Background: Despite recent developments, the role of sirolimus in the heterogeneous spectrum of vascular anomalies is yet to be defined, in terms of indication, dosage, and therapy duration, recognizing both its potential and limitations.Methods: We retrospectively analyzed 16 children with vascular anomalies treated with sirolimus in two pediatric centers between 2014 and 2020 [male: n = 7, the median age at diagnosis: 4.6 months (range, 0–281.4)]. In addition, repetitive volumetric analyses of the vascular anomalies were performed when possible (11 cases).Results: Ten patients were diagnosed with vascular malformations and 6 with vascular tumors. The mean therapy duration was 27.2 months (range, 3.5–65). The mean sirolimus level was 8.52 ng/ml (range, 5.38–12.88). All patients except one with central conducting lymphatic anomaly responded to sirolimus, with the most noticeable volume reduction in the first 4–6 months. Additional administration of vincristine was needed in five patients with kaposiform hemangioendothelioma and yielded a response, even in cases, refractory to sirolimus monotherapy. As a single agent, sirolimus led to impressive improvement in a patient with another vascular tumor—advanced epithelioid hemangioendothelioma. Complicated vascular malformations required long-term sirolimus therapy. Side effects of sirolimus included mucositis and laboratory abnormalities. No major infectious episodes were recorded. An infant with COVID-19, diagnosed while on sirolimus therapy, presented with a mild course.Conclusion: In the current series, we reported limitations of sirolimus as monotherapy, addressing the need to redefine its indications, and explore combination regimens and multimodal treatment strategies. Tools for objective evaluation of response trends over time could serve as a basis for the establishment of future therapeutic algorithms.

Background and Objectives: This study aims to review how the introduction of propranolol as the primary treatment option for children with infantile hemangiomas (IHs) has affected the use of other treatment options at our institution and to determine the indications for surgical treatment of children with IHs in the propranolol era. Patients and Methods: The authors conducted a single-center, noncompeting, historical/retrospective cohort study to review all cases referred to the institution for IH evaluation from 2005 to 2020. The authors analyzed the complete charts of patients who received surgery from 2011 to 2020 and evaluated the reasons for each surgical intervention. Detailed descriptive statistics are provided. Logistic regression analysis and Pearson’s χ ²-test were applied. Results: During the study period, 592 children received treatment. From 2011, oral propranolol (n=268; 74%) and surgery (n=95; 26%) were the only treatments of choice for complicated IH cases. A significant decrease in the frequency of surgical treatment was observed (P=0.01). The authors identified four main indications for surgical treatment: (1) patients with ulceration and IH size appropriate for surgical resection (15%); (2) patients whose parents preferred surgical treatment (19%); (3) patients who presented late and underwent surgery before the age of three (29%); and (4) patients with sequelae after IH involution and excision after the third year of life (37%). Conclusions: Despite the significant decrease in the need for surgical treatment of children with IHs since the introduction of propranolol, there are still several clear indications for treating IH cases where surgery plays a crucial role.

Background: We aimed to gain insights in a co-culture of 10 bacteria and their postbiotic supernatant. Methods: Abundances and gene expression were monitored by shotgun analysis. The supernatant was characterized by liquid chromatography mass spectroscopy (LC-MS) and gas chromatography mass spectroscopy (GC-MS). Supernatant was harvested after 48 h (S48) and 196 h (S196). Susceptibility testing included nine bacteria and C. albicans. Bagg albino (BALBc) mice were fed with supernatant or culture medium. Fecal samples were obtained for 16S analysis. Results: A time-dependent decrease of the relative abundances and gene expression of L. salivarius, L. paracasei, E. faecium and B. longum/lactis and an increase of L. plantarum were observed. Substances in LC-MS were predominantly allocated to groups amino acids/peptides/metabolites and nucleotides/metabolites, relating to gene expression. Fumaric, panthotenic, 9,3-methyl-2-oxovaleric, malic and aspartic acid, cytidine monophosphate, orotidine, phosphoserine, creatine, tryptophan correlated to culture time. Supernatant had no effect against anaerobic bacteria. S48 was reactive against S. epidermidis, L. monocytogenes, P. aeruginosae, E. faecium and C. albicans. S196 against S. epidermidis and Str. agalactiae. In vivo S48/S196 had no effect on alpha/beta diversity. Linear discriminant analysis effect size (LEfSe) and analysis of composition of microbiomes (ANCOM) revealed an increase of Anaeroplasma and Faecalibacterium prausnitzii. Conclusions: The postbiotic supernatant had positive antibacterial and antifungal effects in vitro and promoted the growth of distinct bacteria in vivo.

Neonatal “surgical” malformations are associated with higher costs than major “non-surgical” birth defects. We aimed to analyze the financial burden on the Austrian health system of five congenital malformations requiring timely postnatal surgery. The database of the Austrian National Public Health Institute for the period from 2002 to 2014 was reviewed. Diagnosis-related group (DRG) points assigned to hospital admissions containing five congenital malformations coded as principal diagnosis (esophageal atresia, duodenal atresia, congenital diaphragmatic hernia, gastroschisis, and omphalocele) were collected and compared to all hospitalizations for other reasons. Out of 3,518,625 total hospitalizations, there were 1664 admissions of patients with the selected malformations. The annual mean number was 128 (SD 17, range 110–175). The mean cost of the congenital malformations per hospital admission expressed in DRG points was 26,588 (range 0–465,772, SD 40,702) and was significantly higher compared to the other hospitalizations (n = 3,516,961; mean DRG 2194, range 0–834,997; SD 6161; p < 0.05). Surgical conditions requiring timely postnatal surgery place a significant financial burden on the healthcare system. The creation of a dedicated national register could allow for better planning of resource allocation, for improving the outcome of affected children, and for optimizing costs.

Life-threatening complications (LTCs) and negative results of surgical treatments often go unreported. Minimally invasive repair of pectus excavatum (MIRPE) represents a procedure with a low incidence of adverse outcomes. However, 15 potentially fatal cases of MIRPE-related heart injury have been published. We report a case of cardiac perforation (CP) during MIRPE. A 12-year-old female was admitted for elective repair of a severe asymmetric pectus excavatum. Preoperative computed tomography showed a Haller index of 4.9. MIRPE was performed under bilateral video-assisted thoracoscopy. After the placement of the pectus bar, cardiac arrhythmias, hypotension and bilateral hemothorax occurred. Emergency thoracotomy without pectus bar removal showed CP. The wound sites were repaired and the pectus bar was eventually successfully implanted. The patient was discharged on postoperative day 11. After 10 months, she remains asymptomatic. Reporting rare complications is essential for accurate calculations of the true prevalence of LTCs, maintaining high alertness in pediatric surgeons.

Nasal Glioma (NG) represents a rare congenital abnormality of the neonate, which can be associated with skull defects or even a direct communication to the central nervous system. MRI serves valuable information for differentiation from encephalocele, dermoid cyst and congenital hemangioma. Complete resection remains the treatment of choice. We present two cases of NG, which were both suspected during prenatal ultrasound and MRI. In the first case, postnatal MRI showed a transcranial continuity. Mass excision was performed and the defect was covered by a glabellar flap allowing a good cosmetic result. Postnatal MRI excluded a trans-glabellar communication in the second case. After surgical excision, the resulting skin defect was covered with a full thickness skin graft harvested from the right groin. In cases of NGs complete resection and cosmetic appealing results can be achieved and might necessitate a multidisciplinary approach.