Meningiomas are the most common primary intracranial tumours with an incidence of 2.3–8.3 in 100,000 [5]. Atypical meningiomas are aggressive with high chances of recurrence. Standard of care includes surgical resection followed by adjuvant irradiation partially resected atypical meningiomas. Chemotherapy and other medical therapies are available as salvage treatment once standard options are exhausted; however, the efficacy of these agents remains limited [6]. SWMs account for 11–20% of intracranial meningiomas, classified as “globoid” and “en plaque” tumours. The term spheno-orbital meningiomas (SOM) has been used to describe en plaque meningiomas that arise at the greater sphenoid wing and extend to the orbit with hyperostosis or bone invasion. SWMs has higher mortality, disability and recurrence rate due to their intricate relationship with the cranial nerves and orbital structures. Atypical meningioma involving the orbit and extending to the maxilla, infratemporal fossa is not reported in the literature. The extension of SWMs to the extracranial structures is directly proportional to morbidity and mortality [7, 8]. The surgical approaches described in the literature to excise SWMs included classic pterional, extended pterional approaches and pterional-orbitozygomatic approaches. The three approaches were mainly chosen according to tumour location, invasion and size. The classic pterional approach was the most commonly used surgical approach. Extended pterion approach was mainly chosen for large SWMs with skull base bone invasion or medial SWMs. Sometimes, the intentional incomplete surgical excision is necessary to get an improved postoperative quality of life [9]. There is an upcoming role of 3D fusion imaging in SWMs as a tool for presurgical evaluation, to predict the extent of tumor resection and its outcome [10].
The extent of surgical resection of the maxilla depends on the nature of the tumour, the site of the maxilla involved and the extent of its local spread. Skull base tumours have been considered hopelessly incurable in the past, which is now manageable with the advent of medicines and various skull base approaches, which is anterior, posterior and lateral. These approaches have augmented the surgical access of the nasal and ear tumours with intracranial extension or vice versa. Subtotal or total maxillectomy gives exceptional exposure or wide-field view of the middle compartment of the skull base from the roof of the sphenoid to the 5th cervical vertebrae, exposing the skull base between each Eustachian tube and the carotid canals, and provides ease of access to control haemorrhage [11]. The maxillary tumours can create devastating effects on the patients’ aesthetics and functional well-being as they are not always limited within the boundaries of the maxilla and can involve adjacent structures like the hard and soft palate, skin and orbital contents which may have to be sacrificed [12]. The multidisciplinary approach is commendable, and this relates primarily to the experience and skill of the skull base surgeon, the availability of the operating room and radiotherapeutic equipment, as well as a mutual working relationship among specialty surgeons. The wide external exposure also helps in reaching the areas with very limited in-depth apex and internal exposure and also in the reconstruction of the defect like in this case the skull defect reconstruction was also done with ease. The chances of complete tumour removal are also more with the wide-field exposure. If the tumour removal is incomplete, perhaps a palliative result may be achieved with the additional assistance of radio-chemotherapy. The duration to make a surgical access is also less compared to the other aggressive approaches. The external facial scar falls in natural facial crease lines and is cosmetically acceptable. The limitations of this approach are the risk of exposure to the skull and meninges to nasal and pharyngeal flora that may lead to severe complications along with poor cosmesis due to ectropion and upper lip scarring. An appropriate antibiotic coverage and careful watertight closure of the soft tissues in the operative defect, reinforced by coverage with or without fat, bone or cartilage grafts, have eliminated these complications [13].