Rinosinusitis micótica alérgica con destrucción de base de cráneo / Allergic fungal rhinosinusitis with skull base destruction

La sinusitis micótica alérgica es una enfermedad inflamatoria de la mucosa rinosinusal producida por hongos que pueden aislarse de la cavidad de nasal de individuos sanos. Se produce indirectamente por los hongos que actúan como antígeno y desencadenan una reacción inmunológica mediada por IgE que origina pólipos y una secreción mucosa espesa con detritus e hifas denominada mucina. Su presentación clínica más frecuente es una sinusitis crónica unilateral o bilateral con pólipos. Con menos frecuencia, las sustancias originadas por la desgranulación de los eosinófilos producen remodelación o destrucción ósea y la sinusitis puede simular una neoplasia. Se describe el caso clínico de un paciente que padeció una sinusitis micótica alérgica con destrucción ósea masiva de la base del cráneo y que tuvo extensión intracraneal extradural e intraorbitaria de la enfermedad. Fue tratado con éxito mediante cirugía y corticoides. (AU)

Allergic fungal sinusitis is an inflammatory disease of the rhinosinusal mucosa caused by fungi that can be isolated from the nasal cavity of healthy individuals. The pathology is produced indirectly by the fungus that acts as an antigen and triggers an IgE-mediated allergic reaction that causes polyps and a thick mucous discharge with detritus and hyphae called mucin. Its most common clinical presentation is unilateral or bilateral chronic sinusitis with polyps. Less commonly, substances originated by the degranulation of eosinophils cause bone remodeling or destruction, and sinusitis can simulate a neoplasia. We describe the clinical case of a patient who suffered from allergic fungal sinusitis with massive bone destruction of the skull base and who had intracranial, extradural and intraorbital extension of the disease. He was successfully treated with surgery and corticosteroids.Key words: allergic fungal sinusitis, intracranial extension, endoscopic surgery, transorbital transpalpebral approach. (AU)

Taxonomy of the Lateral Strain Patterns at the Sphenobasilar Synchondrosis for Osteopathic Cranial Manipulative Medicine.

Lateral strain is a type of nonphysiologic cranial dysfunction that occurs at the sphenobasilar synchondrosis. In this dysfunction, the sphenoid and occiput rotate in the same directions along 2 vertical axes. There is currently no consensus on the nomenclature for this cranial dysfunction. In this article, the authors provide a standard nomenclature for lateral strains using the historical writings of pioneers in osteopathic medicine, including William Gardner Sutherland, DO, Anne L. Wales, DO, and Harold Magoun, DO. The authors establish the following consensus: (1) Lateral strains are named for the side to which the basisphenoid shifts; (2) The more prominent greater wing of the sphenoid is on the same side to which the basisphenoid shifts; (3) In vault and fronto-occipital holds, the holds form a parallelogram shape, with the index fingers pointing to the same side as the more prominent greater wing; and (4) The hand that is on the side of the prominent greater wing will shift anteriorly while the hand on the opposite side will shift posteriorly.

Peak Sinus Pressures During Sneezing in Healthy Controls and Post-Skull Base Surgery Patients.

OBJECTIVES/HYPOTHESIS: Patients are frequently advised to sneeze with an open mouth and avoid nose-blowing following an endoscopic endonasal approache (EEA) to the skull base, despite a lack of quantitative evidence. This study applies computational fluid dynamics (CFD) to quantify sinus pressures along the skull base during sneezing. STUDY DESIGN: Case-control series. METHODS: Computed tomography or magnetic resonance imaging scans of four post-EEA patients and four healthy controls were collected and analyzed utilizing CFD techniques. A pressure drop of 6,000 Pa was applied to the nasopharynx based on values in the literature to simulate expiratory nasal airflow during sneezing. Peak pressures along the skull base in frontal, ethmoid, and sphenoid sinuses were collected. RESULTS: Significant increases in skull base peak pressure was observed during sneezing, with significant individual variations from 2,185 to 5,685 Pa. Interestingly, healthy controls had significantly higher pressures compared to post-EEA patients (5179.37 ± 198.42 Pa vs. patients 3,347.82 ± 1,472.20 Pa, P < .05), which could be related to higher anterior nasal resistance in unoperated healthy controls (0.44 ± 0.22 vs. 0.31 ± 0.16 Pa/mL/sec for patients, P = .38). The sinus pressure buildup may be due to airway resistance functioning as a valve preventing air from being released quickly. Supporting this theory, there was a strong correlation (r = 0.82) between peak skull base pressure and the ratio of anterior resistance to total resistance. Within-subject variation in pressures between different skull base regions was much lower (average = ~5%). CONCLUSIONS: This study provided the first quantitative analysis of air pressure along the skull base during sneezing in post-EEA patients through CFD, suggesting that pressure buildup may depend on individual anatomy. LEVEL OF EVIDENCE: 3b Laryngoscope, 130:2138-2143, 2020.

A Case of Autosomal Dominant Osteopetrosis Type 2 with a CLCN7 Gene Mutation

Osteopetrosis is a rare genetic disease characterized by increased bone density and bone fractures due to defective osteoclast function. Autosomal dominant osteopetrosis type 2 (ADO-2), Albers-Schonberg disease, is characterized by the sclerosis of bones, predominantly involving the spine, pelvis and the base of the skull. Here, we report a typical case of osteopetrosis in a 17.7-year-old male who carries a heterozygous c.746C>T mutation in exon 9 in the chloride voltage-gated channel 7 (CLCN7) gene. The patient's spine showed multiple sclerotic changes including sandwich vertebra. His father had the same mutation but his skeletal radiographs were normal. This is the first reported case of ADO-2, confirmed by genetic testing in a Korean patient.

The Craniovertebral Junction in Rheumatoid Arthritis: State of the Art.

Rheumatoid arthritis (RA) is a chronic inflammatory disorder, characterized by polyarticular inflammation causing progressive joint damage and disability. The mechanisms underlying its pathogenesis involve activation of innate and adaptive immunity, microvascular endothelial cell activation, and inflammatory infiltration of lymphocytes and monocytes into the synovium. Spinal involvement in RA is not typical; when it occurs, the main radiological features are (1) atlantoaxial subluxation (AAS), which is the most typical form of cervical spine involvement; (2) cranial settling-also known as basilar impression, atlantoaxial impaction or superior migration of the odontoid-which is the most severe form of associated spinal instability; and (3) subaxial subluxation. A combination of these alterations may occur. Synovitis is characterized by infiltration of innate and adaptive immune cells; joint destruction is a consequence of activation of synovial fibroblasts, which acquire aggressive, inflammatory, invasive features, associated with increased chondrocyte catabolism and synovial osteoclastogenesis.Neck pain is the most frequent symptom of spinal involvement in RA; it occurs in 40-80% of patients and is mostly localized at the craniocervical junction. Other symptoms-caused by compression of neural structures such as the greater occipital nerve (at C2), the nucleus of the spinal trigeminal tract and the greater auricular nerve-are occipital neuralgia, facial pain and ear pain, respectively. Irritation of the lesser occipital nerve (at C1) can cause pain in the suboccipital region. Sometimes patients may complain of a sensation of their head falling down with flexion, weakness, reduced endurance, loss of ability, gait alterations, paraesthesias or other symptoms due to cord and medullary compression, and upper or lower motor neuron signs, or both. Surgical management of RA remains a challenging field.

Ossification of the Posterior Longitudinal Ligament: Analysis of the Role of Craniovertebral and Spinal Instability.

BACKGROUND: This paper reviews an experience of surgically treating ossification of the posterior longitudinal ligament (OPLL) with fixation of the involved spinal segments alone, without resorting to any bony or soft tissue decompression or attempts at direct resection of the OPLL. While in the early part of the experience, stabilization of only the involved subaxial cervical spinal segments was done, in the later part of the experience, atlantoaxial fixation was included in the multisegmental spinal fixation construct. This treatment is based on the understanding that spinal instability that includes atlantoaxial instability forms the nodal point of the pathogenesis and development of OPLL, and maturation of the presenting clinical symptoms. MATERIALS AND METHODS: Twenty-nine patients were treated in this series. There were 28 males and one female, and their ages ranged from 28 to 75 years (average 57 years). All patients presented with symptoms of neck pain, and progressive and disabling myelopathy-related quadriparesis. In the early part of the series (from 2012 to 2014), 14 patients underwent multilevel subaxial cervical spinal fixation by a transarticular technique of facetal fixation. After November 2014, atlantoaxial lateral mass fixation was included in the fixation construct in the subsequent 15 patients. Clinical assessments were done using a visual analogue scale (VAS), the Japanese Orthopaedic Association (JOA) scale and Goel's clinical grading scale. RESULTS: All patients' clinical symptoms improved in the immediate postoperative period, and the improvement was sustained and progressive in 28 patients. CONCLUSION: Atlantoaxial and subaxial spinal instability seems to be the nodal pathogenetic factor in OPLL. Only stabilization of spinal segments that includes the atlantoaxial joint can provide a safe, simple and rational form of treatment.

Association of Obstructive Sleep Apnea With Calvarial and Skull Base Thinning.

Importance: Spontaneous cerebrospinal fluid leaks (sCSF-L) of the temporal bone are associated with obesity, calvarial thinning, and obstructive sleep apnea (OSA), and the incidence has doubled in the past decade. It is currently unknown if OSA is independently associated with skull thinning. Objective: To determine if patients with OSA have thinner skulls than patients without OSA. Design, Setting, and Participants: A retrospective cohort study of patients who underwent a level 1 polysomnogram (PSG) and also had high-resolution computed tomographic (CT) imaging of the head from January 2010 to March 2017 at Indiana University was carried out. Patients with moderate to severe OSA (apnea-hypopnea index [AHI]≥25/h) and without OSA (AHI<5/h) were matched for age and body mass index (BMI, calculated as weight in kilograms divided by height in meters squared). Interventions: Measurement of calvarial thickness, extracranial zygoma thickness, skull base height and tegmen dehiscence (>4 mm) when blinded to OSA status. Main Outcomes and Measures: Primary outcomes were calvarial, skull base, and zygoma thickness differences between patients with OSA vs those without OSA. Results: A total of 22 933 patients had a PSG and 1012 also had head CT imaging. Of the 1012 patients with both PSG and CT, the mean (SD) age was 50.8 (16.2) years and 624 (61.7%) were women. Those patients with moderate to severe OSA (56) and without OSA (58) were matched for mean (SD) age (50.3 [6.5] vs 49.8 [6.1] years]) and BMI (37.4 [8.1] vs 38.6 [6.8]). Patients with OSA had thinner mean (SD) calvaria (2.73 [0.67] vs 2.47 [0.52] mm; difference, -0.26 mm; 95% CI, -0.49 to -0.04; Cohen d, 0.44) and thinner skull bases (5.03 [1.40] vs 4.32 [1.28] mm; difference, -0.71; 95% CI, -1.23 to -0.19; Cohen d, 0.53). The mean (SD) extracranial zygoma thickness was the same (4.92 [0.87] vs 4.84 [0.84] mm; difference, -0.07 mm; 95% CI, -0.39 to 0.24). The tegmen mastoideum was dehiscent in nearly twice as many patients with OSA as those without (37% vs 20%; difference, 17%; 95% CI, 0.4-32). Conclusions and Relevance: Obstructive sleep apnea was independently associated with intracranial bone (calvaria and skull base) thinning and not with extracranial (zygoma) thinning. These findings support a possible role of OSA in the pathophysiologic development of sCSF-L.

Complications and donor site morbidity of 3-layer reconstruction with iliotibial tract of the anterior skull base: Retrospective analysis of 186 patients.

BACKGROUND: Anterior skull base reconstruction after resection of sinonasal cancers may be challenging when pedicled flaps are unavailable. The purpose of the present study was to analyze the complication rate and donor site morbidity of 3-layer reconstruction with the iliotibial tract (ITT). METHODS: We retrospectively reviewed all anterior skull base reconstructions with ITT performed from 2007 to 2015. Donor site morbidity was investigated by a dedicated questionnaire. Factors impacting on cerebrospinal fluid (CSF) leak were assessed using the Fisher's exact test. RESULTS: One hundred eighty-six patients were included. The overall complication rate was 9.7%. A CSF leak occurred in 11 patients (5.8%). Twenty patients (10.8%) and 130 patients (69.9%) underwent previous or adjuvant radiotherapy, respectively. Neither radio(chemo)therapy nor age impacted the risk of CSF leak. Six patients (3.2%) experienced complications at the donor site. The questionnaire demonstrated minimal functional and aesthetic morbidity. CONCLUSION: Three-layer reconstruction with the ITT is a safe procedure with acceptable complication rate and donor site morbidity.

Cirugía endoscópica avanzada de base de cráneo, cavidad nasal y senos paranasales: experiencia de 40 meses en un hospital universitario / Advanced endoscopic surgery of skull base, nasal cavity and paranasal sinus: experience of 40 months in a university hospital / Cirugia endoscopica avançada de base de craneo, cavidade nasal e seios paranasales: experiência de 40 meses num hospital universitário

INTRODUCCIÓN: El uso del endoscopio junto con el avance de la tecnología ha facilitado la extensión de los límites tradicionales en la cirugía de la base del cráneo, la cavidad nasal y los senos paranasales. OBJETIVO: Describir los hallazgos epidemiológicos, clínicos e histopatológicos en los pacientes intervenidos por cirugía endoscópica avanzada en una unidad de Otorrinolaringología en 40 meses. MATERIAL Y MÉTODOS: Estudio retrospectivo. Los protocolos quirúrgicos e historias clínicas fueron revisados. Se realizó un análisis univariado descriptivo con aplicación de test de Chi-cuadrado para significancia estadística (p<0,05)...

INTRODUCTION: The use of the endoscope and the advance of the technology has facilitated the extension of the traditional limits in the surgery of skull base, nasal cavity and paranasal sinuses. OBJECTIVE: To describe the epidemiologic, clinical and histopathological findings in patients intervened under advanced endoscopic surgery in an Otorhinolaryngology unit in 40 months. MATERIAL AND METHODS: Retrospective study. Surgical protocols and medical records were reviewed. A descriptive univariate analysis was performed with the application of Chi-square test for statistical significance (p <0.05)...

INTRODUÇÃO: O uso do endoscópio junto com o avanço da tecnologia facilitou a extensão dos limites tradicionais na cirurgia da base do crânio, a cavidade nasal e os seios paranasais. OBJETIVO: Descrever os achados epidemiológicos, clínicos e histopatológicos em pacientes submetidos a cirurgia endoscópica avançada em uma unidade de Otorrinolaringologia em 40 meses. MATERIAL E MÉTODOS: Estudo retrospectivo. Os protocolos cirúrgicos e os registros médicos foram revisados. Uma análise descritiva univariada foi realizada com a aplicação do teste Qui-quadrado para significância estatística...

Management of Penetrating Skull Base Injury: A Single Institutional Experience and Review of the Literature.

BACKGROUND: Penetrating skull base injury (PSBI) is uncommon among head injuries, presenting unique diagnostic and therapeutic challenges. Although many cases of PSBIs have been reported, comprehensive understanding of its initial diagnosis, management, and outcome is still unavailable. MATERIALS AND METHODS: A retrospective review was performed for patients treated in neurosurgical department of Changzheng Hospital for PSBIs. Presurgical three-dimensional (3D) Slicer-assisted reconstructions were conducted for each patient. Then we reviewed previous literature about all the published cases of PSBIs worldwide and discussed their common features. RESULTS: A total of 5 patients suffering PSBIs were identified. Penetrating points as well as the surrounding neurovascular structures were clearly visualized, assisting in the presurgical planning of optimal surgical approach and avoiding unexpected vascular injury. Four patients underwent craniotomy with foreign bodies removed successfully and 1 patient received conservative treatment. All of them presented good outcomes after proper management. CONCLUSION: Careful physical examination and radiological evaluation are essential before operation, and angiography is recommended for those with suspected vascular injuries. 3D modeling with 3D Slicer is practicable and reliable, facilitating the diagnosis and presurgical planning. Treatment decision should be made upon the comprehensive evaluation of patient's clinicoradiological features and characteristics of foreign bodies.

[Biomechanics analysis of the impact of maxillofacial injury on skull base damage].

OBJECTIVE: To analyze the impact of maxillofacial injury on skull base. METHODS: A three-dimensional(3D)finite-element model of cranio-maxillofacial bone was established by CT scan data. A lead cylinder in base diameter of 3 cm was designed as an impactor. There regions(upper right maxilla, left infraorbital margin and left zygomatic body)subjected to an impact at the speed of 8.6 m/s(about 30 km/h)was simulated. Thirteen landmarks at the skull base were selected. The values of stress at the end of 0.5, 1.0, 1.5, 2.0 ms were obtained, and the results were analyzed. RESULTS: The dynamic process of the fracture of the jaw and the stress distribution and conduction of the skull base were successfully simulated in three parts of the face. When the impact was on the right maxillary bone region, the stress values of the three points(medial foramen rotundum, medial foramen rotundum, anterior clivus reached the peak at each time point, 26.2, 22.4, 21.5 MPa(t=0.5 ms)and 70.0, 55.0, 45.0 MPa(t=1.0 ms)and 38.0, 26.5, 39.5 MPa(t=1.5 ms)and 26.0, 19.0, 23.0 MPa(t=2.0 ms), respectively. When the impact was on the left margo infraorbitalis orbitaeta region, the stress values of the two points(medial left foramen rotundum, posterior clivus)reached the peak at each time point, 8.8, 16.0 MPa(t=0.5 ms)and 10.0, 18.0 MPa(t=1.0 ms)and 5.5, 6.0 MPa(t=1.5 ms)and 11.5, 12.5 MPa(t=2.0 ms), respectively. When the impact was on the body of left zygomatic bone, the stress values of posterior clivus were 45.0 MPa(t=0.5 ms), 40.0 MPa(t=1.0 ms), 12.0 MPa(t=1.5 ms), 42.5 MPa(t= 2.0 ms), respectively. CONCLUSIONS: According to the difference of stress distribution and conduction of maxillofacial and skull base bone, the speed and the path of force transfer to the skull base were different. Finite-element dynamic simulation can be used for the biomechanics research on maxillofacial trauma.

Gorham-Stout Disease of the Skull Base With Hearing Loss: Dramatic Recovery and Antiangiogenic Therapy.

Gorham-Stout disease (GSD) is a rare disorder of unknown etiology. We present a 6-year-old male with GSD involving the skull base who presented with recurrent cerebrospinal fluid (CSF) rhinorrhea, severe hearing loss, and facial palsy secondary to cerebellar herniation into the internal auditory canal. After 2 months of treatment with pegylated interferon (IFN) α-2b (50 µg/week), his hearing recovered dramatically. Two years later, new bone formation appeared radiologically and IFN was switched to sirolimus. One year after the switch, CSF rhinorrhea disappeared. Antiangiogenic therapy might inhibit proliferation of vascular endothelial cells in osteolytic lesions and lead to new bone formation.

Sinonasal and ventral skull base inflammatory pseudotumor: a systematic review.

OBJECTIVES/HYPOTHESIS: Inflammatory pseudotumor is a benign idiopathic inflammatory process often misdiagnosed as an infection or neoplasm. This review analyzes all reported cases of sinonasal and ventral skull base inflammatory pseudotumor to date, and provides a framework for evaluation and management of this uncommon condition. DATA SOURCES: MEDLINE/PubMed database. REVIEW METHODS: A search for articles related to sinonasal and ventral skull base inflammatory pseudotumor, along with bibliographies of those articles, was performed. Demographics, presentation, radiographic findings, treatment, follow-up, and outcome were analyzed. RESULTS: Thirty-three articles were reviewed, including a total of 87 patients. The most common presenting symptom was vision change (58.6%). Sinonasal and ventral skull base inflammatory pseudotumor was found in the cavernous sinus in 46.0% of cases. The lesion appeared isointense (66.7% of cases) and homogeneously enhancing on T1-weighted magnetic resonance imaging (MRI), whereas it appeared hypointense on T2-weighted MRI in 90.7% of cases. Inflammatory pseudotumor appeared hyperdense on computed tomography in 78.9% of cases. Histopathological analysis of biopsied specimens revealed presence of inflammatory cells (94.4%) and fibrosis (80.3%). Corticosteroids alone were the most common treatment modality (55.2%), resulting in disease-free patients in 22.9% of cases over a median follow-up period of 17.6 months. Surgical management alone was uncommon (8.0%), but showed high success rate (57.1%). CONCLUSION: This review is the most comprehensive analysis of sinonasal and ventral skull base inflammatory pseudotumor to date. Radiologic findings and histopathological analysis are essential for diagnosis. Corticosteroids are the most common treatment modality. Surgery, although uncommon, appears to be an efficacious treatment modality.

Perplexing lesions of the sinonasal cavity and skull base: IgG4-related and similar inflammatory diseases.

OBJECTIVE: IgG4-related disease (IgG4RD) causing sinonasal and skull base pathology is uncommonly described. We present a series of suspected IgG4RD patients, with a pertinent review of the literature to highlight diagnostic challenges. STUDY DESIGN: Case series. SETTING: Academic tertiary care center. SUBJECTS AND METHODS: Case series of patients with IgG4RD or suspected IgG4RD involving the sinonasal cavity and skull base. RESULTS: We present 4 patients with atypical sinonasal and/or skull base disease who were noted to have IgG4-positive plasma cell infiltration on immunohistochemistry of biopsy specimens. IgG4RD, a recently described entity affecting multiple organs, is characterized by lymphoplasmacytic infiltration and often elevated serum IgG4. IgG4RD can masquerade as malignancy or infection but responds to glucocorticosteroid and immunosuppressant therapy. IgG4RD has been infrequently reported presenting as sinonasal or skull base lesions, and definitive diagnostic criteria for these regions are not established. In our series, IgG4RD was suspected in all 4 patients, but only 1 met all current criteria for definitive diagnosis. All 4 patients, however, responded to corticosteroid therapy, and 1 was placed on long-term azathioprine. CONCLUSION: IgG4RD is rarely described in the sinonasal cavity and skull base, and specific diagnostic criteria for such disease have not been defined. We present a series of patients with IgG4-positive plasma cell inflammatory pathology who were suspected to have IgG4RD. Our series highlights diagnostic challenges associated with these patients. Tumefactive and destructive sinonasal-skull base lesions with a plasma cell-rich infiltrate should incite suspicion of IgG4RD, and immunohistochemistry for IgG4-positive plasma cells should be performed.

Tension pneumocephalus as a result of endonasal surgery: an uncommon intracranial complication.

Tension pneumocephalus (TP) is a clinical entity characterized by continued build-up of air within the cranial cavity, leading to abnormal pressure exerted upon the brain and subsequent neurologic deterioration, due to development of a mass effect and potentially a herniation syndrome. Intracranial complications of endoscopic sinus surgery (ESS) and other endonasal procedures are fortunately very rare, occurring in less than 3% of cases. We report 4 cases of small bone defects (<3 mm) in the anterior cranial base accompanied by TP, caused by ESS and other endonasal procedures. The pathophysiology and management of this clinical entity is discussed with a pertinent literature. Four patients with small (<3 mm) skull base defects were identified. All patients presented with active cerebrospinal fluid leaks. CT scans showed intracranial tension pneumocephalus. Using image-guided endoscopic techniques, all defects were addressed with multi-layer repair. Closure was achieved in all patients on the first attempt, with an average follow-up of 36 months. Tension pneumocephalus is a rare event that can occur as a result of traumatic or iatrogenic violation of the dura and should be considered in all patients presenting with altered mental status after endoscopic sinus surgery or other surgical and diagnostic procedures that violate either the cranial or spinal dura. Because of the potential for rapid clinical deterioration and death, prompt brain imaging is warranted to rule out the diagnosis, and urgent neurosurgical consultation is indicated for definitive management.

[Scoliotic spine deformity in children and adolescents associated with the craniovertebral junction pathology].

This literature review is devoted to the clinical and pathogenic aspects of the relationship between Chiari type I malformation (CMT) and scoliosis. The view of the clinical presentation development in CMT associated with scoliosis is considered on the basis of both the CSF dynamics disturbances and vascular pathology of the craniovertebral junction. The role of the posterior atlanto-occipital membrane is evaluated. Case reports of the risk factors for scoliosis progression in patients with CMT are presented.

Neurophysiologic, audiometric and vestibular function tests in patients with hyperostosis cranialis interna.

OBJECTIVE: Hyperostosis cranialis interna (HCI) is an autosomal dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina. The aim of this study is to describe the value of several neurophysiological, audiometric and vestibular tests related to the clinical course of the disorder. METHODS: Ten affected subjects and 13 unaffected family members were recruited and tested with visual evoked potentials, masseter reflex, blink reflex, pure tone and speech audiometry, stapedial reflexes, otoacoustic emissions, brainstem evoked response audiometry and electronystagmography. RESULTS: Due to the symmetrical bilateral nature of this disease, the sensitivity of visual evoked potentials (VEPs), masseter reflex and blink reflex is decreased (25-37.5%), therefore reducing the value of single registration. Increased hearing thresholds and increased BERA latency times were found in 60-70%. The inter-peak latency I-V parameter in BERA has the ability to determine nerve encroachment reliably. 50% of the patients had vestibular abnormalities. No patient had disease-related absence of otoacoustic emissions, because the cochlea is not affected. CONCLUSION: In patients with HCI and similar craniofacial sclerosing bone dysplasias we advise monitoring of vestibulocochlear nerve function with tone and speech audiometry, BERA and vestibular tests. VEPs are important to monitor optic nerve function in combination with radiological and ophthalmologic examination. We do not advise the routine use of blink and masseter reflex.

Skull base surgery for tumors in children: long-term clinical and functional outcome.

OBJECT: Skull base tumors in children are rare but require complex approaches with potential morbidity to the developing craniofacial skeleton, in addition to tumor-related morbidity. Reports of long-term clinical and functional outcome following skull base approaches in children are scarce. The authors report long-term outcome in children with tumors undergoing multidisciplinary skull base surgery. METHODS: A retrospective analysis was undertaken of children undergoing surgery at a single institution between 1998 and 2008 for benign and malignant lesions of the anterior, middle, or posterior cranial base. Patients with craniopharyngioma, pituitary tumors, and optic glioma were excluded. Histology, surgical morbidity, length of hospital stay, progression-free survival, and adjuvant therapy were recorded. Functional and cognitive outcome was assessed prospectively using the Late Effects Severity Score (LESS). RESULTS: Twenty-three children ranging in age from 13 months to 15 years underwent skull base approaches for resection of tumors during the study period. The median follow-up duration was 60 months. Tumor types included meningioma, schwannoma, rhabdomyosarcoma, neuroblastoma, angiofibroma, and chordoma. Complete resection was achieved in 12 patients (52%). Thirteen patients (57%) had benign histology. The median hospital stay was 7 days. There were 3 deaths, 1 perioperative and 2 from tumor progression. Two patients had CSF leakage (9%) and 2 developed meningitis. Two children (9%) had residual neurological deficit at last follow-up evaluation. Thirteen (59%) of 22 surviving patients received adjuvant therapy. The majority of the patients remain in mainstream education and 19 of the 20 surviving children have an LESS of 3 or lower. CONCLUSIONS: Children tolerate complex skull base procedures well, with minimal surgical-related morbidity as well as good long-term tumor control rates and functional outcomes from maximal safe resection combined with adjuvant treatment when required.

[The giant fungal body in the sphenoidal sinus and the destruction of the skull base].

The objective of the present study was to estimate the efficacy of the optical systems with the variable visual field angle applied for the endoscopic interventions on the paranasal sinuses. The authors report a clinical observation of the patient presenting with the giant fungal body in the sphenoidal sinus responsible for the partial destruction of the bone canal of the optic nerve and the internal carotid artery. The patient was treated by endoscopic shenoidotomy through the paraseptal approach with the use of a sinuscope with the variable visual field angle. It was shown that the use of optical devices with the variable visual field angle makes it possible to significantly reduce the duration of the surgical intervention, facilitates orientation in the difficult-of-access regions , and ensures adequate control during the removal of neoplasms at the basis of the skull.

Postural evaluation in children with atypical swallowing: radiographic study.

PURPOSE: To evaluate the cranial posture on lateral teleradiographs of children with atypical swallowing in mixed dentition. METHODS: By using cephalometric analysis on lateral teleradiographs, the angles between the odontoid process and cranial base (CC1) and the odontoid process and Frankfurt plane (CC2) were measured in two groups: 55 teleradiographs from the experimental group (with atypical swallowing), and 55 lateral teleradiographs from the control group (normal swallowing). These angular measurements were subjected to statistical analysis. RESULTS: The means of the variable CC1 were 99.95 degrees in the experimental group and 96.42 degrees in the control group, with significant difference between them. The means of the variable CC2 were 90.60 degrees in the experimental group and 86.35 degrees in the control group, which was also statistically significant. CONCLUSION: The angles CC1 and CC2 are increased in the group with atypical swallowing.