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https://www.readbyqxmd.com/read/28445472/unsuspected-osteochondroma-like-outgrowths-in-the-cranial-base-of-hereditary-multiple-exostoses-patients-and-modeling-and-treatment-with-a-bmp-antagonist-in-mice
#1
Sayantani Sinha, Christina Mundy, Till Bechtold, Federica Sgariglia, Mazen M Ibrahim, Paul C Billings, Kristen Carroll, Eiki Koyama, Kevin B Jones, Maurizio Pacifici
Hereditary Multiple Exostoses (HME) is a rare pediatric disorder caused by loss-of-function mutations in the genes encoding the heparan sulfate (HS)-synthesizing enzymes EXT1 or EXT2. HME is characterized by formation of cartilaginous outgrowths-called osteochondromas- next to the growth plates of many axial and appendicular skeletal elements. Surprisingly, it is not known whether such tumors also form in endochondral elements of the craniofacial skeleton. Here, we carried out a retrospective analysis of cervical spine MRI and CT scans from 50 consecutive HME patients that included cranial skeletal images...
April 26, 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28430034/um-chor1-establishment-and-characterization-of-the-first-validated-clival-chordoma-cell-line
#2
John Henry Owen, Christine M Komarck, Anthony C Wang, Waleed M Abuzeid, Richard F Keep, Erin L McKean, Stephen Sullivan, Xing Fan, Mark E P Prince
OBJECTIVE Chordomas are rare malignant tumors thought to arise from remnants of the notochord. They can be located anywhere along the axial skeleton but are most commonly found in the clival and sacrococcygeal regions, where the notochord regresses during fetal development. Chordomas are resistant to many current therapies, leaving surgery as the primary method of treatment. Cancer cell lines have been useful for developing new cancer treatments in a laboratory setting that can then be transferred to the clinic, but there are only 4 validated chordoma cell lines available...
April 21, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28411008/cone-beam-computed-tomography-incidental-findings-of-the-cervical-spine-and-clivus-retrospective-analysis-and-review-of-the-literature
#3
Noura A Alsufyani
OBJECTIVE: The aim of this study was to analyze and describe incidental findings in the cervical spine (C-spine) and the clivus encountered in cone beam computed tomography (CBCT) imaging. The wide range of possible anatomic variants and pathoses is discussed in the context of the medical and dental literature to clarify their radiographic appearance and clinical implications as a guide for the oral and maxillofacial radiologist. STUDY DESIGN: A retrospective analysis of radiographic reports was conducted based on CBCT images from 2 oral and maxillofacial imaging centers...
March 19, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/28410953/spontaneous-cerebrospinal-fluid-fistula-in-the-clivus
#4
A Codina Aroca, J R Gras Cabrerizo, M De Juan Delago, H Massegur Solench
INTRODUCTION: Spontaneous cerebrospinal fluid (CSF) fistulas are infrequent and only 10 cases in the literature have been located in the clivus. We describe two new cases of CSF fistulas in this site and review the literature. CASE REPORT: The first patient was a 52-year-old woman referred to our centre for intermittent rhinorrhea that had been diagnosed after an episode of meningitis. The second case was a 69-year-old man who was visited for rhinorrhea of one-year duration; he also developed meningitis during the preoperative study...
April 11, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28409727/clival-chordomas-considerations-after-16-years-of-endoscopic-endonasal-surgery
#5
Matteo Zoli, Laura Milanese, Rocco Bonfatti, Marco Faustini-Fustini, Gianluca Marucci, Giovanni Tallini, Corrado Zenesini, Carmelo Sturiale, Giorgio Frank, Ernesto Pasquini, Diego Mazzatenta
OBJECTIVE In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach. METHODS All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter...
April 14, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28402528/macrovascular-decompression-of-the-brainstem-and-cranial-nerves-evolution-of-an-anteromedial-vertebrobasilar-artery-transposition-technique
#6
Omar Choudhri, Ian D Connolly, Michael T Lawton
BACKGROUND: Tortuous and dolichoectatic vertebrobasilar arteries can impinge on the brainstem and cranial nerves to cause compression syndromes. Transposition techniques are often required to decompress the brainstem with dolichoectatic pathology. We describe our evolution of an anteromedial transposition technique and its efficacy in decompressing the brainstem and relieving symptoms. OBJECTIVE: To present the anteromedial vertebrobasilar artery transposition technique for macrovascular decompression of the brainstem and cranial nerves...
April 11, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28362929/metastatic-liposarcoma-of-the-skull-base-a-case-report-and-review-of-literature
#7
Nirmeen Zagzoog, Greta Ra, Alex Koziarz, John Provias, Doron Sommer, Saleh A Almenawer, Kesava Reddy
BACKGROUND AND IMPORTANCE: Myxoid liposarcoma is not an uncommon form of sarcoma. However, it usually affects the lower extremity long bones. Scapular involvement is extremely rare, as is a metastasis to the parasellar region. We present a case of liposarcoma of the skull base originating in the scapular region and metastasizing to the sellar and parasellar regions and provide a review of the pertinent literature. CLINICAL PRESENTATION: A 43-year-old female patient diagnosed with left scapular myxoid liposarcoma was treated with surgical resection...
April 1, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28361020/role-of-dynamic-computed-tomography-scans-in-patients-with-congenital-craniovertebral-junction-malformations
#8
Otávio Turolo da Silva, Enrico Ghizoni, Helder Tedeschi, Andrei Fernandes Joaquim
AIM: To evaluate the role of dynamic computed tomography (CT) scan imaging in diagnosing craniovertebral junction (CVJ) instability in patients with congenital CVJ malformations. METHODS: Patients with symptomatic congenital CVJ malformations who underwent posterior fossa decompression and had a preoperative dynamic CT scan in flexion and extended position were included in this study. Measurements of the following craniometrical parameters were taken in flexed and extended neck position: Atlanto-dental interval (ADI), distance of the odontoid tip to the Chamberlain's line, and the clivus-canal angle (CCA)...
March 18, 2017: World Journal of Orthopedics
https://www.readbyqxmd.com/read/28321374/ectopic-pituitary-adenomas-presenting-as-sphenoid-or-clival-lesions-case-series-and-management-recommendations
#9
Bobby A Tajudeen, Edward C Kuan, Nithin D Adappa, Joseph K Han, Rakesh K Chandra, James N Palmer, David W Kennedy, Marilene B Wang, Jeffrey D Suh
Background An ectopic pituitary adenoma presenting as a clival or sphenoid mass is a rare clinical occurrence that may mislead the clinician and result in unnecessary interventions or potential medicolegal consequences. Here, we present one of the largest multi-institutional case series and review the literature with an emphasis on radiological findings and critical preoperative workup. Methods Retrospective chart review. Results Nine patients were identified with ectopic pituitary adenomas of the sphenoid or clivus...
April 2017: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/28317556/skull-5-from-dmanisi-descriptive-anatomy-comparative-studies-and-evolutionary-significance
#10
G Philip Rightmire, Marcia S Ponce de León, David Lordkipanidze, Ann Margvelashvili, Christoph P E Zollikofer
A fifth hominin skull (cranium D4500 and mandible D2600) from Dmanisi is massively constructed, with a large face and a very small brain. Traits documented for the first time in a basal member of the Homo clade include the uniquely low ratio of endocranial volume to basicranial width, reduced vertex height, angular vault profile, smooth nasal sill coupled with a long and sloping maxillary clivus, elongated palate, and tall mandibular corpus. The convex clivus and receding symphysis of skull 5 produce a muzzle-like form similar to that of Australopithecus afarensis...
March 2017: Journal of Human Evolution
https://www.readbyqxmd.com/read/28283722/fanconi-anemia-correlating-central-nervous-system-malformations-and-genetic-complementation-groups
#11
Benjamin A Johnson-Tesch, Rakhee S Gawande, Lei Zhang, Margaret L MacMillan, David R Nascene
BACKGROUND: Congenital central nervous system abnormalities in children with Fanconi anemia are poorly characterized, especially with regard to specific genetic complementation groups. OBJECTIVE: To characterize the impact of genetic complementation groups on central nervous system anatomy. MATERIALS AND METHODS: Through chart review we identified 36 patients with Fanconi anemia with available brain MRIs at the University of Minnesota (average age, 11...
March 10, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28251542/sellar-and-clival-plasmacytomas-case-series-of-5-patients-with-systematic-review-of-65-published-cases
#12
REVIEW
Jane Lee, Edwin Kulubya, Barry D Pressman, Adam Mamelak, Serguei Bannykh, Gabriel Zada, Odelia Cooper
PURPOSE: Parasellar plasmacytomas are rare tumors localized to the sellar region arising from plasma cells. Knowledge of clinical, imaging, surgical, and pathological characteristics is limited to single case reports. METHODS: A retrospective analysis of five primary cases was conducted, followed by systematic review of English language articles using PubMed in accordance with PRISMA guidelines. RESULTS: Five primary case patients include four men and one woman, ages 60-77, followed up to 3 years...
March 1, 2017: Pituitary
https://www.readbyqxmd.com/read/28242132/abducens-nerve-palsy-due-to-inferior-petrosal-sinus-thrombosis
#13
Shivam Om Mittal, Junaid Siddiqui, Bashar Katirji
Isolated unilateral abducens nerve palsy is usually due to ischemia, trauma or neoplasm. Dorello's canal is the space between the petrous apex and superolateral portion of the clivus, bound superiorly by Gruber's ligament. The abducens nerve travels with inferior petrosal sinus (IPS) though the Dorello's canal before entering the cavernous sinus. A 31-year-old man presented with neck pain, and binocular horizontal diplopia, worse looking towards left and at distance. He had a history of intravenous drug abuse but no history of hypertension or diabetes...
February 24, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28239593/ectopic-esthesioneuroblastoma-of-the-sphenoclivus-a-rare-entity
#14
Farah Dayana Zahedi, Balwant Singh Gendeh, Salina Husain, Ramesh Kumar, Thean Yean Kew
Esthesioneuroblastoma is a rare malignant neoplasm of oflactory neuroepithelium and usually located at the olfactory cleft at superior nasal cavity. Ectopic localization of esthesioneuroblastoma is even rarer and usually posed with a diagnostic dilemma and delay in the diagnosis and management, We report a rare case of ectopic esthesioneuroblastoma of the sphenoclivus with the presentation of intermittent unilateral epistaxis, intermittent intractable headache without anosmia. Nasal endoscopy findings showed a pulsatile mass at the anterior face of the sphenoid sinus with extension posteriorly towards the clivus region and occupying the floor of the sphenoid sinus...
March 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28236049/analysis-of-the-venous-channel-within-the-clivus-using-multidetector-computed-tomography-digital-subtraction-venography
#15
Katsuhiro Mizutani, Masahiro Toda, Jun Kurasawa, Takenori Akiyama, Hirokazu Fujiwara, Masahiro Jinzaki, Kazunari Yoshida
PURPOSE: Although neuroradiologists and skull base neurosurgeons are aware of the existence of veins within the clivus, such vessels have seldom been described in the literature. The aim of the present study is to elucidate the detailed venous structure of the clivus. METHODS: Computed tomography digital subtraction venography (CT-DSV) images of 50 unruptured aneurysm cases were examined retrospectively. RESULTS: Eighteen emissary veins were identified in 14 (28...
February 24, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28230588/how-to-expose-the-entire-sella-floor-with-minimal-manipulation-during-an-endoscopic-endonasal-transsphenoidal-approach
#16
Do Hyun Kim, Yong-Kil Hong, Sin-Soo Jeun, Yong Jin Park, Soo Whan Kim, Jin Hee Cho, Boo-Young Kim, Sung Won Kim
OBJECTIVE: A method of opening the posterior ethmoid air cells with minimal manipulation is important for adequate exposure of the sella floor and minimal nasal morbidity. METHODS: Between February 2009 and August 2016, 373 patients with skull-base tumors underwent surgery via endoscopic endonasal transsphenoidal approach with the 2-nostrils/4-hands technique using this technique. RESULTS: A linear incision was made laterally toward one-third of the superior turbinate along the superior border of the sphenoid sinus ostium...
February 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28228324/extended-maxillotomy-for-skull-base-access-in-contemporary-management-of-chordomas-rationale-and-technical-aspect
#17
Muhammad Fahmi Abdul Jalil, Rowan D Story, Myron Rogers
Minimally invasive approaches to the central skull base have been popularized over the last decade and have to a large extent displaced 'open' procedures. However, traditional skull base surgery still has its role especially when dealing with a large clival chordoma where maximal surgical resection is the principal goal to maximize patient survival. In this paper, we present a case of a 25year-old male patient with chordoma in the inferior clivus which was initially debulked via a transnasal endoscopic approach...
February 19, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28176030/endoscopic-approach-to-the-upper-cervical-spine-and-clivus-an-anatomical-study-of-the-upper-limits-of-the-transoral-corridor
#18
Emanuele La Corte, Philipp R Aldana
BACKGROUND: Recent advances in endoscopic techniques have allowed minimally invasive approaches to the cranio-vertebral junction (CVJ) through the oropharynx (ETA) in addition to the transnasal approach (EEA). These minimally invasive endoscopic techniques allow for increased surgical exposure using no visible incisions, with a potential less morbidity. The ability to know preoperatively the limit of the ETA is vital for the surgical planning in order to better address CVJ pathology. The aim of the present study is to determine the anatomical limits of endoscopic dissection of the skull base and upper cervical spine through the transoral corridor and the superior limit reached by adopting this approach...
February 7, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28133560/endoscopic-resection-of-skull-base-teratoma-in-klippel-feil-syndrome-through-use-of-combined-ultrasonic-and-bipolar-diathermy-platforms
#19
Justin A Edward, Alkis J Psaltis, Ryan A Williams, Gregory W Charville, Robert L Dodd, Jayakar V Nayak
Klippel-Feil syndrome (KFS) is associated with numerous craniofacial abnormalities but rarely with skull base tumor formation. We report an unusual and dramatic case of a symptomatic, mature skull base teratoma in an adult patient with KFS, with extension through the basisphenoid to obstruct the nasopharynx. This benign lesion was associated with midline palatal and cerebral defects, most notably pituitary and vertebrobasilar arteriolar duplications. A multidisciplinary workup and a complete endoscopic, transnasal surgical approach between otolaryngology and neurosurgery were undertaken...
2017: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/28120062/craniovertebral-junction-transanasal-and-transoral-approaches-reconstruct-the-surgical-pathways-with-soft-or-hard-tissue-endocopic-lines-this-is-the-question
#20
Massimiliano Visocchi, Giuseppe Barbagallo, Vincenzo Lorenzo Pascali, Pierpaolo Mattogno, Francesco Signorelli, Gerardo Iacopino, Antonino Germano', Giuseppe La Rocca
A variety of pathological conditions may affect the clivus and the craniovertebral junction (CVJ). These include congenital disorders, chronic inflammation, neoplasms, infections, and posttraumatic conditions that could all result in CVJ compression and myelopathy Endoscopic-assisted procedures have been further developed for CVJ decompression and they have now become conventional approaches. The aims of the present study were:(1) to compare "radiological" and "surgical" nasoaxial lines (NAxLs); (2) to introduce an analogous radiological line as a predictor of the superior extension of the transoral approach (palatine inferior dental arch line (PIA); (3) to compare the "radiological" nasopalatine line (NPL) with the "surgical" NPL (SNPL) and surgical PIA (SPIA); (4) to compare "our" SNPL with the NAxL; and (5) to find possible radiological reference points to predict, preoperatively, the maximal extent of superior dissection for the transoral approach (SPIA)...
2017: Acta Neurochirurgica. Supplement
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