Fronto-orbito-zygomatic (FOZ) approach for infratemporal fossa lesions extending to middle cranial fossa: our experience and review of literature.

AIM OF THE STUDY: Tumours of the infratemporal fossa (ITF) are rare and include primary tumours, contiguity lesions and metastases. Surgical resection is the gold standard. The fronto-orbito-zygomatic (FOZ) approach is commonly used in order to obtain safe access to the lateral skull base and ITF to resect intra- and extra-cranial tumours. We here describe our series of ITF lesions extending to the middle cranial fossa and/or orbit, treated by single- or two piece FOZ. MATERIAL AND METHODS: All cases of single- or two-piece FOZ approach for an infratemporal fossa lesion extending to the middle cranial fossa operated at our Institution from January 2014 to January 2018 were retrospectively reviewed. The follow-up was for a minimum of four months and a maximum of 60 months. The inclusion criteria were lesions involving the ITF with an extension to the middle cranial fossa and/or orbit. Baseline characteristics of patients, tumour localisation, tumour extension, diffusion route, histology, extent of tumour resection, postoperative treatment, and post-operative complications were evaluated. RESULTS: Nine patients underwent a surgical procedure with a FOZ approach, two of them with a single-piece approach and the remainder with a two-piece one. All patients had an ITF localisation. Gross total removal (GTR) was achieved in 7/9 patients. Only one patient, with non-total removal (NTR), underwent radiotherapy. CONCLUSIONS: For the treatment of ITF fossa tumours extending to the orbit and or middle cranial fossa, we believe that both FOZ techniques are effective and allow a good medial extension toward the cavernous sinus and parasellar region. But a two-piece craniotomy may ensure a more medial extension and a wider angle of work compared to a one-piece craniotomy.

Access to Meckel's cave for biopsies of indeterminate lesions: a systematic review.

Accessing Meckel's cave (MC) is surgically challenging. Open approaches are complex and often correlated with high morbidity. Endoscopic approaches emerged in the last decade as feasible alternatives to open approaches, especially for sampling indeterminate lesions. This article first analyses available routes to approach Meckel's cave and presents furthermore an illustrative case. We conducted a systematic review and reported according to the guidelines for preferred reporting items for systematic reviews and meta-analyses (PRISMA). Various surgical approaches identified through the search are evaluated and discussed in detail. Additionally, we report on a case of woman with a lesion in MC, which was accessed through an endoscopic transpterygoid approach subsequently diagnosed as a diffuse large B cell lymphoma. Our search delivered 75 articles that included case reports (n = 21), cadaveric studies (n = 32), clinical articles (n = 16), review of the literatures (n = 3), as well as technical notes (n = 2) and a radiological manuscript (n = 1). Open routes included lateral approaches with many variations, mainly intra- and extradural pterional approaches and anterior petrosal, as well as a retrosigmoid intradural suprameatal and a lateral transorbital approach. Endoscopically, MC was reached via approaches that included transpterygoid, transorbital or infraorbital fissure routes. Percutaneous approaches, e.g. through the foramen ovale, were also described. Multiple surgical approaches to MC are currently available. Their different characteristics as well as individual patient factors, such as clinical history and the localization of the disease, have to be considered when choosing a surgical corridor. Studies included in this review highlight the endonasal endoscopic transpterygoidal technique as an excellent corridor for biopsies in the ventral MC.

Same viewing angle, minimal craniotomy enlargement, extreme exposure increase: the extended supraorbital eyebrow approach.

The supraorbital eyebrow approach (SEa) has been commonly used as a straightforward route to reach lesions located in the anterior cranial fossa. The reduced surgical exposure provided by this approach limits its applicability. A modification of the SEa, the extended supraorbital eyebrow approach (X-SEa), allows use of the transylvian corridor to approach parasellar lesions, while maintaining most of the aesthetic advantages of the SEa. To quantify the surgical exposure and maneuverability provided by the X-SEa using a cadaveric study. Eleven heads were used to obtain all stereotactic measurements. Surgical exposure and maneuverability were measured by means of the area of exposure and the angles of attack along key representative points in the anterior circulation. The horizontal angle of attack at the middle cerebral artery provided by the X-SEa was larger than that provided by the SEa (32.6 vs 18.4°, p = 0.009). The X-SEa afforded broader vertical angles of attack at all targets in the anterior circulation (p < 0.05). The total area of exposure provided by the X-SEa was significantly larger than that provided by the SEa (1272 vs 978 ± 156 mm2, p = 0.003). The area of exposure in the ipsilateral trigon and in the midline quadrangle was also significantly larger for the X-SEa (paramedian 195 vs 121 mm2, p = 0.01; midline 1310 vs 778 mm2, p = 0.002). The X-SEa increases the exposure and surgical maneuverability along the anterior and middle cranial fossa when compared to the standard SEa.

Pediatric clear cell meningioma involving the middle cranial fossa in the context of NF2 and SMARCE1 mutations.

Meningiomas are an uncommon entity in children and adolescents. <30 cases of pediatric clear cell meningioma (CCM), a World Health Organization (WHO) Grade II tumor, have been reported in the literature. These tumors are more likely to recur than the more common WHO Grade I meningiomas, especially with incomplete surgical resection. CCMs are most commonly found in the spine and posterior cranial fossa. Recently, SMARCE1 mutations have been linked to the development of CCM. To evaluate the progression of pediatric CCM in the context of emerging genetic knowledge, we reviewed all 45 cases of CCM at our institution for a 23 year period (1997-2019) to identify pediatric cases. Forty-four of the tumors arose in adults from age 34-81 years. The one pediatric case originally presented at age 4 years; the patient was found to have a CCM in the left cavernous sinus projecting into the posterior fossa, associated with a novel germline SMARCE1 mutation and somatic NF1 and DMD mutations. After two years, the patient had a recurrence of the tumor and underwent a second resection. This is the 5th reported case of CCM in the middle cranial fossa, and the only recurrent case, as well as the only reported case of recurrent pediatric CCM associated with a germline SMARCE1 mutation. Further study of the natural history of tumors associated with germline SMARCE1 loss could potentially inform prognosis.

Intracranial Ewing sarcoma with whole genome study.

INTRODUCTION: Ewing sarcoma (ES) as a primary intracranial tumor is very rare. Recently, CNS embryonal tumors with ES-like genomic change have been reported. Patients and methods We report a case of intracranial Ewing sarcoma in a 13-year-old girl who complained of headache and migraine. The tumor had developed in the right middle cranial fossa with a mass effect on the brain with impending transuncal herniation. RESULTS: Undifferentiated small round cell morphology with completely negative results for friend leukemia integration 1 transcription factor (Fli-1) and a nonspecific cytoplasmic CD99-positive staining pattern mislead the diagnosis as central nervous system (CNS) embryonal tumor, NOS. However, whole genome sequencing (WGS) revealed Ewing sarcoma (EWS)-Fli-1 gene fusion, which was confirmed by fluorescence in situ hybridization study and the diagnosis was revised to ES. CONCLUSIONS: This case is a true intracranial but extra-axial ES confirmed by WGS. We report this case of intracranial ES to demonstrate the importance of marker gene studies using FISH or NGS.

Surgical excision of large-to-giant petroclival meningiomas focusing on the middle fossa approaches: The lessons learnt.

INTRODUCTION: Petroclival meningiomas are based on or arising from the petro-clival junction in upper two-thirds of clivus, medial to the fifth cranial nerve. This study focuses on the surgical experience in resecting large-giant tumors >3.5 in size predominantly utilizing middle fossa approaches. MATERIAL AND METHODS: 33 patients with a large or a giant petroclival meningioma (size >3.5 cm) were included. Clinical features, preoperative radiological details, operative findings, and postoperative clinical course at the follow-up visit were reviewed. Group A tumors (n = 17,51.5%) were sized 3.5cm-5cm, and Group B (n = 16,48.48%) tumors were of size >5 cm. Extent of resection was described as 'gross total' (no residual tumor), 'near total' (<10% residual tumor) and 'subtotal resection' (>10% residual tumor). Glasgow outcome scale (GOS) quantitatively scored postoperative neurological outcome (mean follow up: 35.77months; range 1-106 months). RESULTS: 25 (75.8%) patients had tumour extension into both supratentorial and infratentorial compartments. Extension into Meckel's cave (n = 25,75.8%), cavernous sinus (n = 17,48.4%], sphenoid sinus (n = 12,38.7%] and suprasellar area [12,38.7%] was often seen. In 31 (93.9%) patients, the tumor crossed the midline in the premedullary, prepontine, and interpeduncular cisterns. In 20 (60.6%) patients, the tumour extended below and posterior to the internal auditory meatus (IAM), while in 13 (39.4%) patients, the tumor was located above and anterior to the IAM. Kawase's approach was the most commonly used approach in 16 (48.48%) patients and resulted in maximum tumor resection. Other approaches included half-and-half (trans-Sylvian with subtemporal) [n = 6, 18.18%]; frontotemporal craniotomy with orbitozygomatic osteotomy [n = 1, 3%] and retromastoid suboccipital craniectomy (RMSO) [n = 7, 21.21%]. In 2 (6.06%) patients, staged anterior petrosectomy with RMSO; and, in 1, staged presigmoid with half-and-half approach was used. Gross total excision was achieved in 12 (36.36%), near-total excision in 15 (45.45%) and subtotal excision in 6 (18.18%) patients. 20 (60.6%) patients had a good functional outcome; 6 patients succumbed due to meningitis, pneumonitis, perforator injury or a large tumor recurrence. CONCLUSIONS: Half-and-half approach was used in tumors with middle and posterior cranial fossae components often extending to the suprasellar region. Kawase's anterior petrosectomy was utilized in resecting tumors with predominant posterior fossa component (along with a small middle fossa component) that was crossing the midline anterior to the brain stem, and mainly situated superomedial to the IAM. Tumors confined to the posterior fossa, that extended laterally and below the IAM were resected utilizing the RMSO approach. Occasionally, a combination of these approaches was used. Middle fossa approaches help in significantly avoiding morbidity by an early devascularisation and decompression of the tumor. In tumors lacking a plane of cleavage, a thin rim of capsule of tumor may be left to avoid brain stem signs.

Dedifferentiated chordoid meningioma with rhabdomyosarcomatous differentiation on the middle cranial fossa.

A 46-year-old woman presented with headache and right hemiparesis. MRI demonstrated a mass in the left middle fossa. Total resection was performed. A histological examination of the tumor specimen showed several characteristic morphological features. A chordoid meningioma showing an epithelial-like palisade arrangement was observed. An anaplastic short spindle cell tumor exhibiting a fascicular pattern was considered to be a rhabdomyosarcoma. After conventional radiotherapy, the tumor was well controlled without any neurological deficit for 20 months. When subsequent recurrences were observed, the patient was treated by surgery, stereotactic radiosurgery and chemotherapy. Thirty-two months after the initial treatment, the patient died due to intracranial dissemination and an autopsy was performed. The histological examination of the recurrent and autopsy specimens showed a prominent sarcoma component. This case appears to be the first reported intracranial tumor diagnosed as a dedifferentiated chordoid meningioma with rhabdomyosarcomatous differentiation.

[Diagnosis and treatment of diffuse tenosynovial giant cell tumor arising from temporomandibular joints].

OBJECTIVE: To retrospectively analyze the clinical features, treatment and prognosis to the diffuse tenosynovial giant cell tumor (D-TSGCT) arising from the temporomandibular joint (TMJ), and to give a reference for the early diagnosis and treatment of this disease. METHODS: In this study, 15 patients finally diagnosed as D-TSGCT of TMJ histopathologically at the Peking University Hospital of Stomatology from October 2003 to August 2015 were selected and reviewed. Their clinical manifestations, imaging and histological features, diagnoses and differential diagnoses, treatments and follow-ups were summarized and discussed. RESULTS: D-TSGCT of TMJ showed obvious female predominance (12/15), the main symptoms included painful preauricular swelling or mass, limited mouth-opening and mandibular deviation with movement. D-TSGCT on computed tomography (CT) scan often showed ill-defined soft tissue masses around TMJ, enhancement after contrast administration, usually with widening of the joint spaces and with bone destruction of the condyle, the fossa and even the skull base. On magnetic resonance images (MRI), the majority of lesions on T1 weighted images and T2 weighted images both showed the characteristics of low signals (6/11). The lesions could extend beyond the joints (9/11) and into the infratemporal fossa (4/11) and the middle cranial fossa (4/11). Surgical resection was performed in 14 cases and biopsy in 1 case. Postoperative radiotherapy was performed in 3 cases. In follow-ups, 3 cases showed recurrence postoperatively. CONCLUSION: D-TSGCT arising from TMJ should be differentiated with TMJ disorders, other tumors and tumor-like lesions of TMJ and parotid neoplasms, etc. CT and MRI examinations have important values in the diagnosis and treatment design of D-TSGCT. Because of the local aggressive and extensive behavior, complete resection should be performed as soon as possible. Postoperative radiotherapy was helpful for the extensive lesions including destruction of skull base and may be a good supplementary therapy. Because of the possibility of recurrence and malignancy, long-term follow-up was suggested.

Dislocation of Mandibular Condyle Into Middle Cranial Fossa: Two Alternative Methods for Two Patients.

Dislocation of the mandibular condyle into the middle cranial fossa is rare, and the number of cases discussed in published studies is limited. Various treatment routes have been suggested, and the entire published data are based on the presentations of single cases. The present report presents 2 cases of dislocation of the mandibular condyle into the middle cranial fossa. The first case was treated with closed reduction in the early stage; however, the second case, which was treated in the late stage, required open reduction. Both the methods of reduction and the key aspects of diagnosis are discussed.

Juxta-articular Myxoma of the Temporomandibular Joint.

The juxta-articular myxoma represents a benign mesenchymal neoplasm that arises from tissue within or adjacent to a joint space. There have been a number of reported cases involving myxomas of the knee, shoulder, elbow, wrist, and hip. To our knowledge there, however, have been no reported cases of juxta-articular myxomas of the temporomandibular joint (TMJ). This report describes the case of a 57-year-old woman with a juxta-articular myxoma of the left TMJ extending into the infratemporal fossa (ITF). Access to the tumor was accomplished via a preauricular incision and low condylar osteotomy which allowed for displacement of the condyle for direct visualization and excision of the tumor. The postoperative course was benign and the patient demonstrated no cosmetic or functional limitation. Likewise, follow-up at 30 months showed no evidence of recurrence. Benign encapsulated tumors of the ITF can be effectively accessed by means of a modified preauricular incision, low condylar osteotomy, and anterior meniscal release. This direct approach allows for excellent surgical exposure, minimal surgical site morbidity, and maintenance of physiologic joint function and occlusion.

The nasomaxillary complex and the cranial base in artificial cranial deformation: relationships from a geometric morphometric study.

INTRODUCTION: It is widely accepted that there is a relationship between the cranial base and the development of the nasomaxillary complex (NMC). The objective of the present study was to investigate the morphological relationship between these two anatomical units in skulls that have intentionally been subjected to one of two types of artificial deformity of the cranial vault [artificially deformed skulls (ADS)]. MATERIAL AND METHODS: A geometric morphometry study was performed on lateral cephalometric X-rays of three groups of crania: 32 with anteroposterior (AP) deformity, 17 with circumferential (C) deformity, and 39 with no apparent deformity. RESULTS: The cranial base of the ADS showed marked deformity that produced a restriction of AP growth of the NMC, alterations of the roof of the orbit as a consequence of the rotation of anterior cranial fossa, and nasal protrusion. Pronounced morphological differences were found between the three groups: increased vertical development of the maxilla occurred in both ADS groups due to growth of the alveolar process, and rotation of the maxilla and displacement of the orbital rim was observed in the C group. This confirms that the posterior facial plane is regarded as an axial structure that serves as an interface between the middle cranial base and the NMC (Enlow, D.H. and Hans, M.G. (1996) Essential of Facial Growth. WB Saunders Co., Philadelphia, PA). LIMITATIONS: It is important to take into account that these results have been obtained from an archaeological sample, with all the limitations that this implies such as being a small sample and with no absolute certainty regarding the use of the same type of deforming device within each group. Furthermore, this is a lateral two-dimensional study in which transverse development has not been analysed. CONCLUSIONS: Artificial modification of the shape of the vault has repercussions on the NMC that support the theory of an all-inclusive integration of the different cranial units in normal as well as in restricted development.

[Experience of surgical management of trigeminal schwannomas that simultaneously spread to the middle and posterior cranial fossae].

INTRODUCTION: trigeminal schwannomas account for 0.07-0.36% of all intracranial tumors and 0.8-8% of all intracranial schwannomas. Different surgical approaches are used depending on the topographic anatomical variant of the tumor. Dumbbell-shaped tumors that spread both to the middle and posterior cranial fossae are the most challenging ones in terms of their resection. MATERIAL AND METHODS: Five patients with dumbbell-shaped trigeminal schwannomas were operated on at the Burdenko Neurosurgical Institute in 2011-2013. In four cases, tumor resection comprised two stages using the retrosigmoid suboccipital approach (RSA) and lateral extended transsphenoidal endoscopic approach (LETEA); in one case, the tumor was resected in a single session through the LETEA. If there were pronounced symptoms affecting the brainstem and/or cerebellum, tumor resection from the posterior cranial fossa through RSA was resected at the first stage (3 cases). If no symptoms were observed, tumor resection from the middle cranial fossa through LETEA was used as the first stage (2 cases). RESULTS: After two surgical stages, total, subtotal, and partial tumor resection was performed (one case each). Total tumor resection from the middle cranial fossa was achieved through LETEA in two cases. Aggravation of ipsilateral paresis of the craniocerebral nerve VI was observed after the surgery in two cases. No cases of nasal liquorrhea were observed after transnasal surgery. In one case, the cavernous segment of the internal carotid artery was damaged during LETEA, thus requiring endovascular occlusion of the damaged vessel to be performed. CONCLUSION: LETEA is an effective approach that allows resection of tumors from the middle cranial fossa and the cavernous sinus. Combined with RSA, this approach can be used for two-stage resection of dumbbell-shaped trigeminal schwannomas.

Management of iatrogenic tegmen plate defects: our clinical experience and surgical technique.

The objective of our study is to present our recommended approach for surgical management of iatrogenic tegmen plate defects. Patients diagnosed to have symptomatic iatrogenic tegmen plate defects were treated by one of the authors using a one-stage trans-mastoid standardized surgical procedure at Ain Shams University Hospitals. Patients' information records included history, complete examination, computed tomography (CT) and magnetic resonance imaging (MRI) of the temporal bone, and the followup data after the procedure to assess the final outcome in each case. Twelve patients with symptomatic iatrogenic tegmen plate defects were included in our study. The tegment plate defect size in the 12 patients varied from 2.2 to 15 mm (mean 5.6 ± 1.3). Postoperative followup of the patients ranged from 6 months up to 2 years (mean 1.6 ± 0.8). One patient only developed wound infection and was treated with antibiotics and regular dressings, with no other immediate postoperative complications (intracranial hematoma or meningitis). In the entire patient group, no local recurrence of middle fossa encephalocele was recorded. Our surgical trans-mastoid approach using multilayered autologous grafts is successful in closing iatrogenic tegmen plate defects more than 2.2 mm and less than 15 mm.

Supramastoid crest, safety landmark for craniotomy?

BACKGROUND: Bony landmarks are important in identifying and avoiding various structures, and thus, decreasing surgical morbidity. Knowledge of frontal bone was studied to help surgeons with safe craniotomy as temporooccipital region study was rare. OBJECTIVE: To identify usefulness of supramastoid crest and relationship to venous and Labbe's vein. MATERIAL AND METHOD: Twenty fresh cadaveric heads, the vessels were injected with colorized silicone, were studied on both sides, yielded 40 sides. The relationship of the supramastoid crest to the transverse, sigmoid sinus, Labbe's vein was also determined and measured. The following distances were measured utilizing the digital caliper for all measurements. RESULTS: Supramastoid crest was found in all cadavers (100%) and skull opened along supramastoid crest 100% safe from injury to venous sinus. Anterior border of supramastoid crest was close in relation to middle cranialfossa floor, 85% on right side and 90% on left side and the same level of middle cranial. The authors defined supramastoid point with turning of supramastoid crest and found distance from supramastoid point to nearest venous sinuse (always transverse sinus) 1.0-22.41 mm in range and average 12.94 mm on right side and 11.87 mm on left side. The last distance, from supramastoid point to nearest Labbe's vein, was measured and found to be 5.94-24.97 mm in range and average 17.23 mm on both sides. CONCLUSION: Supramastoid crest is bony landmark and easy to identify in the adult cadaver and craniotomy along supramastoid crest and always safe from injuring the venous sinus and Labbe s vein. 87.5% anterior border of supramastoid crest is the same level for middle cranial fossa floor.

An unusual association between growth hormone deficiency and a middle cranial fossa arachnoid cyst.

Middle cranial fossa arachnoid cysts (MFACs) are frequently asymptomatic. Here, we report the case of a 10-year-old boy with growth hormone deficiency (GHD) and an MFAC. His linear growth had followed the -2.5 SD line. On physical examination, his height was 120.8 cm (-2.6 SD), and the level of insulin-like growth factor 1 was low (87 ng/mL). GH provocative tests revealed GHD and brain magnetic resonance imaging (MRI) revealed an MFAC. We started treatment with GH replacement therapy (0.175 mg/kg/week). At the age of 12 years and 9 months, there were no interval changes in the features of the MFAC on the brain MRI. As his height was 145.5 cm (-1.7 SD) at the age of 13 years and 4 months, the therapy seems to be successful without sequelae. GH replacement therapy is suggested to be safe and effective to treat patients with GHD associated with arachnoid cysts.

Rare giant epidermal cyst in the infratemporal fossa and middle cranial fossa.

A 50-year-old man presented with a rare giant crossing cranium-temporal combined epidermal cyst. Physical examination found left facial numbness and temple severely numbness with light pressure. Horizontalis craniocerebral computed tomography demonstrated a mass lesion of 3.0 × 2.0 cm in the middle cranial fossa area; sagittal craniocerebral magnetic resonance scanning demonstrated a mass consisting of 2 leaves (the upper one, 4.0 × 3.0 cm; the lower one, 2.0 × 1.5 cm). Computed tomography angiography showed that the blood supply of the lesion came from superficial temporal artery and middle cranial fossa artery. The clinical diagnosis was neurilemmoma. Surgery revealed a pearly cyst consisting of 2 leaves (connected by a narrowed bridge located at the articular fossa of temporal bone) was 6 × 3 × 3 cm. Histologic examination disclosed disintegrated keratinizing epithelium layer, keratinizing epithelium layer, and stratified squamous epithelium layer from inner to outer and found no hair follicles or sebaceous gland with the diagnosis of epidermoid cyst. Surgery was successfully performed, and the patient was discharged home with severer left facial numbness relatively and left jaw slight opening. The present case suggests that epidermoid cysts can be seen in any location, even giant crossing cranium-temporal combined lesion, and the blood supply should be considered as a factor judging its pathogenesis.

An aneurysm rupturing into a middle cranial fossa arachnoid cyst presenting as an intracystic hemorrhage.

We report a case of an aneurysm rupturing into an arachnoid cyst presenting as a confined intracystic hemorrhage. A 37-year-old man with a history of sporadic headaches for several years complained of a headache of several days' duration. Brain computed tomography and magnetic resonance imaging revealed a cystic lesion in the left side of the middle cranial fossa that included a 3-cm-diameter hematoma in the Sylvian fissure without extracystic hemorrhage. Left carotid artery angiography showed an internal carotid artery-posterior communicating artery (IC-PC) aneurysm and an anterior choroidal artery aneurysm. The cyst wall was excised, and the aneurysms were clipped. The bleeding point was the spot at which the IC-PC aneurysm entered the arachnoid cyst. Right oculomotor nerve palsy developed after surgery, but resolved after 3 months. To the best of our knowledge, this is the only fourth report to date in the literature of a confined aneurysm rupturing into an arachnoid cyst.

Inflammatory myofibroblastic tumor mimicking malignant meningioma in the middle cranial fossa: a case report.

INFLAMMATORY myofibroblastic tumor (IMT), also known as inflammatory pseudotumor or plasma cell granulomas, is an uncommon fibro-inflammatory lesion which is composed of inflammatory cells and myofibroblastic spindle cells. Its pathogenesis is still unknown. The tumor commonly occurs in the lung, upper respiratory tract, live, orbit, abdominal membrane, retroperitoneum, and genitourinary tract.It rarely involves the central nerve system, especially the middle cranial fossa. As far as we know, there are no more than 5 reports of IMT that developed in the middle cranial fossa without involving temporal bone. IMT can occur at any age but primarily affects children and young adults. It is difficult to diagnose IMT by clinical and imaging characteristics only. Here we present an 82-year-old female with intracranial IMT arising from the meninges and without involving temporal bone, which was diagnosed as malignant meningioma before operation.