Kaveh barami, indro chakrabarti, james silverthorn, jeremy ciporen, paul t.Akins.Diagnosis, classification, and management of fourth ventriculomegaly in adults: report of 9 cases and literature review.World neurosurgery (2018).Doi: 10.1016/j.Wneu.2018.05.073 - objective: an enlarged fourth ventricle, otherwise known as fourth ventriculomegaly (4th vm), has been reported previously in the pediatric population, yet literature on adults is scant.We report our experience with 4th vm in adults over an 11-year period and review the literature.Materials and methods: this was a retrospective chart review of adult patients with the diagnosis of 4th vm admitted to the intensive care unit in a tertiary care center.Results: nine patients were identified with 4th vm.Most presented with symptoms in the posterior fossa.Five cases were related to previous shunting and the underlying neurosurgical diseases, and average time interval to develop symptoms was 5.3 years.We divided our cases into primary, acquired, and degenerative based on the pathophysiology involved.Treatments included extended subzero cerebrospinal fluid diversion using a frontal external ventricular drain followed by low-pressure shunt revision, endoscopic third ventriculostomy, suboccipital decompression, and fourth ventricular catheter placement.Literature review identified additional published cases, and there were no reports of a formal classification scheme or treatment algorithm.Conclusions: this case series illustrates a narrow spectrum of etiologies associated with 4th vm in adults.We propose a simple classification scheme dividing 4th vm into 3 categories: primary, acquired, and degenerative.We recommend a stepwise treatment approach starting with extended subzero cerebrospinal fluid diversion followed by shunting for symptomatic primary and acquired 4th vm.Lower success rates and greater morbidity are associated with rescue procedures such as fourth ventricle drainage catheters, endoscopic third ventriculostomies, and skull base decompression.Reported events.A (b)(6) year-old female patient with a (b)(6) intrauterine pregnancy developed darkened skin lesions, chronic cough, low back pain, fever, respiratory failure, and progressive weakness.She presented to an outside hospital and was diagnosed with disseminated coccidioidomycosis based on clinical findings, bronchoscopy specimen cultures, serologies, and involvement of skin (¿desert bumps¿), lung, and blood.She was treated with intravenous amphotericin and switched to oral fluconazole after delivery.She later developed communicating hydrocephalus and underwent bilateral occipital vps at an outside hospital.She remained on high dose oral fluconazole.At age (b)(6) years, she presented to our center after having seizures.She had remained on high-dose oral fluconazole since her initial diagnosis.Mri of the brain was consistent with relapse of her central nervous system (cns) coccidioidomycosis infection, and she was started on liposomal amphotericin treatment intravenously.She developed increased headaches and nausea, and ct demonstrated ventricular enlargement with disproportionate enlargement of the fourth ventricle.She was admitted, and an external ventricular drain (evd) was placed.She was diagnosed with low-pressure hydrocephalus and improved with subzero drainage.A vps was placed.Ct of the chest showed cavitary lung lesions and a hydropneumothorax.She underwent thoracoscopic lung wedge resection.Purulent fibrinous debris was present throughout the right pleural cavity, and a right ruptured lower cavitary lesion with fully exposed cavity was resected.Four months later, she relapsed with headaches and gait imbalance and underwent evd placement and removal of previous shunts.
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