Preoperative Transfusion in Sickle Cell Disease Children Undergoing Adenotonsillectomy.

Objective: To perform a systematic review and meta-analysis comparing two pre-operative transfusion regimens (conservative versus aggressive) in children with sickle cell disease(SCD) undergoing adenotonsillectomy in terms of post-operative complications, complications related to SCD and transfusion related complications. Data Sources and Review Methods: A literature review was performed through PubMed, EMBASE, Cochrane, and Ovid databases using the following phrases: (Adenotonsillectomy OR Tonsillectomy) AND (Sickle Cell Disease OR Sickle Cell Trait). Using predetermined inclusion and exclusion criteria, seven articles were selected for systemic review and two control trials were included in meta-analysis. Results: Out of a total of 3,146 results, seven articles were selected for review and two controlled trials were included in the meta-analysis. There was no statistically significant difference in the rate of primary and secondary hemorrhage between the aggressive and conservative transfusion regimens (RR = 3.1, CI = 0.84-11.4, p-value = 0.089). The rate of sickle cell disease related complications including vaso-occlusive crisis and acute chest syndrome was also not statistically significant between the two transfusion groups (RR = 1.4, CI = 0.7-2.8, p-value = 0.339). Even though, the transfusion related complications did not reach statistical significance, there was a higher complication rate in the group receiving aggressive blood transfusion. Conclusion: In SCD children undergoing adenotonsillectomy, an aggressive transfusion regimen that focuses on reducing the Hemoglobin S ratio to below 30% has not been shown to be more effective in reducing post-operative complications when compared to a conservative transfusion regimen. Therefore, it is reasonable to utilize a conservative transfusion regimen, thereby reducing the transfusion-associated risks.

Pediatric Tracheal Rhabdomyosarcoma Masquerading as a Granuloma.

We describe a posterior wall intratracheal embryonal rhabdomyosarcoma (RMS) arising in a 6-year-old tracheostomized child masquerading as reactive granulation tissue and review all reported cases of pediatric intratracheal RMS. The child underwent laser debulking of the tumor and postoperative radiation and chemotherapy with no evidence of recurrence at 2-year follow-up. A literature review revealed four previous cases of pediatric primary tracheal or intratracheal RMS, and remission was achieved in all but one case with surgery, chemotherapy, and radiation. Pathologic evaluation of tracheal mucosal granulation tissue may merit consideration, particularly in patients with increased risk factors.