RESUMEN
BACKGROUND: COVID-19 pandemic is thought to have changed the epidemiology of some pediatric neurosurgical disease: among them are the intracranial complications of sinusitis and otitis (ICSO). According to some studies on a limited number of cases, both streptococci-related sinusitis and ICSO would have increased immediately after the pandemic, although the reason is not clear yet (seasonal changes versus pandemic-related effects). The goal of the present survey of the European Society for Pediatric Neurosurgery (ESPN) was to collect a large number of cases from different European countries encompassing the pre-COVID (2017-2019), COVID (2020-2021), and post-COVID period (2022-June 2023) looking for possible epidemiological and/or clinical changes. MATERIAL AND METHODS: An English language questionnaire was sent to ESPN members about year of the event, patient's age and gender, presence of immune-deficit or other favoring risk factors, COVID infection, signs and symptoms at onset, site of primary infection, type of intracranial complication, identified germ, type and number of surgical operations, type and duration of medical treatment, clinical and radiological outcome, duration of the follow-up. RESULTS: Two hundred fifty-four cases were collected by 30 centers coming from 14 different European countries. There was a statistically significant difference between the post-COVID period (129 children, 86 cases/year, 50.7% of the whole series) and the COVID (40 children, 20 cases/year, 15.7%) or the pre-COVID period (85 children, 28.3 cases/year, 33.5%). Other significant differences concerned the presence of predisposing factors/concurrent diseases (higher in the pre-COVID period) and previous COVID infection (higher in the post-COVID period). No relevant differences occurred as far as demographic, microbiological, clinical, radiological, outcome, morbidity, and mortality data were concerned. Paranasal sinuses and middle ear/mastoid were the most involved primary site of infection (71% and 27%, respectively), while extradural or subdural empyema and brain abscess were the most common ICSO (73% and 17%, respectively). Surgery was required in 95% of cases (neurosurgical and ENT procedure in 71% and 62% of cases, respectively) while antibiotics in 99% of cases. After a 12.4-month follow-up, a full clinical and radiological recovery was obtained in 85% and 84% of cases, respectively. The mortality rate was 2.7%. CONCLUSIONS: These results suggest that the occurrence of ICSO was significantly increased after the pandemic. Such an increase seems to be related to the indirect effects of the pandemic (e.g., immunity debt) rather than to a direct effect of COVID infection or to seasonal fluctuations. ICSO remain challenging diseases but the pandemic did not affect the management strategies nor their prognosis. The epidemiological change of sinusitis/otitis and ICSO should alert about the appropriate follow-up of children with sinusitis/otitis.
Asunto(s)
Absceso Encefálico , COVID-19 , Empiema Subdural , Otitis , Sinusitis , Niño , Humanos , Pandemias , COVID-19/complicaciones , Absceso Encefálico/epidemiología , Empiema Subdural/etiología , Sinusitis/complicaciones , Otitis/complicaciones , Otitis/epidemiología , Estudios RetrospectivosRESUMEN
We report the cases of two patients who underwent normofractionated radiotherapy for evolutive asymptomatic parasagittal meningiomas. After completion of radiotherapy, both patients presented severe headache and vomiting episodes without papillar edema. We then decided a "wait-and-scan" strategy because of the slit-ventricles, and symptoms regressed spontaneously. MRI showed significant tumor regression a year after radiotherapy with a newly developed collateral venous drainage system in the first patient and a left, unusually large, superior anastomotic vein in the second. These clinical presentation and radiological evolution are compatible with venous stenosis caused by radiation-induced symptomatic edema, fading after the development of a collateral venous drainage system. The relation between pressure-related headaches and venous anatomy remains unclear in parasagittal meningiomas. These observations underline the importance of the study of venous anatomy when pressure-related headaches are suspected. Further clinical descriptions might help the clinicians to treat these patients' symptoms.
Asunto(s)
Hipertensión Intracraneal , Neoplasias Meníngeas , Meningioma , Humanos , Meningioma/diagnóstico por imagen , Meningioma/radioterapia , Neoplasias Meníngeas/diagnóstico por imagen , Neoplasias Meníngeas/radioterapia , Hipertensión Intracraneal/etiología , Encéfalo/patología , Cefalea/etiologíaRESUMEN
BACKGROUND AND PURPOSE: MCA aneurysms are still commonly clipped surgically despite the recent development of a number of endovascular tools and techniques. We measured clinical uncertainty by studying the reliability of decisions made for patients with middle cerebral artery (MCA) aneurysms. MATERIALS AND METHODS: A portfolio of 60 MCA aneurysms was presented to surgical and endovascular specialists who were asked whether they considered surgery or endovascular treatment to be an option, whether they would consider recruitment of the patient in a randomized trial, and whether they would provide their final management recommendation. Agreement was studied using κ statistics. Intrarater reliability was assessed with the same, permuted portfolio of cases of MCA aneurysm sent to the same specialists 1 month later. RESULTS: Surgical management was the preferred option for neurosurgeons (n = 844/1320; [64%] responses/22 raters), while endovascular treatment was more commonly chosen by interventional neuroradiologists (1149/1500 [76.6%] responses/25 raters). Interrater agreement was only "slight" for all cases and all judges (κ = 0.094; 95% CI, 0.068-0.130). Agreement was no better within specialties or with more experience. On delayed requestioning, 11 of 35 raters (31%) disagreed with themselves on at least 20% of cases. Surgical management and endovascular treatment were always judged to be a treatment option, for all patients. Trial participation was offered to patients 65% of the time. CONCLUSIONS: Individual clinicians did not agree regarding the best management of patients with MCA aneurysms. A randomized trial comparing endovascular with surgical management of patients with MCA aneurysms is in order.
Asunto(s)
Embolización Terapéutica , Procedimientos Endovasculares , Aneurisma Intracraneal , Humanos , Toma de Decisiones Clínicas , Reproducibilidad de los Resultados , Incertidumbre , Aneurisma Intracraneal/diagnóstico por imagen , Aneurisma Intracraneal/cirugía , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento , Estudios RetrospectivosRESUMEN
PURPOSE: The optic nerve (ON) is an extension of the central nervous system via the optic canal to the orbital cavity. It is accompanied by meninges whose arachnoid layer is in continuity with that of the chiasmatic cistern. This arachnoid layer is extended along the ON, delimiting a subarachnoid space (SAS) around the ON. Not all forms of chronic intracranial hypertension (ICH) present papilledema. The latter is sometimes asymmetric, unilateral, or absent. The radiological signs of optic nerve sheath (ONS) dilation, in magnetic resonance imaging, are inconsistent or difficult to interpret. The objective of this study was to analyze the anatomy, the constitution, and the variability of the SAS around the ON in its intraorbital segment to improve the understanding of the pathophysiologic mechanism of asymmetric or unilateral or absent papilledema in certain ICH. METHODS: The study was carried out on nine cadaveric specimens. In four embalmed specimens, macroscopic analysis of the SAS of the ONS were performed, with description about density of the arachnoid trabecular meshwork in three distinct areas (bulbar segment, mid-orbital segment and the precanal segment). In three other embalmed specimens, after staining of SAS by methylene blue (MB), we performed macroscopic analysis of MB progression in the SAS of the ONS. Then, in two non-embalmed specimens, light and electron microscopy (EM) analysis were also done. RESULTS: On the macroscopic level, after staining of SAS, we found in all cases that MB progressed on 16 mm average throughout the SAS of the ONS without reaching the papilla. In four embalmed specimens, in the SAS of the ONS, the density of the arachnoid trabecular meshwork showed inter-individual variability (100%) and intra-individual variability with bilateral variability (50%) and/or variability within the same ONS (88%). On the microscopic level, the arachnoid trabeculae of the ONS are composed of dense connective tissue. The EM perfectly depicted its composition which is mainly of collagen fibers of parallel orientation. CONCLUSION: The variability of the SAS around the ONS probably impacts the symmetrical or asymmetrical nature of papilledema in ICH.
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Hipertensión Intracraneal , Papiledema , Electrones , Humanos , Hipertensión Intracraneal/diagnóstico , Hipertensión Intracraneal/etiología , Hipertensión Intracraneal/patología , Microscopía Electrónica , Nervio Óptico/anatomía & histología , Nervio Óptico/diagnóstico por imagen , Papiledema/patología , Espacio Subaracnoideo/anatomía & histología , Espacio Subaracnoideo/diagnóstico por imagenRESUMEN
PURPOSE: Because of its superficial location in the dorsal regions of the scalp, the greater occipital nerve (GON) can be injured during neurosurgical procedures, resulting in post-operative pain and postural disturbances. The aim of this work is to specify the course of the GON and how its injuries can be avoided while performing posterior fossa approaches. METHODS: This study was carried out at the department of anatomy at Bordeaux University. 4 specimens were dissected to study the GON course. Posterior fossa approaches (midline suboccipital, paramedian suboccipital, retrosigmoid and petrosal) were performed on 4 other specimens to assess potential risks of GON injuries. RESULTS: The GON runs around the obliquus capitis inferior (100%), crosses the semispinalis capitis (100%) and the trapezius (75%) or its aponeurosis (25%). Direct GON injuries can be seen in paramedian suboccipital approaches. Stretching of the GON can occur in midline suboccipital and paramedian suboccipital approaches. We found no evidence of direct or indirect GON injury in retrosigmoid or petrosal approaches. CONCLUSION: Our study provides interesting data regarding the risk GON injury in posterior fossa approaches. Direct GON injuries in paramedian suboccipital approaches can be avoided with careful dissection. Placing retractors in contact with the periosteum and performing a minimal retraction may help to avoid excessive GON stretching in midline suboccipital and paramedian suboccipital approaches. Furthermore, the incision for retrosigmoid approaches should be as lateral as possible and not too caudal. Finally, avoiding extreme patient positioning reduces the risk of GON stretching in all approaches.
Asunto(s)
Cabeza , Nervios Espinales , Disección , Cabeza/anatomía & histología , Humanos , Procedimientos Neuroquirúrgicos/efectos adversos , Nervios Espinales/anatomía & histologíaRESUMEN
Formerly called normal pressure hydrocephalus, communicating chronic hydrocephalus (CCH) is a condition affecting 0.1 to 0.5% of patients over 60years of age. The pathophysiology of this disease is poorly understood, but a defect in cerebrospinal fluid (CSF) resorption appears to be commonly defined as the cause of the neurological disorders. The last important discovery is the description of the glymphatic system and its implication in CCH and CSF resorption. Comorbidities (Alzheimer's disease, microangiopathy, parkinsonism) are very frequent, and involve a diagnostic challenge. The clinical presentation is based on the Hakim and Adams triad, comprising gait disorders, mainly impairing walking, cognitive disorders, affecting executive functions, episodic memory, visuospatial cognition, and sphincter disorders as urinary incontinence (detrusor hyperactivity). The diagnosis is suspected through a set of arguments, combining the clinical presentation, the radiological data of the magnetic resonance imaging (MRI) showing a ventriculomegaly associated with signs of transependymomous resorption of the CSF and disappearance of the cortical sulci, and the clinical response to the depletion of CSF. In the presence of all these elements, or a strong clinical suspicion, the standard treatment will be of a permanent CSF shunt, using a ventriculoatrial or ventriculoperitoneal shunt. The effectiveness of this treatment defines the diagnosis. The clinical improvement is better when treatment occurs early after the onset of the disorders, reaching 75 to 90% of motor improvement.
Asunto(s)
Enfermedad de Alzheimer , Hidrocéfalo Normotenso , Derivaciones del Líquido Cefalorraquídeo , Humanos , Hidrocéfalo Normotenso/diagnóstico , Hidrocéfalo Normotenso/epidemiología , Imagen por Resonancia Magnética , Derivación VentriculoperitonealRESUMEN
BACKGROUND: Patients with syndromic faciocraniosynostosis due to the mutation of the fibroblast growth factor receptor (FGFR) 2 gene present premature fusion of the coronal sutures and of the cranial base synchondrosis. Cerebrospinal fluid (CSF) circulation disorders and cerebellar tonsil prolapse are frequent findings in faciocraniosynostosis. OBJECTIVE: We reviewed the medical literature on the pathophysiological mechanisms of CSF disorders such as hydrocephalus and of cerebellar tonsil prolapse in FGFR2-related faciocraniosynostosis. DISCUSSION: Different pathophysiological theories have been proposed, but none elucidated all the symptoms present in Apert, Crouzon and Pfeiffer syndromes. The first theory that addressed CSF circulation disruption was the constrictive theory (cephalocranial disproportion): cerebellum and brain stem are constricted by the small volume of the posterior fossa. The second theory proposed venous hyperpressure due to jugular foramens stenosis. The most recent theory proposed a pressure differential between CSF in the posterior fossa and in the vertebral canal, due to foramen magnum stenosis.
Asunto(s)
Malformación de Arnold-Chiari/etiología , Malformación de Arnold-Chiari/fisiopatología , Craneosinostosis/complicaciones , Craneosinostosis/genética , Hidrocefalia/etiología , Hidrocefalia/fisiopatología , Receptor Tipo 2 de Factor de Crecimiento de Fibroblastos/genética , Acrocefalosindactilia/genética , HumanosRESUMEN
BACKGROUND: The growth of the posterior fossa in syndromic craniostenosis was studied in many papers. However, few studies described the pathophysiological growth mechanisms in non-operated infants with fibroblast growth factor receptor (FGFR) type 2 mutation (Crouzon, Apert or Pfeiffer syndrome), although these are essential to understanding cranial vault expansion and hydrocephalus treatment in these syndromes. OBJECTIVE: A review of the medical literature was performed, to understand the physiological and pathological growth mechanisms of the posterior fossa in normal infants and infants with craniostenosis related to FGFR2 mutation. DISCUSSION: Of the various techniques for measuring posterior fossa volume, direct slice-by-slice contouring is the most precise and sensitive. Posterior fossa growth follows a bi-phasic pattern due to opening of the petro-occipital, occipitomastoidal and spheno-occipital sutures. Some studies reported smaller posterior fossae in syndromic craniostenosis, whereas direct contouring studies reported no difference between normal and craniostenotic patients. In Crouzon syndrome, synchondrosis fusion occurs earlier than in normal subjects, and follows a precise pattern. This premature fusion in Crouzon syndrome leads to a stenotic foramen magnum and facial retrusion.
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Fosa Craneal Posterior/crecimiento & desarrollo , Fosa Craneal Posterior/patología , Craneosinostosis/genética , Craneosinostosis/patología , Receptor Tipo 2 de Factor de Crecimiento de Fibroblastos/genética , Niño , Preescolar , Humanos , Lactante , Recién Nacido , Mutación , Cráneo/anomalías , SíndromeAsunto(s)
Tratamiento Conservador , Hematoma Epidural Craneal/terapia , Adulto , Traumatismos Craneocerebrales/complicaciones , Femenino , Hematoma Epidural Craneal/diagnóstico por imagen , Hematoma Epidural Craneal/etiología , Humanos , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
BACKGROUND: To depict the outcome of patients with WHO grade III meningioma and identify factors that may influence the survival. MATERIAL AND METHODS: Between 1989 and 2007, a retrospective search identified 43 WHO grade III meningiomas after cases exclusion. Nine patients (39.5%) had a previous history of grade I or II meningioma. The patients underwent a total of 89 surgical resections and 83.7% received radiotherapy. Median follow-up was 7.4 years. RESULTS: At the end of the study, 35 patients were deceased (81.4%). One patient died of disseminated metastatic disease of a papillary meningioma. Median overall survival was 4.1 years, 95% CI [1.9, 8.3]. Overall survival probability at 1, 5 and 10 years were respectively: 81.4%, 95% CI [70.6, 93.9], 48.8%, 95% CI [36,66.3] and, 27.5%, 95% CI [16.9, 44.9]. In univariate Cox regression, a previous surgery for WHO grade I or II meningioma (HR=2.05, 95% CI [1.03, 4.07], P=0.04) and the mitosis count (HR=0.3, 95% CI [0.12, 0.79], P=0.02) were associated with the overall survival. However, neither macroscopic gross total resection (HR=0.87, 95% CI [0.4, 1.87], P=0.71), nor radiotherapy (HR=0.75, 95% CI [0.31, 1.83], P=0.53) was associated with an increased survival. CONCLUSION: This series highlights the poor prognosis associate with the diagnosis of malignant meningioma. Patients with primary WHO grade III meningioma demonstrating less than 14 mitosis per 10 high power fields may live longer. We could not confirm the usefulness of complete resection and adjuvant radiotherapy.
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Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/mortalidad , Meningioma/diagnóstico , Meningioma/mortalidad , Recurrencia Local de Neoplasia/mortalidad , Adulto , Anciano , Femenino , Humanos , Masculino , Neoplasias Meníngeas/terapia , Meningioma/terapia , Persona de Mediana Edad , Recurrencia Local de Neoplasia/diagnóstico , Pronóstico , Radioterapia Adyuvante/métodos , Estudios Retrospectivos , Organización Mundial de la SaludRESUMEN
BACKGROUND: Different surgical methods may be used for the treatment of moyamoya disease. The authors report their experience with pial synangiosis revascularization. MATERIAL AND METHODS: We reviewed the clinical, surgical as well as radiographic records of all patients who underwent cerebral revascularization surgery using pial synangiosis for a symptomatic moyamoya disease at a single institution, Bordeaux University Hospital. RESULTS: Over a 6-year period (2007-2013), 17 procedures were performed in 9 patients. Median age at presentation was 11.5 years and six patients presented with an ischemic event. As regards previous medical history, five patients were diagnosed with moyamoya syndrome. Median time between the symptoms onset and the first surgical procedure was 5.9 months. The average age of the first surgery was 11.8 years and the median time between the two synangiosis was 3.5 months. One patient died of a malignant ischemic stroke after the second procedure. At long-term follow-up, no patient experienced any symptoms recurrence. CONCLUSION: Pial synangiosis typically results in an increase in collaterals from the superficial temporal artery or middle meningeal artery to the brain. It is a simple and effective surgical technique to prevent further moyamoya manifestation and to stabilize or improve the neurologic prognosis.
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Arterias Meníngeas/cirugía , Enfermedad de Moyamoya/cirugía , Accidente Cerebrovascular/complicaciones , Arterias Temporales/cirugía , Adolescente , Angiografía Cerebral/métodos , Revascularización Cerebral/métodos , Niño , Preescolar , Femenino , Humanos , Masculino , Arterias Meníngeas/patología , Enfermedad de Moyamoya/diagnóstico , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
STUDY DESIGN: Case report and review of the literature. OBJECTIVE: To prevent and manage a suspected iatrogenic vertebral artery injury during a cervical spine anterior approach. SUMMARY OF BACKGROUND DATA: The anterior spine approach is a common surgery with few complications. One of the rare but significant risks is vertebral artery injury. Consequences of vertebral artery injuries are often delayed. Therefore, it is essential to prevent this complication and to know how when exploring after a suspected vertebral artery injury. METHODS: Report of a case and review of the literature. A 61-year-old woman presented with a cervical schwannoma involving the C5-C6 foramen. She had undergone surgery 22 years before by the posterior approach. We performed an anterior cervical approach. After 12 days, a vertebral artery pseudo-aneurysm occurred. Our review of the literature is focalized on vertebral artery injuries during cervical surgery by the anterior approach. RESULTS: The patient was treated by coil embolization with a good outcome. To our knowledge, only 6 cases of vertebral artery pseudo-aneurysm after surgery have been reported in the literature. CONCLUSION: According to the literature, vertebral artery pseudo-aneurysms resulting in anterior cervical approach are rare but their consequences could be severe. Prevention begins by detailed surgical planning. Peroperative imaging is helpful. Any suspected vertebral artery injury should postpone a contralateral approach before angiographic imaging.
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Aneurisma Falso/etiología , Aneurisma Falso/prevención & control , Procedimientos Endovasculares/efectos adversos , Neurilemoma/cirugía , Complicaciones Posoperatorias/prevención & control , Neoplasias de la Columna Vertebral/cirugía , Arteria Vertebral , Adulto , Aneurisma Falso/terapia , Vértebras Cervicales/cirugía , Embolización Terapéutica , Femenino , Humanos , Complicaciones Posoperatorias/terapiaAsunto(s)
Aneurisma Roto/etiología , Fístula Arteriovenosa/terapia , Testimonio de Experto/legislación & jurisprudencia , Arteria Hepática , Neurocirugia/legislación & jurisprudencia , Procedimientos Neuroquirúrgicos/efectos adversos , Procedimientos Neuroquirúrgicos/legislación & jurisprudencia , Complicaciones Posoperatorias/terapia , Estenosis Espinal/cirugía , Fístula Arteriovenosa/complicaciones , Francia , HumanosRESUMEN
Subarachnoid hemorrhage (SAH) is a sudden and potentially severe event with mortality rates ranging between 24 and 30 % depending on the initial clinical condition. Studies have attempted to assess the possible influence of meteorological parameters on the occurrence of SAH. However, this idea remains very controversial and the results vary widely from one study to another. Our study is the second largest French series, and first performed in a homogeneous series of patients. The aim of our study was to attempt to establish a relationship between the weather (i.e.) temperature variations and daily variations of atmospheric pressure in the days before the onset of SAH and the same day and the occurrence of non-traumatic SAH in a homogeneous population of 236 patients from a single center, over a period of 7 years (2002 to 2008). This retrospective study does not suggest any relationship between the occurrence of SAH and meteorological data studied. Moreover, no relationship was observed between mean changes in temperature or pressure and the occurrence of SAH, that the day of the bleeding or the days preceding the SAH. However, a female predominance was observed and a relatively high mortality rate of 18.3 %. The distribution of the occurrence of an SAH was random. As it seems impossible to provide logistics and organization of care for non-traumatic SAH, the care system must remain vigilant throughout the year.
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Aneurisma Intracraneal/complicaciones , Hemorragia Subaracnoidea/mortalidad , Adulto , Anciano , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Atención al Paciente , Estudios Retrospectivos , Factores de Riesgo , Caracteres Sexuales , Hemorragia Subaracnoidea/complicaciones , Factores de TiempoRESUMEN
INTRODUCTION: Distinguishing between therapeutic contingency and surgical complication is sometimes not easy, especially when rare events occur. We report the case of a patient who presented with an intraperitoneal haemorrhage following laminectomy and discuss the implications of surgery in this complication. CASE REPORT: A 77-year-old woman, suffering from radiculopathy due to lumbar stenosis underwent a laminectomy in the prone position. On admission she presented with high blood pressure and obesity as significant comorbidities. A few hours after surgery, she collapsed and underwent a thoraco-abdominal CT-scan. The examination revealed a ruptured hepatic artery aneurysm. It is the first case published in the medical literature after lumbar surgery. The aim of this article was to discuss the responsibility of the surgeon and surgery, particularly the surgical positioning of the ruptured aneurysm. CONCLUSION: After reviewing the literature we did not find any evidence to attribute the rupture of this hepatic artery aneurysm to lumbar surgery. This adverse event could be attributed to therapeutic contingency. In cases of patient complaint, this situation depends on national solidarity.
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Aneurisma Roto/cirugía , Constricción Patológica/cirugía , Arteria Hepática/cirugía , Laminectomía/efectos adversos , Complicaciones Posoperatorias , Anciano , Aneurisma Roto/diagnóstico , Aneurisma Roto/etiología , Femenino , Humanos , Radiculopatía/etiología , Resultado del TratamientoAsunto(s)
Fístula Arteriovenosa/diagnóstico por imagen , Fístula Arteriovenosa/cirugía , Cabello/trasplante , Malformaciones Arteriovenosas Intracraneales/diagnóstico por imagen , Malformaciones Arteriovenosas Intracraneales/cirugía , Cuero Cabelludo/irrigación sanguínea , Cuero Cabelludo/cirugía , Adulto , Fístula Arteriovenosa/etiología , Humanos , Malformaciones Arteriovenosas Intracraneales/etiología , Masculino , Tomografía Computarizada por Rayos X/métodos , Resultado del TratamientoRESUMEN
Occipital condylar fractures (OCFs) are rare and difficult to diagnose. The routine use of computed tomography (CT) scan in traumatology has however now made their diagnosis easier, with an estimated frequency of 4 to 19% of craniospinal traumatized patients and 0.4 to 0.7% of all severe traumatized patients in emergencies. This paper describes a patient who was not diagnosed with OCF during his first hospitalization after a road accident. However, 15 days later a left sided hypoglossal nerve palsy occurred. In this case report, we underline that an examination of the cranial nerve is a quick and easy procedure to screen each head trauma patient for occipital foramen fractures. Also, careful attention must be paid to X-Rays, CT scans and magnetic resonance imaging of the craniocervical junction.
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Enfermedades del Nervio Hipogloso/terapia , Hueso Occipital/lesiones , Hueso Occipital/patología , Fracturas Craneales/patología , Accidentes , Humanos , Enfermedades del Nervio Hipogloso/diagnóstico , Enfermedades del Nervio Hipogloso/etiología , Enfermedades del Nervio Hipogloso/patología , Imagen por Resonancia Magnética/métodos , Masculino , Fracturas Craneales/complicaciones , Fracturas Craneales/diagnóstico , Fracturas Craneales/terapiaRESUMEN
Myelodysplastic syndrome (MDS) in children is often associated with chromosomal anomalies and trisomy 8 is a characteristic karyotypic feature in up to 20% of the cases. Behçet disease is a rare multisystem inflammatory disorder characterized by recurrent mouth and genital ulcers. MDS with trisomy 8 has been observed in adult patients with Behçet syndrome with some cases developing prior to the clinical manifestations of the latter. We present a female with a similar association and explain the importance of identifying the coexisting conditions. The immunological abnormalities, which may be observed in MDS and their possible mechanisms, are also discussed.