Marston, Alexander P.

Objective: To report the presentation, diagnosis, management, and convalescence of labyrinthine sequestrum (LS) and summarize all previously published cases. Patient(s): Eleven-year-old female with LS. Intervention(s): Multidisciplinary diagnostic evaluation and treatment. Main Outcome Measures: Imaging and laboratory findings, medical and surgical treatment. Results: We describe a case of LS secondary to medically recalcitrant suppurative otitis media in an 11-year-old female and review all eight previously reported cases. The index patient presented after 6 months of otitis media, profound unilateral hearing loss, with symptoms suggesting meningitis. Temporal bone CT demonstrated marked bony destruction of the left otic capsule. Gadolinium-enhanced MRI showed an enhancing process with evidence of meningitis and subdural empyema. The patient was treated with surgical debridement and culture directed antibiotic therapy. Posttreatment imaging showed resolution of intracranial infection with fibrous bony healing of the otic capsule resembling fibrous dysplasia. Conclusion: LS is a rare form of labyrinthitis characterized by centrifugal destruction of the otic capsule. The current index case highlights the importance of combined medical and surgical treatment and describes for the first time in the literature the fibrous ossification of the otic capsule following disease resolution.

OBJECTIVEOver the last 30 years, stereotactic radiosurgery (SRS) has become an established noninvasive treatment alternative for small- to medium-sized vestibular schwannoma (VS). This study aims to further define long-term SRS tumor control in patients with documented pretreatment tumor growth for whom conservative observation failed.METHODSA prospective clinical database was queried, and patients with sporadic VS who elected initial observation and subsequently underwent SRS after documented tumor growth between 2004 and 2014 were identified. Posttreatment tumor growth or shrinkage was determined by a ≥ 2-mm increase or decrease in maximum linear dimension, respectively.RESULTSSixty-eight patients met study inclusion criteria. The median pre- and posttreatment observation periods were 16 and 43.5 months, respectively. The median dose to the tumor margin was 13 Gy (range 12–14 Gy), and the median maximum dose was 26 Gy (range 24–28 Gy). At the time of treatment, 59 tumors exhibited extracanalicular (EC) extension, and 9 were intracanalicular (IC). Of the 59 EC VSs, 50 (85%) remained stable or decreased in size following treatment, and 9 (15%) enlarged by > 2 mm. Among EC tumors, the median pretreatment tumor growth rate was 2.08 mm/year for tumors that decreased or were stable, compared with 3.26 mm/year for tumors that grew following SRS (p = 0.009). Patients who demonstrated a pretreatment growth rate of < 2.5 mm/year exhibited a 97% tumor control rate, compared with 69% for those demonstrating ≥ 2.5 mm/year of growth prior to SRS (p = 0.007). No other analyzed variables were found to predict tumor growth following SRS.CONCLUSIONSOverall, SRS administered using a marginal dose between 12–14 Gy is highly effective in treating VSs in which initial observation fails. Tumor control is achieved in 97% of VSs that exhibit slow (< 2.5 mm/year) pretreatment growth; however, SRS is less successful in treating tumors exhibiting rapid growth (≥ 2.5 mm/year).

Objectives: This case presents a previously undescribed clinical scenario of spontaneous cerebrospinal fluid (CSF) leaks secondary to a lateral sphenoid sinus recess skull base dehiscence and contralateral Sternberg’s canal. This case report aims to characterize the presentation and successful management of these lesions. Methods: The electronic medical record was used to collect information pertaining to the patient’s clinical history. Results: The patient was a middle-aged, obese female with persistent clear rhinorrhea as her only presenting symptom. Neuroradiologic studies localized the defect to the lateral sphenoid sinus recess. CSF opening pressures were within normal limits, but radiographic findings were consistent with elevated intracranial pressure. After an endoscopic transnasal transsphenoidal approach failed to resolve the CSF leak, a transpterygoid approach facilitated CSF leak resolution. The patient then did well for the following 2 years, but later developed a CSF leak through a contralateral Sternberg’s canal. An endoscopic suprapterygoid procedure and ventriculoperitoneal shunt placement led to CSF leak resolution. Conclusion: This case demonstrates one of the only published examples of a sphenoid sinus CSF leak secondary to Sternberg’s canal as it was originally described in the literature. Wide endoscopic surgical exposure and intracranial pressure management ultimately led to CSF leak resolution.