An uncommon etiological factor for aspiration pneumonitis caused by spontaneous sphenoid sinus meningoencephalocele with cerebrospinal fluid rhinorrhea: a case report.

BACKGROUND: Aspiration pneumonitis is an inflammatory disease of the lungs which is difficult to diagnose accurately. Large-volume aspiration of oropharyngeal or gastric contents is essential for the development of aspiration pneumonitis. The role of cerebrospinal fluid (CSF) rhinorrhea is often underestimated as a rare etiological factor for aspiration in the diagnosis process of aspiration pneumonitis. CASE PRESENTATION: We present a case of a patient with 4 weeks of right-sided watery rhinorrhea accompanied by intermittent postnasal drip and dry cough as the main symptoms. Combined with clinical symptoms, imaging examination of the sinuses, and laboratory examination of nasal secretions, she was initially diagnosed as spontaneous sphenoid sinus meningoencephalocele with CSF rhinorrhea, and intraoperative endoscopic findings and postoperative pathology also confirmed this diagnosis. Her chest computed tomography showed multiple flocculent ground glass density shadows in both lungs on admission. The patient underwent endoscopic resection of meningoencephalocele and repair of skull base defect after she was ruled out of viral pneumonitis. Symptoms of rhinorrhea and dry cough disappeared, and pneumonitis was improved 1 week after surgery and cured 2 months after surgery. Persistent CSF rhinorrhea caused by spontaneous sphenoid sinus meningoencephalocele was eventually found to be a major etiology for aspiration pneumonitis although the absence of typical symptoms and well-defined risk factors for aspiration, such as dysphagia, impaired cough reflex and reflux diseases. CONCLUSIONS: We report a rare case of aspiration pneumonitis caused by spontaneous sphenoid sinus meningoencephalocele with CSF rhinorrhea, which can bring more attention and understanding to the uncommon etiology for aspiration, so as to make more accurate diagnosis of the disease and early surgical treatment.

Atlantoaxial Fixation for Treatment of Chiari Formation and Syringomyelia with No Craniovertebral Bone Anomaly: Report of an Experience with 57 Cases.

AIM: In this paper we evaluate the role of atlantoaxial instability in the pathogenesis of Chiari formation type I and the role of atlantoaxial stabilization for treatment of this condition in cases with no obvious bone malformation in the region of the craniovertebral junction. MATERIALS, METHODS AND RESULTS: During the period from January 2010 to July 2016, we identified 57 cases of Chiari formation where there was no bone malformation or evidence of craniovertebral junction instability that could be diagnosed on the basis of an abnormal increase in the atlantodental interval on dynamic imaging. Forty-eight of these patients had syringomyelia. The average duration of follow-up was 42 months. There were 30 males and 27 females in the series. The ages of the patients ranged from 4 to 57 years. The Japanese Orthopaedic Association (JOA), visual analogue scale (VAS) and Goel clinical grading systems were used to assess the patients' clinical status. Atlantoaxial instability was diagnosed on the basis of vertical mobility of the odontoid process on dynamic radiographs, facetal malalignment on imaging or direct bone handling during the surgical procedure. Surgical treatment was achieved using atlantoaxial fixation. Foramen magnum decompression or syrinx manipulation was not done. All patients had immediate postoperative and sustained clinical symptomatic recovery. A reduction in the size of the syrinx was observed in ten patients and regression of tonsillar herniation was observed in 12 of 23 cases in which postoperative magnetic resonance imaging (MRI) was possible. CONCLUSION: Atlantoaxial instability is the prime factor in the genesis of Chiari formation even when there is no bone abnormality in the craniovertebral junction.

An Endoscopic Endonasal Repair of Spontaneous Skull Base Cerebrospinal Fluid Leak.

Idiopathic intracranial hypertension is a disease of elevated intracranial pressure that can lead to spontaneous cerebrospinal fluid leak at the skull base. We present a case of CSF leak in a young female with undiagnosed idiopathic intracranial hypertension that presented as clear rhinorrhea. The defect in the skull base was visualized with computerized tomography and repaired endoscopically with adjuvant placement of a lumboperitoneal shunt. This case outlines the important sequence of repair and postoperative care for spontaneous cerebrospinal fluid leak, and demonstrates the safety and efficacy of endoscopic endonasal repair.

Skull Base Osteomyelitis Caused by an Elegant Fungus.

Malignant otitIs externa (skull base osteomyelitis) is predominantly caused by bacteria while fungal etiology is rare. We report a middle aged diabetic gentleman who succumbed to invasive skull base infection due to Apophysomyces elegans a fungus belonging to Zygomycetes which causes only skin and soft tissue infections. Mortality and invasive infections due to this genus is rarely reported, especially in the ear.

Endoscopic management of pediatric sinonasal schwannoma: case report.

OBJECTIVES: To describe an extremely rare pediatric sinonasal schwannoma, and to reviewmanagement strategies and relevant literature. METHODS: Case report of pediatric sinonasal schwannoma, that was imaged with computed tomography and magnetic resonance imaging and managed endoscopically. Immunohistochemical analysis was performed to determine pathology. RESULTS: A 12-year-old girl presented with a 2-month history of progressive left exophthalmos. Imaging studies showed a large heterogeneous tumour arising from the ethmoid sinus and extending to the base of the skull and to the orbital cavity. The lesion was removed with an endonasal radical excision. The final pathological diagnosis was schwannoma. There was no tumour recurrence or any major complication during the 2-year follow up. CONCLUSION: Schwannoma should be considered in the differential diagnosis for pediatric patients with intranasal masses. Depending on the location and extent of the tumour, endonasal endoscopic excision could be a suitable management strategy.

Double osteotomy of mandibula in the treatment of carotid body tumors with skull base extension.

We report two patients with a carotid body paraganglioma that extended to the skull base, a position that is surgically inaccessible by means of a traditional lateral cervical approach. In both patients we were able to remove the lesion by performing a double mandibular osteotomy. Both patients underwent preoperative embolization to reduce the mass. In our experience, this approach has allowed a safe radical excision of exceptionally high lesions with only minor permanent nerve damage. In our opinion this advantage definitely outweighs the consequences of the increased invasiveness of this technique.

Endoscopic endonasal management of cerebrospinal fluid rhinorrhea.

The authors review their 5 years' experience with endonasal endoscopic repair of the anterior skull base fistulas presenting with cerebrospinal fluid (CSF) rhinorrhea. A total of 12 patients were managed endoscopically between 2004 and 2008. Seven patients (58.3%) had nonsurgical posttraumatic CSF rhinorrhea, 2 patients (16.7%) had CSF rhinorrhea due to surgical/iatrogenic trauma, and 3 patients (25%) had spontaneous onset of CSF rhinorrhea. Radiosurgical correlation for CSF fistula identification was positive in all patients. The most common site of leak was the fovea ethmoidalis. The repair method consisted of an extradural underlay closure of a defect with fascia lata. The largest diameter of a defect to be closed was 15 mm. Immediate results were good in all patients, but later in the follow-up, CSF rhinorrhea recurred in 2 patients, and each patient had a revision 2 times. In the first revisions, transcranial approach was used, whereas in the second revisions endonasal endoscopic route was resorted. The primary closure rate was 83.3%, and the overall closure rate was 100%. The average follow-up period thus far is 21 months. Endonasal endoscopic technique well known to otolaryngologists should be considered as the first choice of surgery in the repair of CSF rhinorrhea because of low morbidity and a higher closure rate. The possibility of revision with the same technique makes this approach ideal for the repair of cranionasal osteodural defects.

Imaging of the Postoperative Skull Base and Cerebellopontine Angle.

For pathologic conditions affecting the skull base and cerebellopontine angle, imaging techniques have advanced to assess for residual disease, disease progression, and postoperative complications. Knowledge regarding various surgical approaches of skull base tumor resection, expected postoperative appearance, and common postsurgical complications guides radiologic interpretation. Complexity of skull base anatomy, small size of the relevant structures, lack of familiarity with surgical techniques, and postsurgical changes confound radiologic evaluation. This article discusses the imaging techniques, surgical approaches, expected postoperative changes, and complications after surgery of the skull base, with emphasis on the cerebellopontine angle, anterior cranial fossa, and central skull base regions.

Conservative management of transnasal intracranial injury.

The purpose of this study was to explore the conservative management for an unusual case of transnasal intracranial injury. A 3-year-old female child presenting with transnasal injuries after a domestic accident whereby she apparently fell while holding a large pair of scissors, which then penetrated her left nasal cavity, piercing her nasal cavity, ethmoid sinus, and skull base. The scissors were removed from her nasal cavity. The patient had scant cerebrospinal rhinorrhea and no other additional neurologic deficits noted at the time, as well as no long-term developmental deficits. This report highlights the occurrence of this rare condition. The role of radiologic studies such as computed tomographic scans and plain films in diagnosis and management of this case is affirmed. The strategy of minimally invasive treatment of this injury can be a reasonable treatment option.

Special MRI Techniques to Suss out Spontaneous Cerebrospinal Fluid Leaks.

ABSTRACT: Anterior skull base cerebrospinal fluid (CSF) fistulas result from skull base osteodural defects, allowing subarachnoid space CSF to escape into pneumatized cavities such as the paranasal sinuses and nasal fossa. Precise localization, characterization, and effective treatment of CSF leaks is essential to prevent meningitis, treatment failure, or recurrence. Advances in magnetic resonance imaging have improved radiologists' ability to localize and characterize anterior skull base CSF fistulas. This article reviews new imaging techniques enabling diagnostic location of CSF fistulas, with an emphasis on magnetic resonance imaging-based techniques.

IgG4-Related Disease of the Skull and Skull Base-A Systematic Review and Report of Two Cases.

OBJECTIVE: IgG4-related disease (IgG4-RD) is an inflammatory process that uncommonly can present in the skull base and calvarium and mimic a tumor but the nature of this condition is not well summarized in the neurosurgical literature. METHODS: A review was performed of 2 cases of IgG4-RD in the skull base highlighting the diagnostic challenges with assessment of these skull base lesions, and a systematic review of relevant literature was carried out. RESULTS: A systematic review of the literature conducted in accordance with PRISMA guidelines identified 113 articles, with 184 cases of IgG4-RD in the skull base or calvarium. The most commonly affected locations include the meninges, cavernous sinus, base of the posterior fossa, clivus, and mastoid bone. Headache, visual and auditory disturbances, cranial nerve dysfunction, and seizures were the most common presenting symptoms. Medical treatment was highly successful and most commonly consisted of corticosteroids coadministered with immunosuppressive agents such as rituximab. Prevalence seemed to be equal between sexes, and serum IgG4 levels were increased in 61% of patients. Delayed diagnosis and a need for multiple biopsies were reported in numerous cases. Two cases of skull base IgG4-RD from the authors' institution show the variable presentations of this disease. More invasive surgical biopsies were required in both cases, and corticosteroid treatment led to significant clinical improvement. CONCLUSIONS: IgG4-RD is an uncommon condition with an increasing body of reported cases that can affect the skull base and calvarium and should be in the differential diagnosis, because delay in diagnosis and treatment may be common.

[Otogenic meningitis and bilateral spontaneous dehiscences of the lateral skull base. Two case reports]. / Otogene Meningitis bei bilateralen knöchernen Dehiszenzen der Laterobasis. Zwei Fallberichte.

Meningitis in the case of spontaneous dehiscences of the lateral skull base is rare. We report on two patients with otogenic meningitis. High-resolution CT (HR-CT) showed bilateral dehiscences at the lateral skull base in both cases. Infection along the skull base was assumed. In the first case sufficient repair was achieved using a multilayered technique, while in the second case conservative treatment on an intensive care unit was successful. Conservative therapy can prove successful in exceptional cases.

Insidious onset of headache, diplopia and Horner's syndrome: a rare case of petrous bone osteomyelitis.

We present an unusual case of skull base osteomyelitis in an 88-year-old woman. She presented with gradual onset unilateral headache and diplopia. On examination, there was evidence of a left-sided Horner's and ipsilateral sixth nerve palsy. In addition to persistent raised inflammatory markers, an MRI neck identified signal change in the petrous bone confirming a diagnosis of skull base osteomyelitis. Skull base osteomyelitis should be considered in presentations of subacute raised inflammatory markers in the context of ipsilateral cranial nerve signs.

Bilateral traumatic abducens nerve palsy associated with skull base fracture--case report.

A 29-year-old man presented with skull base fractures involving the bilateral petrous bones and clivus to the posterior clinoid process manifesting as bilateral abducens nerves palsy. Conservative treatment resulted in residual bilateral abducens nerves palsy. Posterolateral impact probably resulted in strain-stress in the neighborhood of the foramen lacerum, resulting in a clivus fracture of the posterior clinoid process and bilateral petrous bone fractures. Chalasia of Gruber's ligament then exerted mechanical pressure on the bilateral abducens nerves.

[Endoscopic treatment of cerebral rhinorrhea]. / Endoskopowe leczenie plynotoku nosowego.

INTRODUCTION: The frequency of different factors causing cerebrospinal fluid rhinorrhea (CFR) has lately changed. The incidence of iatrogenic CFR has reached 10% of all cases of CFR, due to an increasing number of endoscopic operations of the sinuses and skull base, while idiopathic CFR is nowadays very rare. The current treatment method for CFR is surgical repair of the fistula. Endoscopic surgery of the anterior skull base has become the standard procedure for the repair of cerebrospinal fluid (CSF) leaks of various origins. The aim of this study was to analyse results of endoscopic surgical technique used in our department for the treatment of CFR. MATERIAL AND METHODS: Records of 5 patients aged from 46 to 69 (mean 58.2) years treated in the department between April 2004 and March 2006 were analysed retrospectively. 4 individuals had underwent endoscopic sinus surgery for sinus problems which resulted in iatrogenic CSF leak. One patient had idiopathic CFR. 3 fistulas localised in the neighbourhood of the cribriform plate were closed using an "underlay" technique with synthetic dura, and covered with free mucosal grafts from the nasal septum, kept in place by fibrin glue. The fistula in the neighbourhood of the sphenoid sinus posterior wall was closed using an ,,overlay" technique with surgical, covered with synthetic dura. RESULTS: In the 3 patients with cribriform plate fistulas the closure was successful and CFR did not recur during 6 to 9 month's follow-up. In the patient with sphenoid sinus fistula CFR recurred on exertion after 4 months. In one patient with cribriform plate fistula, CFR resolved spontaneously during preparation to surgery. CONCLUSIONS: Endoscopic closure of the skull base fistula represents a minimally invasive and highly successful procedure. Our experience suggests thet the optimal surgical technique in the region of cribriform plate consists in performing an "underlay" procedure with synthetic dura and covering the graft with free mucosal grafts from the nasal septum.

Advanced Imaging Techniques of the Skull Base.

Although conventional imaging can depict the anatomy of the head and neck with exquisite detail, it often falls short in its ability to characterize tissue physiology and abnormality; this is especially seen in the posttherapy setting where benign posttreatment changes and recurrent tumors can show intense postcontrast enhancement and similar features on conventional imaging. Advanced imaging can evaluate tissue physiology and, along with conventional imaging, provide a more accurate assessment of the skull base. This article describes the technical details and clinical applications of different advanced imaging techniques with a primary focus on diffusion-weighted imaging.

Overview of Sinonasal and Ventral Skull Base Malignancy Management.

Significant technological advances have fostered a movement toward minimally invasive surgical interventions for the management of ventral skull base malignancies. The care of patients with these lesions ideally involves an interdisciplinary skull base team that includes otolaryngologists, neurologic surgeons, radiation oncologists, and medical oncologists. This article describes considerations essential for diagnosis, prognosis, and preoperative evaluation. Furthermore, surgical nuances, strategies for skull base reconstruction, and nonsurgical options are briefly discussed. This overview may be useful as an up-to-date description of the challenging clinical scenarios associated with these lesions.

Long-Standing Scalp Retraction Technique Using an External Fixator for Sunken Skin Flap Syndrome.

BACKGROUND AND IMPORTANCE: The authors report a novel external scalp retraction technique for sunken skin flap syndrome (SSFS). CLINICAL PRESENTATION: A 48-year-old male patient suffered neurological deterioration due to SSFS after decompressive craniectomy. Cranioplasty is the gold standard for therapeutic management of SSFS, but could not be performed due to intracranial wound infection. The patient underwent external fixation of a metal plate as a frame for the skull and the scalp was pulled outward to the frame by skin suture. The patient returned to daily activities at home for 3 months with this scalp traction frame, which was needed for infection control. CONCLUSION: External scalp traction is useful for the temporary relief of pressure caused by SSFS.

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.

Excavating meningoencephaloceles: A newly recognized entity.

BACKGROUND: Spontaneous cerebrospinal fluid (CSF) leaks are largely attributed to idiopathic intracranial hypertension and typically present as skull base defects with or without prolapse of intracranial contents. However, in our practice, we have encountered a distinct type of spontaneous CSF leak that presents in a different manner. OBJECTIVE: To discuss a newly-classified, difficult to treat, subset of spontaneous CSF leaks that present as excavation of the bone of the skull base in a tunnel- or canal-like fashion by a meningocele or meningoencephalocele instead of as a localized area of bony dehiscence. METHODS: A retrospective review was performed at a tertiary care rhinology practice to identify a subset of CSF leak patients with an excavating/canal-like skull base defect visualized radiographically on computed tomography (CT) scan or magnetic resonance imaging and/or endoscopically in the operating room. RESULTS: The cohort of patients consisted of 7 females and 1 male with an average age of 53.6 years and a self-reported race of 4:3:1 African-American:Caucasian:Indian. All patients presented with CSF rhinorrhea. The most common leak site was the cribriform and upper septum. Six of the 8 patients had multiple defects and/or progression of their skull base defects, and 5 patients required multiple and/or repeat repairs in the operating room. Seven of the 8 patients underwent a cisternogram because the excavating nature of the leaks made it difficult to identify the specific leak site on high-resolution CT scan alone. CONCLUSION: In spontaneous CSF leaks that are difficult to identify or recur soon after a proper repair, an excavating pattern should be considered. Failure to recognize this type of leak and all of its tributaries, to fully unroof the excavated bone to completely resect the meningocele, and to visualize and close the site of origin will likely result in failure and recurrence of CSF leak.