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Surgery of the skull base.

Tumors involving the base of the skull are often occult and may become quite large before detection is possible. Symptomology varies depending upon the nature of the tumor and its placement. These neoplasms have frequently been considered inoperable simply because of their location, but in recent years microsurgical technique and high speed air drills have allowed the modern temporal bone surgeon to gain improved access to the skull base. A wide variety of surgical approaches to tumors in this area have been described in the past 20 years: translabyrinthine, middle fossa, transcochlear, retrolabyrinthine, retrosigmoid, transpalatal-transclival, and through the jugular bulb. The purpose of this paper is to review the indications, complications, and results of each of these procedures in relation to specific tumors involving the skull base. Case reports illustrate the diagnosis and surgical management of a variety of unusual neoplasms, including an extradural meningioma of the temporal bone and clivus, a low grade squamous cell carcinoma on the tegmen in a radical cavity, a large primary cholesteatoma, an osteoblastoma of the temporal and occipital bones, an XIth nerve neuroma in the jugular bulb area, and an osseous hemangioma involving the facial nerve at the geniculate ganglion.

Adolescent↗

Evaluation of quality of life and psychiatric morbidity in patients with malignant tumours of the skull base.

In general, patients with malignant tumours of the skull base have a poor prognosis. Treatment may have a disfiguring physical and disabling mental effect on patients. To evaluate the effect of treatment on physical and mental quality of life in patients with skull base malignancy, we conducted a cross-sectional patient survey of 18 patients treated for such tumours, using the University of Washington (version 4) quality of life questionnaire and the hospital anxiety and depression scale. The total quality of life score (median value) was 980 (550-1125). Patients with anterior skull base malignancy scored lower than those with lateral skull base malignancy (p=0.003). In general, the worst individual domain scores were: mood (64 per cent); activity (69 per cent); and, specifically for patients with anterior skull base malignancy, taste (54 per cent, p=0.004) and anxiety (60 per cent, p=0.034). One-third of skull base cancer patients were at risk of suffering from mental distress and psychiatric morbidity (indicated as a score of more than seven on the hospital anxiety and depression scale).

Adult↗

Cranionavigator combining a high-speed drill and a navigation system for skull base surgery--technical note.

Drilling of the skull base bone without damaging the important inside structures and with the correct orientation is very difficult even with the help of the anatomical landmarks. Monitoring of the location and direction of the drill tip and indications of the removed part of the bone during the drilling procedure enhances safety and achieves less invasive neurosurgery. We have developed a novel cranionavigator by combining a high-speed drill with a neurosurgical navigation system. To reduce the positional error to less than 1.5 mm, the position sensor (magnetic field sensor) must be attached 5 cm from the metallic fan portion of the drill and the sensor kept at least 10 cm away from the operating microscope. Simulation studies with the cranionavigator using two dried skulls and three cadaver heads were performed before clinical application. Clinically, this surgical instrument was used in four patients with the skull base tumor. The cranionavigator helped to safely drill the skull base bone in a shorter time by dynamic and real-time display of the precise operating site and extent of bone drilling on the preoperative computed tomography scans or magnetic resonance images. The cranionavigator is a very helpful instrument for skull base surgery in the hands of neurosurgeons with extensive expertise and anatomical knowledge.

Craniotomy↗

Endonasal endoscopic management of a large meningocephalocele in a patient with concomitant middle skull base defect.

The presence of a skull base defect can lead to major complications such as cerebrospinal fluid leak, meningocele, encephalocele and meningitis. It is exceptional to find the existence of two concomitant defects in the skull base. We present the case of a patient with concomitant spontaneous defects of the anterior and middle skull base that were surgically repaired. After 18 years of right rhinorrhea the patient was referred after being diagnosed with a large right nasal fossa meningoencephalocele, which was surgically removed by functional endoscopic sinus surgery. Following the surgery the patient complained about unilateral ear fullness. A paracentesis revealed a highly suspicious cerebrospinal fluid collection. High resolution scans revealed a defect in the mastoid tegmen; subsequently a transmastoid approach was carried out. Greater defects or those lying around the internal auditory canal, are best treated via the middle fossa approach. In the anterior cranial fossa the treatment of choice is provided by endoscopic procedures, but frontal bone craniotomy should be considered if the defect is in the frontal sinus or greater than 5 cm in size.

Cerebrospinal Fluid Rhinorrhea↗

Imaging of giant tumours involving the anterior skull base.

Tumours involving the anterior skull base are a challenge in diagnosis and treatment. They may arise from the bony skull base itself, intracranially or from the sinonasal tract and orbit. It is often difficult to determine the site of origin of giant tumours as anatomical boundaries are frequently breached. Accurate imaging evaluation is useful in planning treatment and may help in the differential diagnosis. We review those CT and MRI features of giant anterior cranial fossa tumours which may be helpful in identifying a pre-operative diagnosis.

Chondrosarcoma↗

Expression of Fas ligand in patients with evident skull base involvement of nasopharyngeal carcinoma.

We investigated whether skull base involvement in patients with nasopharyngeal carcinoma (NPC) is correlated with expression of Fas ligand (FasL) in NPC cells. A prospective assessment of FasL expression was determined by immunohistochemistry and in situ hybridization in 98 patients with newly diagnosed NPC. Among these patients, 21 had evident skull base involvement. Expressions of human apoptosis-related genes and FasL were confirmed by reverse transcription-polymerase chain reaction. Relation between the frequency of skull base involvement and FasL expression was analyzed by Chi-square and multivariate analyses. FasL expression was detected in 32 (32.6%) of 98 pathological sections. Compared to patients with low FasL expression in tumors, patients with notable FasL expression had higher incidence of skull base involvement (28.6 vs. 71.4%, p<0.005). Expression of FasL in tumor cells was correlated with the higher frequency of skull base involvement in patients with NPC.

Adult↗

Tumors of the skull base in children and adolescents.

OBJECT: Published data obtained in children with tumors of the skull base are sparse. In the majority of the available reports, the authors focus on the technical application of skull base approaches, but they contribute a paucity of information on the management of specific tumors, especially malignant skull base lesions. The purposes of this report are to increase the collective experience with the treatment of these tumors and to identify successful management paradigms. METHODS: The authors retrospectively reviewed the clinical records, pathological reports, and diagnostic images obtained in 24 children (< or = 19 years of age) with tumors arising from the cranial base in whom resection was part of their management between 1992 and 2002. Surgery-related complications and outcomes were analyzed with regard to tumor type and surgical approach. The median age of the group was 14 years. Tumors involved the anterior skull base in eight (33%), the middle skull base in 10 (42%), both the anterior and middle skull base in four (17%), and the posterior skull base in two patients (8%). Benign lesions were discovered in 11 patients (46%) and malignant neoplasms in 13 (54%). The tumors were most commonly of mesenchymal origin (21 [87.5%] of 24 tumors). Thirty surgical procedures were performed using a number of skull base approaches. A gross-total resection was achieved in 23 procedures (77%) and a subtotal resection in five (17%); a biopsy procedure was performed in one case; and the disease process could not be accessed in one case. One patient died in the perioperative period. Minor complications (Karnofsky Performance Scale score > or = 90, no prolongation of hospital stay, and no further surgery needed) occurred following 10 (33%) of the 30 surgical procedures. These affected 10 (42%) of the 24 patients and resulted in persistent or prolonged deficits in only five patients (21%). In nine patients (38%) the tumor recurred after a mean duration of 23 months. CONCLUSIONS: Skull base tumors in children affect mainly the anterior and middle cranial fossa. Sarcomas account for the majority of malignant tumors. Treatment of skull base tumors in children and adolescents needs to be tailored to patient age, tumor location, and tumor type.

Adolescent↗

[Reconstruction methods in skull base surgery].

Surgery for extensive skull base lesions inevitably creates multiple tissue defects that require reconstruction. In this paper reconstruction methods applied in 41 skull base surgery procedures are presented. Anterior cranial fossa was involved in 13 operations (4 malignant and 9 non-malignant tumours), middle and posterior cranial fossa in 28 (9 malignant and 19 non-malignant). As a rule all the defects were reconstructed in one stage, immediately after tumour removal. Titanium mesh was the only alloplastic material used to cover bone defects in 7 patients. Otherwise only autografts were used. Postoperatively in all the patients in whom excision and repair of dura was necessary, continuous lumbar drainage of cerebrospinal fluid was maintained for 4-6 days. In anterior cranial fossa area large pediculated, vascularized periosteal flap allowed for reliable separation of upper airways from intracranial cavity. Titanium mesh was used to cover orbital wall defects. In the area of middle cranial fossa and temporal bone the most important aspects of reconstruction were watertight closure of the dura, separation of the surgical cavity from nasopharynx with rotated temporalis muscle, and facial nerve reconstruction (the nerve was reconstructed in 7 cases). Additionally in 1 patient hypoglossal nerve was sutured end-to-end. In 2 patients the invaded segment of internal carotid artery was excised and replaced with prosthesis. In 13 patients the surgical procedure was performed in an area that was previously operated upon and/or irradiated. Delayed healing was seen in 6 cases, but none required additional procedures and the wounds were completely healed with local treatment. Reconstruction methods presented in this paper are relatively simple and do not necessitate modern materials. Even in extensive procedures proper wound healing and satisfactory cosmetic result were obtained.

Humans↗

Skull base growth in craniosynostosis.

INTRODUCTION: The interrelationship of skull base growth and craniosynostosis is one that is not often taken into account in the clinical management of patients with craniofacial disorders by neurosurgeons and craniofacial surgeons. Very early on in our medical training we are taught the anatomical differences between the calvarial unit and skull base portion of the skull. The inherent differences in both underlying tissue components and the different growth characteristics of these two uniquely different structures are critical in understanding skull base growth in craniosynostosis and the inherent potential craniofacial growth in these unique children. REVIEW: This paper will review some of the basic anatomy of these zones of growth plus review some of the prevalent theories of the effect of skull base growth on craniosynostosis and the converse. CONCLUSION: While the theories of these growth patterns have yet to be finalized an understanding of their potential influences and abnormal growth patterns remain key to providing a good surgical outcome in surgery for craniosynostosis.

Cranial Sutures↗

Multiple skull base meningioma: case report.

BACKGROUND: The incidence of multiple skull base meningiomas varies from 1 to 3% in different series. Skull base meningiomas are rare. The pathogenetic role of low-dose radiation seems to be fairly well established in the oncogenesis of meningiomas. Calvarial location and multiplicity seem to be among the distinctive features of radiation-induced meningiomas. Skull base location is a very rare occurrence, mainly because the path of irradiation does not significantly involve this region. CASE REPORT: We describe a rare case of simultaneous occurrence of two skull base meningiomas in a 66-year-old female. This patient underwent low-dose irradiation for tinea capitis when she was 8 years old. The patient complained of nuchal pain, paresthesias in both hands, and progressive weakness on her right side. She was admitted to the hospital in September 1994. An MRI showed two masses, one located at the level of the tuberculum sellae and the other at the foramen magnum. These seemed very likely to be multiple meningiomas. The latter lesion, which was more symptomatic and dangerous, was operated on first. Six months later, elective treatment of the suprasellar meningioma was performed with success. CONCLUSION: The actual role of previous head irradiation in the oncogenesis of the present meningiomas remains somewhat unclear. Proper management and judicious use of skull base surgery techniques were key factors in the successful treatment of the patient.

Aged↗

Skull-base metastases.

Metastasis to the skull-base particularly affects patients with carcinoma of the breast and prostate. Clinically, the key feature is progressive ipsilateral involvement of cranial nerves. Five syndromes have been described according to the metastatic site including the orbital, parasellar, middle-fossa, jugular foramen and occipital condyle syndromes. Magnetic resonance imaging (MRI) is nowadays the most useful examination to establish the diagnosis but plain films, CT scans with bone windows and isotope bone scans remain helpful to demonstrate bone erosion. Normal imaging studies do not exclude the diagnosis. The treatment depends on the nature of the underlying tumor. Radiotherapy is generally the standard treatment, while some patients with chemosensitive or hormonosensitive lesions benefit from chemotherapy or hormonotherapy and selected patients from surgical removal. Gamma Knife radiosurgery is sometimes a useful alternative, particularly for previously irradiated skull-base regions, and for small tumors (diameter < 30 mm). The overall prognosis is poor, with an overall median survival of about 2.5 years, probably because skull-base metastases appear late in the course of the disease.

Humans↗

Use of microvascular free flaps in the reconstruction of the anterior and middle skull base.

Different reconstruction techniques of the anterior and middle skull base as consequence of a defect after surgical treatment of neoplastic pathologies are described in the literature. The aim of the present study is to present our experience regarding the use of microvascular free flaps for reconstruction of the anterior or middle skull base after large defects caused by removal of malignant neoplasms. From 2000 to 2004, in the Department of Maxillo-Facial Surgery of the University of Rome "La Sapienza" and "Tor Vergata," 13 surgical procedures for reconstruction of anterior and middle skull base defects by free flaps were performed in 11 patients. Data on patient demographics, histopathology, location and size of defect, type of reconstruction, and postoperative complications were obtained from medical record charts. A safe soft tissue closure of the intracranial space was achieved in all patients. Defect repair was accomplished by revascularized transfer of rectus abdominis flaps in seven cases, latissimus dorsi muscle flaps in two patients, radial forearm flap in one case, and fibula flap in one case. There were two total flap losses; the secondary defect repair was accomplished in both cases by revascularized transfer of latissimus dorsi muscle flap. No donor site complications were observed in all the flaps. The mean operation time was 85 hours; patients were hospitalized for a mean period of 14 days. The method of choice for the reconstruction of anterior or middle skull base defect should be based upon careful evaluation of the single case and, particularly, the localization and entity of the residual defect. For defects that require large amounts of soft tissue, the latissimus dorsi free flap and the rectus abdominis free flap are the best appropriate choices for reconstructive procedures for anterior and middle skull base tumors.

Adolescent↗

Chondrosarcoma of the skull base: long-term follow-up.

OBJECTIVE: Chondrosarcoma of the skull base is an uncommon neoplasm comprising 0.15% of all intracranial tumors and 6% of skull base neoplasms. The outcome of treatment is difficult to assess because the slow growth rate means that there is a long interval before detecting the recurrence. We describe the use of lateral skull base techniques for these lesions and examine the long-term outcomes. The pathological features, radiological findings, and radiotherapy options are also discussed. STUDY DESIGN: Retrospective case review with current follow-up where possible. SETTING: Tertiary referral neurotologic private practice. PATIENTS: Eight patients with histologically confirmed skull base chondrosarcoma operated on since 1979. At the time of the surgery, the five women and three men ranged in age from 31 to 63 years, with a mean of 42.9 years. The follow-up ranged from 9 months to 25 years and 5 months. INTERVENTIONS: All patients underwent surgical removal; the earliest patient underwent surgical removal via transcochlear and retrosigmoid approaches, and seven via an infratemporal fossa approach, with or without exenteration of the otic capsule. MAIN OUTCOME MEASURES: Number of patients with residual and recurrent tumors. RESULTS: In the patient who underwent surgery via the transcochlear approach, recurrence was noted within 3 months, and new exploration using the retrosigmoid approach was performed for the removal of the recurrent lesion. This patient showed no signs of recurrence for 25 years since last surgery. Of the seven patients who underwent surgery via the infratemporal fossa approach, one had residual tumor found at 1 year, but remained alive and well for 21 years after the surgery. The other six had gross total resection and showed no signs of recurrence at the last known follow-up. One patient died as a result of an unrelated cause 18 years after surgery for the chondrosarcoma. CONCLUSION: The gross total resection of these lesions is possible because of the evolution of lateral skull base techniques and can be routinely accomplished with the infratemporal fossa approach. The improved exposure afforded by this approach seems to have resulted in more complete extirpation of the tumor and a decrease in the recurrence rate.

Adult↗

Selective reconstructive options for the anterior skull base.

Carcinomas of the ethmoid, frontal, or maxillary sinuses sometimes invade the anterior skull base. It is necessary to perform en-bloc resection for this invasive carcinoma according to the concepts of surgical treatment for head and neck cancer. The anterior skull base consists of two parts, the orbital roof as the lateral portion and the roofs of the frontal sinus, ethmoid sinus, and/or sphenoid sinus as the central portion. Selective reconstructive options for the anterior skull base depend on the size of the defect of the skull base. A dural defect is repaired by a fascia lata or a pericranial flap. After the dura has been tacked up, reconstruction of the anterior skull base is performed simultaneously with augmentation of the defect of extracranial structures. Larger defects that consist of both central and lateral portions with orbitomaxillary structures are reconstructed by a bulky musculocutaneous flap such as a rectus abdominis or latissimus dorsi flap. The bony reconstruction of supraorbital structures is also to be considered esthetically. On the other hand, intraorbital tissues are basically preserved in cases of central defects of the anterior skull base. These defects are reconstructed by a free forearm flap or a local flap such as a de-epithelialized midline forehead flap or a pericranial flap. We have selected and applied these flaps in 37 patients as reconstructive options for the anterior skull base since 1989. Eleven of the 37 patients had larger defects and 26 had central defects. De-epithelialized midline forehead flaps were used in 20 patients and were recognized to be a very useful and reliable reconstructive option for central defects of the anterior skull base.

Child, Preschool↗

[Impact of dose boost in skull base on recurrence of stage T4 nasopharyngeal carcinoma].

BACKGROUND & OBJECTIVE: The recurrence rate in skull base is high for nasopharyngeal carcinoma (NPC) patients with cavernous sinus or/and sphenoid sinus involvement. This study was conducted to explore the impact of dose boost in skull base on the recurrence in skull base and survival of NPC patients with cavernous sinus or/and sphenoid sinus involvement. METHODS: A total of 120 stage T4 NPC patients with cavernous sinus or/and sphenoid sinus involvement proved by histopathology and computed tomography (CT) were treated in our hospital from Oct. 1996 to Nov. 1998. The irradiation dose was (71.55+/-3.09) Gy in nasopharynx and (58.95+/-6.16) Gy in neck. Of the 120 patients, 27 received irradiation (6-10 Gy) in skull base after radiotherapy (boost group), 93 did not receive irradiation in skull base (control group). Fifty-three patients, 41 in control group and 12 in boost group, received cisplatin-based chemotherapy for 1-3 cycles. The survival rates and the recurrence of the 2 groups were calculated by Kaplan-Meier method and log-rank test. RESULTS: The clinical characteristics of patients were similar in the 2 groups. The 1-, 3-, 5-year skull base control rates were significantly higher in boost group than in control group (100% vs. 93.4%, 91.8% vs. 76.4%, and 86.8% vs. 65.1%, respectively, P=0.045). The 1-, 3-, 5-year disease-freely survival rates were slightly higher in boost group than in control group (85.2% vs. 75.3%, 59.3% vs. 45.2%, and 51.9% vs. 31.2%, respectively, P=0.084). The median disease-freely survival time was longer in boost group than in control group (60 months vs. 30 months). The prevalences of irradiation-induced brain damage were 7.4% in boost group and 4.3% in control group with no significant difference (P=0.514). CONCLUSION: Dose boost in skull base can reduce the recurrence of stage T4 NPC in skull base and tends to enhance the disease-freely survival rate for NPC patients with cavernous sinus or/and sphenoid sinus involvementû it is recommended to such patients.

Adult↗

Masked pseudomonal skull base osteomyelitis presenting with a bilateral Xth cranial nerve palsy.

Skull base osteomyelitis classically presents as a complication of severe external otitis, middle ear, mastoid or sinus infection and can lead to multiple lower cranial nerve palsies when the jugular foramen is involved as a consequence of widespread involvement of the skull base. Bilateral skull base osteomyelitis is a recognized phenomenon, but has not previously been reported secondary to pseudomonal infection in the absence of a clinically obvious focus of infection. We report the case of a 77-year-old diabetic patient who presented with dysphonia and dysphagia and had a bilateral Xth cranial nerve palsy. No focus of infection was evident on presentation. Subsequent radiological investigation confirmed the diagnosis of bilateral skull base osteomyelitis.

Aged↗

Occult malformations of the skull base.

Occult malformations of the skull base are rare anomalies. They are often not detected until they give rise to complications such as meningitis or cerebrospinal fluid rhinorrhea. We used high-resolution computed tomography, fluorescein endoscopy, cisternography, magnetic resonance imaging, and duraplasty to diagnose occult malformations of the skull base in 4 patients. The four patients had had between 4 and 6 attacks of meningitis. Cerebrospinal fluid rhinorrhea was confirmed in 3 cases. The following occult malformations were found: (1) an encephalocele of the glabella/cribriform plate and a meningocele at the petrous bone apex, (2) a meningoencephalocele at the petrous bone apex, (3) dural lesions in the regions foramen rotundum/sphenoid sinus and frontal sinus/cribriform plate, and (4) a dural lesion of the sphenoid sinus. In patients presenting with recurrent meningitis, meningitis with isolation of upper airway pathogens, or cerebrospinal fluid rhinorrhea, modern diagnostic methods should be used to search for dural lesions. Diagnosis of an occult malformation makes it possible to perform the necessary surgical repair and thus prevent the further occurrence of potentially fatal episodes of meningitis.

Adolescent↗

Linear accelerator radiosurgery for recurrent malignant tumors of the skull base.

The efficacy of linear accelerator-based radiosurgery for patients who have preirradiated recurrent nasopharyngeal carcinomas and unresectable recurrent sarcomas invading the base of skull was assessed. Thirteen patients were treated: 8 patients had carcinomas arising from the nasopharynx (lymphoepithelioma, 4; squamous cell carcinoma, 2; adenoid-cystic, 2); 5 patients had sarcomas (rhabdomyosarcoma, 1; chordoma, 1; chondrosarcoma, 1; hemangiopericytoma, 2). All patients had had repeated tumor resections or irradiation, hindering any further conventional fractionated radiotherapy or surgery. Convergent-beam irradiation was performed with a modified linear accelerator (8-MeV photons). Because of irregular tumor configuration, multiple (up to seven) isocenters had to be used in 10 of 13 patients to match the target volume with the reference isodose (60%-80%). Each isocenter was irradiated with 6 to 10 arcs. The median planning target volume was 33 mL (4-128 mL) and the median dose was 15 Gy (9-24 Gy). Median survival time was 9 months in 8 patients who had recurrent nasopharyngeal carcinomas. Three patients who had complete or partial tumor remission survived 1.5 to 3.5 years. All of the sarcoma patients responded to radiosurgery. After a follow-up of 28 to 67 months, 4 of 5 patients are alive. This investigation demonstrates that radiosurgery is an effective tool in palliative treatment for patients who have recurrent, extensively pretreated nasopharyngeal cancer. Patients who have recurrent sarcomas of the base of skull may be treated for long-term palliation or even for cure.

Adult↗