Provisionally unclassified vascular anomalies (PUVA) are a group of diseases with unique characteristics that make them unclassifiable within vascular tumors or malformations. We describe a PUVA as the cause of recurrent pericardial effusion and its response to sirolimus. A 6-year-old girl was referred with a cervico-thoracic vascular anomaly, a violaceous and irregular lesion in the neck and upper chest, diagnosed as "hemangioma". She had pericardial effusion at the neonatal age that required pericardiocentesis, propranolol and corticosteroids. She remained stable for 5 years, when she presented with a severe pericardial effusion. A magnetic resonance visualized a diffuse vascular image in the cervical and thoracic region with mediastinal extension. The pathological study showed a vascular proliferation in the dermis and hypodermis with positive staining for WT1 and negative for Glut-1. Genetic testing found a variant in GNA14, for which the diagnosis of PUVA was established. When a pericardial drain was placed without response, treatment with sirolimus was started with resolution of the effusion. Sixteen months later, the malformation is stable and there has been no recurrence of pericardial effusion. In a significant group of patients, definitive diagnosis is not possible despite pathological and genetic analysis. mTOR inhibitors may become a therapeutic option if symptoms are severe enough, with a low rate of reported side effects.
New Insights and the Importance for the Pediatric Surgeon
In patients with vascular anomalies in whom it is not possible to reach a diagnosis despite multiple tests, a safe and effective option is to try mTOR inhibitors if symptoms are severe enough, considering the low rate of reported side effects with their use.
Purpose: There is controversy about the necessity of nighttime appendectomy. The aim of this study was to determine whether timing of appendectomy performance plays a role on postoperative complications.
Methods: A retrospective single-center comparative study was performed in children who underwent surgery for acute appendicitis between 2017-2021. Patients were divided into groups based on the time slot in which surgery was performed: morning (8:00h-15:00h), afternoon (15:00h-22:00h) and night (22:00h-08:00h). Demographics, intraoperative data, length of hospital stay and postoperative complications were analyzed and compared.
Results: A total of 1643 patients were included: 337 were operated in the morning, 751 in the afternoon and 555 at night. We found no demographic differences. When comparing the intraoperative data, no differences were observed in the percentage of complicated appendicitis. Night group patients presented a higher percentage of open appendectomies (64.5%) when compared to afternoon (49.6%) and morning (46.2%) groups (p<0.001). Surgery time was also significantly shorter in the night group (45.2min ± 18,9min) (p<0.001). There were no differences in length of hospital stay, postoperative complications rate or readmission rate.
Conclusion: These results show that in our institution time slot in which the appendectomy is performed has no consequences in postoperative outcomes and complications.
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