scholarly journals Endoscopic endonasal extreme far-medial approach for a lower clivus osteochondroma in a patient with hereditary multiple exostoses: illustrative case

2021 ◽  
Vol 1 (20) ◽  
Author(s):  
Yusuke Morinaga ◽  
Hiroyoshi Akutsu ◽  
Hiroyoshi Kino ◽  
Shuho Tanaka ◽  
Hidetaka Miyamoto ◽  
...  
Keyword(s):  
Jugular Foramen ◽  
Neurological Deficits ◽  
Occipital Condyle ◽  
Illustrative Case ◽  
Endoscopic Endonasal ◽  
Hereditary Multiple Exostoses ◽  
Lateral Tilt ◽  
Multiple Exostoses ◽  
The Right ◽  
Lower Clivus

BACKGROUND The authors reported on the use of endoscopic endonasal surgery (EES) for clivus osteochondroma in a patient with hereditary multiple exostoses (HME), a rare pediatric disorder characterized by the formation of osteochondromas adjacent to the growth plates of the axial and appendicular skeletal elements. OBSERVATIONS A 26-year-old man with a family history of HME reported progressive hoarseness and dysphagia over the previous 6 months. He was referred to us after magnetic resonance imaging (MRI) showed a bone tumor in the lower clivus. MRI revealed tumor proliferation in the lower clivus and its extension to the bilateral occipital condyle and jugular tubercle. The hypoglossal canal and jugular foramen were encased on the right side, whereas the medulla oblongata was compressed. The tumor was subtotally resected with EES, and the brainstem was successfully decompressed. The pathological diagnosis was exostoses. Transient postoperative worsening of dysphagia improved within 1 month without other neurological deficits. The patient underwent posterior occipitoaxial fixation 3 months after EES to correct instability and local lateral tilt of the right atlanto-occipital joint. LESSONS The authors’ experience showed that EES is effective for resection of lower clivus osteochondromas, including the cartilaginous cap, and may improve clinical outcomes in patients with HME.

scholarly journals Chordoma of the Craniocervical Junction: Endoscopic Endonasal Approach

2019 ◽  
Vol 80 (S 04) ◽  
pp. S372-S374
Author(s):  
Eduard H. Voormolen ◽  
Pierre Olivier Champagne ◽  
Sebastien Froelich
Keyword(s):  
Craniocervical Junction ◽  
University Hospital ◽  
Neurological Deficits ◽  
Preoperative Imaging ◽  
Classical Type ◽  
Endoscopic Endonasal Approach ◽  
Endonasal Approach ◽  
Endoscopic Endonasal ◽  
Postoperative Mri ◽  
Lower Clivus

Objective This study was aimed to achieve gross-total removal of a chordoma of the craniocervical junction via an endonasal approach (Fig. 1). Design The present study is a case report. Setting The study was conducted at neurosurgical clinic of university hospital. Participant A 40-year-old male, with normal neurologic exam and no prior medical history, presented with a 2-year history of cervicalgia. On preoperative imaging, a midline lesion, with image characteristics of chordoma, was seen in the lower clivus and odontoid. It had limited lateral extension. Main Outcome Measures This study measures postoperative neurological deficits and postoperative tumor volume on magnetic resonance imaging (MRI). Results A binostril approach to the upper nasopharynx was performed using endoscope at 30- and 45-degree angles. Subsequently, a heart-shaped mucosal flap was made and the clivus was drilled to expose the lesion (Fig. 2). After initial debulking, the ring of C1 was slightly drilled to reach the tumor in and around the odontoid. Postoperative MRI showed that a gross-total resection was achieved. The patient had no neurologic deficits postoperatively. Pathologic examination revealed a chordoma of the classical type. Conclusion An endoscopic endonasal approach, utilizing the heart-shaped flap and angled endoscopes and instruments, can be considered for resection of select cases of craniocervical junction chordoma with limited lateral expansion.The link to the video can be found at: https://youtu.be/rwVoZJRBIEo.

scholarly journals AB0775 MANY RARE AND SERIOUS LOCALIZATIONS IN ONE CASE WITH HEREDITARY EXOSTOSES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1414.2-1414
Author(s):  
R. Sarraj ◽  
M. Ghali ◽  
R. Ben Chehida ◽  
G. Mouna ◽  
I. Bejia ◽  
...  
Keyword(s):  
Case Report ◽  
Bone Growth ◽  
Functional Disability ◽  
Axial Skeleton ◽  
Iliac Bone ◽  
Spinal Cord Atrophy ◽  
Hereditary Multiple Exostoses ◽  
Multiple Exostoses ◽  
One Year ◽  
The Right

Background:Multiple hereditary exostoses (MHE) is an inherited disorder of bone growth. People who have MHE grow exostoses or boney bumps on their bones that vary in size, location and number. Some localizations are rare but potentially serious such as ribs, spine or pelvis due to proximity to important structures.Objectives:The aim of this case report was to illustrate the clinical and radiographic features of some rare localizations of MHE.Methods:We are reporting a rare case of a thirty one-year-old male with severe localizations of MHE.Results:A thirty one-year-old male affected by hereditary multiple exostoses., not followed up, came to our consultation complaining about chest pain. On examination, he walks with a limp because of the unequalness of his limbs.The osteoarticular examination revealed numerous palpable osseous masses at the distal and proximal end of the 2 arms, 2 forearms, 2 thighs and 2 legs and at the right iliac wing. Neurological examination revealed vivid deep tendon reflexes, bilateral positive Hoffmann’sign and Babinski reflex associated with right hemiparesis. The pulmonary exam was normal. Medullary MRI showed medullary compression with spinal cord atrophy and sign of myelopathy of c1 c2 segment. The ct –scan revealed multiple exostosis, the two largest lesions were located in the second rib measuring 45mm * 35mm axially and 52mm in height compromising blood vessels, and in the right iliac bone with a retroperitoneal growing mass measuring 126 * 94 mm axially and 100 mm in height leading to a serious entrapment of the axial skeleton and the abdominal organs. It also confirmed thickening of the cartilage cap of the right iliac bone suggestive of malignant chondrosarcomatous transformation.Conclusion:Exostoses are the most common benign bone tumors. Its complications are rare but can cause a functional disability or even a vital risk.References:[1]Eke GK, Omunakwe HE, Echem RC. Hereditary multiple exostoses in a 15-year-old boy: A case report and review of literature. Niger J Paediatr. 2016;43(4):295.Disclosure of Interests:None declared

scholarly journals Morphometric Study of the Jugular Foramen in Northwest Indian Population

2012 ◽  
Vol 46 (4) ◽  
pp. 165-171 ◽  
Author(s):  
Anjali Aggarwal ◽  
Tulika Gupta ◽  
Harjeet Kaur ◽  
Anjali Singla ◽  
Daisy Sahni
Keyword(s):  
Indian Population ◽  
Bone Growth ◽  
Anatomical Variations ◽  
Styloid Process ◽  
Jugular Foramen ◽  
Occipital Condyle ◽  
Morphometric Study ◽  
Carotid Canal ◽  
Jugular Fossa ◽  
The Right

ABSTRACT Context Most of the approaches for skull base surgeries are designed to drill the bone around the jugular foramen for proper exposure. In order to achieve this, an understanding of normal morphometric dimensions of jugular foramen is necessary. Aim To conduct the morphometric analysis and anatomical variations of jugular foramen (JF) in Northwest Indian population. Settings and design Anatomic study using human skulls. Materials and methods Anteroposterior and mediolateral diameter of jugular foramen, depth (if domed) and width of jugular fossa were measured with the help of digital vernier caliper. Statistical analysis used The mean, standard deviation (SD) and range of each dimension was computed. A comparison between right and left jugular foramina was made by using student's t-test. Results Mediolateral diameter of jugular foramen and width of jugular fossa was significantly higher on the right side. An abnormal unilateral blockage of jugular foramen by a bone growth converting it into a slit was noted with anteroposterior (AP) diameter of 2.37 mm in one skull. Bilateral complete septation of jugular foramen into two and three compartments was observed in 2 (4%) and 3 (6%) of the skulls respectively. The domed bony roof was noticed in 66% of the skulls on both the sides. Conclusion The observed variations of JF are possibly due to constitutional, racial or genetic factors. Knowledge of the observed variations of this foramen may be important for neurosurgeons, radiologists and anthropologists. Key message The total subdivision of jugular foramen is not common in our environment. The jugular foramen is generally larger on right side. This study supports reported morphometric variations of jugular foramen, besides adding data on the Northwest Indian population. Abbreviation CC: carotid canal; BO: basi-occiput; OC: occipital condyle; JF: jugular foramen; S: styloid process. How to cite this article Singla A, Sahni D, Aggarwal A, Gupta T, Kaur H. Morphometric Study of the Jugular Foramen in Northwest Indian Population. J Postgrad Med Edu Res 2012;46(4):165-171.

Posttraumatic Delayed Jugular Foramen Syndrome in a Toddler

2021 ◽  
Author(s):  
Cristina Toledo-Gotor ◽  
Nerea Gorría ◽  
Miren Oscoz ◽  
Katia Llano ◽  
Pablo la Fuente Rodríguez-de ◽  
...  
Keyword(s):  
Physical Examination ◽  
Cranial Nerves ◽  
Jugular Foramen ◽  
Exceptional Case ◽  
Cerebellar Tonsil ◽  
Occipital Condyle ◽  
Lower Cranial Nerve ◽  
Cranial Nerve Palsies ◽  
Blunt Head Trauma ◽  
Condyle Fracture

Abstract Background Multiple lower cranial nerve palsies have been attributed to occipital condyle fractures in older children and adults, but no clinical details of other possible mechanisms have been described in infants. Case Report A 33-month-old boy suffered blunt head trauma. A bilateral skull base fracture was diagnosed, with favorable outcome during the first days after trauma. On the sixth day, the patient began to refuse drinking and developed hoarseness. Physical examination and additional investigations revealed paralysis of left VII, IX, X, and XI cranial nerves. A follow-up computed tomography (CT) scan disclosed a left petrous bone fracture involving the lateral margin of the jugular foramen, and a cranial magnetic resonance imaging (MRI) study showed a left cerebellar tonsil contusion. He improved after methylprednisolone was started. Three months later, he was asymptomatic, although mild weakness and atrophy of the left sternocleidomastoid and trapezius muscles remained 1 year later. Discussion A posttraumatic “jugular foramen syndrome” is rare in children, but it has been reported shortly after occipital condyle fracture, affecting mainly IX, X, and XI cranial nerves. In this toddler, delayed symptoms appeared with unilateral involvement. While an occipital fracture was ruled out, neuroimaging findings suggest the hypothesis of a focal contusion as a consequence of a coup-contrecoup injury. Conclusion This exceptional case highlights the importance of gathering physical examination, anatomical correlation, and neuroimaging to yield a diagnosis.

scholarly journals Gasperini Syndrome: A Case Report and Systematic Review and Proposing a New Definition

2020 ◽  
Vol 12 (3) ◽  
pp. 433-439
Author(s):  
Riwaj Bhagat ◽  
Siddharth Narayanan ◽  
Marwa Elnazeir ◽  
Thong Diep Pham ◽  
Robert Paul Friedland ◽  
...  
Keyword(s):  
Systematic Review ◽  
Brain Mri ◽  
Cranial Nerves ◽  
Clinical Manifestations ◽  
The Other ◽  
Neurological Deficits ◽  
Brainstem Stroke ◽  
Neurological Features ◽  
The Right ◽  
Core Features

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V–VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V–VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg’s syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.

scholarly journals RARE-29. PRIMARY CENTRAL NERVOUS SYSTEM NON-HODGKIN LYMPHOMA IN AN 11-YEAR-OLD BOY: A CASE REPORT

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii448-iii448
Author(s):  
Jorge Luis Ramírez-Melo ◽  
Regina M Navarro-Martin del Campo ◽  
Manuel D Martinez-Albarran ◽  
Fernando Sánchez-Zubieta ◽  
Ana L Orozco-Alvarado ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hodgkin Lymphoma ◽  
Central Nervous System Lymphoma ◽  
Complete Response ◽  
High Dose ◽  
Illustrative Case ◽  
Tumor Biopsy ◽  
Non Hodgkin Lymphoma ◽  
The Right

Abstract BACKGROUND Primary central nervous system lymphoma (PCNSL) are very rare in children. CLINICAL CASE: An 11-year-old male presented with a 2 months history with myoclonic movements in the upper right limb, and a sudden frontal headache, gait disturbance due to right hemiparesis and an ipsilateral convulsive episode. Upon admission he had critical condition, with hypertensive skull syndrome, Glasgow of 12, Karnofsky 40%, right hemiparesis, swallowing disorder, facial paralysis, and loss of photo motor reflex and unilateral amaurosis. A CT and MRI showed a huge tumor mass in the left tempo-parietal region, infiltrating the white matter and shifting the midline. A Tumor biopsy was done, and reported diffuse small cell non-Hodgkin lymphoma of high-grade, Burkitt type. Systemic lymphoma workup was negative. He received six cycles of chemotherapy based on high dose methotrexate, rituximab and triple intrathecal.After the second cycle an ophthalmologic evaluation was done, and found infiltration to the right retina, for which 6 cycles of intra vitreous chemotherapy with methotrexate were applied, he showed an excellent response, and recovered all his neurological functions except that right hemianopia persist. Control MRI showed partial response at 2nd cycle and complete response after the 4th cycle. No Radiation was performed. CONCLUSION This report highlights the fact that pediatric PCNSL may be effectively treated by a combination of HDMTX and rituximab-based chemoimmunotherapy without irradiation. Lack of awareness of this rare entity may lead to extense resections of brain, and potential permanent secuelae that were avoided in this illustrative case.

The Infratemporal Retro-Eustachian Transposition of the Temporoparietal Fascial Flap for Clival Reconstruction After Endoscopic Endonasal Approach: An Anatomic Conceptual Technique

2021 ◽  
Author(s):  
Giulio Cecchini ◽  
Huy Q Truong ◽  
Francesco Di Biase ◽  
Antonio Musio ◽  
Juan C Fernandez Miranda
Keyword(s):  
Infratemporal Fossa ◽  
Surgical Techniques ◽  
Pedicled Flap ◽  
Reconstruction Technique ◽  
Clinical Validation ◽  
Endoscopic Endonasal Approach ◽  
Endoscopic Endonasal ◽  
Fascial Flap ◽  
The Times ◽  
Lower Clivus

Abstract BACKGROUND Reconstruction after endoscopic endonasal approaches is a key element. Lower clivus reconstruction is difficult and most of the times a pedicled flap is not available. As the complexity and the dimensions of the exposure increase, a reliable reconstruction technique becomes more and more important. OBJECTIVE To describe the anatomic and technical nuances of the transposition of the temporoparietal fascial flap for lower clivus reconstruction. METHODS A specific temporoparietal fascial flap (TPFF) design and tunneling technique has been studied using 4 head specimens, microscopic and endoscopic surgical techniques, and neuronavigation. RESULTS The L-shaped flap offers several advantages. It can be tunneled directly toward the lower clivus passing through the infratemporal fossa. CONCLUSION The infratemporal retro-eustachian transposition of an L-shaped TPFF provides a vascularized tissue virtually without dimension limits. This is the only technique that allows the flap to be tunneled directly in the lower clivus with the most vascular portion being at the bottom of the defect. Clinical validation is still required since more issues may become relevant in a real-surgery setting. Though, due to its possible complications, this methodology needs further testing and should not be attempted in less experienced hands.

scholarly journals Ruptured Bilateral Pericallosal Artery Aneurysm in Mirror Position: Case Report

2018 ◽  
Vol 37 (03) ◽  
pp. 263-266
Author(s):  
Lucas Meguins ◽  
Linoel Valsechi ◽  
Ronaldo Fernandes ◽  
Dionei Morais ◽  
Antonio Spotti
Keyword(s):  
Case Report ◽  
Subarachnoid Hemorrhage ◽  
External Ventricular Drain ◽  
Artery Aneurysm ◽  
Operative Management ◽  
Neurological Deficits ◽  
Pericallosal Artery ◽  
Digital Subtraction Arteriography ◽  
The Many ◽  
The Right

Introduction Pericallosal artery (PA) aneurysms represent 2 to 9% of all intracranial aneurysms, and their management remains difficult. Objective The aim of the present study is to describe the case of an adult woman with subarachnoid hemorrhage and bilateral PA aneurysm in mirror position. Case Report A 46-year-old woman was referred to our institution 20 days after a sudden severe headache. She informed that she was treating her arterial hypertension irregularly, and consumed ∼ 20 cigarettes/day. The patient was neurologically intact at admission. A non-contrast computed tomography (CT) on the first day of the onset of the symptoms revealed hydrocephaly and subarachnoid hemorrhage (Fisher III). An angio-CT/digital subtraction arteriography showed bilateral PA aneurysms in mirror position. The patient was successfully treated with surgery via the right interhemispheric approach (because the surgeon is right-handed); the surgeon performed the proximal control with temporary clipping, and introduced an external ventricular drain at the end of the surgery. The patient was discharged on the fourth postoperative day without any additional neurological deficits or ventricular shunts. Conclusion Ruptured PA aneurysm is a surgically challenging aneurysm due to the many anatomical nuances and risk of rebleeding. However, the operative management of ruptured bilateral PA aneurysms is feasible and effective.

scholarly journals Cerebellar peduncle abscess secondary to disseminated strangles in a six-week-old miniature foal

2011 ◽  
Vol 1 (1) ◽  
pp. 12
Author(s):  
Brianne Henderson
Keyword(s):  
Animal Health ◽  
Clinical Signs ◽  
Acute Onset ◽  
Abscess Formation ◽  
Neurological Deficits ◽  
Post Mortem ◽  
Streptococcus Equi ◽  
The Central Nervous System ◽  
Cerebellar Peduncle ◽  
The Right

During a strangles outbreak within a herd of minature horses, a six week old foal developed acute onset clinical signs of sepsis and neurological deficits. The foal was euthanized and submitted for post-mortem at the Animal Health Laboratories, Guelph Ontario. Gross <em>post-mortem</em> examination noted severe bronchopneumonia, hypopyon of the right eye and a singular cerebellar peduncle abscess. Culture of the lungs and cerebellum produced a pure growth of <em>Streptococcus equi</em> ssp. <em>equi</em>. <em>Streptococcus equi</em> ssp. <em>equi</em>, the causative agent of equine strangles, produces an acute pyrexia, purulent lymphadenopathy of submandibular and retropharyngeal lymph nodes. Commonly, lymph node abscesses rupture and resolve without complication. Rarely, complications may include: dissemination of the bacteria with diffuse abscess formation, immune mediated disease (purpura haemorrhagica), rarely abscess formation within the central nervous system (CNS) can occur. These can be managed medically with appropriate antibiotics and drugs to reduce intra-cranial pressure, however surgical drainage and debulking of the abscess has been attempted successfully in a few cases.

Sign in / Sign up

Export Citation Format

Share Document