SDS below his target height at the age of 16 years. CONCLUSION: The diagnosis, treatment, and follow-up of TSHomas are challenging, and short stature due to accelerated bone maturation is a complication of paediatric TSHomas.

Assessment of the Effect of Subthalamic Deep Brain Stimulation on Sleep Quality of Parkinson's Disease Patients.

AIM: To investigate the effects of subthalamic deep brain stimulation (STN DBS) therapy on sleep quality of Parkinson?s Disease (PD) patients and the relationship between sleep, motor symptoms, depression, and adverse effects of dopamine replacement therapies. MATERIAL AND METHODS: A total of 26 PD patients have been included and assessed using various tools both 1 week before and 8 months after the STN DBS therapy. The data collection tools were the Unified Parkinson?s Disease Rating Scale (UPDRS), Beck Depression Inventory (BDI), Montreal Cognitive Assessment (MoCA), Parkinson?s Disease Questionnaire (PDQ-39), Pittsburgh Sleep Quality Index (PSQI), Insomnia Severity Index (ISI), Epworth Sleepiness Scale (ESS) and Polysomnography. RESULTS: PSQI, ISI, and ESS scores were found to have significantly improved after the STN DBS therapy (p=0.002, p=0.006, p < 0.001, respectively), as were the scores obtained from several PSQI sub-scales, that is, sleep duration, sleep disturbance and daytime dysfunction (p=0.023, p=0.005, p=0.032, respectively). Additionally, Wake Times After Sleep Onset (WASO) (p=0.047) and Rapid Eye Movement (REM) sleep latency values (p=0.005) were found to have decreased after the STN DBS treatment, whereas REM sleep durations (p=0,028) and REM sleep percentages (p=0.007) were found to have increased, after the STN DBS therapy. No correlation was found between the ESS scores and Levodopa Equivalent Dosage (LED) or between the scores obtained from the sleep scales and the scores obtained from the UPDRS and BDI. There was also no correlation between sleep scores and other PD-related factors. CONCLUSION: The findings of this study indicated that STN DBS therapy positively affected the PD patients? sleep. This result was attributed to the neuromodulatory effects of the STN DBS independent of the motor symptoms, depression levels, and LED decrease.

A primary third ventricle mixed germ cell tumor with leptomeningeal dissemination of immature teratoma component.

A 17-year-old male patient presented to the clinic with a headache, nausea, and vomiting. Magnetic resonance imaging demonstrated a fat-containing and -enhancing heterogeneous tumor in the third ventricle, and fat droplets within the ventricles and the subarachnoid space. Obstructive hydrocephalus was also present. Emergency subtotal removal of the mass was performed via interhemispheric transcallosal approach. The histopathological diagnosis was a mixed germ cell tumor that was composed of embryonal carcinoma, yolk-sac tumor, germinoma, and immature teratoma containing a large amount of mature elements. The patient was referred for postoperative chemoradiotherapy. A mixed germ cell tumor is a rare type of nongerminomatous germ cell tumor that is made up of at least two different types of germ cell tumors. These may include germinoma, choriocarcinoma, embryonal carcinoma, yolk sac tumor, mature teratoma, immature teratoma, or teratoma with malignant degeneration. As far as we know, this is the first reported case of a primary third ventricle mixed germ cell tumor with leptomeningeal dissemination of the immature teratoma component that contains grossly visible mature elements at admission.

Anterior third ventricular height and infundibulochiasmatic angle: two novel measurements to predict clinical success of endoscopic third ventriculostomy in the early postoperative period.

OBJECTIVE: The authors sought to develop a set of parameters that reliably predict the clinical success of endoscopic third ventriculostomy (ETV) when assessed before and after the operation, and to establish a plan for MRI follow-up after this procedure. METHODS: This retrospective study involved 77 patients who had undergone 78 ETV procedures for obstructive hydrocephalus between 2010 and 2015. Constructive interference in steady-state (CISS) MRI evaluations before and after ETV were reviewed, and 4 parameters were measured. Two well-known standard parameters, fronto-occipital horn ratio (FOHR) and third ventricular index (TVI), and 2 newly defined parameters, infundibulochiasmatic (IC) angle and anterior third ventricular height (TVH), were measured in this study. Associations between preoperative measurements of and postoperative changes in the 4 variables and the clinical success of ETV were analyzed. RESULTS: Of the 78 ETV procedures, 70 (89.7%) were successful and 8 (10.3%) failed. On the preoperative MR images, the mean IC angle and anterior TVH were significantly larger in the successful procedures. On the 24-hour postoperative MR images of the successful procedures, the mean IC angle declined significantly from 114.2° to 94.6° (p < 0.05) and the mean anterior TVH declined significantly from 15 to 11.2 mm (p < 0.05). The mean percentage reduction of the IC angle was 17.1%, and that of the anterior TVH was 25.5% (both p < 0.05). On the 1-month MR images of the successful procedures, the mean IC angle declined significantly from 94.6° to 84.2° (p < 0.05) and the mean anterior TVH declined significantly from 11.2 to 9.3 mm (p < 0.05). The mean percentage reductions in IC angle (11%) and anterior TVH (16.9%) remained significant at this time point but were smaller than those observed at 24 hours. The 6-month and 1-year postoperative MR images of the successful group showed no significant changes in mean IC angle or mean anterior TVH. Regarding the unsuccessful procedures, there were no significant changes observed in IC angle or anterior TVH at any of the time points studied. Reduction of IC angle and reduction of anterior TVH on 24-hour postoperative MR images were significantly associated with successful ETV. However, no clinically significant association was found between FOHR, TVI, and ETV success. CONCLUSIONS: Assessing the IC angle and anterior TVH on preoperative and 24-hour postoperative MR images is useful for predicting the clinical success of ETV. These 2 measurements could also be valuable as radiological follow-up parameters.

Is endoscopic endonasal transsphenoidal surgery increases the susceptibility to rhinosinusitis.

The aim of the study was to analyze whether the measurement of changes in the anatomical position and volume of middle concha, the volume changes in the area between the middle concha and lamina papyracea, the evaluation of opacification in major paranasal sinuses, and osteomeatal complex occlusion in cases with middle concha by out-fracture technique during endoscopic endonasal transsphenoidal approach is a minimally invasive surgery, and also to find out whether these changes lead to the development of tendency to rhinosinusitis. It was a retrospective clinical study. Forty-five cases, between 2013 and 2015, planned for endoscopic endonasal transsphenoidal surgery due to hypophyseal pathology at the Neurosurgery Departments of Marmara University Hospital were evaluated retrospectively. The patients were evaluated for the changes in the anatomy of the middle concha and the effects of these changes to paranasal sinuses by paranasal computed tomographies were studied at the preoperative second week and postoperative 12 month. The Lund-Mackay scoring system was used for the evaluation of opacification in the five major paranasal sinuses and occlusion of the osteomeatal complex in the pre- and postoperative period. The Lund-Mackay scoring system was used to analyze the paranasal computed tomography of the patients at the preoperative 2 weeks and postoperative first year. According to the Lund-Mackay scoring system, no significant difference was detected between the preoperative and postoperative opacification of paranasal sinuses (p > 0.05). Besides, there was also no significant difference between the preoperative and postoperative osteomeatal complex occlusion (p > 0.05). Considering the distance between middle concha and lamina papyracea following the out-fracture of the middle concha, a significant lateralization of 0.5 mm between the preoperative and postoperative period was observed (p < 0.05). In addition, a significant change was also detected in the volume of middle concha (p < 0.05). The volume of the area between the middle concha and lamina papyracea was decreased with a statistical significance (p < 0.05). The endoscopic endonasal transsphenoidal surgery causes some variations in the structures of the middle concha, paranasal sinuses, and OMC, but these changes do not lead to significant rhinologic pathologies.

In vivo effect of pregnancy on angiogenesis potential of arteriovenous malformation tissue samples: an experimental study.

BACKGROUND: Increased angiogenic potential of cerebrovascular malformations during pregnancy may help to explain the complications of arteriovenous malformations (AVMs) in this group of patients. This experimental study investigated the effect of pregnancy on angiogenic activity of implanted AVM tissue samples. METHODS: A subject group of 10 pregnant rats and 10 non-pregnant rats as controls were used. Surgical AVM resection samples were implanted into the micropocket created in both eyes of each animal. Vascular development was assessed by vessel count throughout the study period. In addition, immunohistochemical studies were done for vascular endothelial growth factor (VEGF), platelet derived growth factor (PDGF), and their receptors (VEGFR, PDGFR). RESULTS: Statistically significant increase in the number of vessels was found in both groups (P<0.0001); however, the increase in the pregnant group was greater (P=0.0032). The difference between the two groups was evident at the 25th day of the experiment. Despite both groups showed increased level, there was no difference with the level of VEGF, VEGF receptor, PDGF, or PDGF receptor (P>0.05 for all comparisons). CONCLUSIONS: Findings of this study suggest that angiogenic activity of AVM tissues may increase during late pregnancy, hence physicians should inform pregnant patients with AVM of the potential risk.

Surgical Exposure Gained in an Extended Retrosigmoid Approach to the Cerebellopontine Angle Compared to the Traditional Retrosigmoid Approach.

AIM: Retrosigmoid approach to the posterior fossa has been a popular and dependable approach for accessing the cerebellopontine angle (CPA) and petroclival region. Although this approach is commonly used, it requires cerebellar retraction and has limitations when the lesion is located ventral to the brainstem. The aim was to quantify the angle of view provided by extended retrosigmoid approach in comparison to the traditional approach. A secondary objective was to identify a strategic initial burr hole site for craniotomy. MATERIAL AND METHODS: Ten adult human cadaver heads (20 sides) were used. First, traditional retrosigmoid approach was performed and the angle of exposure was measured on cranial computerized tomography (CT). Following, extended retrosigmoid approach was performed with mastoid bone drilling and reflection of venous sinuses. Angle of exposure was measured on CT. Two measurements of both approaches were compared. RESULTS: Mean angle of view for the traditional retrosigmoid approach (31.4° ± 4.1°) was significantly smaller than that of the extended approach (46.0° ± 4.7°) (p < 0.001). Site of strategic burr hole was 5 mm below and 15 mm lateral to the asterion was defined. CONCLUSION: Extended retrosigmoid approach offers neurosurgeons approximately 50% larger angle of view and shorter working distance than the traditional approach provides. This modification permits better access to the CPA and ventral brain stem without cerebellar retraction.

Hereditary spastic paraplegia with recessive trait caused by mutation in KLC4 gene.

We report an association between a new causative gene and spastic paraplegia, which is a genetically heterogeneous disorder. Clinical phenotyping of one consanguineous family followed by combined homozygosity mapping and whole-exome sequencing analysis. Three patients from the same family shared common features of progressive complicated spastic paraplegia. They shared a single homozygous stretch area on chromosome 6. Whole-exome sequencing revealed a homozygous mutation (c.853_871del19) in the gene coding the kinesin light chain 4 protein (KLC4). Meanwhile, the unaffected parents and two siblings were heterozygous and one sibling was homozygous wild type. The 19 bp deletion in exon 6 generates a stop codon and thus a truncated messenger RNA and protein. The association of a KLC4 mutation with spastic paraplegia identifies a new locus for the disease.

Deep brain stimulation as treatment for dystonic storm in pantothenate kinase-associated neurodegeneration syndrome: case report of a patient with homozygous C.628 2 T > G mutation of the PANK2 gene.

Pantothenate kinase-associated neurodegeneration (PKAN) syndrome is an autosomal-recessive neurodegenerative disease that causes progressive generalized dystonia. Currently, the disorder remains pharmacologically intractable. Herein we report the first case in which deep brain stimulation helped to relieve dystonic storm in a patient with PKAN syndrome who had homozygous c.628 2 T > G mutation of the PANK2 gene. A 10-year-old boy with PKAN disease presented with dystonic storm and was admitted to the emergency department. Examination revealed generalized dystonia and impaired breathing due to involvement of the respiratory muscles. The patient underwent surgery for bilateral globus pallidus internus deep brain stimulation. The patient showed marked response to treatment.

The Supraorbital Keyhole Approach.

AIM: The major aim of the present anatomical study was to demonstrate the anatomical structures that can be visualized using the supraorbital keyhole approach, both endoscopically and microscopically, from an eyebrow incision to intracranial structures. Furthermore, it defines an optimal craniotomy for surgery. METHODS: Fine dissection was performed on each side of 5 formalin-fixed adult cadavers according to the surgical procedures of the supraorbital keyhole approach, and each step was documented both endoscopically and microscopically. Furthermore, the distance between the superior temporal line and the supraorbital notch/foramen was measured from the 10 total sides of the 5 cadavers and from the 118 sides of the 59 autopsies. RESULTS: Tumors and aneurysms of the anterior cranial fossa can be visualized during the supraorbital keyhole approach. The average distance between the superior temporal line and the supraorbital notch/foramen was measured. The distance obtained from the autopsies on the 25 females was 31.56 ±â€Š4.03  mm on the right side and 31.04 ±â€Š5.40  mm on the left side. The average distance obtained from the autopsies on the 34 males was 34.00 ±â€Š4.59  mm on the right side and 33.59 ±â€Š5.41  mm on the left side. There was no statistically significant difference between right and left in the female and male autopsies or between sexes. CONCLUSIONS: This anatomical study showed that structures in the anterior and middle cranial fossa can be reached via the supraorbital keyhole craniotomy approach with minimal brain retraction and adequate exposure and with minimal craniotomy size.

Neural tube defect family with recessive trait linked to chromosome 9q21.12-21.31.

PURPOSE: Meningomyelocele is one of the most common and socioeconomically, psychologically, and physically debilitating neurodevelopmental diseases. A few chromosomal locus and genes have been identified as responsible for the disease; however, clear evidence still needs to be produced. This study aimed to show evidence of a strong genetic linkage in a novel chromosomal locus in a family with this neural tube defect. METHODS: We identified a neural tube defect family in eastern Turkey, where two of six offspring had operations due to thoracolumbar meningomyelocele. The parents were of a consanguineous marriage. We collected venous blood from six offspring of the family. Whole genome linkage analysis was performed in all offspring. RESULTS: A theoretical maximum logarithm of an odds score of 3.16 was identified on chromosome 9q21.12-21.31. This result shows a strong genetic linkage to this locus. CONCLUSIONS: Our results identified a novel chromosomal locus related to meningomyelocele and provide a base for further investigations toward the discovery of a new causative gene.

Superior medullary velum: anatomical-histological study in the sheep brain and a preliminary tractographic study in the human brain.

AIM: To study the anatomy, histology and fiber relations of the superior medullary velum. MATERIAL AND METHODS: Ten previously frozen and formalin-fixed sheep brains were used. The fiber dissection was done using the operating microscope at the Rhoton Anatomy Laboratory of Marmara Faculty of Medicine. A tractographic study was conducted on five volunteer patients to see the fiber anatomy of the superior medullary velum. RESULTS: The average thickness and length was found to be 0.296 mm (range 0.09-1 mm) and 4.25 mm (range 3.25-4.5 mm) respectively. Histologically, the superior medullary velum consisted of cuboidal layer of ependymal cells on the anterior surface related to fourth ventricle. The subependymal layer contained hypocellular fibrillary zone with few glial cells, and the outer layer consisted of thin layer of fibroblasts. Under the hypocellular fibrillary zone, abundant axons and organized structures were observed. Tractographically, only a few fibers were identified on diffusion-tensor imaging (DTI). CONCLUSION: We could not identify a distinct tract anatomically and neuron cells histologically. Neuron-like cells and organized structures were observed on immunohistochemical analysis. Also a few tracts were observed on DTI study as an ascending pathway from spinal tracts to the superior medullary velum. Further studies including human cadaveric, histologic and fiber tractographic investigations are needed to say that it is harmless to divide this anatomical structure.

Anterior transpedicular screw fixation of cervical spine: Is it safe? Morphological feasibility, technical properties, and accuracy of manual insertion.

OBJECT Due to lack of construct stability of the current anterior cervical approaches, supplemental posterior cervical approaches are frequently employed. The use of an anterior-only approach with anterior transpedicular screws (ATPSs) has been proposed as a means of providing 3-column fixation. This study was designed to investigate the feasibility of anterior transpedicular screw (ATPS) fixation of cervical spine, to obtain the morphological measurements for technical prerequisites, and to evaluate the accuracy of the ATPS using fluoroscopy. METHODS The study included both radiological and anatomical investigations. The radiological investigations were based on data from cervical spine CT scans performed in 65 patients. Technical prerequisites of ATPS were calculated using OsiriX for Mac OS. In the anatomical part of the study, 30 pedicles (C3-7) from 6 formalin-preserved cadavers were manually instrumented. Measurements obtained included pedicle width (PW), pedicle height (PH), pedicle transverse angle (PTA), distance of the entry point from the midline (DEPM), and distance of the entry point from the superior endplate (DEPSEP). The authors also analyzed screw position in the manually instrumented vertebrae. RESULTS The mean PW and PH values showed a tendency to increase from C-3 to C-7 in both males and females. The means were significantly larger for both PW and PH in males than in females at all levels (p = 0.001). The overall mean PTA value was significantly lower at C-7 (p < 0.0001). The mean value for the distance of entry point from the midline (DEPM) represented a point at the contralateral side of the pedicle for every level except C-7. The mean DEPSEP values showed significant differences between all levels (p < 0.0001). Seven of the 30 screws were identified as breaching the pedicle (23.3%); these screw malplacements were seen at C-3 (3 screws), C-4 (2 screws), and C-5 (2 screws). CONCLUSIONS The morphological measurements of this study demonstrated that ATPS fixation is feasible in selected cases. They indicate that ATPS insertion using a fluoroscopy-assisted pedicle axis view is safe at the C-6 and C-7 levels, but the results at the other levels did not prove the safety of this technique.

A sine-wave-shaped skin incision for inserting deep-brain stimulators.

BACKGROUND: The sine-wave-shaped skin incision is a technique that minimizes skin-related complications near burr hole caps after electrode placement for deep-brain stimulation (DBS). METHODS: Between 2011 and 2013, 54 DBS electrodes were implanted in 27 consecutive patients with Parkinson's disease (PD), essential tremor, or dystonia. The sine-wave incision was used in 26 patients and conventional bilateral linear scalp incisions were used in one patient. RESULTS: None of the patients whose operations involved sine-wave-shaped incisions developed hardware-linked complications such as skin infection or skin erosion. The one patient who underwent conventional bilateral linear scalp incisions developed a skin infection. CONCLUSION: By preserving the vascular anatomy of the scalp and reducing skin tension at the wound site, the sine-wave-shaped incision promotes wound healing.

The dentate nucleus and its projection system in the human cerebellum: the dentate nucleus microsurgical anatomical study.

BACKGROUND: Neurosurgical management of cerebellar lesions remains challenging. Thus, it is important to have sound knowledge of the microsurgical anatomy of the cerebellum and dentate nucleus (DN) and to define different types of exposure in a variety of surgical interventions. OBJECTIVE: To examine the anatomy of the DN from a neurosurgical viewpoint using fiber tracking techniques. METHODS: Ten formalin-fixed human hemispheres were dissected with the Ludwig and Klingler fiber dissection technique under × 6 to × 40 magnification. Anatomic images were created with 3-dimensional diffusion tensor imaging. The relationships of the DN to tentorium and suboccipital and lateral surfaces of the cerebellum and its spatial positioning relative to different surgical approaches in the cerebellum and fourth ventricle were examined. The fiber tracts terminating at and surrounding the DN were defined. RESULTS: The DN is at greater risk of being injured in the transvermian and supratonsillar approaches to the cerebellum and fourth ventricle, with lesser risk in the telovelar and subtonsillar approaches. Superior approaches are safer compared with other approaches. CONCLUSION: The DN represents an important anatomic structure in surgical interventions involving the posterior fossa, particularly in the elderly because of the common occurrence of atrophy-related problems in this age group. Functionally and anatomically, the DN is closely related to the superior and middle cerebellar peduncles. The inferior cerebellar peduncle poses positional risks because it follows an anterior and superior course relative to the DN. The telovelar approach is a safer procedure for interventions involving the pathological lesions of the fourth ventricle floor.

Falcine venous plexus within the falx cerebri: anatomical and scanning electron microscopic findings and clinical significance.

BACKGROUND: Only limited attention has been paid to the anatomy and clinical importance of the falcine venous plexus. The aim of this study was to evaluate the falcine venous plexus anatomically using scanning electron microscopy (SEM), and to provide guidance for neurosurgical approaches. METHODS: Latex or ink was injected into the superior and inferior sagittal sinus. The falcine venous plexus lying within the connective tissue of the falx cerebri was observed by dividing the falx into thirds (anterior, middle and posterior). Further, the SEM appearance of the falcine venous plexus was evaluated. RESULTS: The anterior third of the falx cerebri consisted of small diameter falcine venous vessels. These vessels were localized close to either the superior or inferior sagittal sinus, and none extended as far as mid-falx cerebri levels in any of the 16 cases. They communicated with either superior or inferior sagittal sinuses, but not with both of these sinuses. In the middle third of the falx cerebri, the majority of the vessels of the falcine venous plexus had larger diameter compared to those of the anterior third. These vessels extended the length of the falx cerebri levels. They communicated with both superior and inferior sagittal sinuses. In the posterior third of the falx cerebri, the vessels of the falcine venous plexuses had the largest diameter and were located at the junction of the inferior sagittal sinus and the straight sinus. They were localized at the lower two-thirds of the falx cerebri. In all cases, the dense venous networks communicated with the inferior sagittal sinus but not with the superior sagittal sinus. The falcine venous plexus observed in the posterior third of the falx cerebri was denser than in the anterior and middle portions. The SEM revealed small vessels whose diameter ranged between 42 and 138 µm. The vessels of the falcine venous plexus in the anterior third had a mean diameter of 0.42 ± 0.1 mm, in the middle third a mean diameter of 0.87 ± 0.17 mm, and in the posterior third, 1.38 ± 0.21 mm. CONCLUSION: The falcine venous plexus is a network of venous channels that exists within the connective tissue of the falx; the sizes and patterns of communication of these structures showed regional differences. Neurosurgeons should be aware of the regional differences when making an incision or puncturing the falx during a surgical approach.