Fluorescence-guided lymph node sampling is feasible during up-front or delayed nephrectomy for Wilms tumor.

BACKGROUND: Lymph node sampling is critical to surgical staging in Wilms tumor; failure to sample lymph nodes is associated with under-staging and an increased incidence of local relapse. However, no standard lymphatic mapping method is currently being utilized for Wilms tumor to aid identification of regional draining lymph nodes. Herein, we describe the use of fluorescence-guided lymphatic mapping for Wilms tumor. MATERIALS AND METHODS: Two tertiary level referral centers independently began indocyanine green (ICG) fluorescence-guided nodal mapping. In one center, this was achieved with ipsilateral intra-parenchymal (IP) injection of ICG during minimally invasive tumor nephrectomy (MIN) following neoadjuvant chemotherapy and in the other, with Peri­Hilar (PH) injection during upfront, open tumor nephrectomy (ON). Successful lymph node mapping was defined as the presence of fluorescence signal in draining lymph nodes. RESULTS: Eight patients (median age of 2.5 years) underwent fluorescence-guided lymphatic mapping (four IP and four PH injection). Lymphatic mapping was successful in seven patients (88%) including each of the four patients with IP injection. CONCLUSIONS: Fluorescence-guided lymphatic mapping of Wilms tumor drainage is feasible by both IP injection and PH injection techniques. However, whether lymphatic mapping improves the precision of lymph node sampling is unknown and should be studied in prospective trials.

A single centre experience using internal traction sutures in managing long gap oesophageal atresia.

AIM: Thoracoscopically placed internal traction sutures (ITS) for the initial management of long gap oesophageal atresia (LGOA), not amenable to primary anastomosis, was first described in 2015. Here we describe our experience using ITS both thoracoscopically and at thoracotomy where the gap between upper and lower oesophagus is too wide for primary anastomosis. METHOD: The case notes of all infants treated with ITS for oesophageal atresia (01/10/2015 to 01/12/2019) were reviewed. Gaps considered too wide for an anastomosis had ITS placed between the two pouches as described by Patkowski in 2015. All patients were gastrostomy fed.  Patients returned to theatre with an expectation to complete the anastomosis or re-tighten the traction sutures. RESULTS: Seven patients (4 OA, 1 OA with proximal fistula, 2 OA/distal TOF) median birthweight 2.28 kg (1.2-3.6 kg) were managed using ITS. Median gap length 4.5 (3-9) vertebral bodies.  ITS were placed thoracoscopically in 5 cases and at thoracotomy in 2 at median 46 days (1-120) old. In all cases, ITS was associated with significant intra-thoracic adhesions. Five patients leaked from the traction sutures. Four patients had a delayed primary anastomosis performed at thoracotomy and 3 required a cervical oesophagostomy. The median length of stay was 159 days (98-282).  All patients started thoracoscopically eventually required thoracotomy. CONCLUSION: The use of ITS in our department was associated with significant complications, particularly intra-thoracic leaks and adhesions.  In our hands ITS did not improve the feasibility of thoracoscopic repair for LGOA and has been abandoned by us. LEVEL OF EVIDENCE: Level IV Case Series.

Ultrasound-guided percutaneous insertion of Broviac lines in infants less than 5kg: Prospective study of 100 consecutive procedures.

AIM: Ultrasound-guided (USG) percutaneous insertion of Broviac lines (cuffed tunnelled silastic central venous catheters, TCVC) has increasingly been adopted throughout the UK. However, vascular access remains a challenge in small babies and in some units is still performed by open cutdown. Our vascular access team, established in 2004, consists of consultant surgeons, anaesthetists and interventional radiologists, who provide all permanent vascular access by the USG technique. We reviewed the outcome in our last 100 patients less than 5 kg. METHOD: A prospective database of TCVC insertions in patients <5 kg weight recorded age, gestation, weight, diagnosis, type of catheter and complications within 28 days of insertion. A standardised technique of USG insertion is used by all operators. RESULTS: One-hundred patients <5 kg had TCVC inserted between 1/1/2018 and 31/3/2020. Median age 46(range0-316)days, gestation 36.5(23-42)weeks, weight 3(0.66 to 5)kg. INDICATION: parenteral nutrition(75), long term antibiotics(14), cardiac medication(6), chemotherapy(3), other(2). All were tunnelled silicone lines of single 2.7fr(51) and 4.2fr(46) or double lumen 7fr(3). Uncomplicated insertion in 94/100 cases. In 6 patients difficulties were encountered with cannulating the vein. In 4 cases an experienced colleague was called and managed to cannulate the vein; in 1 case a new successful attempt was made on the opposite internal jugular vein, and in 1 the femoral vein was used. No patient required an open cutdown. There were no cases of line sepsis requiring removal but 1 replacement was required for blockage within 28days. CONCLUSION: The USG approach in infants<5 kg is safe and can be used exclusively for venous access even in the most tiny babies. It is, however, a technically challenging procedure therefore we would recommend establishing a consultant delivered vascular access team to provide this service. Open venous cutdown in a tertiary children's hospital is no longer necessary for the insertion of TCVC and should be abandoned altogether. LEVELS OF EVIDENCE: Level I Prognosis Study.

Controlled aspiration of large paediatric ovarian cystic tumours.

AIM: Cystic ovarian masses in children may be physiological or neoplastic. It is mandatory that suspected neoplastic lesions are resected without tumour spillage. However, a large midline incision is cosmetically unappealing incision to young women. Here we describe our experience of using controlled drainage without spillage that allows a cosmetic pfannenstiel approach without compromising oncological principles. METHOD: All girls treated with large ovarian cystic masses since 2008 in our centre were identified and data was collected prospectively. A small pfannenstiel incision was performed followed by peritoneal washings; tissue glue was used to stick an Opsite™ dressing to the cyst surface and fluid drained so there was no leakage back into the patient. Once aspirated the cyst was delivered and an ovarian preserving cystectomy was performed where possible. RESULTS: Twenty-three girls (median age 14.5 years (8.1 to 16.5 years) were included. Pre-operative MRI scan showed a complex lesions with median volume of 1169 ml (range 252-7077 ml). At surgery 22/23 cysts were intact and removed without spillage. HISTOLOGY: mature teratoma (11), serous cyst (3), mucinous cyst adenocarcinoma (2), mucinous cystadenoma (5), Sertoli-Leydig tumour, sclerosing stromal tumour. One girl with pre-operative rupture of a mucinous adenocarcinoma subsequently died. Ovarian sparing cystectomy was performed in 17/23 girls. All other patients are well without evidence of recurrence. CONCLUSION: This is the largest series in children and adolescents using controlled drainage of cystic ovarian tumours. Though there were a range of diagnoses we have shown that these can be removed safely with a cosmetic pfannenstiel approach while following oncological principles.

Paediatric major incident triage: UK military tool offers best performance in predicting the need for time-critical major surgical and resuscitative intervention.

BACKGROUND: Children are frequently injured during major incidents (MI), including terrorist attacks, conflict and natural disasters. Triage facilitates healthcare resource allocation in order to maximise overall survival. A critical function of MI triage tools is to identify patients needing time-critical major resuscitative and surgical intervention (Priority 1 (P1) status). This study compares the performance of 11 MI triage tools in predicting P1 status in children from the UK Trauma Audit and Research Network (TARN) registry. METHODS: Patients aged <16 years within TARN (January 2008-December 2017) were included. 11 triage tools were applied to patients' first recorded pre-hospital physiology. Patients were retrospectively assigned triage categories (P1, P2, P3, Expectant or Dead) using predefined intervention-based criteria. Tools' performance in <16s were evaluated within four-yearly age subgroups, comparing tool-predicted and intervention-based priority status. FINDINGS: Amongst 4962 patients, mortality was 1.1% (n = 53); median Injury Severity Score (ISS) was 9 (IQR 9-16). Blunt injuries predominated (94.4%). 1343 (27.1%) met intervention-based criteria for P1, exhibiting greater intensive care requirement (60.2% vs. 8.5%, p < 0.01) and ISS (median 17 vs 9, p < 0.01) compared with P2 patients. The Battlefield Casualty Drills (BCD) Triage Sieve had greatest sensitivity (75.7%) in predicting P1 status in children <16 years, demonstrating a 38.4-49.8% improvement across all subgroups of children <12 years compared with the UK's current Paediatric Triage Tape (PTT). JumpSTART demonstrated low sensitivity in predicting P1 status in 4 to 8 year olds (35.5%) and 0 to 4 year olds (28.5%), and was outperformed by its adult counterpart START (60.6% and 59.6%). INTERPRETATION: The BCD Triage Sieve had greatest sensitivity in predicting P1 status in this paediatric trauma registry population: we recommend it replaces the PTT in UK practice. Users of JumpSTART may consider alternative tools. We recommend Lerner's triage category definitions when conducting MI evaluations.

Exomphalos major: Conservative management using Manuka honey dressings and an outreach surgical nursing team.

INTRODUCTION: Controversy exists over the best dressing for conservative management of exomphalos major. Here we describe our experience of using Manuka Honey. METHODS: Our regimen involved covering the sac with Manuka honey (Advancis Medical™) wrapped with gauze and crepe bandage. Initially, dressings were changed 3 times a week and then twice weekly until full epithelialisation. Babies went home after reaching full feeds, with our outreach nurses continuing dressings in clinic until the parents were trained to do them alone. Only patients needing management of co-morbidities were transferred to our unit. Patients would be reviewed by video consultation. Data was prospectively collected. RESULTS: From 2011-2019, 24 consecutive patients (11:13 M:F; median gestation 37 weeks, birth weight 3.1 kg) with exomphalos major were managed with honey dressings. Fourteen babies had significant associated anomalies of which 10 died of problems unrelated to the exomphalos. Time to full feeds 6 (2-58) days; time to discharge 21(7-66) days if no associated anomalies; time to epithelialisation 73 (27-199) days. Dressings were well tolerated. Definitive closure occurred at 17(11-38) months and was uneventful. No patient required fundoplication and all patients were orally fed. Only one patient developed a clinically significant infection. CONCLUSION: This is the largest report of using Manuka honey for the management of exomphalos major. Benefits include early feeding, early discharge and a 'normalisation' of the neonatal period. Key to our success was the surgical outreach service supporting parents doing the dressings, first at the local hospital and then at home.

A single centre matched pair series comparing minimally invasive and open surgery for the resection of pediatric renal tumours.

INTRODUCTION: Minimally invasive nephrectomy (MIN)for the resection of pediatric renal tumors is controversial. We compared patients who underwent laparoscopic MIN according to our unit protocol with matched patients who underwent open surgery (ON). METHOD: A single centre retrospective analysis of prospectively collected data for MIN. Tumor volume was calculated from pre-operative imaging and volume matched ON patients were identified from the regional tumor database. Demographics, complications, operative time, length of stay, number of nodes resected, status of margins, radiotherapy (RT)requirement, length of follow-up and outcomes were analysed. RESULTS: Fourteen MINs performed between September 2016 and 11/2019 (M:F 5:9; median age 37 (range 2-73)months) were matched with ON controls (7:7 M:F, median age 42 (1-74)months). Diagnoses in the MIN group were Wilms Tumour (WT)in11 and one each of mesoblastic nephroma (MN), malignant rhabdoid tumour of the kidney (MRTK), nephroblastomatosis and in the ON group were 13 WT and 1 MN. Median operating time for MIN was 180 (125-300)minutes, with 4 conversions and 155 (1-3-480)minutes in ON. There were no intra-operative ruptures. Three tumors in each group had positive margins. A median of 4 (0-6) nodes were removed during MIN and 4 (2-22) during ON. Median length of stay (LoS)was 4 (2-15)days following MIN and 4 (2-9) following MIN. Six ON and 3 MIN patients required radiotherapy. Differences were not significant. Median follow up of 30 (6-44)months for MIN and 73 (34-109)months for ON patients was significantly different. There have been no local recurrences but the child with MRTK died following brain tumor recurrence. CONCLUSION: MIN for pediatric renal tumors has similar short term outcomes to ON in carefully selected patients.

Biodegradable Stents in the Management of Refractory Esophageal Strictures in Children.

Background: Management of refractory esophageal strictures in children is challenging and mostly involves frequent and prolonged dilatations that are associated with significant physical and psychological complications. Biodegradable stents (BDS) have recently been used and it is particularly attractive in children as stent removal is avoided due to their dissolution. Report of Cases: We report our experience with BDS use in 3 children with severe refractory esophageal strictures. Two of the patients had strictures as a result of caustic ingestion and 1 after long-gap esophageal atresia repair. Median number of dilatations prestent is 5 per year. Custom-made uncovered BDS were inserted under general anesthesia. Median follow-up period was 23 months. Results: Minor postprocedure complications occurred in 1 patient. Stent insertion led to temporary improvement in swallowing in all cases. However, all of the patients developed recurrent symptoms as severe as before stenting and need for dilatations remained unchanged. They also experienced mucosal hyperplasia that contributed to their recurrent symptoms. Conclusion: All of the cases in our series did not have a favorable outcome with BDS use. BDS is yet to be proven as an effective management option for refractory esophageal strictures in children from our experience as well as most other published reports.

Damage control surgery in neonates: Lessons learned from the battlefield.

INTRODUCTION: Mortality for neonates requiring surgery for serious pathology such as NEC, remains high. Damage control surgery (DCS) has evolved as an operative strategy in battlefield trauma that sacrifices the completeness of the initial surgery to address the deadly triad of acidosis, hypothermia and coagulopathy. This approach is now used routinely in sick adults with nontrauma surgical emergencies. Here we describe our experience of using DCS in neonates. METHOD: Neonates undergoing DCS at our hospital from 1/8/2010 to 30/11/17 had data collected prospectively. RESULTS: 27 neonates (median age 21 days; gestation 29 weeks; weight 1200 g; M:F 18:9) underwent DCS. Diagnosis (NEC 23, volvulus 2, meconium peritonitis 1, spontaneous perforation 1). Preoperative physiology: median temperature 35.5 °C, lactate 3.7, Activated prothrombin time 49; on a median of 1 inotrope (range 0 to 4); 19 had surgery on the intensive care unit. Surgery involved resection of dead bowel with the ends ligated and the abdomen left open. Operation took 38 min (26-80 min) and crew-resource management techniques were used to optimize efficiency. Second look occurred at 48 h (24-108 h) when the physiology had normalized. There were a total of 32 anastomoses in 18 patients with one leak; 3 patients had stomas for distal rectal disease. Overall mortality was 15% (4/27) or 18% in the NEC group (4/23). CONCLUSION: Though techniques such as "clip and drop" exist, they have not been routinely incorporated into an operative strategy for sick neonates based on physiological derangement. The two benefits from our DCS approach were a low mortality and an avoidance of stomas. This approach deserves more investigation to see whether it is as effective in babies and children with nontrauma associated abdominal catastrophes as it is in adults. TYPE OF STUDY: Case controlled study. LEVEL OF EVIDENCE: Level III.

Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders.

Totally implantable vascular access devices (TIVADs) are commonly used in conjunction with enzyme replacement therapy (ERT) for lysosomal storage disorders (LSDs). This case series describes potential complications associated with long-term TIVAD use, such as compromise of skin integrity, infection, or port failures. Best practices and skilled specialists are essential for minimizing complications from long-term TIVAD use for ERT.

Characterization of the upper pouch tracheo-oesophageal fistula in oesophageal atresia.

AIM: A small proportion of infants with oesophageal atresia (OA) are thought to have a proximal tracheoesophageal fistula (TOF). Failure to recognize these can hamper mobilization of the upper pouch and lead to life-threatening episodes of aspiration once oral feeding starts. We reviewed our experience of upper pouch fistulae to identify characteristic features of proximal TOF. METHODS: A retrospective review of TOF/OA patient notes and bronchoscopy photographs and videos, identified from our database from 01/01/2006 to 12/31/2015, was performed. RESULTS: Eight (6.1%) infants were identified (M:F 5:3) from a total population of 131 newly diagnosed TOF/OA infants during the period. Their median gestational age was 33 (range 28-39) weeks, and median birth weight was 1647g (range 1100-3400g). Five were initially diagnosed with pure OA and 3 with a distal TOF. All patients underwent rigid bronchoscopy at the initial surgery but only one proximal fistula was identified. The 7 missed proximal fistulae were subsequently found either during on-table oesophagograms for gap assessment (n=2), at the time of thoracotomy when mobilizing the upper pouch (n=3), or during subsequent bronchoscopy for symptoms post OA repair (n=2). Two patients needed a further operation to divide the fistula. Review of the bronchoscopy videos identified four characteristic differences between upper and lower pouch fistulae. Proximal fistulae are found just distal to the vocal cords, are very small, often no more than a pit, do not open and close with ventilation, and are best identified by insufflation of the esophagus. CONCLUSION: Upper pouch fistulae are relatively easy to miss because of different characteristics compared with H-type or distal fistulae that have not previously been mentioned in the literature. LEVEL OF EVIDENCE: level IV.

Is adjuvant chemotherapy indicated in ovarian immature teratomas? A combined data analysis from the Malignant Germ Cell Tumor International Collaborative.

BACKGROUND: There is a debate regarding the management of ovarian immature teratomas (ITs). In adult women, postoperative chemotherapy is standard except for stage I, grade 1 disease, whereas surgery alone is standard in pediatric patients. To determine the role of chemotherapy, a pooled analysis of pediatric and adult clinical trials was conducted. METHODS: Data from 7 pediatric trials and 2 adult trials were merged in the Malignant Germ Cell International Collaborative data set. Four trials included patients with newly diagnosed pure ovarian ITs and were selected (Pediatric Oncology Group/Children's Cancer Group Intergroup Study (INT 0106), Second UKCCSG Germ Cell Tumor Study (GC2), Gynecologic Oncology Group (GOG 0078 and GOG 0090). Adult and pediatric trials were analyzed separately. The primary outcome measures were event-free survival (EFS) and overall survival (OS). RESULTS: One hundred seventy-nine patients were included (98 pediatric patients and 81 adult patients). Ninety pediatric patients were treated with surgery alone, whereas all adult patients received chemotherapy. The 5-year EFS and OS were 91% and 99%, respectively, for the pediatric cohort and 87% and 93%, respectively, for the adults. There were no relapses in grade 1 patients, regardless of the stage or age. Only 1 adult patient with a grade 2 IT relapsed. Among grade 3 patients, the 5-year EFS was 0.92 (0.72-0.98) for stage I/II and 0.52 (0.22-0.75) for stage III in the pediatric cohort (P = .005) and 0.91 (0.69-0.98) for stage I/II and 0.65 (0.39-0.83) for stage III/IV in the adult cohort (P = .01). Postoperative chemotherapy did not decrease relapses in the pediatric cohort. CONCLUSIONS: The grade was the most important risk factor for relapse in ovarian ITs. Among grade 3 patients, the stage was significantly associated with relapse. Adjuvant chemotherapy did not decrease relapses in the pediatric cohort; its role in adults remains unresolved. Cancer 2016;122:230-237. © 2015 American Cancer Society.

Pediatric and Adolescent Extracranial Germ Cell Tumors: The Road to Collaboration.

During the past 35 years, survival rates for children with extracranial malignant germ cell tumors (GCTs) have increased significantly. Success has been achieved primarily through the application of platinum-based chemotherapy regimens; however, clinical challenges in GCTs remain. Excellent outcomes are not distributed uniformly across the heterogeneous distribution of age, histologic features, and primary tumor site. Despite good outcomes overall, the likelihood of a cure for certain sites and histologic conditions is less than 50%. In addition, there are considerable long-term treatment-related effects for survivors. Even modest cisplatin dosing can cause significant long-term morbidities. A particular challenge in designing new therapies for GCT is that a variety of specialists use different risk stratifications, staging systems, and treatment approaches for three distinct age groups (childhood, adolescence, and young adulthood). Traditionally, pediatric cancer patients younger than 15 years have been treated by pediatric oncologists in collaboration with their surgical specialty colleagues. Adolescents and young adults with GCTs often are treated by medical oncologists, urologists, or gynecologic oncologists. The therapeutic dilemma for all is how to best define disease risk so that therapy and toxicity can be appropriately reduced for some patients and intensified for others. Further clinical and biologic insights can only be achieved through collaborations that do not set limitations by age, sex, and primary tumor site. Therefore, international collaborations, spanning different cooperative groups and disciplines, have been developed to address these challenges.

Revised risk classification for pediatric extracranial germ cell tumors based on 25 years of clinical trial data from the United Kingdom and United States.

PURPOSE: To risk stratify malignant extracranial pediatric germ cell tumors (GCTs). PATIENTS AND METHODS: Data from seven GCT trials conducted by the Children's Oncology Group (United States) or the Children's Cancer and Leukemia Group (United Kingdom) between 1985 and 2009 were merged to create a data set of patients with stage II to IV disease treated with platinum-based therapy. A parametric cure model was used to evaluate the prognostic importance of age, tumor site, stage, histology, tumor markers, and treatment regimen and estimate the percentage of patients who achieved long-term disease-free (LTDF) survival in each subgroup of the final model. Validation of the model was conducted using the bootstrap method. RESULTS: In multivariable analysis of 519 patients with GCTs, stage IV disease (P = .001), age ≥ 11 years (P < .001), and tumor site (P < .001) were significant predictors of worse LTDF survival. Elevated alpha-fetoprotein (AFP) ≥ 10,000 ng/mL was associated with worse outcome, whereas pure yolk sac tumor (YST) was associated with better outcome, although neither met criteria for statistical significance. The analysis identified a group of patients age > 11 years with either stage III to IV extragonadal tumors or stage IV ovarian tumors with predicted LTDF survival < 70%. A bootstrap procedure showed retention of age, tumor site, and stage in > 94%, AFP in 12%, and YST in 27% of the replications. CONCLUSION: Clinical trial data from two large national pediatric clinical trial organizations have produced a new evidence-based risk stratification of malignant pediatric GCTs that identifies a poor-risk group warranting intensified therapy.

Management of complex abdominal wall defects associated with penetrating abdominal trauma.

INTRODUCTION: The paradigm of Damage Control Surgery (DCS) has radically improved the management of abdominal trauma, but less well described are the options for managing the abdominal wall itself in an austere environment. This article describes a series of patients with complex abdominal wall problems managed at the UK-led Role 3 Medical Treatment Facility (MTF) in Camp Bastion, Afghanistan. METHOD: Contemporaneous review of a series of patients with complex abdominal wall injuries who presented to the Role 3 MTF between July and November 2012. RESULTS: Five patients with penetrating abdominal trauma associated with significant damage to the abdominal wall were included. All patients were managed using DCS principles, leaving the abdominal wall open at the end of the first procedure. Subsequent management of the abdominal wall was determined by a multidisciplinary team of general and plastic surgeons, intensivists and specialist nurses. The principles of management identified included minimising tissue loss on initial laparotomy by joining adjacent wounds and marginal debridement of dead tissue; contraction of the abdominal wall was minimised by using topical negative pressure dressing and dermal-holding sutures. Definitive closure was timed to allow oedema to settle and sepsis to be controlled. Closure techniques include delayed primary closure with traction sutures, components separation, and mesh closure with skin grafting. DISCUSSION: A daily multidisciplinary team discussion was invaluable for optimal decision making regarding the most appropriate means of abdominal closure. Dermal-holding sutures were particularly useful in preventing myostatic contraction of the abdominal wall. A simple flow chart was developed to aid decision making in these patients. This flow chart may prove especially useful in a resource-limited environment in which returning months or years later for closure of a large ventral hernia may not be possible.