Case of split notochord syndrome: a neonate with thoracic neuroenteric cyst, abdominal duodenal duplication cyst, malrotation and vertebral anomalies.

The authors describe a case of a male neonate with split notochord syndrome presenting with cervico-thoracic deformity, thoracic neuroenteric cyst, separate abdominal duodenal duplication cyst and concurrent intestinal malrotation. This combination of abnormalities is very rare. When these lesions are suspected, patients must be investigated carefully.This case is presented not only to recount an infrequent combination of structural abnormalities but also to raise awareness of the signs that should point to clinical suspicion and prompt diagnosis.Following surgical excision of the thoracic neuroenteric cyst, the patient has made a good recovery.

Comprehensive strategies to minimize radiation exposure during Interventional electrophysiology procedures: state-of-the-art review.

INTRODUCTION: Cardiac electrophysiology (EP) procedures are frequently performed in patients with cardiac arrhythmias, chronic heart failure, and sudden cardiac death. Most EP procedures involve fluoroscopy, which results in radiation exposure to physicians, patients, and EP lab staff. Accumulated radiation exposure is a known health detriment to patients and physicians. AREA COVERED: This review will summarize radiation exposure, dose metrics, complications of radiation exposure, factors affecting radiation exposure, minimizing radiation exposure, zero or near-zero fluoroscopy strategies, and up-to-date research in the area of reducing radiation exposure and best practices. EXPERT COMMENTARY: Comprehensive strategies should be implemented in EP laboratories to minimize radiation exposure with standard fluoroscopy. There are routine techniques that can mitigate significant amounts of radiation exposure using standard equipment within the EP lab. The operators need to emphasize that EP practices routinely incorporate non-ionizing radiation sources for cardiac imaging (e.g. magnetic resonance imaging, advanced electroanatomical mapping systems, intracardiac ultrasonography) in addition to other novel technologies to mitigate radiation exposure to patients and physicians.

Synthetic Anion Transporters as Endoplasmic Reticulum (ER) Stress Inducers.

Cl--ion transporters (2a-2h) were synthesized based on the binding motifs of prodigiosin. Transporter 2e clearly displays Cl--ion transportation activity across both model and live cell membranes. Furthermore, 2e can disrupt Ca2+ homeostasis and increase the intracellular concentration of Ca2+ in the DLD-1 cell. This disruption can lead to Caspase-dependent apoptosis supported by CHOP expression (a marker of ER stress) and the appearance of the cleaved forms of Caspase 3 and PARP.

The Cambridge experience with buried bumpers.

AIM: Buried Bumper (BB) is a complication of percutaneous endoscopic gastrostomy (PEG) that leads to tube dysfunction and major morbidity. Although many techniques have been described to manage BB, none are universally adopted, and laparotomy remains the mainstay. We introduce a novel endoscopic technique in paediatric surgery that avoids laparotomy. METHODS: A retrospective review of medical records of patients who presented with BB to Cambridge University Hospital, UK, between January 2012 and June 2018 was done. Data collected included: demographics, tube size and type, interval between insertion and diagnosis of BB, hospital stay, technique used, and postoperative complications. The technique involved using an endoscopic snare passed from inside the stomach lumen through the PEG lumen to the outside, guided if required by a stiff nylon thread if no part of the PEG was visible, grasping the PEG tube externally after cutting it short, followed by a retrograde pull to remove the buried tube via the mouth. MAIN RESULTS: Fifteen BBs were found in ten patients. Median patient age was 5.25 years (1.2-16.6). Median time between gastrostomy insertion and diagnosis of BB was 9 months (1-32). Twelve BBs were removed endoscopically with no postoperative complications. Patients had a replacement inserted through the original track and were discharged within 24 h. Two underwent laparotomies performed by surgeons unfamiliar with endoscopic technique, and one was converted to laparotomy owing to inability to transverse an encrusted and closed PEG tube lumen. CONCLUSION: Endoscopic retrograde BB removal is a safe, easy, and quick technique with minimal complications. We strongly advocate widespread adoption of the technique before considering a laparotomy. LEVEL OF EVIDENCE: Treatment study: Level IV.

Factors affecting prognosis in locoregional recurrence of oral squamous cell carcinoma.

BACKGROUND: Recurrence of oral squamous cell carcinoma (rOSCC) after primary treatment is associated with poor survival outcomes. Salvage treatment with further surgery, radiotherapy and chemotherapy has high morbidity, making patient selection crucial. However, in the recurrence setting, reliable stratification data are scarce. Decision-making in this context is consequently complex. We investigated factors influencing overall survival after rOSCC. METHODS: Retrospective cohort study of patients with rOSCC (n = 83) at the Queen Elizabeth Hospital Birmingham, UK between 2006 and 2016. Associations with overall survival were analysed using univariate and multivariate analyses to identify important clinical prognostic indicators. RESULTS: Overall survival at 1 year was 32.5% and at 5 years was 18.1% after a median follow-up of 7.4 months. Multivariate analysis identified four independent predictors of overall survival following rOSCC: size of primary tumour (HR 2.077; 95% CI 1.034-4.172), extent of recurrent disease (HR 3.286; 95% CI 1.545-6.991), history of moderate alcohol consumption (HR 0.351; 95% CI 0.162-0.763), and close or positive margins at primary resection (HR 1.955; 95% CI 1.063-3.595). CONCLUSIONS: We identified four key factors that help prognostication and risk stratification of rOSCC. Given the high morbidity associated with salvage treatment, we recommend that the multidisciplinary team (MDT) and the patient weigh these factors carefully when considering further treatment. Further investigation of the biology underlying these oncophenotypes may contribute to better patient stratification.

Features and prognostic utility of biopsy in oral squamous cell carcinoma.

BACKGROUND: Traditional approaches in oral squamous cell carcinoma (OSCC) management utilize biopsy tissue for diagnostic purposes only. Adverse prognostic features, such as the tumor depth, are usually determined from final resection specimens, but are seldom studied in biopsy tissue. METHODS: A preliminary study of 139 consecutive biopsies compared biopsy size with T classification, tumor site, and operator grade, and biopsy tumor depth with the true tumor depth. RESULTS: This study demonstrated that biopsy size is independent of T classification (p = .44), subsite (p = .86), and operator grade (p = .10). The biopsy tumor depth significantly underrepresented true tumor depth (2.5 mm, 95% confidence interval [CI] = 2.4-2.9 vs 8.2 mm, 95% CI = 6.5-9.9; p < .001), confirming the limited prognostic utility of biopsies in OSCC. CONCLUSION: A future clinical trial will compare the routine biopsy technique with standardized deeper biopsy techniques using punch biopsy to sample invasive fronts and investigate opportunities for up-front staging using a combination of histological features and epithelial and stromal molecular biomarkers in OSCC. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1857-E1862, 2016.

Congenital lung lesions: preoperative three-dimensional reconstructed CT scan as the definitive investigation and surgical management.

AIM OF STUDY: The aim of this study was to review our experience of postnatal investigations and management of congenital lung lesions. METHODS: All children with antenatal diagnosis undergoing surgical management were identified from hospital records. Antenatal diagnosis and serial antenatal ultrasound findings were noted, postnatal chest X-ray (CXR) and computed tomographic (CT) scan were reviewed. Pearson correlation coefficient (r) was used to look into relation between CT scan and per-operative findings. Surgical management and outcome of these lesions were assessed. RESULTS: A total of 38 children were identified between January 2000 and December 2011; 22 were males and 16 were females. The mean gestational age at diagnosis was 21 weeks (range 18 to 26 weeks). Five children showed complete resolution antenatally. Four children were symptomatic at birth. Postnatal CXR showed an abnormality in only 17 infants. CT scan with three-dimensional (3D) reconstructions was performed at the mean age of 7.7 months (range 1 day to 42 months). CT scan correlated well with per-operative findings and provided adequate anatomical information r = 0.98. Open thoracotomy and lobectomy/excision was performed in 23, and 15 had thoracoscopic lobectomy/excision. The mean age of operation was 18 months (range 2 days to 96 months). Twenty patients had signs of recurrent preoperative infection with pleural adhesions and hilar thickening resulting in conversion of 10 thoracoscopic cases to open surgery. Histology confirmed 26 congenital cystic adenomatoid malformations, 2 hybrid lesions, 7 sequestrations, and 3 bronchopulmonary malformations. CONCLUSIONS: Antenatal resolution and normal postnatal CXR are not reliable indicators of resolution of the lesion. Early postnatal CT scan preferably with 3D reconstruction and early surgical treatment are suggested, as delaying the operation may result in repeated infection making thoracoscopic approach more difficult.

Identification of novel genes in Hirschsprung disease pathway using whole genome expression study.

AIM: This study aims to identify new genes not described previously that may be relevant in the etiology or pathophysiology of patients with Hirschsprung disease (HD). This was done by identifying differences in gene expression between normal and abnormal segments of bowel in HD patients compared with controls. METHODS: Full-thickness colonic tissue samples were taken from HD patients, both from the diseased (Ds) and normal segment of the colon (Nr), and from controls (Ct). Samples were further dissected into mucosa (MUC) and muscle (MUS). RNA was extracted and analyzed on Affymetrix Gene Chip Human Gene 1.0 ST arrays. Statistical analyses using ANOVA with a fold change cut off of 2 was applied to detect a number of differentially expressed genes. Selected genes were revalidated by quantitative real-time reverse transcriptase polymerase chain reaction. RESULTS: Thirty-four samples (18 MUS and 16 MUC) were analyzed. MUC (1.64 ± 0.46 µg/mg) and MUS (0.83 ± 0.48 µg/mg) showed good RNA extraction yield and quality. Of the 24,987 filtered on expression genes, MUS showed 220 genes with expression difference of 2-fold, out of which 120 genes were significant with P ≤ .05. Similarly, MUC demonstrated 206 genes with 2-fold changes and 9 had P ≤ .05. Some genes showing differential expression between groups and therefore subject to further analysis were RELN, GAL, GAP43, NRSN1, and GABRG2. CONCLUSION: Analyzed data showed significant differences in expression of above sets of genes with up- and down-regulation, which has not been described before in HD and could have a role in pathogenesis of this condition.

Early experience of pediatric thoracoscopic lobectomy in the UK.

PURPOSE: The aim of this study was to report on the early experience of pediatric thoracoscopic lobectomy in two UK centers (Royal Hospital for Sick Children, Edinburgh, and Addenbrookes Hospital, Cambridge). METHODS: Twelve patients between February 2000 and November 2005 were treated with a lobectomy for pulmonary disease. RESULTS: Diagnoses included 7 congenital cystic adenomatous malformations, 4 patients with bronchiectasis, and 1 thoracic mature teratoma. The patients' ages ranged from 8 months to 15 years. In all patients, a thoracoscopic lobectomy was attempted. In all cases, the lobectomy was completed; however, in 6 patients, the conversion to either video-assisted thoracoscopic surgery (VATS) or open thoracotomy was required. Of note, 9 of the 12 patients had had previous lung infections prior to lobectomy. Five of 6 that required a conversion to VATS or open thoracotomy had had significant previous pulmonary infection, causing hilar lymphadenopathy and adhesions that complicated the dissection. The other case requiring a conversion to thoracotomy had abnormal hilar anatomy with an incomplete oblique fissure. CONCLUSIONS: Patients with a previous history of pulmonary infection can cause difficulty in dissection of the hilum that can necessitate a conversion to VATS or open thoracotomy. An infection prior to lobectomy can cause difficulty in completing the procedure safely thoracoscopically. Consideration of patients with pulmonary disease for lobectomy should be made prior to the onset of infectious complications. The thoracoscopic lobectomy can still be performed in patients with a preceding history of infectious complications, though a higher rate of conversion is likely.