Metachronous Wilms Tumor, Glioblastoma, and T-cell Leukemia in an Child With Constitutional Mismatch Repair Deficiency syndrome due to Novel Mutation in MSH6 (c.2590G>T).

Constitutional mismatch repair deficiency (CMMRD) is an autosomal recessively inherited childhood cancer predisposition syndrome results from biallelic germline mutations affecting the key DNA mismatch repair gene: MLH1, MSH2, MSH6, or PMS2. CMMRD is associated with a high risk of developing early onset of central nervous system tumors, hematologic, and intestinal tract tumors. Clinical manifestations, genetic screening, and cancer prevention strategies are limited. In this report we present a patient with metachronous Wilms tumor, glioblastoma, and acute T-cell lymphoblastic leukemia. He had cutaneous features of neurofibromatosis type 1 (NF1). Molecular testing revealed a novel homozygous mutation in MSH6 (c.2590G>T; p.G864*) that has not been reported previously. CMMRD should be considered in patients with cutaneous features similar to NF1 if tumor is found other than expected tumors in NF, early onset cancer, and strong family history of cancer.

Early diagnosis of Grisel's syndrome in children with favorable outcome.

PURPOSE: The painful torticollis only itself may be a major sign for the diagnosis of Grisel's syndrome (GS). It is known as an inflammatory atlantoaxial rotatory subluxation following upper respiratory tract infections (URTI) and surgical otolaryngological procedures. PATIENTS AND METHODS: The analysis of 16 pediatric GS patients were reviewed retrospectively by considering the diagnosis, the treatment modality, and the prognosis at the Department of Neurosurgery and Otorhinolaryngology in Mersin University, Faculty of Medicine between 2008 and 2018. In addition to the clinical cases, five cadavers were used to demonstrate atlantoaxial region, particularly the ligamentous complex and articulation of the atlas-axis, for the mechanism of these rare entities. RESULTS: The most common etiological factor of GS was URTI with 81.25% among 16 patients. Painful torticollis was the primary symptom of pediatric patients at admission. The X-Ray, computerized tomography (CT), and magnetic resonance imaging (MRI) investigations were used for the definitive diagnosis in the first week of admission, except one. No morbidity, mortality, and deformity were reported in this series. CONCLUSIONS: Early diagnosis is the principle of GS for avoiding of permanent neck deformity and complex surgical procedures. If GS can be diagnosed without any doubt by only considering patient's history and clinical examination, CT scan is not recommended due to harmful effects of radiation. The treatment was achieved by reduction, external fixation under analgesia, or sedoanalgesia accompanying with antibiotic and anti-phylogistic treatment.

Treatment Response to Acute Total Ophthalmoplegia in Primary Pituitary Lymphoma: A Case Report and Review of the Literature.

Primary pituitary lymphoma (PPL) is an extremely rare localized lymphoma without systemic involvement. The most common clinical presentations of PPL are hypopituitarism, headaches, and ophthalmoplegia. Diagnosing PPL without a biopsy is almost impossible. There is no study that has specifically investigated and reviewed treatment responses to the ophthalmological symptoms of PPL patients. Herein, we present a 66-year-old female patient who had acute-onset total ophthalmoplegia and headache as admission symptoms, which was diagnosed as PPL after subtotal resection. In the present study, we discussed the response of ophthalmological symptoms to treatment with a review of the literature. Only 18 reported cases had postoperative ophthalmological examination, and in 94.4% of these cases, ophthalmoplegia resolves after surgery and chemotherapy. The complete resection rate of the PPL in the literature was found to be as low as 12.3% in this region because of the hard and adhesive nature of the tumor. Our review unveiled that complete recovery of ophthalmoplegia can be achieved even in the late phase of the symptoms. In the present case, ophthalmoplegia resolved completely following subtotal resection and rituximab, high-dose methotrexate, and cytarabine treatment.

A new classification of parasagittal bridging veins based on their configurations and drainage routes pertinent to interhemispheric approaches: a surgical anatomical study.

OBJECTIVE: Opening the roof of the interhemispheric microsurgical corridor to access various neurooncological or neurovascular lesions can be demanding because of the multiple bridging veins that drain into the sinus with their highly variable, location-specific anatomy. The objective of this study was to propose a new classification system for these parasagittal bridging veins, which are herein described as being arranged in 3 configurations with 4 drainage routes. METHODS: Twenty adult cadaveric heads (40 hemispheres) were examined. From this examination, the authors describe 3 types of configurations of the parasagittal bridging veins relative to specific anatomical landmarks (coronal suture, postcentral sulcus) and their drainage routes into the superior sagittal sinus, convexity dura, lacunae, and falx. They also quantify the relative incidence and extension of these anatomical variations and provide several preoperative, postoperative, and microneurosurgical clinical case study examples. RESULTS: The authors describe 3 anatomical configurations for venous drainage, which improves on the 2 types that have been previously described. In type 1, a single vein joins; in type 2, 2 or more contiguous veins join; and in type 3, a venous complex joins at the same point. Anterior to the coronal suture, the most common configuration was type 1 dural drainage, occurring in 57% of hemispheres. Between the coronal suture and the postcentral sulcus, most veins (including 73% of superior anastomotic veins of Trolard) drain first into a venous lacuna, which are larger and more numerous in this region. Posterior to the postcentral sulcus, the most common drainage route was through the falx. CONCLUSIONS: The authors propose a systematic classification for the parasagittal venous network. Using anatomical landmarks, they define 3 venous configurations and 4 drainage routes. Analysis of these configurations with respect to surgical routes indicates 2 highly risky interhemispheric surgical fissure routes. The risks are attributable to the presence of large lacunae that receive multiple veins (type 2) or venous complex (type 3) configurations that negatively impact a surgeon's working space and degree of movement and thus are predisposed to inadvertent avulsions, bleeding, and venous thrombosis.

Calvarial Tumors: A Retrospective Analysis and Clinical Experience.

AIM: To elucidate the prognosis, frequency, and diverse nature of pathologies for calvarial tumors among different age groups. MATERIAL AND METHODS: Seventy-six patients who underwent surgery for calvarial lesions between January 2007 and March 2021 are included in this study. Clinical data obtained retrospectively from patients? electronic records. Radiological images and surgical notes are reviewed to determine extent of the tumor and resection. RESULTS: Among 76 patients, 33 (43.4%) were male and 43 (56.6%) were female. The mean age was 36.0 years (range: 1?81 years) at the time of initial operation. Children consisted 28.9% (n=22) of the patients. In children, 59.1% (n=13) had tumor-like pathologies, while 27.3% (n=6) had benign pathologies, and 13.6% (n=3) had malignant tumors. In the adult population, 42.6% (n=23) had malignant tumors, 31.5% (n=17) had benign tumors, 16.7% (n=9) had tumor-like pathologies, and 9.2% (n=5) had intermediate-grade tumors. F-fluorodeoxyglucose positron emission tomography (FDG-PET) scan was performed in 16 patients, 10 cases underwent whole-body bone scintigraphy (WBBS), and 4 cases underwent both. Among these examinations, 16 (80%) of the FDG-PET scans and 5 (35.7%) of the WBBS scans revealed an extracranial pathological lesion. A calvarial tumor was diagnosed in 13 of 18 cases of metastatic lesions (72.2%) before the primary tumor detection. CONCLUSION: Lesions of the calvarium include malignant tumors, intermediate grade tumors, tumor-like lesions, and benign tumors. These masses may be the first presentation in patients with underlying primary tumors. In our study, the malignant tumor rate in the calvaria was 34.2%, and 72.2% of the metastatic tumors were diagnosed with a calvarial resection before the primary tumor was found. Operating a calvarial lesion and making an early diagnosis are crucial for the treatment of the primary lesions.

Transcavernous Approach for Gross Total Resection of a Dumbbell-Shaped Giant Trigeminal Schwannoma.

Trigeminal schwannomas are rare nerve sheet tumors that represent the second most common intracranial site of occurrence after vestibular nerve origins. Microsurgical resection of giant dumbbell-shaped trigeminal schwannomas often requires complex skull base approaches. The extradural transcavernous approach is effective for the resection of these giant tumors involving the cavernous sinus. The patient is a 72-year-old man with headache, dizziness, imbalance, and cognitive decline. Neurological examination revealed left-sided sixth nerve palsy, a diminished corneal reflex, and wasting of temporalis muscle. Magnetic resonance imaging (MRI) showed a giant homogeneously enhancing dumbbell-shaped extra-axial mass centered within the left cavernous sinus, Meckel's cave, and the petrous apex, with extension to the cerebellopontine angle. There was a significant mass effect on the brain stem causing hydrocephalus. Computed tomography (CT) scan showed erosion of the petrous apex resulting in partial anterior autopetrosectomy ( Figs. 1 and 2 ). The decision was made to proceed with tumor resection using a transcavernous approach. Gross total resection was achieved. The surgery and postoperative course were uneventful, and the patient woke up the same as in the preoperative period. MRI confirmed gross total resection of the tumor. The histopathology was a trigeminal schwannoma, World Health Organization (WHO) grade I. The patient continues to do well without any recurrence at 15-month follow-up. This video demonstrates important steps of the microsurgical skull base techniques for resection of these challenging tumors. The link to the video can be found at https://youtu.be/TMK5363836M.

Role of Extradural Clinoidectomy and Optic Unroofing in Resection of an Anterior Clinoidal Meningioma with Encasement of the Internal Carotid Artery and Its Branches.

Anterior clinoidal meningiomas (ACMs) remain a major neurosurgical challenge. The skull base techniques, including extradural clinoidectomy and optic unroofing performed at the early stage of surgery, provide advantages for improving the extent of resection, and thereby enhancing overall outcome, and particularly visual function. Additionally, when the anterior clinoidal meningiomas encase neurovascular structures, particularly the supraclinoid internal carotid artery and its branches, this further increases morbidity and decreases the extent of resection. Although it might be possible to remove the tumor from the artery wall despite complete encasement or narrowing, the decision of whether the tumor can be safely separated from the arterial wall ultimately must be made intraoperatively. The patient is a 75-year-old woman with right-sided progressive vision loss. In the neurological examination, she only had light perception in the right eye without any visual acuity or peripheral loss in the left eye. MRI showed a homogeneously enhancing right-sided anterior clinoidal mass with encasing and narrowing of the supraclinoid internal carotid artery (ICA). Computed tomography (CT) angiography showed a mild narrowing of the right supraclinoid ICA with associated a 360-degree encasement. The decision was made to proceed using a pterional approach with extradural anterior clinoidectomy and optic unroofing. The surgery and postoperative course were uneventful. MRI confirmed gross total resection ( Figs. 1 and 2 ). The histopathology was a meningothelial meningioma, World Health Organization (WHO) grade I. The patient continues to do well without any recurrence and has shown improved vision at 15-month follow-up. This video demonstrates important steps of the microsurgical skull base techniques for resection of these challenging tumors. The link to the video can be found at https://youtu.be/vt3o1c2o8Z0.

Level I to III craniofacial approaches based on Barrow classification for treatment of skull base meningiomas: surgical technique, microsurgical anatomy, and case illustrations.

OBJECT: Although craniofacial approaches to the midline skull base have been defined and surgical results have been published, clear descriptions of these complex approaches in a step-wise manner are lacking. The objective of this study is to demonstrate the surgical technique of craniofacial approaches based on Barrow classification (Levels I-III) and to study the microsurgical anatomy pertinent to these complex craniofacial approaches. METHODS: Ten adult cadaveric heads perfused with colored silicone and 24 dry human skulls were used to study the microsurgical anatomy and to demonstrate craniofacial approaches in a step-wise manner. In addition to cadaveric studies, case illustrations of anterior skull base meningiomas were presented to demonstrate the clinical application of the first 3 (Levels I-III) approaches. RESULTS: Cadaveric head dissection was performed in 10 heads using craniofacial approaches. Ethmoid and sphenoid sinuses, cribriform plate, orbit, planum sphenoidale, clivus, sellar, and parasellar regions were shown at Levels I, II, and III. In 24 human dry skulls (48 sides), a supraorbital notch (85.4%) was observed more frequently than the supraorbital foramen (14.6%). The mean distance between the supraorbital foramen notch to the midline was 21.9 mm on the right side and 21.8 mm on the left. By accepting the middle point of the nasofrontal suture as a landmark, the mean distances to the anterior ethmoidal foramen from the middle point of this suture were 32 mm on the right side and 34 mm on the left. The mean distance between the anterior and posterior ethmoidal foramina was 12.3 mm on both sides; the mean distance between the posterior ethmoidal foramen and distal opening of the optic canal was 7.1 mm on the right side and 7.3 mm on the left. CONCLUSIONS: Barrow classification is a simple and stepwise system to better understand the surgical anatomy and refine the techniques in performing these complex craniofacial approaches. On the other hand, thorough anatomical knowledge of the midline skull base and variations of the neurovascular structures is crucial to perform successful craniofacial approaches.

Comparison of the effects of different electrocautery applications to peripheral nerves: an experimental study.

BACKGROUND: This study was designed to investigate the effects of bipolar and mononopolar electrocauterization on peripheral nerve tissue. The comparison on the deleterious effects of the different cautery modalities and the importance of probe tip placement are evaluated using electrophysiological, electron microscopic and biochemical assessment parameters. METHODS: Ninety-eight male Wistar albino rats, each weighing 250-275 g, were randomly divided into 14 groups. Each group consisted of seven animals. Monopolar and bipolar electrocautery were performed at 15 watts. The application was performed either directly on the nerve or 1 mm lateral to the longitudinal axis of the nerve for 'near the nerve groups', respectively. RESULTS: The electrophysiological findings showed that the mean amplitudes were at the lowest value in the first day for all the groups. At the end of the 3rd week, we recognised that the electrophysiological recovery continued. Electron microscopic evaluation showed myelin disruption in all groups. Myelin disruption of healthy neurons was at the highest level in the 1st day of application in accordance with the electrophysiological findings. Biochemical evaluation revealed statistical significance between the control and the two of the 'near the nerve groups' (GIII and GV) for NO (nitrite and nitrate) serum level. CONCLUSION: The data of the present study might suggest that electrocautery, independent of the type and form of application, may result in significant damage in histological and electrophysological basis. Although the relative proportions cannot be ascertained, the time course of recovery suggests that both axon and myelin damage have occurred. The probable electrocautery damage may be of substantial importance for the situation that the nerves are displaced by tumor masses or atypical neural traces.

Microsurgical anatomy of the vein of Labbé.

OBJECTIVE: Venous drainage of the temporal lobe is of great importance in various neurosurgical and combined skull base approaches. The most significant draining vein of the temporal lobe is the inferior anastomotic vein (vein of Labbé). The purpose of this study was to examine the detailed anatomy and variations of the vein of Labbé (VL) from microsurgical perspective. METHODS: Fourteen fixed human cadaver heads (28 sides) with perfused vessels were included to define microsurgical anatomy and variations of the VL. RESULTS: The main findings of the present study were as follows: (1) drainage pattern of the VL was found to be very variable in cadaveric dissections; (2) VL drained around the sinus confluence at the tentorium in one specimen (3.5%), into the large meningeal vein in the occipital dura mater in another specimen (3.5%). The VL rarely (7%) drains into the superior petrosal sinus (SPS) which may make combined skull base approaches very difficult or impossible. CONCLUSION: Results of this study suggest that careful and thorough evaluation of the VL is of great importance, especially in surgeries combining a subtemporal route with petrosal approaches by sectioning the SPS and the tentorium.

Demonstration of Microsurgical Technique and Nuances for the Resection of a Midbrain Tectal Glioma via the Transcollicular Approach: 3-Dimensional Operative Video.

Tectal gliomas are a rare subset of intrinsic brainstem lesions. The microsurgical resection of these lesions remains a major challenge.1,2 Transcollicular approaches on one side, via the superior or inferior colliculi or both, are neurologically well tolerated without obvious or major auditory or oculomotor consequences. However, any postoperative acute visually triggered saccadic abnormalities caused by iatrogenic superior colliculus damage generally resolve during the postoperative period, as other oculomotor structures compensate for these functions in unilateral lesions. In this surgical video, we present a 37-yr-old man with long-standing seizures, new onset headaches, progressive ataxic gait, and imbalance. Magnetic resonance imaging (MRI) showed a circumscribed nonenhancing dorsal midbrain cystic mass with compression on the aqueduct causing hydrocephalus. The lesion had a low signal intensity on T1-weighted images and a high signal intensity on T2-weighted images. The patient first underwent an endoscopic third ventriculostomy. Although his headaches greatly improved after the third ventriculostomy, he remained quite symptomatic in terms of gait imbalance and ataxia. The patient underwent a supracerebellar, infratentorial, transcollicular approach for resection of the tectal tumor. Simultaneously, motor and somatosensory evoked potentials were monitored. Both the surgery and the postoperative course were uneventful, with postoperative MRI showing gross total resection of the mass, and histopathology indicating a WHO (World Health Organization) grade I pilocytic astrocytoma. The patient continued to do well without recurrence at 2-yr follow-up. In this video, we demonstrate step-by-step microsurgical techniques for resecting these challenging tectal gliomas via the infratentorial-supracerebellar-transcollicular approach. The patient consented to the procedure and publication of his images.

Navigating a Carotico-Clinoid Foramen and an Interclinoidal Bridge in the Endonasal Endoscopic Approach: An Anatomical and Technical Note.

Objective The carotico-clinoid foramen and interclinoid bridge are two anatomical variants of the sellar region. If these anatomical variants go unrecognized and are not managed safely by the surgeon during expanded endoscopic endonasal surgery for a posterior clinoidectomy, a carotid artery injury may occur. We summarize a method to safely navigate in the presence of the carotico-clinoid foramen and interclinoid bridge in an endoscopic endonasal approach. Study Design The study involves cadaveric dissection and management of the anatomical variants. Setting The study took place in a cadaveric dissection laboratory. Participants The object of study is one cadaveric head. Main Outcome Measures After discovering the two variants in both cavernous sinuses of a cadaveric head, we established a stepwise coping strategy to avoid carotid artery injury while performing an endoscopic endonasal bilateral interdural pituitary transposition, and we report the final view after endoscopic management. Results Debulking of the middle clinoid process can decrease the obstacle effect, and the pituitary transposition can be performed medial to the ossified carotico-clinoid ligament. Disconnection of the interclinoid bridge is the prerequisite to an effective posterior clinoidectomy, and distinguishing the transition between the sellar diaphragm and the interclinoid bridge is essential. Conclusion In the presence of both the carotico-clinoid foramen and the interclinoid bridge, a bilateral interdural pituitary transposition can still be performed, although preoperative strategic preparation, full inspection, and stepwise disconnections are of paramount importance in such a scenario to avoid cavernous carotid artery injury.

Intraoperative perineural infiltration of lidocaine for acute postlaminectomy pain: preemptive analgesia in spinal surgery.

STUDY DESIGN: Prospective, cohort, and clinical study. SUMMARY OF BACKGROUND DATA: It was proven that effective control of postoperative pain in lumbar disk surgery improves the patient's recovery. Despite the many advances in surgical and nonsurgical techniques, the most accurate approach in pain relief is still under debate. OBJECTIVE: In this study, our aim was to determine whether neural root blockade before the onset of noxious stimuli could inhibit the production of pain. METHODS: Forty-five patients undergoing unilateral 1 spinal level (lumbar 5) hemipartial laminectomy were included in the study. In 20 of the patients (group 2), 0.5 mL 2% lidocaine was infiltrated onto the neural root immediately after the exposure; the 25 patients in the control group (group 1) were not injected. All patients were monitored regarding pain determination using a visual analog scale, and the exact time of analgesic requirement during the first postoperative day was noted. Total analgesic dose given during the first postoperative day was also recorded. RESULTS: Perineural lidocaine infiltration extended the early postoperative analgesic period. Although the pain was not completely suppressed, the lidocaine infiltration helped to manage the postoperative pain more effectively. The patients (group 2) who received lidocaine infiltration intraoperatively onto the neural root had a statistically significant longer time before analgesia requested (P<0.001) and also required significantly less analgesic when compared with the control group (P<0.001). CONCLUSIONS: For preemptive analgesia for acute postoperative pain in laminectomy surgery, which remains a major concern, we suggest that lidocaine infiltration onto the dorsal neural sheath immediately before retraction of the root may extend the time before analgesia requested and the total analgesic drug consumption.

Intraoperative supratentorial epidural haematoma during removal of a huge posterior fossa dermoid cyst.

Epidural haematomas may occur following cranial operations, and most of them are located near the craniotomy or burr-hole areas. There are very few cases of supratentorial epidural haematoma following the resection of giant tumours located in the posterior fossa. In this case report, we present a patient who developed an acute left temporoparietal epidural haematoma in the perioperative period during the excision of a huge dermoid cyst in the posterior fossa.

Grapefruit Training Model for Distal Anterior Cerebral Artery Side-to-Side Bypass.

OBJECTIVE: Simulation models enable trainees to master microsurgical skills before performing surgeries. Vascular bypass is a critical component of cerebrovascular and many nonneurologic procedures. However, most available bypass training models lack important spatial, tactile, and physiologic aspects of real surgery. Animal and placental models provide true physiology but are expensive. While some models adequately simulate superficial temporal artery-middle cerebral artery bypass, there is no model for side-to-side distal anterior cerebral artery bypass. The objective is to create a realistic and inexpensive training model for this important procedure. METHODS: The depth of interhemispheric fissures in cadaver brains was compared with the grapefruit radii. Grapefruits were dissected to simulate the operative field within the deep and narrow interhemispheric fissure. Pericallosal arteries were mimicked with chicken wing vessels or synthetic tubing, with an aquarium pump providing closed circulation. Twelve board-certified neurosurgeons who were given bypass training using the grapefruit model were blindly surveyed on model realism and training suitability. RESULTS: Grapefruit depths from pith to central column were comparable with interhemispheric cadaveric fissure depths. Approximate preparation time of grapefruit training models was 5-10 minutes. Surveyed neurosurgeons rated the model a better replicate for cerebral artery bypass (P < 0.02) and more challenging than common training models (P < 0.01). They also rated the grapefruit model as likely to be superior for improving surgical skills before surgery (P < 0.05). CONCLUSIONS: This grapefruit model provides a realistic simulation of side-to-side distal anterior cerebral artery bypass procedure that can be inexpensively and easily implemented in nearly any resource environment.

Radioanatomic Assessment of the Geniculate Ganglion Dehiscence and Dimension: A Cadaveric Study.

OBJECTIVE: The main aim of this study placed on cadavers was to compare the data related to geniculate ganglion (GG) dehiscence and dimension obtained from computed tomography (CT) with dissection values. METHODS: This study was conducted on 20 temporal bones obtained from 10 cadavers (4 female, 6 male) aged between 45 and 92 (71.50 ± 15.98) years. All the measurements related to GG dimension were performed with a CT scanner and microdissection. RESULTS: The size of GG including its area, length, and width did not show statistically significant differences in terms of sex, side, and assessment method (CT and cadaveric dissections). The dehiscent GG was observed in 6 (30%) and 5 (25%) out of 20 temporal bones in CT and cadaveric dissections, respectively. The presence and absence of GG dehiscence in CT and dissection were similar in 75%. CONCLUSIONS: Our findings based on dissection data suggest that radiologic evaluation of dehiscent GG detection might be erroneous by 25%, which highlights that surgeons should be careful when lifting the dura to prevent GG injury during middle cranial fossa surgical approaches. On the other hand, there was no statistical difference between CT and dissection measurements related to GG dimension.

Unusual location of hydatid cysts in pediatric patients.

OBJECTIVE/AIMS: To emphasize the importance of diagnosis and treatment of unusually localized hydatid cysts in pediatric cases. METHODS: Hydatid cyst patients of two departments were listed who had undergone surgery between January 2001 and December 2008. Of the 7 pediatric patients, 3 were chosen as the ones with unusual localization. Cyst removal with Dowling's technique was performed in 2 cases and total removal of the cyst wall was achieved after cyst aspiration in the other patient. RESULTS: Two patients did not show any signs of recurrence. Some of the cranial multiple cysts of the patient who had undergone her first surgery in another clinic with cyst rupture were successfully removed in our clinic. Six months later, she was admitted with spinal seedings. CONCLUSION: Hydatid cyst removal without rupture should be the surgical goal in all cases. Radiological evaluation is of utmost importance for differential diagnosis. When a cystic lesion is found in the central nervous system on radiological evaluation, hydatid disease must be considered in countries where the disease is endemic and surgery is to be planned emergently especially for pediatric cases with increased intracranial pressure. The study focuses on the strategy for the correct diagnosis and the appropriate treatment of unusually localized hydatid cysts.

Rare Cause of Acute Tetraplegia and Respiratory Arrest: Cervicomedullary Neuroenteric Cyst in a Child: Case Report.

BACKGROUND: Neuroenteric cysts (NCs) are benign, rare congenital lesions which are most commonly found in the lower cervical and upper thoracic segments. Even though these are benign lesions, it may present with fatal neurologic deterioration particularly in pediatric patients. CASE REPORT: We here report a 16-year-old boy who has unique manifestation with acute tetraplegia and respiratory arrest in emergency room. According to English literature, this is the first case of cervicomedullary intradural neuroenteric cyst which causes acute tetraplegia and respiratory arrest in a child. CONCLUSION: The NCs can be manifested with unpredictable, life threating clinical presentation. The exact pathophysiology of an acute neurological deterioration is still unclear, however prolonged latent period with minor symptom should be considered carefully in child population.

The Comparison of the Right and Left Sigmoid Sinus Cross-Sectional Areas in Fetal Period and the Factors Affecting the Venous Dominance.

OBJECTIVES: Skull base is an important and a challenging area for surgeons. Success in skull base surgery depends on various factors such as pre-operative evaluation, appropriate surgical technique, anesthesia duration, intraoperative neuromonitorization and wound care. MATERIALS AND METHODS: This study was performed in the Anatomy dissection laboratory of M.U. Medical Faculty (Ethical committee approval number 2010-103). Twelve fetuses between 17-33 gestational weeks fixed with formaldehyde were enrolled to the study. RESULTS: This study was planned to investigate the cross sectional areas of the sigmoid sinus in three levels to compare the right-left sides and the probable relationship among the levels in fetuses to further delineate the developmental factors on jugular foramen asymmetry. The cross-sectional measurements of sigmoid sinus lumen were done on 3 levels which are described as A1 level; sinodural angle, A2 level; the midpoint between the sinodural angle and endocranial orifice and A3 level as the entrance (endo-cranial orifice) of the jugular foramen. There is a strong positive correlation between left (L) A1 and L A2 and also the same for L A1 and right (R) A2. These strong and positive correlations are all valid between L A2-L A3, L A2-R A2, L A2-R A3, L A3-R A3, R A1-R A2. CONCLUSION: Multicenter studies would be beneficial to investigate the topic with greater number of fetuses also on the different regions for genetic differences.

The work horse of skull base surgery: orbitozygomatic approach. Technique, modifications, and applications.

OBJECT: The aim of this study was to describe the microsurgical anatomy of the orbitozygomatic craniotomy and its modifications, and detail the stepwise dissection of the temporalis fascia and muscle and explain the craniotomy techniques involved in these approaches. METHODS: Nine cadaveric embalmed heads injected with colored silicone were used to demonstrate a stepwise dissection of the 3 variations of orbitozygomatic craniotomy. The craniotomies and dissections were performed with standard surgical instruments, and the microsurgical anatomy was studied under microscopic magnification and illumination. RESULTS: The authors performed 2-piece, 1-piece, and supraorbital orbitozygomatic craniotomies in 3 cadaveric heads each. Stepwise dissection of the temporalis fascia and muscle, and osteotomy cuts were shown and the relevant microsurgical anatomy of the anterior and middle fossae was demonstrated in cadaveric heads. Surgical case examples were also presented to demonstrate the application of and indications for the orbitozygomatic approach. CONCLUSIONS: The orbitozygomatic approach provides access to the anterior and middle cranial fossae as well as the deep sellar and basilar apex regions. Increased bone removal from the skull base obviates the need for vigorous brain retraction and offers an improved multiangled trajectory and shallower operative field. Modifications to the orbitozygomatic approach provide alternatives that can be tailored to particular lesions, enabling the surgeon to use the best technique in each individual case rather than a "one size fits all" approach.