Sarcoidosis of the pineal region, dorsal mesencephalon, and periaqueductal region: Case report and systematic review

Background: The central nervous system is an unusual location of sarcoidosis, which commonly affects the cranial nerves, meninges, hypothalamus, and pituitary gland. Involvement of the pineal region is extremely rare. This systematic review focused on the diagnosis and management of pineal region sarcoidosis, dorsal mesencephalon, and periaqueductal region. Objectives: This study aimed to discuss diagnostic modalities and best management tools of the aforementioned pathology. Methods: ScienceDirect, PubMed, and Google Scholar databases were searched for English or French articles about sarcoidosis of the pineal region, dorsal mesencephalon, and periaqueductal region. The clinical case of a patient managed at our department that we believe is directly relevant to this review is also presented. Patients’ demographics, clinical presentations, presence of hydrocephalus, other sarcoidosis locations in the central nervous system, and medical treatment were collected. Surgical management, surgical approach, and outcomes and complications of each procedure were also obtained. This study was conducted in agreement with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Results: Fifteen cases were examined. The study sample consisted of nine (60%) male and six (40%) female, and the mean age was 32 years. Eight (53%) patients had hydrocephalus, and the predominant clinical presentations were signs of increasing intracranial pressure (headaches, vomiting, and papilledema). Six (40%) patients had diplopia, and convergence–retraction nystagmus was noted in three (20%) patients. Argyll Robertson sign was present in one patient and suspected in another patient (13%). Medical treatment consisted mainly of steroids (93% of cases). Open surgery on the pineal region was performed in five patients, and four of them reported to have serious complications (such as ophthalmoplegia, hemianopsia, hemiparesis, bilateral third cranial nerve paresis, and cerebellar syndrome). Endoscopic management was performed in two patients without complications. Conclusion: To treat hydrocephalus, brain imaging is mandatory in patients with sarcoidosis if intracranial hypertension is suspected. In pineal region sarcoidosis, management of hydrocephalus is the priority, followed by medical treatment of the lesion. Open surgery of any approach presents a high risk of complications; thus, an endoscopic approach is the preferred management, as it treats hydrocephalus and makes biopsy possible with minimal risk.