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Colistin is still a therapeutic cornerstone against multidrug-resistant gram-negative bacteria (MDRGN), mostly when other antibiotics do not gain adequate activity on these strains. In the present study, we evaluated in a cohort of burn patients the relationship between colistin therapy, survival and requirement of renal replacement therapy (CRRT). Retrospective study of 133 burn patients treated with iv colistimethate sodium (loading dose 9.0 × 10 IU, maintenance dose 4.5 × 10 IU BID) and 35 treated with other antibiotics for MDRGN infection including , , and between January 2008 and December 2017. Multivariate analysis with logistic regression was used to determine the effect of the predictors such as age, total body surface area (TBSA), third-degree burn areas, Revised Baux score, Charlson comorbidity score, length of stay, colistin dose and duration of treatment, mechanical ventilation, and need of CRRT on in-hospital mortality. To investigate the relationship between colistin and renal function, we focused on survivor patients as the completion of the therapeutic course of colistin represented the basic requirement to analyze its impact on the kidney. Out of 133 colistin- and 35 other antibiotics-treated patients, 83 (62.4%) and 31 (88.6%) survived, and 53 (39.8%) and 3 (9.7%) required CRRT, respectively. The severity of burns, as well as CRRT requirement and mortality, was significantly higher in colistin-treated patients than in other antibiotics-treated patients. Age and TBSA% were the significant predictors of mortality. Out of 83 colistin-treated survivors, 19 (22.9%) required CRRT (9 before and 10 after the start of colistin), and 64 (77.1%) had a normal renal function. No difference about the colistin dose and baseline characteristics, but the revised Baux score was found between the 9 patients requiring CRRT before the colistin course and the 10 patients after. Similarly, among the 64 patients not undergoing CRRT, no difference was found between the patients treated with the cumulative dose of colistin <99.0 × 10 IU (n = 33, median daily dose of 4.0 × 10 IU) and >99.0 × 10 IU (n = 31, median daily dose of 9.0 × 10 IU) about the baseline characteristics and the daily median plasma creatinine over 24 days of therapy. Colistin therapy was associated with more severe burns, mortality, and CRRT requirement. A short course therapy, at appropriate cumulative dosage, can lead to clinical success without a significant association with severe renal impairment.

The diagnosis of vanishing gastroschisis is made when in the presence of a full-thickness intrauterine abdominal wall defect the eviscerated loops are incarcerated in the fascial interruption. Four types of vanishing gastroschisis are described (A–D). We report on the case of a newborn with a vanishing gastroschisis-D. Gastroschisis was diagnosed at the 19th week of gestation, confirmed at the 30th, when the herniated loops previously visible to the right of the funiculus were no longer visualized. At the 32nd week, delivery was induced. The neonate weighed 1600 g, and the abdomen was distended, free from skin defects. On surgical exploration, the jejunum was 13 cm in length, with a blind ending. The post-atretic intestine measured 22 cm. A jejunostomy and a colostomy were built. The child received total parenteral nutrition for 13 months due to short bowel syndrome and was then subjected to intestinal lengthening procedure when she was 18 months old. Vanishing gastroschisis is a rare entity with a worse prognosis of the “classic” gastroschisis.

Introduction Colorectal carcinoma (CC) is a rare disease in the pediatric population, with an annual incidence of 1 in 10 million adolescents, and it accounts for approximately 1% of pediatric solid neoplasms. It is the most common primary gastrointestinal malignancy in children with the vast majority of CCs being adenocarcinoma (CA). Unfortunately, the proportion of poorly differentiated, mucinous type, signet-ring cell containing carcinomas is higher in younger patients than in adults. Moreover, due to the low awareness of the disease, diagnosis is usually delayed until advanced stages, resulting in an extremely poor prognosis. Surgery is the only curative modality for localized CAs, whereas adjuvant chemotherapy is the standard of care for patients with stage III cancer to eradicate micro-metastases. Patients and methods In the last 10 years, we treated 3 patients diagnosed with CA: a 14-year-old female, a 15-year-old male, and a 15-year-old female. All patients presented to our Emergency Department with nonspecific symptoms of abdominal pain and vomiting. Results All patients were subjected to laparoscopic tumor resection to relief intestinal obstruction. In the male patient, laparoscopy was converted to laparotomy to safely assess the anatomy because of strong peritoneal adhesions. No stomas were created, in order to improve quality of life. Oxaliplatin and 5-fluorouracil-based regimens were among the most commonly used chemotherapy combinations. The 15-year-old female and the 15-year-old male died 1 year after the surgical resection, while the 14-year-old female is still on follow-up. Conclusions CAs behave aggressively in children; they not only show a poorer response to chemotherapy, but are also associated with extensive intramural spread and peritoneal carcinomatosis. Lack of awareness and timely intervention remain the main challenges for early diagnosis and improved prognosis of CA.

Study objectiveOvarian torsion (OT) in pediatric age is a challenging condition to diagnose and treat. To date, there is still no clear consensus about its management. Our aim was to assess some possible associated factors that can help surgeons in decision-making.DesignWe conducted a retrospective multicentric study of pediatric OT surgically treated between 2010 and 2020 in six Italian and German institutions, comparing our findings with a literature review of the last 10 years (2010–2020).ParticipantsPatients aged 0–18 years with a diagnosis of OT intraoperatively confirmed and surgically treated at the involved institutions.ResultsNinety-seven patients with a mean age at diagnosis of 8.37 years were enrolled in the study. Severe abdominal pain was present in 82 patients (84.5%). Eighty children (82.5%) presented an enlarged ovary with an US diameter > 5 cm and only 32 (40%) of them underwent conservative surgery. A laparoscopic approach was performed in 60 cases (61.9%) although in 15 (15.5%) conversion to open surgery was deemed necessary. A functional cyst was present in 49 patients (50.5%) while 11 children (11.3%) suffered from OT on a normal ovary.ConclusionsOur results showed that a post-menarchal age (p = .001), a pre-operative US ovarian size < 5 cm, (p = .001), the presence of severe abdominal pain (p = .002), a laparoscopic approach (p < .001), and the presence of a functional cyst (p = .002) were significantly associated with conservative surgery.

SD has unknown aetiology and is characterised by a single segment of dilated intestine with threefold to fourfold increase, from duodenum to the distal colon, with abrupt transition to normal bowel and no evidence of intrinsic obstruction or deficient innervation.3 4 The differential diagnosis of SD includes more common causes of intestinal obstruction, such as midgut volvulus and Hirschsprung’s disease.3 ‘Bowel loop sign’ is the radiological diagnostic hallmark of SD. Ethics approval All procedures performed in this study were in accordance with the ethical standards of the Institutional and National Research Committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Enteric nervous system, interstitial cells of Cajal, and smooth muscle vacuolization in segmental dilatation of jejunum.

Pediatric liver transplantation is a challenging surgical procedure requiring complex post-transplant patient management. Liver transplantation in children should ensure long-term survival and good health-related quality of life (HR-QOL), but data in the literature are conflicting. With the aim of investigating survival and psychosocial outcomes of patients transplanted during childhood, we identified 40 patients with ≥ 20-year follow-up after liver transplantation regularly followed up at our Institution. Clinical charts were reviewed to retrieve patients’ data. Psychosocial aspects and HR-QOL were investigated by an in-person or telephonic interview and by administering the WHOQOL-BREF questionnaire through an online form. Ten- and 20-year patient survival was 97.5% (95% CI 92.8–100%), whereas 10- and 20-year graft survival was 77.5% (65.6–91.6%) and 74.8% (62.5–89.6%), respectively. At last follow-up visit, 31 patients (77.5%) were receiving a tacrolimus-based immunosuppression. Twelve (32.4%) patients obtained a university diploma or higher, whereas 19 (51.4%) successfully completed high school. 81.1% of patients were active workers or in education, 17.5% had children, and 35% regularly practiced sport. 25 patients answered to the WHOQOL-BREF questionnaire. More than 60% of respondents did not report any disability and the perceived physical status was invariably good or very good. Median scores for physical health, psychological health, social relationships, and environment were 16.6, 14.7, 16, and 15, respectively. Pediatric liver transplantation is associated with excellent long-term survival and good HR-QOL. Psychological health and environment represent areas in which support would be needed to further improve HR-QOL.

DICER1 syndrome is a rare genetic disorder that predisposes patients to the development of malignant and non-malignant diseases. Presently, DICER1 syndrome diagnosis still occurs late, usually following surgical operations, affecting patients’ outcomes, especially for further neoplasms, which are entailed in this syndrome. For this reason, herein we present a multicenter report of DICER1 syndrome, with the prospective aim of enhancing post-surgical surveillance. A cohort of seven patients was collected among the surgical registries of Pediatric Surgery at the University of Pisa with the General and Oncologic Surgery of Federico II, University of Naples, and the Pediatric Surgery, Regina Margherita Hospital, University of Turin. In each case, the following data were analyzed: sex, age at diagnosis, age at first surgery, clinical features, familial, genetic investigations, and follow-up. A comprehensive literature review of DICER1 cases, including case reports and multicenter studies published from 1996 to June 2022, was performed. Eventually, the retrieved data from the literature were compared with the data emerging from our cohort of patients.

IntroductionOver the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations.MethodsFollowing a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery.Results39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers.ConclusionThoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.