Introduction: To present a complex case of giant MENINGOENCEPHALOCELE after a canal wall down mastoidectomy and describe our preferred approach to repair meningoencephalic herniation of the temporal bone. Case Report: A 20-year-old patient, who had previously undergone type III tympanoplasty with total ossicular reconstruction prosthesis for an attic cholesteatoma, presents with clinical and imaging features compatible with the diagnosis of a giant temporal MENINGOENCEPHALOCELE. We performed a combined approach –, transmastoid plus minicraniotomy-to repair the skull base defect. A multilayer reconstruction of the defect with septal cartilage and temporal fascia was performed. After a 48 months follow-up, the patient remains symptom free without signs of tissue herniation. Conclusions: Transmastoid plus minicraniotomy combined approach is a safe and feasible technique in case of large and anterior skull base defects with low surgical morbidity, allowing a safe and multilayered reconstruction, even in the context of a simultaneous active chronic otitis media.

Congenital skull base MENINGOENCEPHALOCELEs (MECs) are exceedingly rare lesions, occurring in approximately 1/35000 live births. Basal encephaloceles include spheno-orbital, sphenoethmoidal, sphenomaxillary, transethmoidal, and transsphenoidal lesions. Among them, the transsphenoid MENINGOENCEPHALOCELE (TSME) are the rarest, occurring in 1/700000 live births. . .

Purpose: To report a case of cerebrospinal fluid (CSF) leakage during dacryocystorhinostomy (DCR) in a patient with Mobius syndrome and history of MENINGOENCEPHALOCELE. Patient and findings: This 12-year-old girl had undergone an operation for MENINGOENCEPHALOCELE at infancy by a neurosurgeon and was referred because of nasolacrimal drainage obstruction. She underwent DCR surgery at age 10 and 12 years. CSF leakage was detected during surgery. The authors describe the course and management of this patient. Conclusion: In patients with any history of surgery or trauma in the periorbital region, the ophthalmologist should be ready to manage CSF leakage during DCR.