RESUMO
Rosai-Dorfman disease (RDD) with craniocervical junction involvement is a rare clinical entity. We present herein a case of a pediatric patient with craniocervical junction RDD which was surgically treated. A 10-year-old female with a history of B-cell acute lymphoblastic leukemia (B-ALL) in remission and RDD presented with frontal migraine headaches. She previously had a right posterior chest wall lesion which was biopsy-proven RDD. She was found on imaging to have a dural-based right craniocervical junction lesion. Given her history of B-ALL, after a multidisciplinary discussion, the decision was made to proceed with resection with possible initiation of cobimetinib or clofarabine. The patient underwent a suboccipital craniotomy, C1 laminectomy, and resection of the dural-based lesion. Gross total resection was achieved, and histopathology confirmed the diagnosis of RDD. She was discharged home on postoperative day 4. No recurrence was seen on follow-up imaging at 3 months. We conducted a systematic literature review examining all cases of pediatric intracranial RDD and all cases of craniocervical junction RDD. This represents, to the best of our knowledge, only the second case of pediatric craniocervical junction RDD. Although RDD is often self-limiting, medical treatment is often considered for intracranial disease, but tissue confirmation is necessary. Surgical resection provides histopathologic diagnosis and can sometimes serve as definitive treatment for a particular lesion.
RESUMO
OBJECTIVES: To describe the mechanisms and visual outcomes of recreational and sports-related open globe injuries (OGIs). METHODS: A retrospective case series of eyes experiencing OGI secondary to recreational and sports-related activities at Memorial Hermann Hospital - Texas Medical Center (MHH-TMC) from January 1st, 2010 through March 31st, 2015 was conducted. Exclusionary criteria included no documented ophthalmologic examination upon presentation and repairs performed by services other than ophthalmology. A two-tailed t-test and Fisher's exact test were utilized to assess for statistical significance (p < 0.05). RESULTS: A total of 20 eyes from 20 patients experiencing OGIs secondary to recreational and sports-related activities were included. Thirteen eyes (65 %) presented with OGIs from penetrating objects while seven eyes (35 %) had injuries from blunt injuries. Males comprised most of the total study group (17 of 20 patients), and zone 3 injuries were the most common location of OGI. While eyes with OGIs from blunt trauma underwent evisceration/enucleation procedures more frequently than OGIs from penetrating mechanisms (71% vs 23 %) (p = 0.10), the final visual outcomes were similarly poor between groups. Only three eyes in this series experienced an improvement from baseline VA; all three eyes had lacked initial findings consistent with severe injury. CONCLUSIONS: Recreational and sports related OGIs most commonly occur in zone 3 and in young males, regardless of injury type. OGIs due to both penetrating and blunt trauma mechanisms implicate poor functional outcomes, but the absence of certain presenting injury features may indicate possibility of eventual visual recovery.
