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1.
Pituitary ; 27(1): 70-76, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38006472

RESUMEN

PURPOSE: Pituitary transposition is a novel surgical approach to access the retroinfundibular space and interpeduncular cistern. Few studies have evaluated post-surgical outcomes, including incidence of hyponatremia, following pituitary transposition. METHODS: This is a retrospective study including 72 patients who underwent endoscopic endonasal surgery involving pituitary transposition for non-pituitary derived tumors over a decade at the University of Pittsburgh Medical Center. Anterior pituitary deficiencies and replacement therapy, tumor pathology and pre-operative serum sodium (Na) were recorded. Na was assessed at postoperative day 1, 3, 5, 7, and 10. Anatomical/surgical parameters included sellar height, sellar access angle to approach the tumor, and cranial extension of the tumor above the sellar floor (B) compared to the height of the gland (A) (B/A). T-test (normally distributed variables) and Wilcoxon rank-sum test (not-normally distributed) were applied for mean comparison. Logistic regression analyzed correlations between anatomical/surgical parameters and postoperative hyponatremia. RESULTS: 55.6% of patients developed post-operative transient hyponatremia. Two patients (5%) developed severe hyponatremia (sodium level < 120 mmol/L). Eleven (15.3%) patients required desmopressin replacement immediately post-operatively, and 2 other patients needed desmopressin after discharge and after sodium nadir developed. Hyponatremia was inversely associated with sellar access angle (p = 0.02) and the tumor cranial extension above the sellar floor showing a trend towards significance (p = 0.09). CONCLUSION: More than half of patients who had pituitary transposition developed transient hyponatremia. Hyponatremia was more common in those with narrower sellar access angle and smaller cranial extension of the tumor above the sellar floor. Anatomical/surgical parameters may allow risk-stratification for post-operative hyponatremia following pituitary transposition.


Asunto(s)
Hiponatremia , Neoplasias , Enfermedades de la Hipófisis , Neoplasias Hipofisarias , Humanos , Hiponatremia/epidemiología , Hiponatremia/etiología , Estudios Retrospectivos , Incidencia , Desamino Arginina Vasopresina/uso terapéutico , Base del Cráneo/patología , Sodio , Neoplasias Hipofisarias/patología
2.
Eur Arch Otorhinolaryngol ; 281(3): 1221-1229, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37668755

RESUMEN

PURPOSE: While extensive research with accurate classification has been done in mycoses of the paranasal sinuses and anterior skull base, a similar understanding of lateral skull base fungal pathologies is lacking due to relative rarity and diagnostic difficulties. We introduce a series of eleven cases and two different invasive entities of Aspergillus temporal bone diseases-fungal skull base osteomyelitis (SBO)/malignant otitis externa (MOE) and chronic invasive granulomatous fungal disease (CIGFD). METHODOLOGY: A retrospective observational study was conducted at the neuro-otology unit of a tertiary care referral center between July 2017 and November 2022. Diagnosed cases of lateral skull base osteomyelitis with atypical symptoms and lack of response to culture-directed antibiotics were evaluated for fungal origin. Patient data, including history, laboratory findings, serum galactomannan assay, CT and MRI imaging findings, clinical examination findings, and co-morbidities, were analyzed. The treatment course and response were assessed. RESULTS: A total of 11 cases were included in the study. Of these, 9 were cases of Aspergillus-induced skull base osteomyelitis (SBO) and 2 of Aspergillus-induced chronic invasive granulomatous fungal disease (CIGFD). CIGFD presented with persistent ear discharge and slowly progressive post-aural swelling, while all patients of fungal SBO had lower cranial nerve palsies. CIGFD responded to excision and antifungals, while SBO responded well to conservative anti-fungal treatment. CONCLUSION: In cases of lateral SBO not responding to antibiotic therapy, the possibility of fungal etiology should be considered. Aspergillus spp. seems to be the major fungal pathogen.


Asunto(s)
Aspergilosis , Micosis , Osteomielitis , Otitis Externa , Humanos , Base del Cráneo/diagnóstico por imagen , Base del Cráneo/patología , Aspergilosis/diagnóstico , Aspergilosis/tratamiento farmacológico , Micosis/diagnóstico , Otitis Externa/patología , Osteomielitis/diagnóstico , Osteomielitis/tratamiento farmacológico
3.
Eur Arch Otorhinolaryngol ; 281(5): 2755-2759, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38381152

RESUMEN

BACKGROUND: Osteoradionecrosis (ORN) of the skull base is a rare complication after head and neck radiation with a broad variety of subsequent complications. METHODS: A 68-year-old woman with a complex oncological history (right-sided sphenoid meningioma; left-sided neck metastasis of a Cancer of Unknown Primary-CUP) was admitted with a right-sided epi-/ oropharyngeal mass and severe pain exacerbations for further evaluation. CT scan revealed an advanced ORN of the skull base with subsequent abruption of the ventral part of the clivus. This dislocated part of the clivus wedged in the oropharynx for 48 h and then moved towards the larynx, resulting in dyspnea and almost complete airway obstruction. RESULTS: Due to the dangerous airway situation, an urgent exploration and removal of the dislocated clivus was necessary. After a potential cervical spine instability was ruled out, the patient's airway was initially secured with an awake tracheotomy and the clivus was removed transorally. The tracheostomy tube was removed during the ongoing inpatient stay, and the patient was discharged with significant pain relief. CONCLUSIONS: The present case illustrates an orphan complication of skull base ORN resulting in a major airway emergency situation.


Asunto(s)
Laringe , Osteorradionecrosis , Femenino , Humanos , Anciano , Osteorradionecrosis/diagnóstico por imagen , Osteorradionecrosis/etiología , Osteorradionecrosis/cirugía , Base del Cráneo/diagnóstico por imagen , Base del Cráneo/patología , Fosa Craneal Posterior , Laringe/patología , Dolor
4.
HNO ; 72(4): 265-271, 2024 Apr.
Artículo en Alemán | MEDLINE | ID: mdl-38393669

RESUMEN

BACKGROUND: Extended endoscopic endonasal surgery (EEES) is an essential part of treatment of various pathologies of the anterior skull base. In addition to significant improvements in the quality of life of affected patients and a lower complication profile compared to open skull base surgery, the therapeutic results are comparable if the indications are correct. MATERIALS AND METHODS: Data of all endoscopic endonasal skull base procedures performed at the University Skull Base Center Hamburg under the direction of the Department of Otorhinolaryngology between June 2018 and November 2022 were retrospectively collected. RESULTS: A total of 50 cases were identified. Of these, 56% (28/50) were malignant tumors, 24% (12/50) were benign pathologies with direct skull base involvement, and 20% (10/50) were anterior skull base defects with rhinoliquorrhea. In 96% (48/50) of cases, the preoperatively set goal of surgery (representative biopsy, complete resection, closure of the skull base defect) could be achieved. Complications grade III or higher according to Clavien-Dindo occurred in 4/50 cases. During the observation period, n = 5 olfactory neuroblastomas were diagnosed, all of which were exclusively and successfully operated on endoscopically. CONCLUSION: In recent years, the spectrum of endoscopically resectable pathologies of the anterior skull base has steadily expanded. In particular, midline-related tumors such as olfactory neuroblastoma or iatrogenic/idiopathic skull base defects with cerebrospinal fluid rhinorrhea are treated completely endoscopically with very good results. Nevertheless, there are also limitations to this technique. Due to high variance in the scope of frontobasal surgery, the extent, and the complex anatomy, as well as the overlapping responsibilities of the specialist disciplines, establishment of certified skull base centers and bundling of frontobasal surgery at these centers is highly relevant for quality assurance.


Asunto(s)
Rinorrea de Líquido Cefalorraquídeo , Calidad de Vida , Humanos , Estudios Retrospectivos , Base del Cráneo/cirugía , Base del Cráneo/patología , Endoscopía/métodos , Rinorrea de Líquido Cefalorraquídeo/etiología , Rinorrea de Líquido Cefalorraquídeo/patología , Rinorrea de Líquido Cefalorraquídeo/cirugía
5.
Fetal Pediatr Pathol ; 43(3): 251-256, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38345043

RESUMEN

BACKGROUND: Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl. CASE REPORT: A 12-year-old female with sickle cell disease and Jeavons syndrome presented with seizures. Imaging and partial resection revealed an expansile benign granular cell tumor (GCT) involving the sphenoid body, pterygoid process, and central skull base. The disease has remained stable after 36-month follow up. DISCUSSION: GCT primarily involving the osseous sphenoid/skull base has not been previously reported in a child. Although mostly benign, some are aggressive, with malignant transformation in 1-2%. Surgery is the mainstay of treatment, but in the skull base this may be limited by adjacent critical structures. Decision-making is guided by anatomic extent, histology, and clinical behavior.


Asunto(s)
Tumor de Células Granulares , Neoplasias de la Base del Cráneo , Hueso Esfenoides , Humanos , Femenino , Niño , Tumor de Células Granulares/patología , Tumor de Células Granulares/diagnóstico , Tumor de Células Granulares/cirugía , Hueso Esfenoides/patología , Neoplasias de la Base del Cráneo/patología , Neoplasias de la Base del Cráneo/diagnóstico , Neoplasias de la Base del Cráneo/cirugía , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/patología , Base del Cráneo/patología , Base del Cráneo/diagnóstico por imagen
6.
No Shinkei Geka ; 52(2): 327-334, 2024 Mar.
Artículo en Japonés | MEDLINE | ID: mdl-38514122

RESUMEN

With the development of endoscopic and peripheral instruments, endonasal or transcranial endoscopic surgery for skull-base tumors has become more common. Preoperative simulation makes it relatively easy to understand the anatomical relationship between skull base tumors and the surrounding vital structures, which vary with each case. This may lead to the avoidance of complications and an improvement in the removal rate. Especially in cases of skull base tumors where multiple surgical approaches are possible, the three-dimensional model can be used to confirm the surgical field for each approach and consider the most appropriate. With the development of endovascular treatment and radiotherapy, experience in craniotomy has decreased. Young neurosurgeons need to develop skills to learn as efficiently as possible from their limited experience. Therefore, it is extremely useful to provide an environment that allows for easier preoperative simulations.


Asunto(s)
Neoplasias de la Base del Cráneo , Humanos , Neoplasias de la Base del Cráneo/cirugía , Neoplasias de la Base del Cráneo/patología , Endoscopía/métodos , Nariz/cirugía , Craneotomía , Base del Cráneo/cirugía , Base del Cráneo/patología
7.
No Shinkei Geka ; 52(2): 320-326, 2024 Mar.
Artículo en Japonés | MEDLINE | ID: mdl-38514121

RESUMEN

Preoperative simulation images creates an accurate visualization of a surgical field. The anatomical relationship of the cranial nerves, arteries, brainstem, and related bony protrusions is important in skull base surgery. However, an operator's intention is unclear for a less experienced neurosurgeon. Three-dimensional(3D)fusion images of computed tomography and magnetic resonance imaging created using a workstation aids precise surgical planning and safety management. Since the simulation images allows to perform virtual surgery, a déjà vu effect for the surgeon can be obtained. Additionally, since 3D surgical images can be used for preoperative consideration and postoperative verification, discussion among the team members is effective from the perspective of surgical education for residents and medical students. Significance of preoperative simulation images will increase eventually.


Asunto(s)
Neoplasias de la Base del Cráneo , Base del Cráneo , Humanos , Base del Cráneo/diagnóstico por imagen , Base del Cráneo/cirugía , Base del Cráneo/patología , Imagenología Tridimensional/métodos , Neoplasias de la Base del Cráneo/cirugía , Tomografía Computarizada por Rayos X/métodos , Procedimientos Neuroquirúrgicos/métodos , Imagen por Resonancia Magnética/métodos
8.
Genesis ; 61(1-2): e23509, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36622051

RESUMEN

Craniofacial anomalies (CFAs) are a diverse group of disorders affecting the shapes of the face and the head. Malformation of the cranial base in humans leads CFAs, such as midfacial hypoplasia and craniosynostosis. These patients have significant burdens associated with breathing, speaking, and chewing. Invasive surgical intervention is the current primary option to correct these structural deficiencies. Understanding molecular cellular mechanism for craniofacial development would provide novel therapeutic options for CFAs. In this study, we found that enhanced bone morphogenetic protein (BMP) signaling in cranial neural crest cells (NCCs) (P0-Cre;caBmpr1a mice) causes premature fusion of intersphenoid synchondrosis (ISS) resulting in leading to short snouts and hypertelorism. Histological analyses revealed reduction of proliferation and higher cell death in ISS at postnatal day 3. We demonstrated to prevent the premature fusion of ISS in P0-Cre;caBmpr1a mice by injecting a p53 inhibitor Pifithrin-α to the pregnant mother from E15.5 to E18.5, resulting in rescue from short snouts and hypertelorism. We further demonstrated to prevent premature fusion of cranial sutures in P0-Cre;caBmpr1a mice by injecting Pifithrin-α through E8.5 to E18.5. These results suggested that enhanced BMP-p53-induced cell death in cranial NCCs causes premature fusion of ISS and sutures in time-dependent manner.


Asunto(s)
Anomalías Craneofaciales , Base del Cráneo , Proteínas Morfogenéticas Óseas/metabolismo , Cresta Neural/metabolismo , Cresta Neural/patología , Proliferación Celular , Proteína p53 Supresora de Tumor/antagonistas & inhibidores , Proteína p53 Supresora de Tumor/metabolismo , Masculino , Femenino , Animales , Ratones , Animales Recién Nacidos , Transducción de Señal , Apoptosis , Condrocitos/metabolismo , Proteínas Smad/metabolismo , Unión Proteica , Anomalías Craneofaciales/metabolismo , Anomalías Craneofaciales/patología , Embarazo , Base del Cráneo/anomalías , Base del Cráneo/metabolismo , Base del Cráneo/patología , Hipertelorismo/metabolismo , Hipertelorismo/patología
9.
J Vasc Surg ; 77(2): 523-528, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36087829

RESUMEN

OBJECTIVE: In the present study, we aimed to confirm the findings reported by Kim et al. They stated that the tumor's distance to the base of the skull was predictive of injury to the cranial nerves and their branches during carotid body tumor resection in an Austrian cohort. METHODS: In the present retrospective observational trial, we included all consecutive patients who had been discharged from our tertiary care teaching hospital with the diagnosis of a carotid body tumor (CBT) between January 2004 and December 2019. Tumor-specific parameters were measured from the preoperative contrast-enhanced computed tomography or magnetic resonance imaging studies. Patient-specific data were obtained from the patients' medical records. The effect of these parameters on the occurrence of cranial nerve injuries was calculated using univariate logistic regression analysis. Parameters significant on univariate analysis were included in a multivariate model. RESULTS: A total of 48 CBTs had been resected in 43 patients (29 women [67.4%] and 14 men [32.6%]), with a mean age of 55.6 years (95% confidence interval, 51.8-58.5). The mean distance to the base of the skull was 43.2 mm (95% confidence interval, 39.9-46.5). A total of 18 injuries to the cranial nerves and their branches in 10 CBTs were detected. The tumor-specific parameters that were significant on univariate analysis were the distance to the base of the skull (P = .009), craniocaudal tumor diameter (P = .027), and tumor volume (P = .036). Stepwise multivariate logistic regression analysis revealed that the distance to the base of the skull was the only parameter that remained statistically significant. CONCLUSIONS: We found that the distance to the base of the skull is a highly predictive parameter for injuries to the cranial nerves and their branches during CBT resection and should be included in the surgical risk assessment and patient information.


Asunto(s)
Tumor del Cuerpo Carotídeo , Traumatismos del Nervio Craneal , Masculino , Humanos , Femenino , Persona de Mediana Edad , Estudios Retrospectivos , Tumor del Cuerpo Carotídeo/diagnóstico por imagen , Tumor del Cuerpo Carotídeo/cirugía , Resultado del Tratamiento , Traumatismos del Nervio Craneal/etiología , Base del Cráneo/diagnóstico por imagen , Base del Cráneo/cirugía , Base del Cráneo/patología
10.
Histopathology ; 82(3): 420-430, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36217885

RESUMEN

De-differentiated chordoma is defined as a high-grade sarcoma lacking notochordal differentiation, which arises in association with conventional chordoma. The mechanism underlying de-differentiation remains unclear. We immunohistochemically investigated trimethylation at lysine 27 of histone 3 (H3K27me3) in nine de-differentiated chordomas. The tumours occurred at the skull base (n = 5) or the sacrum (n = 4) in four men and five women with a median age of 50 years. De-differentiation occurred de novo in four cases and at recurrence/metastasis in five cases. Five tumours retained H3K27me3, whereas four showed complete loss of H3K27me3 only in the de-differentiated component, while the conventional chordoma component retained H3K27me3. All the H3K27me3-negative tumours showed co-loss of dimethylation at H3K27 (H3K27me2), consistent with inactivation of polycomb repressive complex 2. Two genetically analysed H3K27me3-negative tumours harboured EED homozygous deletions. All four H3K27me3-negative de-differentiated chordomas affected the skull base of young or middle-aged women. Unlike dense proliferation of highly pleomorphic spindle or epithelioid cells in the H3K27me3-positive de-differentiated chordomas, all H3K27me3-negative tumours displayed swirling fascicles of relatively uniform spindle cells with alternating cellularity and perivascular accentuation, resembling malignant peripheral nerve sheath tumour (MPNST). Rhabdomyoblastic differentiation was present in one H3K27me3-negative tumour. We identified a novel group of de-differentiated chordomas in the skull base that lost H3K27me3/me2 only in the de-differentiated component, which was associated with EED homozygous deletion and MPNST-like histology. Our data suggest a distinct 'polycomb-type' de-differentiation pathway in chordoma, similar to a recently described de-differentiated chondrosarcoma with H3K27me3 loss.


Asunto(s)
Neoplasias Óseas , Cordoma , Neurofibrosarcoma , Persona de Mediana Edad , Masculino , Humanos , Femenino , Histonas/metabolismo , Cordoma/genética , Neurofibrosarcoma/metabolismo , Homocigoto , Biomarcadores de Tumor/análisis , Metilación de ADN , Eliminación de Secuencia , Diferenciación Celular , Neoplasias Óseas/metabolismo , Base del Cráneo/química , Base del Cráneo/metabolismo , Base del Cráneo/patología
11.
Strahlenther Onkol ; 199(2): 160-168, 2023 02.
Artículo en Inglés | MEDLINE | ID: mdl-36149438

RESUMEN

BACKGROUND: This study aimed to compare the results of irradiation with protons versus irradiation with carbon ions in a raster scan technique in patients with skull base chordomas and to identify risk factors that may compromise treatment results. METHODS: A total of 147 patients (85 men, 62 women) were irradiated with carbon ions (111 patients) or protons (36 patients) with a median dose of 66 Gy (RBE (Relative biological effectiveness); carbon ions) in 4 weeks or 74 Gy (RBE; protons) in 7 weeks at the Heidelberg Ion Beam Therapy Center (HIT) in Heidelberg, Germany. The median follow-up time was 49.3 months. All patients had gross residual disease at the beginning of RT. Compression of the brainstem was present in 38%, contact without compression in 18%, and no contact but less than 3 mm distance in 16%. Local control and overall survival were evaluated using the Kaplan-Meier Method based on scheduled treatment (protons vs. carbon ions) and compared via the log rank test. Subgroup analyses were performed to identify possible prognostic factors. RESULTS: During the follow-up, 41 patients (27.9%) developed a local recurrence. The median follow-up time was 49.3 months (95% CI: 40.8-53.8; reverse Kaplan-Meier median follow-up time 56.3 months, 95% CI: 51.9-60.7). No significant differences between protons and carbon ions were observed regarding LC, OS, or overall toxicity. The 1­year, 3­year, and 5­year LC rates were 97%, 80%, and 61% (protons) and 96%, 80%, and 65% (carbon ions), respectively. The corresponding OS rates were 100%, 92%, and 92% (protons) and 99%, 91%, and 83% (carbon ions). No significant prognostic factors for LC or OS could be determined regarding the whole cohort; however, a significantly improved LC could be observed if the tumor was > 3 mm distant from the brainstem in patients presenting in a primary situation. CONCLUSION: Outcomes of proton and carbon ion treatment of skull base chordomas seem similar regarding tumor control, survival, and toxicity. Close proximity to the brainstem might be a negative prognostic factor, at least in patients presenting in a primary situation.


Asunto(s)
Condrosarcoma , Cordoma , Neoplasias de Cabeza y Cuello , Radioterapia de Iones Pesados , Terapia de Protones , Neoplasias de la Base del Cráneo , Masculino , Humanos , Femenino , Protones , Cordoma/diagnóstico por imagen , Cordoma/radioterapia , Cordoma/tratamiento farmacológico , Condrosarcoma/tratamiento farmacológico , Condrosarcoma/etiología , Iones , Carbono/uso terapéutico , Neoplasias de la Base del Cráneo/diagnóstico por imagen , Neoplasias de la Base del Cráneo/radioterapia , Neoplasias de la Base del Cráneo/tratamiento farmacológico , Base del Cráneo/patología , Radioterapia de Iones Pesados/efectos adversos , Radioterapia de Iones Pesados/métodos
12.
Ann Vasc Surg ; 94: 223-228, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-36906133

RESUMEN

BACKGROUND: Depending on the size and location of the tumor, carotid body tumor (CBT) resection can come with various complications, mostly intraoperative bleeding, and cranial nerve injuries. In the present study, we aim to evaluate 2 fairly new variables, tumor volume, and distance to the base of the skull (DTBOS), with operative complications of CBT resection. METHODS: Patients who underwent CBT surgery in Namazi hospital from 2015 to 2019 were studied using standard databases. Tumor characteristics and DTBOS were measured via computed tomography or Magnetic resonance imaging. Outcomes, including intraoperative bleeding and cranial nerve injuries, along with perioperative data were collected. RESULTS: A total of 42 cases of CBT were evaluated with an average age of 53.21 ± 12.8 and mostly female (85.7%). Based on Shamblin scoring, 2 (4.8%) were classified as group I, 25 (59.5%) as group II, and 15 (35.7%) as group III. The amount of bleeding significantly increased with an increase in the Shamblin scores (P = 0.031; median: I: 45 cc; II: 250 cc, III: 400 cc). Also, there was a significant positive correlation between the size of the tumor and the estimated amount of bleeding (correlation coefficient = 0.660; P < 0.001), and also a significant reverse correlation with between bleeding and DTBOS (correlation coefficient = -0.345; P = 0.025). During the follow-up of the patients, 6 (14.3%) had abnormalities in their neurological evaluation. Receiver operating characteristic curve analysis revealed the size of tumor cutoff level 32.7 cm3 (3.2 cm radius) to be most predictive of postoperative neurological complication with an area under the curve = 0.83, sensitivity = 83.3%, specificity = 80.6%, a negative predictive value = 96.7%, and positive predictive value of 41.7%, and an accuracy of 81.0%. Furthermore, based on the predictive power of the models in our study, we demonstrated that a combination model including the tumor size, DTBOS, along with the Shamblin score had the most predictive power for neurological complications. CONCLUSIONS: By evaluating CBT size and DTBOS, paired with the use of the Shamblin classification, a better, more insightful understanding of possible risks and complications of CBT resection can be obtained, leading to deserved levels of patient care.


Asunto(s)
Tumor del Cuerpo Carotídeo , Traumatismos del Nervio Craneal , Enfermedades del Sistema Nervioso , Humanos , Femenino , Adulto , Persona de Mediana Edad , Anciano , Masculino , Tumor del Cuerpo Carotídeo/diagnóstico por imagen , Tumor del Cuerpo Carotídeo/cirugía , Procedimientos Quirúrgicos Vasculares/efectos adversos , Estudios Retrospectivos , Resultado del Tratamiento , Base del Cráneo/patología , Complicaciones Posoperatorias/etiología , Traumatismos del Nervio Craneal/etiología
13.
Childs Nerv Syst ; 39(10): 2779-2787, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37584742

RESUMEN

Very few clinical entities have undergone so many different treatment approaches over such a short period of time as craniosynostosis. Surgical treatments for this condition have ranged from simple linear craniectomies, accounting for the specific role of cranial sutures in assuring the normal growth of the skull, to more complex cranial vault reconstructions, based on the perceived role of the skull base in affecting the growth of the skull. While a great deal of evolution has occurred, there remains controversy regarding the ideal treatment including the best surgical technique, the optimal age for surgery, and the long-term morphological and neurodevelopmental outcomes. The evolution of the surgical management of craniosynostosis in the last 50 years has been affected by several factors. This includes the awareness of needing to operate on affected children during infancy to achieve the best results, the use of multistage operations, the availability of more sophisticated surgical tools, and improved perioperative care. In some forms of craniosynostosis, the operations can be carried out at a very young age with low morbidity, and with the postoperative use of a molding helmet, springs, or distractors, these operations prove to be as effective as traditional larger cranial reconstructions performed in older children. As a consequence, complex surgical operations have become progressively less utilized. A second relevant advance was the more recent advent of a molecular diagnosis, which allowed us to understand the pathogenesis of some associated malformations and neurodevelopmental issues that were observed in some children despite appropriate surgical treatment. Future research should focus on improving the analysis of longer-term outcomes and understanding the natural history of craniofacial conditions, including what issues persist despite optimal surgical correction. Progress in molecular investigations concerning the normal and pathological development of cranial sutures could be a further significant step in the management of craniosynostosis, possibly favoring a "medical" treatment in the near future. Artificial intelligence will likely have a role in establishing the diagnosis with less reliance on radiographic studies and in assisting with surgical planning. Overall, much progress has been made, but there remains much to do.


Asunto(s)
Craneosinostosis , Neurocirugia , Humanos , Niño , Lactante , Inteligencia Artificial , Craneosinostosis/diagnóstico por imagen , Craneosinostosis/cirugía , Craneosinostosis/patología , Cráneo/cirugía , Suturas Craneales/diagnóstico por imagen , Suturas Craneales/cirugía , Base del Cráneo/patología
14.
Neurosurg Rev ; 46(1): 55, 2023 Feb 13.
Artículo en Inglés | MEDLINE | ID: mdl-36781550

RESUMEN

Synchronous or metachronous growth of multiple tumors (≥ 2) is found in up to 20% of meningioma patients. However, biological as well as histological features and prognosis are largely unexplored. Clinical and histological characteristics were retrospectively investigated in 95 patients harboring 226 multiple meningiomas (MMs) and compared with 135 cases of singular meningiomas (SM) using uni- and multivariate analyses. In MM, tumors occurred synchronously and metachronously in 62% and 38%, respectively. WHO grade was intra-individually constant in all but two MMs, and histological subtype varied in 13% of grade 1 tumors. MM occurred more commonly in convexity/parasagittal locations, while SM were more frequent at the skull base (p < .001). In univariate analyses, gross total resection (p = .014) and high-grade histology in MM were associated with a prolonged time to progression (p < .001). Most clinical characteristics and rates of high-grade histology were similar in both groups (p ≥ .05, each). Multivariate analyses showed synchronous/metachronous meningioma growth (HR 4.50, 95% CI 2.26-8.96; p < .001) as an independent predictor for progression. Compared to SM, risk of progression was similar in cases with two (HR 1.56, 95% CI .76-3.19; p = .224), but exponentially raised in patients with 3-4 (HR 3.25, 1.22-1.62; p = .018) and ≥ 5 tumors (HR 13.80, 4.06-46.96; p < .001). Clinical and histological characteristics and risk factors for progression do not relevantly differ between SM and MM. Although largely constant, histology and WHO grade occasionally intra-individually vary in MM. A distinctly higher risk of disease progression in MM as compared to SM might reflect different underlying molecular alterations.


Asunto(s)
Neoplasias Meníngeas , Meningioma , Humanos , Meningioma/cirugía , Meningioma/patología , Neoplasias Meníngeas/cirugía , Neoplasias Meníngeas/patología , Estudios Retrospectivos , Pronóstico , Base del Cráneo/patología
15.
Acta Neurochir (Wien) ; 165(8): 2299-2307, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37074392

RESUMEN

AIMS: Post-operative CSF leak remains a significant problem following endoscopic skull base surgery, particularly when there is a high-flow intra-operative CSF leak. Most skull base repair techniques are accompanied by the insertion of a lumbar drain and/or the use of nasal packing which have significant shortcomings. Our aim was to review the results of a large series of endoscopic skull base cases where a high-flow intra-operative CSF leak rate was encountered and repaired to assess if modifications in technique could reduce the post-operative CSF leak rate. METHODS: A retrospective review of a prospectively maintained database of skull base cases performed by a single surgeon over a 10-year period was performed. Data regarding patient demographics, underlying pathology, skull base repair techniques and post-operative complications were analysed. RESULTS: One hundred forty-two cases with high-flow intra-operative CSF leak were included in the study. The most common pathologies were craniopharyngiomas (55/142, 39%), pituitary adenomas (34/142, 24%) and meningiomas (24/142, 17%). The CSF leak rate was 7/36 (19%) when a non-standardised skull base repair technique was used. However, with the adoption of a standardised, multi-layer repair technique, the post-operative CSF leak rate decreased significantly (4/106, 4% vs. 7/36, 19%, p = 0.006). This improvement in the rate of post-operative CSF leak was achieved without nasal packing or lumbar drains. CONCLUSION: With iterative modifications to a multi-layered closure technique for high-flow intra-operative CSF leaks, it is possible to obtain a very low rate of post-operative CSF leak, without lumbar drains or nasal packing.


Asunto(s)
Neoplasias Meníngeas , Neoplasias de la Base del Cráneo , Humanos , Pérdida de Líquido Cefalorraquídeo/etiología , Pérdida de Líquido Cefalorraquídeo/cirugía , Endoscopía/efectos adversos , Endoscopía/métodos , Base del Cráneo/cirugía , Base del Cráneo/patología , Neoplasias de la Base del Cráneo/cirugía , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/prevención & control , Complicaciones Posoperatorias/patología , Estudios Retrospectivos , Neoplasias Meníngeas/patología
16.
Eur Arch Otorhinolaryngol ; 280(11): 5135-5138, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37491647

RESUMEN

OBJECTIVE: This is a case report of a dermoid cyst located in the infratemporal fossa and its surgical removal using infratemporal fossa type B approach. CASE REPORT: A 15-year-old male was referred from a local clinic after an incidental finding of a mass lesion in the skull base area on computed tomography (CT). Pre-operative magnetic resonance imaging showed a large cystic mass lesion, expanding to the foramen ovale with fat component in the right infratemporal fossa region. The lesion was completely excised using an infratemporal fossa type B approach. CONCLUSION: An extremely rare case of dermoid cysts of the infratemporal fossa was managed with infratemporal fossa type B approach without severe complication.


Asunto(s)
Quiste Dermoide , Fosa Infratemporal , Neoplasias de la Base del Cráneo , Masculino , Humanos , Adolescente , Quiste Dermoide/diagnóstico por imagen , Quiste Dermoide/cirugía , Quiste Dermoide/patología , Base del Cráneo/patología , Neoplasias de la Base del Cráneo/diagnóstico por imagen , Neoplasias de la Base del Cráneo/cirugía , Neoplasias de la Base del Cráneo/patología
17.
J Craniofac Surg ; 34(3): e296-e298, 2023 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-36882924

RESUMEN

Vascular tumors represent a challenging pathologic subset for surgical treatment as they show a propensity for profuse bleedings. This is especially applicable to the skull base region, where surgical access is difficult due to its complex anatomy. To overcome this problem, the authors introduced the use of a harmonic scalpel in endoscopic skull base surgery for vascular tumors. Here, the authors report the outcomes of endoscopic harmonic scalpel-assisted surgery in 6 juvenile angiofibromas and 2 hemangiomas. All surgeries were performed using Ethicon Endo-Surgery HARMONIC ACE 5 mm Diameter Shears. The median intraoperative blood loss was 400 mL (range: 200-1500 mL). The median length of hospital stay was 7 days (range: 5-10 days). Recurrence was recorded in 1 patient with juvenile angiofibroma, which was successfully resolved with revision surgery. In this institutional experience, ultrasonic technology showed precise cutting with minimal bleeding, resulting in reduced surgical morbidity compared with conventional endoscopic instruments.


Asunto(s)
Angiofibroma , Neoplasias Vasculares , Humanos , Neoplasias Vasculares/cirugía , Endoscopía/métodos , Instrumentos Quirúrgicos , Angiofibroma/cirugía , Angiofibroma/patología , Procedimientos Neuroquirúrgicos , Base del Cráneo/patología
18.
J Craniofac Surg ; 34(7): e628-e630, 2023 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-37236622

RESUMEN

Giant cell tumor (GCT) is a benign tumor that originates from undifferentiated mesenchymal cells of the bone marrow. The craniums as well as temporal bone are extremely rare locations for GCTs. Clinical, radiological, and anatomical diagnosis of this locally aggressive disease poses a major challenge in clinical practice. In this article, we present a clinical study for a 35-year-old female who was found to have left-sided temporal bone GCT with extension to middle cranial fossa and temporomandibular joint (TMJ) with its clinical features and management.


Asunto(s)
Tumor Óseo de Células Gigantes , Neoplasias de la Base del Cráneo , Femenino , Humanos , Adulto , Tumor Óseo de Células Gigantes/diagnóstico por imagen , Tumor Óseo de Células Gigantes/cirugía , Base del Cráneo/patología , Neoplasias de la Base del Cráneo/diagnóstico por imagen , Neoplasias de la Base del Cráneo/cirugía , Hueso Temporal/diagnóstico por imagen , Hueso Temporal/patología , Fosa Craneal Media/diagnóstico por imagen
19.
J Craniofac Surg ; 34(1): e10-e15, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36608087

RESUMEN

Sphenoorbital meningiomas are a challenge to access and reconstruct. Although there is much neurosurgical literature on resection of such tumors, there is little discussion on the best methods for the reconstruction of consequent defects, which are often extensive due to large areas of hyperostosis requiring resection. We performed a retrospective analysis of patients who underwent resection and reconstruction of a sphenoorbital meningioma by the senior authors (C.S. and D.A.S.) between 2010 and 2020. Surgical access in all cases included an orbitozygomatic osteotomy. The study cohort consisted of 23 patients (20 female, 3 male) with an average age of 50 (range: 37-72) years at the time of surgery. Most patients had progressive proptosis before the ablative operation. Orbital reconstruction was with a combined titanium-Medpor implant in 18 patients, split calvarial bone graft in 3 patients, and a Medpor implant in 2 patients. Calvarial reconstruction was performed with titanium mesh in 21 patients, split calvarial bone graft and titanium mesh in 1 patient, and craniotomy bone and titanium plate in 1 patient. Reoperation was required in 7 patients due to hypoglobus or enophthalmos (N=2), orbital implant malposition (N=1), abscess (N=1), pain (N=1), intracranial fat graft modification (N=1), and soft tissue deformities (N=2). Our experience demonstrates that sphenoorbital meningiomas can require broad areas of resection of the skull base and calvarium and necessitate comprehensive reconstruction of the anterior cranial fossa, orbital walls, and cranium. Collaboration between craniofacial surgeons and neurosurgeons can achieve optimal results.


Asunto(s)
Neoplasias Meníngeas , Meningioma , Neoplasias de la Base del Cráneo , Humanos , Masculino , Femenino , Persona de Mediana Edad , Meningioma/cirugía , Meningioma/patología , Estudios Retrospectivos , Titanio , Neoplasias de la Base del Cráneo/cirugía , Base del Cráneo/patología , Neoplasias Meníngeas/cirugía , Neoplasias Meníngeas/patología , Resultado del Tratamiento
20.
Pediatr Emerg Care ; 39(11): 869-874, 2023 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-37857457

RESUMEN

OBJECTIVES: We describe 10 new cases of otogenic (n = 8) and nonotogenic (n = 2) skull base osteomyelitis (SBO) in previously healthy children and review the literature on SBO in the pediatric population. METHODS: We retrospectively analyzed the medical records of 10 children (age range 0.9-12.8 years) discharged with a diagnosis of SBO between 2015 and 2020 in 2 children's hospitals in central Israel. RESULTS: Five patients presented with fever and 5 with otological signs and symptoms. All 10 children underwent a comprehensive clinical evaluation, imaging studies (computerized tomography or magnetic resonance imaging) and laboratory investigations. The physical examination revealed neurologic findings, including nuchal rigidity, papilledema, and apathy, in 4 patients. All 8 otogenic patients underwent surgical intervention and the 2 nonotogenic patients, who were diagnosed as having deep neck and throat infections, responded well to treatment consisting of antibiotics without surgery. CONCLUSIONS: Early diagnosis of pediatric SBO can be challenging because the symptoms are often nonspecific. The final diagnosis relies mainly on imaging, preferably magnetic resonance imaging. Surgical intervention is usually mandatory in the otogenic patients, whereas the nonotogenic patients respond well to medical management alone.


Asunto(s)
Osteomielitis , Base del Cráneo , Humanos , Niño , Lactante , Preescolar , Estudios Retrospectivos , Base del Cráneo/diagnóstico por imagen , Base del Cráneo/patología , Base del Cráneo/cirugía , Osteomielitis/diagnóstico , Osteomielitis/tratamiento farmacológico , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos X/efectos adversos
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