Outcome of patients with undifferentiated embryonal sarcoma of the liver treated according to European soft tissue sarcoma protocols

Background

To assess the outcomes of pediatric patients with undifferentiated embryonal sarcoma of the liver (UESL) and treatment including at least surgery and systemic chemotherapy.

Methods

This study included patients aged up to 21 years with a pathological diagnosis of UESL prospectively enrolled from 1995 to 2016 in three European trials focusing on the effects of surgical margins, preoperative chemotherapy, use of radiotherapy (RT), and chemotherapy.

Results

Out of 65 patients with a median age at diagnosis of 8.7 years (0.6–20.8), 15 had T2 tumors, and one had lymph node spread, 14 were Intergroup Rhabdomyosarcoma Study (IRS) I, nine IRS II, 38 IRS III, and four IRS IV. Twenty-eight upfront surgeries resulted in five operative spillages and 11 infiltrated surgical margins, whereas 37 delayed surgeries resulted in no spillages (p = .0119) and three infiltrated margins (p = .0238). All patients received chemotherapy, including anthracyclines in 47. RT was administered in 15 patients. With a median follow-up of 78.6 months, 5-year overall and event-free survivals (EFS) were 90.1% (95% confidence interval [CI]: 79.2–95.5) and 89.1% (95% CI: 78.4–94.6), respectively. Two out four local relapses had previous infiltrated margins and two out of three patients with metastatic relapses received reduced doses of alkylating agents. Infiltrated margins (p = .1607), T2 stage (p = .3870), use of RT (p = .8731), and anthracycline-based chemotherapy (p = .1181) were not correlated with EFS.

Conclusions

Multimodal therapy improved the outcome of UESL. Neoadjuvant chemotherapy for pediatric patients increases the probability of complete surgical resection. The role of anthracyclines and RT for localized disease remains unclear.     

Thymus and mediastinal node involvement in childhood langerhans cell histiocytosis: Long‐term follow‐up from the French national cohort

Background

Mediastinal involvement (MI) in Langerhans cell histiocytosis (LCH) has been rarely reported. Here, we describe the clinical, radiological, and biological presentation, and the outcome of childhood LCH with MI.

Method

From the French LCH register, which includes 1,423 patients aged less than 18 years, we retrieved the medical charts of patients with mediastinal enlargement detected on chest X‐rays.

Results

Thirty‐seven patients were retrieved, including 18 males; median age of diagnosis was 0.7 years, and median follow‐up time was 6.2 years. The prevalence of MI varied with the age at diagnosis, ranging from 7% below 1 year old to less than 1% at >5 years. Thirteen cases (35%) were diagnosed because of MI‐related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10). CT scans performed in 32 cases at diagnosis showed tracheal compression (N = 5), cava thrombosis (N = 2), and/or calcification (N = 16). All patients presented multi‐system disease at LCH diagnosis, and 35/37 were initially treated with vinblastine and corticosteroids. Death occurred in five cases, due to MI (N = 1) or hematological refractory involvement (N = 4). The overall 5‐year survival was 87.1%, and immunodeficiency was not detected as a sequel.

Conclusions

MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications. Pediatr Blood Cancer 2013;60:1759–1765. © 2013 Wiley Periodicals, Inc.     

Effectiveness of a transluminal endoscopic fundoplication for the treatment of pediatric gastroesophageal reflux disease

Purpose  

Although laparoscopic Nissen fundoplication is the standard surgical treatment for gastroesophageal reflux disease (GERD), surgical complications and post-operative pain are not uncommon, especially for those patients who are neurologically impaired (NI) or undergoing re-operative procedures. To address this challenge, we utilized the transoral incisionless fundoplication (TIF) procedure to treat GERD via an endoscopic approach.     

Chronic bilious vomiting in children in developing countries due to high bowel obstruction: not always malrotation or tuberculosis

Background  

Bilious vomiting, in conjunction with abdominal pain is considered to be a surgical problem, unless proved otherwise. In children, besides tuberculosis (TB), we have found jejunal stricture (JS) due to non-specific jejunoileitis (NSJI) to be an important cause of chronic high small bowel obstruction and bilious vomiting.     

Comparative analysis between laparoscopic (UCL) and open (UCO) technique for the treatment of ulcerative colitis in pediatric patients

Purpose  

The aim of this study is to compare laparoscopic with open approach in the surgical treatment of ulcerative colitis (UC)     

B‐type natriuretic peptide for assessment of haemodynamically significant patent ductus arteriosus in premature infants

Aim

Haemodynamically significant patent ductus arteriosus (hsPDA) is frequently observed in premature infants. This study was conducted to explore whether the blood BNP can be a valuable biomarker to assess the necessity of treatment for hsPDA in premature infants.

Methods

Serial measurements of the blood BNP were performed during the first 5 days of life in premature infants with hsPDA (Group I) and those without hsPDA (Group N). The definition of the hsPDA was the PDA requiring treatment, such as indomethacin administration and/or surgical ligation.

Results

Forty‐six subjects were enrolled. Compared with Group N, Group I showed significantly higher level of blood BNP at postnatal 24–96 h and demonstrated the peak value at postnatal 24–48 h. With the ROC curve using the data at postnatal 24–48 h in Group I, we deduced the predictive value of 250 pg/mL of blood BNP for indomethacin treatment. Similarly, with the ROC curve using the maximal value of blood BNP within the first 5 days of life, the predictive value of 2000 pg/mL for surgical ligation was deduced.

Conclusions

Blood BNP during early postnatal period can be a useful biomarker to assess the necessity of treatment for hsPDA in premature infants.     

Ostomy complicat?ons in patients with anorectal malformations

Purpose  

Ostomy is an important step in the treatment of patients with anorectal malformation (ARM). Sometimes this basic surgical procedure may cause a devastating complication.     

Outcomes analysis after percutaneous abdominal drainage and exploratory laparotomy for necrotizing enterocolitis in 4,657 infants

Purpose  

Necrotizing enterocolitis (NEC) is a common acquired gastrointestinal disease of infancy that is strongly correlated with prematurity. Both percutaneous abdominal drainage and laparotomy with resection of diseased bowel are surgical options for treatment of NEC. The objective of the present study is to compare outcomes of patients who were treated either with bowel resection/ostomy (BR/O), percutaneous drainage (PD) or Both procedures for NEC in a retrospective analysis.     

Chylothorax in congenital diaphragmatic hernia

Purpose  

Following surgical repair of congenital diaphragmatic hernia (CDH), chylothorax can be present in 7–28% of the cases. It has been associated with prenatal diagnosis, the use of ECMO and prosthetic patches during reparatory surgery. The objective is to present a neonatal unit experience in handling this complication and the search for predictive factors for its appearance in our patients.     

Re-operation for Hirschsprung’s disease: experience in 24 patients from China

Purpose  

The aim of this study is to review the authors’ 12-year experience with re-operative surgery for Hirschsprung’s disease (HD) including indications of re-operation and surgical technique.     

Recurrent rectal prolapse following primary surgical treatment

Purpose  

The management of recurrent rectal prolapse following initial surgical procedures remains unclear. We present a series of pediatric patients with rectal prolapse and describe their surgical management, and the subsequent care for those children developing recurrence.     

Evaluation of the Implementation of a Multicomponent Intervention to Improve Health Care Provider Communication About Human Papillomavirus Vaccination

Objective

To evaluate the relative use, usefulness, and facilitators and barriers to use as perceived by providers of 5 different components in a human papillomavirus vaccine communication intervention—which was found to be effective at improving human papillomavirus vaccination rates.

Outcome of renal transplant in patients with abnormal urinary tract

Purpose  

To determine whether surgical procedures of the lower urinary tract in patients with uropathies affect evolution of the graft in renal transplantation.     

Surgical intervention strategies for pediatric ovarian tumors: experience with 60 cases at one institution

Purpose  

The aim of this study was to assess the surgical intervention strategies for pediatric ovarian tumors.     

Systemic postoperative pain management following minimally invasive pectus excavatum repair in children and adolescents: a retrospective comparison of intravenous patient-controlled analgesia and continuous infusion with morphine

Purpose  

Optimal postoperative pain management following minimally invasive surgical repair of pectus excavatum is not established. We compared efficacy and adverse effects in patients treated with patient-controlled analgesia (PCA) with those treated with continuous infusion (CI) with morphine in addition to nonsteroidal anti-inflammatory drugs.     

Image-defined risk factors in localized thoracic neuroblastoma and ganglioneuroma

Background

The pretreatment International Neuroblastoma Risk Group Staging System (INRGSS) discriminates localized tumors L1/L2 depending on the absence/presence of image-defined risk factors (IDRFs) at diagnosis. Referring to this new staging system, we assessed initial imaging of localized thoracic neuroblastoma (NB) and ganglioneuroma (GN) and the extent of initial tumor resection.

Methods

Patients with localized thoracic NB/GN from the German clinical trials NB97 and NB2004 were included. Imaging at diagnosis and operative reports were reviewed retrospectively. IDRFs were assessed centrally and correlated to International Neuroblastoma Staging System (INSS) stage and extent of tumor resection. Additionally, we analyzed data on surgery-related complications.

Results

Imaging series of 88 patients were available for central review. In 18 children, no IDRF was present, 28 exhibited one IDRF, 42 two or more IDRFs, resulting in 70 patients with L2 disease. The most frequently observed IDRF was encasement of any vessel (n = 38). Initial surgical resection was aimed for in 45 patients (L1: n = 11; L2: n = 34). Complete and gross total resection rates were higher children with L2 NB (n = 8/25 L1, n = 17/25 L2 vs. n = 2/15 L1, n = 13/15 L2, respectively). The proportion of surgical complications was very similar between INRGSS L1 and L2 (n = 4/11 vs. n = 17/34). All complications were manageable, and no surgery-related deaths were observed.

Conclusion

In this retrospective cohort, the extent of resection and the rate of surgical complications did not differ substantially between patients classified as L1/L2, indicating that INRGSS L2 does not equate unresectability. It appeared that individual IDRFs differ in value. Larger studies are needed to assess the significance and therapeutic/prognostic impact of such findings.     

Hydatid cysts of the liver in children: percutaneous treatment with ultrasound follow-up

Background  

Hydatid disease is a serious public health problem in endemic areas. Percutaneous treatment is an effective alternative to surgical and medical therapy in hydatid liver disease (HLD).     

Transumbilical laparoendoscopic single-site pyeloplasty in infants and children: initial experience and short-term outcome

Background  

Scar-free abdominal wall surgery is a research hotspot in recent years. This study presented surgical skills of transumbilical laparoendoscopic single-site pyeloplasty (LESS-P) for pediatric patients with ureteropelvic junction obstruction (UPJO) and its clinical application.     

Minimally invasive technique for the concealed penis lead to longer penile length

Objectives  

The aim of this study is to report a simple and minimally invasive surgical technique for congenital concealed penis repair.     

Surgical complications after living donor liver transplantation in patients with biliary atresia: a relatively high incidence of portal vein complications

Background/purpose  

The aim of this study is to present the surgical complications in living donor liver transplantation (LDLT) for biliary atresia (BA) as a treatment for end stage liver disease.