Diagnostic and management strategies for congenital H-type tracheoesophageal fistula: a systematic review.

BACKGROUND: H type tracheoesophageal fistula (H-TEF) is a rare congenital anomaly. Management may be complicated by late diagnosis and variation(s) in the therapeutic strategy. A systematic review of published studies explores the utility of diagnostic studies, operations and postoperative complications. METHODS: Medline and PubMed database(s) were searched for ALL studies reporting H-TEF during 1997-2020. Using PRISMA methodology, manuscripts were screened for eligibility and reporting. RESULTS: Forty-seven eligible studies were analysed. Primary diagnosis varied widely with surgeons performing oesophagography and trachea-bronchoscopy. Preoperative localisation techniques included fluoroscopy, guidewire placement and catheterisation. A cervical approach (209 of 272 cases), as well as thoracotomy, thoracoscopy and endoscopic fistula ligation, were all described. Morbidity included fistula recurrence (1.7%), leak (2%), tracheomalacia (3.4%) and respiratory sequelae (1%). The major adverse complication in all studies was vocal cord palsy secondary to laryngeal nerve injury (18.5%) yet strikingly few centres routinely reported undertaking vocal cord screening pre or postoperatively. CONCLUSION: This study shows that paediatric surgeons record low volume activity with H type tracheoesophageal fistula. Variation(s) in clinical practice are widely evident. Laryngeal nerve injury and its subsequent management warrant special consideration. Care pathways may offset attendant morbidity and define 'best practice.'

Necrotising enterocolitis-A 15-year outcome report from a UK specialist centre.

AIMS: Necrotising enterocolitis (NEC) is a disease associated with high mortality and morbidity, low birthweight and prematurity are risk factors. This study reports outcomes of babies having emergency laparotomy for NEC, examining institutional trends and exploring impact of multiple variables on mortality at 30 days and 1 year post-operatively. METHODS: Case records of babies with ICD coding for NEC were examined from 2000 to 2015. After exclusions, 243 cases were identified-confirmed by operative findings and histology. Cohort demographics and trends in mortality were investigated, and the relationship of common variables to mortality was modelled with univariate and multivariate logistic regression to generate a mortality prediction tool. RESULTS: Mean gestational age was 28 + 4 weeks. A 30-day mortality was 18.9%. Gestation, birthweight and area of bowel affected were significant of outcome (mortality), and the presence of pre-operative pneumoperitoneum was strongly correlated. Year of surgery and congenital cardiac pathology requiring intervention were not significant. Using multivariate regression modelling, a mortality outcome prediction tool has been developed. CONCLUSION: Good survival following operation for NEC (>70%) is feasible, even in those babies born extremely premature (<28 weeks) and post-operatively re-located to tertiary NICUs. With increasing gestational age (>32 weeks), mortality is uncommon.

CDK inhibitors reduce cell proliferation and reverse hypoxia-induced metastasis of neuroblastoma tumours in a chick embryo model.

Neuroblastoma is a paediatric cancer with a poor prognosis. This is in part due to widespread metastasis at time of presentation, which is refractory to current treatment modalities. New therapeutic agents that can control not only tumour growth but also metastasis are urgently needed. The differentiation therapy, retinoic acid, is currently used in clinic, leading to terminal differentiation of neuroblastoma cells thus reducing tumour growth in the primary tumour as well as at metastatic sites. However, retinoic acid only works in a subset of patients. We investigated the potential of CDK inhibitors, Palbociclib and RO-3306, on neuroblastoma cell differentiation, tumour progression and metastasis by utilising a 3R compliant cost effective preclinical chick embryo model. In both SK-N-AS and BE(2)C cell lines, when engrafted on the chorioallantoic membrane of chick embryos, we observed a reduction of tumour cell proliferation as well as a reduction in hypoxia preconditioning-driven metastasis by 60%. In addition, the expression of a panel of genes with known roles in metastasis, which increased upon hypoxia-preconditioning, was largely reduced by a CDK1 inhibitor. These results provide a promising alternative to currently existing therapies and might aid the development of new treatment protocols for retinoic acid-resistant patients.

Unilateral acute angle closure glaucoma.

A 52-year-old gentleman presented with 1-week history of severe right-sided headache associated with reduced vision in his right, amblyopic eye. Examination revealed raised intraocular pressure at 64 mm Hg. The anterior chamber (AC) was shallow and there was a dense cataract with no red reflex or fundal view. The contralateral eye had a deep anterior chamber with normal pressure and a clear lens. He was treated initially for acute angle closure glaucoma. The anterior chamber remained shallow and the intraocular pressure uncontrolled, despite maximum medical therapy. Owing to the absent fundal view and unilateral AC shallowing, further imaging was performed and a choroidal mass was found to be responsible for anterior displacement of the lens and shallowing of the angle. He went on to have an enucleation of the right eye, and histological examination confirmed the diagnosis of choroidal melanoma.