Objective To perform a systematic review of the literature evaluating clinical characteristics and management of cervical ganglioneuromas (CGNs). Data Sources PubMed, Embase, and Cochrane Library databases were searched. Data such as patient demographics, imaging, and treatments were obtained. Review Methods Pertinent studies were downloaded, and the full text was reviewed by 4 authors (N.P., S.S., C.F., D.T.). Results were reported via the PRISMA guidelines (Preferred Reporting Items for Systematic Reviews and Meta-analyses). Results Fifty-two studies with 58 patients were identified in the literature. Of the 58 patients, 22 were adults and 36 were pediatric. The most common reported location of CGN was within the parapharyngeal space (76%), followed by the retropharyngeal (19%) and paravertebral/prevertebral (7%) spaces. The most common presenting symptoms included a nontender mass (29.3%), dysphagia (17.2%), and hoarseness (10.3%). Interestingly, the average tumor volume for patients with postoperative Horner’s syndrome was 183 mm3 (n = 21, 47.7%) vs 946 mm3 in patients without Horner’s syndrome (n = 23, 52.3%). This represents a statistically significant finding ( P = .018). There exists no significant difference in tumor volumes between adult and pediatric patients with Horner’s syndrome ( P = .645). Conclusion CGN is a rare tumor of the sympathetic nervous system. Management should involve complete surgical excision with biopsy. We found that patients with small-volume CGNs are significantly more likely to experience postoperative Horner’s syndrome. This finding is independent of age and should therefore be taken into consideration in any patient with suspected CGN.
Sickle cell disease is a disorder of hemoglobin. The abnormal hemoglobin S disrupts blood flow, thereby resulting in acute painful sickle cell crisis. These episodes frequently prompt packed red blood cell transfusions to replace a patient's functional hemoglobin stores. Production of alloantibodies and autoantibodies to these transfusions can result in a rare, but serious, complication known as hyperhemolysis syndrome. Hyperhemolysis syndrome presents several challenges in regard to its acute management and the consequent difficulties in finding future compatible blood products. We report a case of recurrent hyperhemolysis syndrome. Both episodes occurred following orthopedic procedures, and the recurrent episode proved refractory to multiple treatments.
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