Dragon 1 Protocol Manuscript: Training, Accreditation, Implementation and Safety Evaluation of Portal and Hepatic Vein Embolization (PVE/HVE) to Accelerate Future Liver Remnant (FLR) Hypertrophy.

STUDY PURPOSE: The DRAGON 1 trial aims to assess training, implementation, safety and feasibility of combined portal- and hepatic-vein embolization (PVE/HVE) to accelerate future liver remnant (FLR) hypertrophy in patients with borderline resectable colorectal cancer liver metastases. METHODS: The DRAGON 1 trial is a worldwide multicenter prospective single arm trial. The primary endpoint is a composite of the safety of PVE/HVE, 90-day mortality, and one year accrual monitoring of each participating center. Secondary endpoints include: feasibility of resection, the used PVE and HVE techniques, FLR-hypertrophy, liver function (subset of centers), overall survival, and disease-free survival. All complications after the PVE/HVE procedure are documented. Liver volumes will be measured at week 1 and if applicable at week 3 and 6 after PVE/HVE and follow-up visits will be held at 1, 3, 6, and 12 months after the resection. RESULTS: Not applicable. CONCLUSION: DRAGON 1 is a prospective trial to assess the safety and feasibility of PVE/HVE. Participating study centers will be trained, and procedures standardized using Work Instructions (WI) to prepare for the DRAGON 2 randomized controlled trial. Outcomes should reveal the accrual potential of centers, safety profile of combined PVE/HVE and the effect of FLR-hypertrophy induction by PVE/HVE in patients with CRLM and a small FLR. TRIAL REGISTRATION: Clinicaltrials.gov: NCT04272931 (February 17, 2020). Toestingonline.nl: NL71535.068.19 (September 20, 2019).

Bleeding after circumcision is more likely in children with lichen sclerosus (balanitis xerotica obliterans).

INTRODUCTION: Over 27,000 circumcisions were performed in England in 2012-13. The complication rate is generally perceived to be low, although published figures vary widely. Balanitis xerotica obliterans, more correctly termed Lichen Sclerosus et atrophicus (LS), is one of the commonest indications for medical circumcision. To test the hypothesis that children undergoing circumcision for LS have a higher rate of postoperative bleeding than those undergoing the procedure for other reasons, we retrospectively reviewed records for patients undergoing circumcision. METHODS: The disease and procedure coding system was used to identify patients who underwent circumcision (ICD10 code N303) between 2000-2010. Cases with a diagnosis unrelated to circumcision and children circumcised during hypospadias repair were excluded. Bleeding which required return to theatre for surgical arrest was considered significant. Cases were identified by review of medical records if there was: a second procedure during the same admission, or readmission coded for circumcision within 2 weeks. Only cases with histologically confirmed LS were included in the LS cohort. GraphPad online calculator was used for statistical analysis (two tailed Fisher's exact test. RESULTS: 2385 boys with a median age of 4 years (range 0-16) were included in the study. Indication for circumcision included religious (1305, 54.7%), phimosis or redundant prepuce (512, 21.5%), suspected LS (366, 15.4%) and balanoposthitis (202, 8.5%). LS was histologically confirmed in 262 (10.9%) boys. Fourteen (0.6%) patients returned to theatre for surgical arrest of bleeding following circumcision; 6 had LS and 8 did not (Table 1). The bleeding rate was higher in those with LS (2.3%) than in those without (0.3%), P = 0.0003 with a relative risk of 6.08. CONCLUSION: Post-operative complications are distressing, especially if further surgery is required. Published figures for complications following circumcision vary widely making counseling regarding risk difficult. Since LS includes an inflammatory element and circumcision in widespread LS can be challenging, the observation of more post-operative bleeding in patients with histologically confirmed LS during a previous audit prompted the hypothesis that this may be a significant finding. Thus we reviewed all patients requiring return to theatre within 2 weeks of circumcision, finding that whilst the overall bleeding rate was low, circumcision for LS significantly increased the risk. Although factors such as the severity of LS and surgical technique were not assessed, this is still a notable finding which should be reflected during pre-operative counseling.

Retained central venous lines (CVLs) after attempted removal: an 11-year series and literature review.

BACKGROUND: Central venous lines (CVLs) are frequently used in the management of many neonatal and pediatric conditions. Failure to remove the luminal part of the line (retained CVL) is rare. Consequently, there is lack of experience and consensus in its optimal management. AIM: To document the incidence and management of retained CVLs in a tertiary pediatric surgical centre with access to interventional cardiology services. To review the literature and report efficacy/morbidity of attempted extraction of retained CVLs. METHODS: Children with retained CVLs were identified from departmental morbidity and mortality records over an 11-year period. A literature search was performed in PubMed and Scopus to identify studies reporting retained CVLs (earliest date to 1 January 2012). This was supplemented by scanning bibliographies of retrieved articles. RESULTS: The 11-year incidence of retained CVL was 0.3% (n=10; median duration in-situ 66.5 {range 47-146} months). The underlying pathology in 8 was cystic fibrosis. Antegrade transfemoral snare retrieval was successful in 6 of 7 attempts. In the remaining 3, a conservative approach was adopted following consultation with the family. None of the 4 with retained CVL developed complications (median follow-up 7.5 {range 1-53} months). The literature describes 38 pediatric index cases (including 10 from the current series). Seventeen (49%) were managed conservatively either intentionally or by default after failed endovascular removal attempt (n=4). No complications directly attributed to retained CVLs have been reported (median follow-up 40 {range 1-120} months). Reported morbidity associated with endovascular retrieval includes: procedural failure 30%, line embolization 8%, and intra-operative thrombo-embolism 8%. CONCLUSION: Literature regarding management of retained CVLs is anecdotal. Although uncommon, the complication should feature in consent for removal of CVLs. Conservative management carries long-term risks of infection, thrombosis, and even migration, albeit unquantified over a child's lifetime. Endovascular retrieval is feasible with appropriate expertise.

Urinary incontinence - an unusual indication for early vaginoplasty in late presenting congenital adrenal hyperplasia.

BACKGROUND: Congenital adrenal hyperplasia (CAH) is an autosomal recessive condition leading to deficient cortisol with an incidence of 1/16,000. Patients with CAH typically present early with ambiguous genitalia or as an emergency with adrenal crisis. CASE: We report an atypical late presentation of a 4-year-old girl with pubertal-like symptoms and urinary incontinence, due to a persistent urogenital sinus (UGS). An early vaginoplasty was successfully performed allowing the patient to achieve continence. CONCLUSION: Literature describing the symptoms of CAH with UGS is scarce. The case is unusual in demonstrating pubertal-like symptoms and urinary incontinence due to the late presentation of a persistent UGS, highlighting the need for an open mind in assessment of children with urinary incontinence. Timing of surgery is controversial, and cases need to be considered on an individual basis.

A complicated simple fall--an atypical case of serogroup Y meningococcal pneumonia with secondary septicaemia and literature review.

An elderly lady was admitted for pain management and rehabilitation following a fall. During her stay she developed a new heart murmur and sepsis. Subacute bacterial endocarditits was excluded, empirical ciprofloxacin was initiated and later converted to aztreonam with gentamicin for clinical deterioration. Subsequent investigations revealed meningococcal Y septicaemia secondary to pneumonia, with a possible oropharynx focus. Upon discharge she had returned to baseline state. The case reflects an unusual and increasing cause of pneumonia. A steady increase of infective serogroup Y isolates over the past 12-years in England, with tendency towards elderly makes it a significant differential among the general medical population. This trend corresponds with the US, but is yet unknown whether to be a periodic cycle or true change in dominance and, or, virulence among serogroups. If the latter were true, it would support the inclusion of serogroups beyond menigitides C in the vaccination program.