Derived neutrophil-to-lymphocyte ratio (dNLR) as a diagnostic biomarker of pediatric acute appendicitis.

Appendicitis stands as the most common surgical emergency in pediatric populations. Despite the existence of numerous diagnostic biomarkers, their utility is constrained by limitations in cost-effectiveness, potentially leading to therapeutic delays. This research aims to determine the diagnostic accuracy of the derived neutrophil-to-lymphocyte ratio (dNLR) in appendicitis. Although its role in this context has been recently described, this is the first study to compare its performance against acute-phase reactants routinely employed in clinical practice. Following approval from the Research Committee (2023/390894), a diagnostic study was conducted including patients under 15 years old undergoing surgery for acute appendicitis (AA) and those presenting with non-surgical abdominal pain (AP).

Risk factors for sequelae after surgery for lymphatic malformations in children.

OBJECTIVE: The first-line treatment of lymphatic malformations (LMs) is pharmacological or interventional; however, surgery is still necessary in selected cases. Our aim was to identify factors associated with the occurrence of permanent postoperative complications. METHODS: This was a case series study of children operated on for LMs between 2001 and 2021 and followed-up in our institution. Patients who presented sequelae derived from surgical treatment (cases) and those who did not (controls) were compared. RESULTS: We included 112 children who underwent surgery for LMs in different centers. Forty-nine cases and 63 controls were included (58% male), with a mean age of 34 months. Patients younger than 1 year presented more complications than older children, 59% (n = 29/49) vs 41% (n = 24/49), respectively (P = .02). LMs were in the cervicofacial region in seven patients in the control group compared with 30 of the cases (P ≤ .001), with microcystic malformations the most associated with sequelae (n = 11/15; P = .019). Concerning permanent complications, 88% were neurological (n = 43/49), mainly peripheral facial palsy (n = 17). There was greater postoperative residual disease in controls compared with cases (65% vs 14%, respectively; P ≤ .0001). However, following a second procedure in the control group, there was no significant difference in long-term cure rates (P = .38). CONCLUSIONS: The risk of sequelae following surgery for LM increases significantly in patients younger than 12 months in cervicofacial and microcystic malformations. Because non-radical resections are associated with fewer complications and an optimal long-term cure rate, we consider that aggressive surgical approaches should be avoided if the absence of sequelae is not guaranteed.

Lymphatic Malformations in Parkes Weber's Syndrome: Retrospective Review of 16 Cases in a Vascular Anomalies Center.

INTRODUCTION: Parkes Weber's syndrome (PWS) is a rare genetic disorder characterized by overgrowth and vascular malformations, primarily affecting the extremities. While PWS is known to be associated with arteriovenous and capillary malformations, the potential involvement of lymphatic malformations (LMs) has not been previously reported. The objective of this study is to investigate the presence of lymphatic anomalies in PWS patients and their role in the development of limb asymmetry. MATERIALS AND METHODS: This is a retrospective study of patients diagnosed with PWS in a Vascular Anomalies Center from 1994 to 2020. Clinical data were obtained from medical records including diagnostic imaging, lymphoscintigraphy, and genetic testing. The Institutional Review Board and Ethics Committee have approved this study. RESULTS: A total of 16 patients aged 18 interquartile range 14.7 years diagnosed with PWS were included (50% female). Six of the 16 patients with PWS had clinical and imaging data suggestive of LM (37.5%) and 3 of them had genetic variants in RASA1 (2/3) or KRAS (1/3). Limb asymmetry was greater in patients with isolated PWS (2.6 ± 0.8 cm) than in the PWS-lymphatic anomalies population (2 ± 0.7 cm), although not significant (p = 0.247). One in 6 patients with PWS-LM required amputation (16.6%) versus 1 in 10 in isolated PWS (10%). CONCLUSION: Lymphatic anomalies may be present in a significant number of patients with PWS and could have a role in limb asymmetry and outcomes. It is paramount to investigate their existence and distinguish them from true overgrowth.

Antibiotic treatment of post-appendectomy abscesses in children, regardless of size: a twelve years' experience.

Intra-abdominal abscesses are a common issue after appendectomy. Antibiotics have shown efficacy in treating smaller abscesses, while larger ones have traditionally been treated with drainage. This study assesses the efficacy of antibiotics for post-appendectomy intra-abdominal abscess (PAA) in children regardless of size. Case-control study of children with PAA admitted at our hospital from 2010 to 2022. The efficacy of antibiotics was compared between abscesses less and more than 6 cm in diameter. The Institutional Review Board has approved this study. A total of 1766 appendectomies were performed from 2010 to 2022 with an incidence of PAA of 5% (n = 89): age 9.3 IQR 5.8, 63% male (n = 56). Sixty-seven patients presented with a ≤ 6 cm abscess (controls) and 22 children had a > 6 cm PAA (cases). Length of intravenous antibiotics were higher in cases (15 IQR 7 days) than controls (12 IQR 4 days), p = 0.003. The efficacy of antibiotics in controls was 97% whereas 86.4% in cases (p = 0.094), reoperation was needed in 2/67 controls and 3/22 cases, with no differences in complications or readmission. The length of stay was longer in cases (15 IQR 6 days) than controls (13 IQR 5 days), p = 0.042. Antibiotics seem a safe treatment for PAA in children regardless of the size. However, this approach is associated with a longer period of intravenous antibiotics and hospital stay, although not with a higher rate of therapeutic failure, complications or reoperations.

Successful Sirolimus Treatment for Recurrent Pericardial Effusion in a Large Cervicomediastinal Provisionally Unclassified Vascular Anomaly: A Case Report.

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 cervicothoracic 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 Wilms' Tumor 1 Protein (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. Mammalian target of rapamycin inhibitors may become a therapeutic option if symptoms are severe enough, with a low rate of reported side effects.