Aggressive fibromatosis (fibrosarcoma) of the facial nerve

Aggressive fibromatosis of the facial nerve is a very rare tumor. Three cases have been previously reported. The tumor is locally recurrent and often has a fatal outcome. This report is of a ten-year-old boy whose tumor originally developed in the parotid area with subsequent spread to the base of the skull, the neck and the cerebellopontine angle. Treatment was by wide surgical excision, radiation therapy and chemotherapy. Despite treatment, the patient died. The clinical features of this case will be described. Only wide surgical excision early in the course of the disease may offer a chance for cure.     

The "ART" mandibular nerve block: a new approach to accomplishing regional anesthesia

A study was conducted from February 29, 1996, to March 28, 1996, at the University of Brno's Pathology Institute in the Czech Republic to explore the possible application of craniofacial intracranial endoscopic techniques through minimal skin incisions and trephines in fresh cadavers (3 to 12 hours old). Through the trephines the dura was totally dissected from the bone. After this dissection a standard bicoronal incision and a full craniotomy was performed to assess the integrity of the meninges. This minimally invasive dissection of the skull base with the aid of an endoscope is characterized by fewer skin incisions, thereby avoiding the exposure of subcutaneous tissue, muscle, cranial bone, and meninges. We find that it is possible to accomplish an accurate and extensive intracranial dissection with the aid of an endoscope. The potential of this technique is important for craniofacial surgery as well as for neurosurgery. The objectives of this study were [1] to explore endoscopically the craniofacial anatomy to determine the best approach and the optimal method for dissecting endocranial structures, and [2] to achieve a safer and more accurate dissection of the skull base, evaluating advantages of endoscopic surgery as an alternative method for the treatment of craniofacial and neurosurgical pathologies.     

Childhood fibrous dysplasia presenting as blindness: a skull base approach for resection and immediate reconstruction

Fibrous dysplasia is an abnormal fibroosseous process of bone of unknown cause. The incidence of skull involvement varies, painless enlargement being the most common presenting symptom. Change in vision is a rare but recognized finding. We report a 3-year-old boy with extreme fibrous dysplasia involving the skull base, who presented with blindness. He underwent exposure osteotomies of the frontal bones and orbits to provide access for skull base tumor removal. The orbital roofs were reconstructed with microplate-fixed cranial grafts. One and one half years after tumor excision followed by immediate reconstruction, the boy retains facial symmetry, and his ocular function has not deteriorated.     

Indications for, contraindications to, and interruption of craniofacial procedures

In spite of increasing experience with skull base surgery, some of the guidelines for indications for operations may vary according to the institution. One-hundred two patients underwent craniofacial oncologic resections at our institution from 1982 to 1995. A retrospective analysis of the indications for and contraindications to these procedures was undertaken. The main indications for malignant tumors were skin lesions with direct invasion of the anterior or lateral skull base (69%) and nasal-paranasal sinus tumors (21%). The main indications for benign tumors were glomus lesions (26%), menigiomas (22%), and fibro-osseous lesions of the anterior skull base (19%). The main contraindications were extensive invasion of the central nervous system, invasion of the cavernous sinus and/or internal carotid artery by aggressive malignancies, and bilateral orbital invasion in a nonblind patient. Also, 6 patients had their procedures interrupted during craniotomy for several reasons - extensive central nervous system invasion (2 cases), bilateral orbital invasion (1), lack of brain retraction (1), lack of histologic diagnosis during the operation (1), and purulent discharge at the frontal sinus (1). Craniofacial oncologic operations are extensive surgical procedures that have to be properly indicated in order to obtain low levels of morbidity and mortality. The selection of cases is of paramount importance. In some instances, it seems advisable even to interrupt these operations in the first phase.     

Differential expression of costimulatory molecules in chronic inflammatory periodontal disease tissue

OBJECTIVES: To present the technique of the extended subcranial approach to the anterior skull base and to review the results in 55 patients who underwent the procedure. STUDY DESIGN: Retrospective review of the records of 55 patients who underwent the extended subcranial approach to the anterior skull base between 1994 and 1998 for the treatment of various neoplasms originating in the nasal cavity, nasopharynx, paranasal sinuses, orbit, or meninges, as well as for the repair of complex craniofacial trauma and/or cerebrospinal fluid (CSF) leak. Preoperative patient evaluation and the surgical technique are also reviewed. METHODS: Patient records were retrospectively reviewed and tabulated for age, sex, and indications for procedure, with special focus on early outcome and complications. RESULTS: Twenty-six patients underwent oncologic resections, 22 patients had reduction of complex fronto-naso-orbital and skull base fractures, and seven patients had repair of CSF leak. Significant complications in the oncologic group consisted of one hematoma requiring needle aspiration and two cases of temporary nontension pneumocephalus. In the fracture group, one patient died because of extensive intracerebral damage and multiorgan failure, and one patient had nontension pneumocephalus coupled with CSF leakage and one patient had temporary nontension pneumocephalus. The most common late complication in all three groups was anosmia. CONCLUSIONS: Based on their review, the authors conclude that the extended subcranial approach to the anterior skull base is a safe, versatile, and effective procedure for the surgical treatment of various pathological conditions involving the anterior skull base.     

The surgical management of giant cervicofacial lymphatic malformations

Giant cervicofacial lymphatic malformation, a potentially life-threatening congenital malformation, historically has been removed in staged resections. However, complete surgical extirpation is desirable and can be achieved with the aid of a multidisciplinary team. The authors present the case of a 12-year-old girl who had an extensively enlarged cervicofacial lymphatic malformation. Effective treatment involved aggressive surgical excision, facilitated by mandibular osteotomies to provide access to the floor of the mouth, the tongue, and the pharyngeal wall. In addition, total removal of involved facial and neck skin was performed. The remaining soft tissue defect was reconstructed primarily with an abdominal musculocutaneous free flap. In the 5 years since the procedure, there has been no recurrence. Based on this experience, excision of involved skin and mandibular access osteotomies are important techniques to help completely remove cervicofacial lymphatic malformations with oral involvement, thus potentially reducing the number of recurrences. In addition, these cases require the best efforts of a multidisciplinary team to achieve a successful result so that complete and radical excision can be performed with the hope of avoiding the pitfalls of multiple resections.     

Managing hearing loss in a patient with Alzheimer disease

Advances in combined transcranial and transfacial (craniofacial) approaches for malignant tumors involving the anterior skull base have demonstrated improved survival. The technique allows adequate assessment of the intracranial extent of the tumor through an appropriate craniotomy. Vital structures, such as the dura, brain, and blood vessels, can be protected or resected and reconstructed safely. An en bloc excision can be accomplished. Dural defects and/or tears are satisfactorily repaired under direct vision, ensuring a watertight closure. Finally, adequate closure of the soft tissue defect is obtained, thus segregating the cranial cavity from the potentially infected nasal cavity and the nasopharynx with a resultant decrease in morbidity. Operative mortality is low, although complication rates are high. The technique is safe and continues to be improved to reduce morbidity. To evaluate the true impact of this surgical procedure on improvement in survival as well as quality of life, a multiinstitutional registry with uniform indications is indicated. With increasing experience and well-defined indications, improvement in survival (from 50% to 60%) and reduction in morbidity (from 30% to 40%) can be demonstrated through multiinstitutional, cooperative efforts.     

Lymphoblastoid alpha-interferon for chronic hepatitis C: a randomized controlled study

The orientation of the sella nasion (SN) line in lateral skull radiographs is important for accurate assessment of antero-posterior and vertical facial relationship. Lateral skull radiographs of a random sample were traced and digitized to produce a mean shape template of the sample, with the craniofacial centroid line (CFC) used as a base for superimposition of the sample. A geometrical triangle was formed using the sella nasion line, craniofacial centroid line and the mandibular plane as the sides of the triangle. Simple geometric calculations were done to re-orient both the mandibular plane and sella nasion line in cases with balanced clinical profiles, while the S-N-A and S-N-B measurements indicate otherwise.     

Structure-based redesign of the catalytic/metal binding site of Cfr10I restriction endonuclease reveals importance of spatial rather than sequence conservation of active centre residues

Thirty years ago, submental fat deposition and platysma band formation were inadequately addressed by surgeons attempting facial rejuvenation procedures. Simple skin envelope tightening as practiced commonly at that time resulted in reappearance of submental bands within months, disappointing both patient and surgeon. During each subsequent decade, existing techniques for aesthetic surgical treatment of the submental and neck areas were refined, and new techniques were introduced. The earliest in this evolution of surgical approaches to rejuvenate the aging submental region was direct excision of redundant anterior cervical skin and platysma muscle bands. Later, periauricular incision approaches without anterior skin excision were used to address platysma muscle bands and remove excess submental fat. Today, the standard approach for submental and anterior neck rejuvenation is some variation of "corset" platysmaplasty and suction-assisted lipectomy both superficial and deep to the platysma muscles. The introduction of endoscopy to plastic surgery has stimulated interest in performing many surgical procedures through limited-incision approaches. While endoscopic techniques may not have a clear application for the submental area, redundant submental soft tissues producing obliquity of the cervicomandibular angle may be treated through a single submental incision approach under direct vision. A technique practiced by the author since 1981 for performing submental lipectomy and platysmaplasty through a single limited anterior submental incision without skin excision is described and discussed. Postoperative compression of the submental area with an elastic garment facilitates smooth skin contraction and redraping for all patients except those with poor or no residual skin elasticity.     

Vagal paraganglioma: a review of 46 patients treated during a 20-year period

BACKGROUND: Vagal paragangliomas (VPs) arise from paraganglia associated with the vagus nerve. Approximately 200 cases have been reported in the medical literature. Because of their rarity, most information regarding these tumors has arisen from case reports and small clinical series. OBJECTIVE: To detail the clinicopathologic features of 46 patients with VP with an emphasis on the role of a multidisciplinary skull base team in both the successful extirpation and rehabilitation. DESIGN: Retrospective review of 46 patients with VP managed by a single skull base team. SETTING: An academic tertiary medical center. RESULTS: Forty-six patients were treated over a 20-year period (1978-1998). Ten (22%) demonstrated intracranial extension. There was a history of familial paragangliomas in 9 (20%) of the patients. The incidence of multicentric paragangliomas was 78% in patients with familial paragangliomas vs 23% in patients with nonfamilial paragangliomas. Management of this group of 46 patients consisted of surgery (n = 40), radiation therapy (n = 4), and observation (n = 2). The operative approach consisted of a transcervical excision often combined with a transtemporal or lateral skull base approach as dictated by the tumor extent. Postoperative cranial nerve deficits were common, and, as such, aggressive rehabilitation was a vital component in the management of these tumors. CONCLUSIONS: The management of VP and its associated cranial nerve deficits remains a difficult clinical problem. Options for treatment include surgical resection, radiation therapy, and, in selected cases, observation. Surgical extirpation requires a multidisciplinary skull base team to achieve complete tumor resection. Radiation therapy is reserved for elderly patients and patients at risk for bilateral cranial nerve deficits. Rehabilitation of cranial nerve deficits is an integral part of the management of VP.     

Modern imaging of craniofacial malformations

Due to progress in the field of medical imaging of craniofacial malformations, the place of these investigations in the assessment of these abnormalities needs to be revised. 3D CT scan currently remains the fundamental element of the assessment by providing a truly anatomical dissection of each bone. In the field of craniostenoses, the study of the base of the skull has allowed a new assessment of lesions of skull base and craniofacial sutures and the resulting skeletal deformities: they provide restrospective justification for an extensive approach to the surgery of this group of malformations and a basis for reflection concerning extension to direct skull base surgery. Craniofacial clefts constitute a heterogeneous groupe of anomalies in which Tessier's concepts have allowed a methodical approach. 3D CT allows better definition of certain subgroups of malformations within this group (midfacial clefts) and provides a clearer understanding of the skeletal defects of maxillary clefts, especially in the laterofacial region. This imaging is currently undergoing rapid development. Improvement of 3D CT scanning techniques (direct 3D image acquisition, improvement of the images obtained), development and combination of 3D MRI, after being superimposed onto the skeletal image, will allow total dissection of the malformation. Development of 3D cephalometric analysis techniques and growth analysis software will allow really predictive "image-assisted surgery". Finally, antenatal imaging (B-mode and 3D ultrasonography) makes a considerable contribution to this field of anomalies by allowing the diagnosis of serious or severely disabling anomalies and by elucidating the antenatal development of certain anomalies (especially craniostenoses) and their consequences.     

Implementation of guidelines for No-CPR orders by a general medicine unit in a teaching hospital

Improvements in reconstruction of the skull base have made craniofacial surgery safe. Reconstruction of the anterior skull base must provide a seal between the cranial cavity and upper respiratory tract, as well as offer structural support for the brain. A wide variety of local flaps have been designed. The choice of flap in individual cases depends on the location and size of the defect. We report a reconstructive technique for the anterior skull base with vertical median forehead flaps which we used to treat two patients, one patient with adenocarcinoma and the other with leiomyosarcoma. Both were lesions of the ethmoid sinuses and nasal cavity.     

Average African American three-dimensional computed tomography skull images: the potential clinical importance of ethnicity and sex

The production of average 'normative' three-dimensional (3D) computed tomography surface images of the bony skull has only recently been explored. The authors wish to determine the effect of using sex- and ethnicity-specific adult average 3D skull images for comparisons with patient images at various stages of craniofacial surgical management (i.e., diagnosis, treatment planning, prosthetic design, image-guided operative procedures, and outcomes assessment). Craniofacial surgical reconstruction for abnormal patterns of development, cancer resection, or trauma are most likely to benefit from these comparisons. To morphometrically test the significance of separating normative 3D skull data by sex and ethnicity, the authors collected 52 3D, anatomical landmarks from 3D computed tomography scans of dry skulls of 20 Americans of European ethnicity and 20 Americans of primarily African (i.e., primarily African and some European) ethnicity. A Procrustes-based morphometric analysis of shape detects 1.2 times as much interethnic variance as intersex variance. The African American sample presents 4.2% more dolichocephaly, wider orbits, flatter nasal area, larger gnathic anatomy, and more procumbent dentition. Pooling the sexes across both groups, it is seen that men tend to have less bulbous crania, more protruding brows, noses, and masticatory muscle attachments, and relatively less protrusive palettes and anterior mandibles. Despite a small sample size (N = 40), the authors' results are statistically significant (P approximately 0.001 overall) for both of the main factors, sex and ethnicity, separately.     

Hemifacial dismasking flap for anterior skull base tumor--technical note

A new approach to malignant tumor in the anterior skull base using a hemifacial dismasking flap is described. A bicoronal incision is extended unilaterally down to the neck, degloving the craniofacial tissue to widely expose the skeleton underneath, allowing easy resection of an extensive tumor without postoperative scarring of the face. This method has been used successfully on five patients.     

Model-based surgical planning and simulation of cranial base surgery

Plastic skull models of seven individual patients were fabricated by stereolithography from three-dimensional data based on computed tomography bone images. Skull models were utilized for neurosurgical planning and simulation in the seven patients with cranial base lesions that were difficult to remove. Surgical approaches and areas of craniotomy were evaluated using the fabricated skull models. In preoperative simulations, hand-made models of the tumors, major vessels and nerves were placed in the skull models. Step-by-step simulation of surgical procedures was performed using actual surgical tools. The advantages of using skull models to plan and simulate cranial base surgery include a better understanding of anatomic relationships, preoperative evaluation of the proposed procedure, increased understanding by the patient and family, and improved educational experiences for residents and other medical staff. The disadvantages of using skull models include the time and cost of making the models. The skull models provide a more realistic tool that is easier to handle than computer-graphic images. Surgical simulation using models facilitates difficult cranial base surgery and may help reduce surgical complications.     

Anterior approach for CT-guided biopsy of skull base and parapharyngeal space lesions

At our institution we use an anterior approach to biopsy of the parapharyngeal space or skull base lesions because it provides more direct access than the traditional lateral approach through the mandibular notch. The anterior approach follows a course lateral to the alveolar ridge of the maxilla and lateral pterygoid plate, and inferior to the zygomatic process of the maxilla. Biopsy was performed on 15 patients with either a skull base or a parapharyngeal space mass, none of which could be palpated externally or through the oral cavity by the ear, nose, and throat surgeon. In 12 patients the needle biopsy correlated with the surgical pathology. Three needle biopsies were nondiagnostic.     

Comparative effects of neonatal and prepubertal castration on craniofacial growth in rats

The role of endogenous testosterone in the craniofacial growth of the young male rat was investigated. First, the effect of neonatal surgical castration was examined in a randomized, cross-sectional study in which male Wistar rats were allocated to be either castrated or sham-operated 4 h after birth. Then, the effect of prepubertal chemical castration was analysed in a second, randomized longitudinal study in which male Wistar rats were randomly allocated either to a control group or to two experimental groups, one injected with triptorelin at day 25 and the other injected on day 25 and on day 45. Every tenth day between 20 and 70 days of age for the first study, and between 30 and 110 days of age for the second, body length and weight were measured, cephalometric X-rays taken, and blood samples obtained. Neonatal and prepubertal castration resulted in decreased plasma concentrations of testosterone and in delayed growth of somatic and craniofacial components. The initiation, duration and magnitude of the effect was dependent on individual bones (cranial base, skull roof) and on the lower incisor, and related to the testosterone concentrations. These results suggest that testosterone effects participate in the process of normal craniofacial growth, particularly during puberty.     

p53-mediated regulation of proliferating cell nuclear antigen expression in cells exposed to ionizing radiation

BACKGROUND: The complications associated with anterior craniofacial resections for benign and malignant tumors were reviewed in 104 patients treated between January 1981 and June 1996. METHODS: Information regarding patient characteristics, histologic type, history of prior therapy, extent of the disease, extent of surgical procedure, and type of reconstruction were entered in a microcomputer database. To better understand and stage postoperative complications, we divided them into early (<14 days) and late (>14 days) according to the time of presentation, into major and minor depending on the morbidity potential of complication, and into local and systemic ones. Comparison between risk factors associated with complications was made using chi-square analysis with Yates' correction for continuity. Survival analysis was performed using the Kaplan-Meier product limit method. RESULTS: There were 8 (7.6%) postoperative deaths, with only 1 occurring from systemic complications. Complications occurred in 53 (48.6%) patients. Local major complications occurred in 49 (45%) patients, local minor in 29 (26.6%), and systemic in 11 (10%). Early complications occurred in 40 (38.5%) patients and late complications in 13 (12.5%) patients. These complications developed during a period ranging from 1 day to 5 months. More than one complication occurred in a number of patients. Bacterial contamination leading to local septic complications was the principal cause of morbidity, accounting for 54.7% (29/53) of complications. Major complications included meningitis in 8 patients associated with cerebrospinal fluid leak in 7, cerebral abscess in 2, sepsis in 1, and subdural hemorrhage in 1, all of which resulted in death except for one case. The extent of the craniofacial resection (p = .011) was the most important factor associated with major complications. Invasion of the dura and the type of reconstruction of the anterior skull base were the most important factors related to cerebrospinal fluid leakage (p = .048 and p = .032) and meningitis (p = .011). CONCLUSION: Contemporary surgical approaches and methods of reconstruction have enabled skull base surgeons to extend their cranial base resections and increase the 5-year survival rates of patients. Nevertheless, significant complications persist. Knowledge and high index of suspicion together with early recognition of these complications are essential for effective management of patients undergoing craniofacial resection. The factors related to major complications found in this study stressed the need to develop more effective methods to prevent contamination of intracranial structures.     

The biotype and genetic characteristics of an isolate of the cowpox virus causing infection in a child

The study included 138 patients operated on for endo-extracellular pituitary adenomas which extend both intracranially and into the structures of the base of the skull. Operations via transcranial and transsphenoidal access to various tumor sites were performed in 38 patients (a main group), while 100 patients (a control group) underwent one of these operations. Two-stage operations, followed by removal of the suprasellar and basal regions of a tumor, are expedient for enhancing the efficiency of surgical treatment, reducing the incidence of complications associated with traumatic attempts at removing tumor parts hard-to-reach by transcranial or transsphenoidal approaches, as well as at reducing the number of relapses. At the first stage of surgical treatment it is advisable to make an intervention via transcranial access especially in cases of complex configuration of the suprasellar part of a tumor. The recommended interval between transcranial and transsphenoidal surgeries is 3-5 months. Two-stage surgical treatment does not lead to significant structural changes and to the increased number of complications, and to higher mortality rates as compared to one-stage surgery (transcranial or transsphenoidal surgeries alone).     

Cyclin D1 overexpression related to retinoblastoma protein expression as a prognostic marker in human oesophageal squamous cell carcinoma

Aspergillus sp. sinusitis is not uncommon in immunocompromised patients but is unusual in patients who are not immunocompromised. The disease may occur as a saprophytic condition, as an allergic sinusitis or as a potentially lethal invasive disease. The differentiation between non-invasive and invasive Aspergillus sp. sinusitis is crucial and this distinction is fully discussed. The treatment options are also considered. Invasive disease requires aggressive treatment with long-term antifungal agents in sufficient doses combined with wide surgical excision. We present a patient who presented with invasive Aspergillus fumigatus sinusitis and subsequently developed cranial neuropathies and skull base osteitis. She was initially treated with oral itraconazole (400 mg daily) for 18 months but due to lack of response this was changed to a new experimental oral azole (voriconazole) which was continued for a further 14 months. She has since remained well for the last five years.