This report describes the case of a 10-month-old boy who was diagnosed to have kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt syndrome (KMS), which is a rare pediatric vascular tumor with a high mortality rate. Although both KHE with KMS were resistant to various therapies, such as oral prednisolone, sclerotherapy, and chemotherapy, repeated radiation therapy with methylprednisolone pulse therapy did reduce the volume of KHE and improved the symptoms of KMS. Unfortunately, a regrowth of KHE with KMS was observed 4 months after the cessation of treatment and the patient thereafter died from an intracranial hemorrhage and Pneumocystis carinii pneumonia, which is a complication related to repetitive radiation and steroid therapy. A histopathologic examination of autopsy specimens confirmed a diagnosis of KHE and immunohistologic staining was positive for vascular endothelial growth factor receptor (VEGFR)-2 and VEGFR-3. These findings may provide the rationale to further investigate the role of VEGFRs in the pathogenesis of KHE and also to elucidate its prognostic value, along with the application of inhibitors for VEGFRs for the treatment of refractory KHE.
Our results indicate that treatment with inflammatory agents, in combination with an antigen-dependent immune response, could induce nasal polyp formation associated with eosinophil infiltration and mucosal hypertrophy. The gene expression profile supported the clinical relevance of this model.
Objectives
Treatments for unilateral vocal fold paralysis (UVFP) include conservative voice rehabilitation, vocal fold injection, and laryngeal framework surgery. We proposed basic fibroblast growth factor (bFGF) injection as a potential novel treatment for UVFP and have reported the short‐term results. In this study, we present the long‐term results and safety of vocal fold bFGF injection as a treatment for UVFP.
Methods
This retrospective study included 42 patients (25 males and 17 females) with UVFP who were administered a local injection of bFGF. The injection regimen involved injecting FGF (0.5 μg/ml in 0.5 ml per side) into the bilateral vocal folds using a 23‐gauge injection needle. Phonological outcomes were evaluated 6 months and 12 months after the injection.
Results
Overall, 26 patients received a single injection of bFGF, six patients received an additional injection, and 10 patients received the additional framework surgery. Maximum phonation time, mean flow rate, pitch range, jitter and shimmer percentages, the total GRBAS (grade, roughness, breathiness, asthenia, strain) score, and voice handicap index scores improved significantly in the long term. In patients who received the additional injection or framework surgery, the effects of bFGF injection were temporary, but did not interfere with the performance of the framework surgery.
Conclusion
In total, 42 patients who underwent vocal fold bFGF injections were reviewed. The bFGF injections were effective and safe in the long‐term results for UVFP in the selected cases. Some patients with severe symptoms benefited from the additional framework surgery but not the additional bFGF injection.
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