Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review.

BACKGROUND: Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tumors. However, if DICER 1 anomalies have been reported in patients with Wilms tumor (WT), to date, no cases of PPB, WT, and DICER1 mutations have been reported in the same patient. CASE PRESENTATION: We report the case of a 3-year-old patient, initially managed for metastatic WT. During his clinical course, the diagnosis of a PPB was made after detecting the DICER1 mutation and subsequent management was therefore modified. CONCLUSION: This case highlights that in case of simultaneous discovery of a renal tumor and a pulmonary lesion in a child, the DICER 1 mutations should be looked for as these could help adapt management and schedule the surgical procedures.

Pancreatic fracture: a rare complication following scoliosis surgery.

STUDY DESIGN: Grand Round case report. OBJECTIVE: We report a pancreatic fracture associated with Wirsung duct disruption, following a scoliosis surgery in a cerebral palsy adolescent. Spinal fusion surgery is the standard treatment for severe neuromuscular scoliosis. Many complications such as digestive ones account for its complexity. Postoperative acute pancreatitis is well described, although its pathophysiology remains unclear. To our knowledge, pancreatic fracture following scoliosis correction has never been described to date. Clinical presentation is not specific, and management is not consensual. CASE REPORT: A 14-year-old adolescent had posterior spinal fusion for neuromuscular scoliosis due to cerebral palsy. During the postoperative course, she developed progressive nonspecific abdominal symptoms. The abdominal CT scan demonstrated a pancreatic fracture and a surgical exploration was decided as perforations of the bowel were highly suspected. Drains were placed around the pancreatic area as the retrogastric region was out of reach due to local inflammation. Conservative management led to the occurrence of a pseudocyst in the following weeks as the pancreatic leakage progressively dropped. DISCUSSION: Two hypotheses have been proposed: direct iatrogenic trauma from lumbar pedicle screws and pancreatic rupture related to the correction of the spinal deformity. As the latter seems the most likely, spinal surgeons should be aware of this occurrence following severe scoliosis correction. CONCLUSION: Spinal fusion for severe neuromuscular scoliosis is a difficult procedure, with a high rate of complications. Among them, pancreatic fracture should be considered when abdominal pain persists in the postoperative period. Conservative management is advocated especially in case of a poor general condition.

Non-operative Management for Uncomplicated Appendicitis: An Option to Consider.

AIM: The main goal of our study was to assess a 7 days long course of antibiotics for acute uncomplicated appendicitis. MATERIALS AND METHODS: From March 2014 to November 2015, all patients diagnosed with acute appendicitis have been considered to be treated by only antibiotics. Inclusion criteria included clinical (tenderness), biological (C-reactive protein [CRP] < 50), and radiological features (diameter > 6 mm). All patients were treated with intravenous amoxicillin and clavulanic acid (100 mg/kg/day) for 2 days (six doses). At the end of the treatment, clinical and paraclinical examinations included blood samples at day 7 and ultrasound (US) scan at 3 months. RESULTS: A total of 166 patients were treated and followed up prospectively during the study period. Mean age at diagnosis was 10.8 ± 0.6 years. All children, but four were discharged with a clinical improvement after 48 hours and six intravenous antibiotics injection according to our protocol. Four children required surgery during the initial hospitalization period.Initial ultrasound scan showed a mean diameter of 7.85 ± 1.6 mm, with inflamed fat in 124 patients (74.7%). At Day 7, the diameter was 5.2 ± 1.6 mm (p < 0.0001).During a median follow-up of 18.8 months (3.5-18), 22 patients (13.25%) had to be managed for a novel episode of acute appendicitis after a median period of 138 days (13-270). None had to be managed for a complicated appendicitis. CONCLUSION: Non-operative treatment (NOT) is a safe alternative for the management of uncomplicated acute appendicitis in children. Further study should be conducted to determine relapse risk factors.

Engineered acellular collagen scaffold for endogenous cell guidance, a novel approach in urethral regeneration.

UNLABELLED: The treatment of congenital malformations or injuries of the urethra using existing autologous tissues can be associated with post-operative complications. Using rat-tail collagen, we have engineered an acellular high-density collagen tube. These tubes were made of 2 layers and they could sustain greater burst pressures than the monolayered tubes. Although it remains a weak material this 2 layered tube could be sutured to the native urethra. In 20 male New Zealand white rabbits, 2cm long grafts were sutured in place after subtotal excision of the urethra. This long-term study was performed in Lausanne (Switzerland) and in Kuala Lumpur (Malaysia). No catheter was placed post-operatively. All rabbits survived the surgical implantation. The animals were evaluated at 1, 3, 6, and 9months by contrast voiding cysto-urethrography, histological examination and immunohistochemistry. Spontaneous re-population of urothelial and smooth muscle cells on all grafts was demonstrated. Cellular organization increased with time, however, 20% of both fistula and stenosis could be observed post-operatively. This off-the shelf scaffold with a promising urethral regeneration has a potential for clinical application. STATEMENT OF SIGNIFICANCE: In this study we have tissue engineered a novel cell free tubular collagen based scaffold and used it as a urethral graft in a rabbit model. The novelty of our technique is that the tube can be sutured. Testing showed better burst pressures and the grafts could then be successfully implanted after a urethral excision. This long term study demonstrated excellent biocompatibility of the 2cm graft and gradual regeneration with time, challenging the current literature. Finally, the main impact is that we describe an off-the-shelf and cost-effective product with comparable surgical outcome to the cellular grafts.

Tubular Compressed Collagen Scaffolds for Ureteral Tissue Engineering in a Flow Bioreactor System.

Ureteral replacement by tissue engineering might become necessary following tissue loss after excessive ureteral trauma, after retroperitoneal cancer, or even after failed reconstructive surgery. This need has driven innovation in the design of novel scaffolds and specific cell culture techniques for urinary tract reconstruction. In this study, compressed tubular collagen scaffolds were evaluated, addressing the physical and biological characterization of acellular and cellular collagen tubes in a new flow bioreactor system, imitating the physiological pressure, peristalsis, and flow conditions of the human ureter. Collagen tubes, containing primary human smooth muscle and urothelial cells, were evaluated regarding their change in gene and protein expression under dynamic culture conditions. A maximum intraluminal pressure of 22.43 ± 0.2 cm H2O was observed in acellular tubes, resulting in a mean wall shear stress of 4 dynes/cm(2) in the tubular constructs. Dynamic conditions directed the differentiation of both cell types into their mature forms. This was confirmed by their gene expression of smooth muscle alpha-actin, smoothelin, collagen type I and III, elastin, laminin type 1 and 5, cytokeratin 8, and uroplakin 2. In addition, smooth muscle cell alignment predominantly perpendicular to the flow direction was observed, comparable to the cell orientation in native ureteral tissue. These results revealed that coculturing human smooth muscle and urothelial cells in compressed collagen tubes under human ureteral flow-mimicking conditions could lead to cell-engineered biomaterials that might ultimately be translated into clinical applications.

Bilateral anterior sternothoracotomy (clamshell incision): a suitable alternative for bilateral lung sarcoma metastasis in children.

BACKGROUND: The aim of our study was to assess the postoperative course of bilateral anterior sternothoracotomy (BAT) in children with sarcoma metastases, in a curative care perspective. METHODS: We reviewed the records of seven patients younger than 18 years old, who underwent surgical procedures for sarcoma metastasis to the lung between 2000 and 2012. We compared the postoperative course of the BAT group with that of patients who underwent unilateral posterolateral thoracotomies (PLTs) for the same etiology. RESULTS: Of 17 surgical procedures, there were seven BAT and 10 unilateral PLT. Mean ages at the time of the procedures were 12.9 ± 5.4 years old for BAT, and 17.4 ± 1.9 years old for PLT. Mean operative time was 173 ± 37 minutes in the BAT group, and 145 ± 39 minutes in the PLT group (P = 0.19). Patients received epidural analgesia in all cases; this was for a mean time of 3.8 ± 1.3 days in the BAT group, and 3.21 ± 4 days in the PLT group (P = 0.36). Chest tubes were removed after 3.6 ± 1.3 days in the BAT group, and 3 ± 1.2 days in the PLT group (P = 0.69). Total hospital stay was 7.7 ± 6.6 days in the BAT group, and 7 ± 1.2 days in the PLT group (P = 0.72). CONCLUSION: In our experience, BAT seems suitable and shows outcomes similar to those of PLT for sarcoma metastasis resection. The BAT procedure allows the manual exploration of both lungs during a single surgical intervention, and so reduces the delay of further therapies.

Osteosarcoma metastasis causing ileo-ileal intussusception.

Osteosarcoma metastasis causing intussusception is a very rare entity, with a pejorative prognosis. Based on a case, we performed a literature review in order to better assess this situation. We conclude that, in patients with a history of osteosarcoma lung metastasis, echographic and/or computed tomography scan evidence of a small bowel obstruction with intussusception should lead to an open surgical procedure if the laparoscopic approach does not allow to accurately explore and resect the lesion, in order to prevent misdiagnosis and to avoid further delay in the management.