Renal Oncocytosis in a Pediatric Patient: Case Report and Review of the Literature

Renal oncocytosis is a rare condition in which the kidney develops numerous oncocytomas. We present a case of a 12-year-old female who presented with right-sided flank pain of one-year duration. Imaging revealed several masses in the right kidney. Tissue biopsy confirmed multiple benign oncocytomas. Due to the presence of multiple oncocytomas throughout the kidney, a radical nephrectomy was performed. Given the rarity of this condition, as well as its known association with von Hippel-Lindau disease and Birt-Hogg-Dube syndrome, genetic investigations were pursued but failed to identify any abnormalities. This patient remains well and disease free six years after surgery. A review of the literature of this rare condition was performed

Pulmonary Sequestration Causing Severe Cardiac Failure Requiring Lobectomy in an Extreme Preterm Infant

We report a case of a large, extralobar pulmonary sequestration in a preterm infant born at 25 weeks gestational age. A computed tomography (CT) angiogram demonstrated that the arterial supply arose from the celiac trunk while an abnormally large, single left pulmonary vein drained the sequestration. This, along with the large patent ductus arteriosus (PDA), created a double left to right shunt, which resulted in severe, high output cardiac failure. Despite aggressive medical management for 3 weeks, he remained critically ill and developed renal failure. Therefore, after multiple, extensive multi-disciplinary discussions with the family, resection was offered as the only possibility for survival. He underwent a left thoracotomy and resection of the extra-lobar sequestration, which was occupying the lower two-thirds of his left hemithorax. To our knowledge, this is the youngest patient in the literature to undergo resection of an extra-lobar sequestration. Management challenges in terms of balancing the cardiac failure against the timing, approach and success of surgical intervention are also discussed along with a review of the literature

Hair epilation versus surgical excision as primary management of pilonidal disease in the pediatric population

Pilonidal disease is a chronic, acquired inflammatory process of the skin due to entrapped hair at the natal cleft. Reported recurrence rates are as high as 30%, and recurrence has been attributed to persistent hair near the surgical site. Although conservative measures, such as meticulous hair control and improved perineal hygiene, have been shown to be effective, these techniques typically require much effort on behalf of the patient. Laser hair epilation (LE) might solve this issue of poor patient compliance while helping patients to avoid surgical excision. In this article, we discuss recurrence rates of pilonidal disease in children treated with LE versus surgical excision in relation to findings from our institution between 2005 and 2013 as well as patient satisfaction with the treatment method

Laparoscopic-assisted percutaneous endoscopic gastrostomy: Insertion of a skin-level device using a tear-away sheath

Background: This study describes our experience with the placement of a skin-level gastrostomy device (MIC-KEY) in a single procedure. Methods: We identified infants, children and young adults who underwent laparoscopicassisted percutaneous endoscopic gastrostomy (LAPEG) tube insertion between October 2009 and June 2013. The steps of this procedure include upper endoscopy, single-port laparoscopy, gastropexy via percutaneous T-fasteners and placement of a skin-level gastrostomy device (MIC-KEY) using a push technique with a tearaway sheath. Results: We included 92 patients in our study. Mean age was 3.7 years (range 3 wk- 5 yr), and mean weight was 11.2 (range 2.8-54) kg. Median procedural time was 20 (range 12-76) minutes. Total median duration for the most recent 25 procedures was lower than that of the first 25 (62 v. 79 min, p = 0.004). There were no intraoperative complications or conversions to open surgery. Postoperative complications were observed in 6 (6.5%) patients. Three retained T-fasteners were assessed endoscopically (n = 1) or removed via local excision (n = 2). Two patients experienced early dislodged feeding tubes that were replaced via interventional radiology (n = 1) or repeat LAPEG (n = 1). There was also 1 intra-abdominal fluid collection that was drained percutaneously but ultimately required a laparotomy and washout. There were no major complications in the most recent 50 procedures. Conclusion: Our results suggest that LAPEG is a safe, minimally invasive procedure for infants, children and young adults. This approach allows for immediate use of a skin-level gastrostomy device without the need for postoperative tube exchanges

Making the transition from video-assisted thoracoscopic surgery to chest tube with fibrinolytics for empyema in children: Any change in outcomes?

Background: There is ongoing variation in the use of video-assisted thoracoscopic surgery (VATS) and chest tube with fibrinolytics (CTWF) for empyema in children. Our objective was to report outcomes from a centre that recently made the transition from VATS to CTWF as the primary treatment modality. Methods: We conducted a historical cohort study of children with empyema treated with either primary VATS (between 2005 and 2009) or CTWF (between 2009 and 2013). Results: Sixty-seven children underwent pleural drainage for empyema during the study period: 28 (42%) were treated with primary VATS, and 39 (58%) underwent CTWF. There were no significant differences between the VATS and CTWF groups for length of stay (8 v. 9 d, p = 0.61) or need for additional procedures (4% v. 13%, p = 0.19). Length of stay varied widely for both VATS (4-53 d) and CTWF (5-46 d). Primary VATS failed in 1 (4%) patient, who required an additional chest tube, and CTWF failed in 5 (13%) patients. Additional procedures included 3 rescue VATS, 2 additional chest tubes and 1 thoracotomy. All patients recovered and were discharged home. Conclusion: Primary VATS and CTWF were associated with similar outcomes in children with empyema. There appears to be a subset of children at risk for treatment failure with CTWF. Further research is needed to determine if these patients would benefit from primary VATS

Neuroendocrine tumor of the pancreas causing biliary obstruction in a 12 year-old girl: A case report and literature review

Pancreatic tumors are uncommon in children and rarely result in biliary obstruction. A previously well 12-year old female presented with a one-week history of fatigue, pruritis, and painless jaundice. Abdominal ultrasound demonstrated a mass in the pancreatic head associated with dilation of the common bile duct. Further workup included abdominal MRI, CT and endoscopic retrograde pancreaticogram (ERCP) with biliary stenting. Octreotide scan did not reveal uptake in the pancreatic tumor. Percutaneous biopsies were consistent with a grade 2 pancreatic neuroendocrine tumor (NET). Preoperative imaging demonstrated involvement of the portal vein. The patient was brought the operating room for a pancreaticoduodenectomy and portal vein resection. Final pathology revealed a T3N1M0 pancreatic NET. The patient recovered uneventfully

Renal ganglioneuromas in a pediatric patient: Case report and review of the literature

AbstractGanglioneuromas are rare benign tumors originating from the sympathetic nervous system and neural crest cells. A 4-year-old girl presented with numerous urinary tract infections. Ultrasound and computed tomography revealed a large mass within the right kidney. A right nephrectomy and sampling of surrounding lymph nodes were performed. Pathology confirmed that the mass was a mature ganglioneuroma. The patient remains disease-free, more than 2 years after surgery. We present this rare case of renal ganglioneuroma as well as a review of the literature

The effects of tracheal occlusion on Wnt signaling in a rabbit model of congenital diaphragmatic hernia

Purpose: Tracheal occlusion (TO)reverses pulmonary hypoplasia (PH)in congenital diaphragmatic hernia (CDH), but its mechanism of action remains poorly understood. Wnt signaling plays a critical role in lung development, but few studies exist. The purpose of our study was to a)confirm that our CDH rabbit model produced PH which was reversed by TO and b)determine the effects of CDH +/− TO on Wnt signaling. Methods: CDH was created in fetal rabbits at 23 days, TO at 28 days, and lung collection at 31 days. Lung body weight ratio (LBWR)and mean terminal bronchiole density (MTBD)were determined. mRNA and miRNA expression was determined in the left lower lobe using RT-qPCR. Results: Fifteen CDH, 15 CDH + TO, 6 sham CDH, and 15 controls survived and were included in the study. LBWR was low in CDH, while CDH + TO was similar to controls (p = 0.003). MTBD was higher in CDH fetuses and restored to control levels in CDH + TO (p \u3c 0.001). Reference genes TOP1, SDHA, and ACTB were consistently expressed within and between treatment groups. miR-33 and MKI67 were increased, and Lgl1 was decreased in CDH + TO. Conclusion: TO reversed pulmonary hypoplasia and stimulated early Wnt signaling in CDH fetal rabbits. Type of study: Basic science, prospective. Level of evidence: II

Hyoscine butylbromide versus acetaminophen for nonspecific colicky abdominal pain in children: a randomized controlled trial.

BACKGROUND: Less than two-thirds of children with abdominal pain in the emergency department receive analgesia. We sought to determine whether hyoscine butylbromide was superior to acetaminophen for children with nonspecific colicky abdominal pain. METHODS: We randomly allocated children aged 8-17 years with nonspecific colicky abdominal pain who presented to the pediatric emergency department of London Health Sciences Centre, London, Ontario to receive hyoscine butylbromide, 10 mg given orally, or acetaminophen, 15 mg/kg given orally (maximum 975 mg). We considered the minimal clinically important difference for the primary outcome (self-reported pain at 80 min) to be 13 mm on a 100 mm visual analogue scale. Secondary outcomes included administration of rescue analgesia, adverse effects and pain score less than 30 mm at 80 minutes. RESULTS: A total of 236 participants (120 in the hyoscine butylbromide group and 116 in the acetaminophen group) were included in the trial. The mean visual analogue scale scores at 80 minutes were 29 mm (standard deviation [SD] 26 mm) and 30 mm (SD 29 mm) with hyoscine butylbromide and acetaminophen, respectively (adjusted difference 1, 95% confidence interval -7 to 7). Rescue analgesia was administered to 4 participants (3.3%) in the hyoscine butylbromide group and 1 participant (0.9%) in the acetaminophen groups ( INTERPRETATION: Hyoscine butylbromide was not superior to acetaminophen in this setting. Both agents were associated with clinically important pain reduction, and either can be considered for children presenting to the emergency department with nonspecific colicky abdominal pain

Appendectomy versus non-operative treatment for acute uncomplicated appendicitis in children: Study protocol for a multicentre, open-label, non-inferiority, randomised controlled trial

Background Appendectomy is considered the gold standard treatment for acute appendicitis. Recently the need for surgery has been challenged in both adults and children. In children there is growing clinician, patient and parental interest in non-operative treatment of acute appendicitis with antibiotics as opposed to surgery. To date no multicentre randomised controlled trials that are appropriately powered to determine efficacy of nonoperative treatment (antibiotics) for acute appendicitis in children compared with surgery (appendectomy) have been performed. Methods Multicentre, international, randomised controlled trial with a non-inferiority design. Children (age 5–16 years) with a clinical and/or radiological diagnosis of acute uncomplicated appendicitis will be randomised (1:1 ratio) to receive either laparoscopic appendectomy or treatment with intravenous (minimum 12 hours) followed by oral antibiotics (total course 10 days). Allocation to groups will be stratified by gender, duration of symptoms (≫ or \u3c48 hours) and centre. Children in both treatment groups will follow a standardised treatment pathway. Primary outcome is treatment failure defined as additional intervention related to appendicitis requiring general anaesthesia within 1 year of randomisation (including recurrent appendicitis) or negative appendectomy. Important secondary outcomes will be reported and a cost-effectiveness analysis will be performed. The primary outcome will be analysed on a non-inferiority basis using a 20% non-inferiority margin. Planned sample size is 978 children. Discussion The APPY trial will be the first multicentre randomised trial comparing non-operative treatment with appendectomy for acute uncomplicated appendicitis in children. The results of this trial have the potential to revolutionise the treatment of this common gastrointestinal emergency. The randomised design will limit the effect of bias on outcomes seen in other studies. Trial registration number clinicaltrials.gov:NCT02687464. Registered on Jan 13th 2016