Michael Stanton

Conservative management of antenatally diagnosed cystic lung malformations

Aim To review the outcome of all antenatally diagnosed conservatively managed congenital lung malformations (CLMs) managed at our centre. Methods All patients diagnosed antenatally with cystic lung malformations from 2001 to 2011, at a tertiary referral paediatric surgical centre practising a policy of conservative management of asymptomatic cases, were retrospectively reviewed. Data were collected from medical case notes and radiology reports. Ethical approval was obtained from our institutional research and development department. Results The complete records of 74 fetuses antenatally diagnosed with CLM were reviewed. There were 72 live births, at a median gestation of 39.6u2005weeks. Emergency lobectomy was performed in one symptomatic neonate. Elective lobectomies were performed at parental request in three asymptomatic infants, one of whom had a family history of synovial sarcoma. Two patients developed pneumonia in the affected lobe during early childhood and proceeded to lobectomy at the age of 3u2005years. One patient with a bronchopulmonary sequestration required embolisation for cyanotic episodes. The remaining 65 patients have been conservatively managed to date, and none have required hospital admission. Less than a quarter report mild respiratory symptoms such as cough or wheeze. Median follow-up is 5u2005years. Conclusions This retrospective cohort study of 74 consecutive CLMs diagnosed antenatally over a 10-year period demonstrates that most of these lesions will remain asymptomatic throughout childhood. Although the natural history of CLMs in later years remains to be elucidated, we hope that this report on medium-term outcomes will be useful to clinicians who undertake antenatal counselling and may inform the discussion on how best to manage these children.

Is interval appendicectomy justified after successful nonoperative treatment of an appendix mass in children? A systematic review

BACKGROUND/PURPOSEnInterval appendicectomy (IA) is commonly performed after successful nonoperative treatment of appendix mass (AM); although, this approach has recently been challenged. We systematically reviewed the pediatric literature with regard to the justification for this practice.nnnMETHODSnUsing a defined search strategy, studies were identified and data were extracted independently by 2 reviewers. Incidences of recurrent appendicitis, complications after IA, and carcinoid tumor were estimated accounting for interstudy heterogeneity. Cost and length of stay of IA were analyzed.nnnRESULTSnThree studies (127 cases) reporting routine nonsurgical treatment were identified; all were retrospective. There was marked interstudy heterogeneity and variable follow-up. After successful nonoperative treatment of AM, the risk of recurrent appendicitis is 20.5% (95% confidence interval [CI], 14.3%-28.4%). The incidence of complications after IA (23 studies, n = 1247) is 3.4% (95% CI, 2.2-5.1), and the incidence of carcinoid tumor found at IA (15 studies, n = 955) is 0.9% (95% CI, 0.5-1.8). No reports compared costs. Mean length of stay for IA was 3 days (range, 1-30 days).nnnCONCLUSIONSnProspective studies comparing routine IA with nonoperative treatment without IA in children are lacking. Available data suggest that 80% of children with AM may not need IA. A prospective study to evaluate the natural history of this condition compared with the morbidity and costs of IA is warranted.

Malrotation and intestinal atresias.

This review encompasses four congenital conditions which present with symptoms of bowel obstruction in the neonatal period. The antenatal and postnatal features of malrotation, jejuno-ileal atresia, duodenal atresia and colonic atresia are discussed. Each condition is outlined including the classification, epidemiology, aetiology and presentation, and a summary of the surgical management is described.

Anatomy and physiology of the peritoneum

The peritoneum is commonly encountered in abdominal surgery. The development and rotation of the primitive gut tube lead to the normal adult arrangement of the peritoneal cavity, which forms bloodless planes allowing the retroperitoneal portions of the bowel to be safely mobilised. The arrangement of the peritoneum also forms spaces in which infected fluid or pus can collect. The microcirculation of peritoneal fluid is now well understood, and the large absorptive surface of the peritoneum can be exploited in peritoneal dialysis. The absorption of gas by the peritoneum following abdominal surgery is faster in neonates than in older children, and understanding this process contributes to the interpretation of post-operative radiographs.

The argument for a non-operative approach to asymptomatic lung lesions

The controversy surrounding the management of congenital lung malformations (CLMs) centre on how best to manage the increasing population of asymptomatic antenatally detected infants. Should elective surgery be offered? Or is a watch-and-wait policy safe? This will be addressed in this review by examining the reported complications of surgery, the risk of symptom development if lesions are left in situ and whether this may alter surgical outcomes, and importantly whether there is any long-term risk of malignancy that can be negated by surgical resection in infancy.

Does thoracoscopy have advantages over open surgery for asymptomatic congenital lung malformations? An analysis of 1626 resections

AIMnThe apparent incidence of antenatally diagnosed congenital lung malformations (CLM) is rising (1 in 3000), and the majority undergo elective resection even if asymptomatic. Thoracoscopy has been popularized, but early series report high conversion rates and significant complications. We aimed to perform systematic review/meta-analysis of outcomes of thoracoscopic vs open excision of asymptomatic CLMs.nnnMETHODSnA systematic review according to PRISMA guidelines was performed. Data were extracted for all relevant studies (2004-2015) and Rangel quality scores calculated. Analysis was on intention to treat basis for thoracoscopy and asymptomatic lung lesions. Meta-analysis was performed using the addon package METAN of the statistical package STATA14™; p<0.05 was considered significant.nnnRESULTSn36 studies were eligible, describing 1626 CLM resections (904 thoracoscopic, 722 open). There were no randomized controlled trials. Median quality score was 14/45 (IQR 6.5) poor. 92/904 (10%) thoracoscopic procedures were converted to open. No deaths were reported. Meta-analysis showed that regarding thoracoscopic procedures, the total number of complications was significantly less (OR 0.63, 95% CI 0.43, 0.92; p<0.02, 12 eligible series, 912 patients, 404 thoracoscopic). Length of stay was 1.4days shorter (95%CI 2.40, 0.37;p<0.01). Length of operation was 37 min longer (95% CI 18.96, 54.99; p<0.01). Age, weight, and number of chest tube days were similar. There was heterogeneity (I2 30%, p=0.15) and no publication bias seen.nnnCONCLUSIONSnA reduced total complication rate favors thoracoscopic excision over thoracotomy for asymptomatic antenatally diagnosed CLMs. Although operative time was longer, and open conversion may be anticipated in 1/10, the overall length of hospital stay was reduced by more than 1day.nnnLEVEL OF EVIDENCEn4 (based on lowest level of article analyzed in meta-analysis/systematic review).

Barotrauma-associated posterior tension pneumomediastinum, a rare cause of cardiac tamponade in a ventilated neonate: a case report and review of the literature

Ventilation‐associated neonatal barotrauma comprises a spectrum of conditions including pneumothorax, pulmonary interstitial emphysema, pneumopericardium and pneumomediastinum. Whilst pneumothorax is common, mediastinal and particularly posterior mediastinal air collections are rare. We report the case of a neonate, presenting with life threatening pericardial tamponade secondary to posterior tension pneumomediastinum. The infant was successfully resuscitated with an emergency left lateral thoracotomy and chest drain insertion. We believe this to be the first such case reported in the literature. We discuss aspects of pathophysiology, diagnosis and management relating to posterior pneumomediastinum.

Total Colonic Aganglionosis: A 15-Year Single Center Experience

PURPOSEnTotal colonic aganglionosis (TCA) is a rare condition, which is challenging to manage. Outcome data are sparse. We aimed to review the demographics, treatment, and outcomes of TCA in our center.nnnPATIENTS AND METHODSnA retrospective case note review of 15 years from a single center was undertaken.nnnRESULTSnA total of nine patients (five male) were managed. Gestational age at birth was 39 weeks (range, 32.5-41 weeks). All patients were referred with distal intestinal obstruction at a median of day 2 (range, 1-6 days) of life. Two patients were managed with a long-term stoma. One died with persistent functional obstruction (despite a ganglionic stoma). Of the nine patients, seven patients underwent staged pull-through: three Soave, three Duhamel, and one Martin procedure with no short-term complications. All patients had at least one readmission with enterocolitis, diarrhea, or high stoma output. Further procedures were required in four of the seven patients. Only one child (older than 3 years) has achieved continence. Two children (both aged 8 years) requested reformation of a stoma to manage incontinence.nnnCONCLUSIONnIn this series, we observed high morbidity and poor functional outcome, which should be anticipated in TCA. Patients with TCA have a high probability of requiring a long-term stoma and this should be considered as a management option.

CONTRACT Study - CONservative TReatment of Appendicitis in Children (feasibility): study protocol for a randomised controlled Trial

BackgroundCurrently, the routine treatment for acute appendicitis in the United Kingdom is an appendicectomy. However, there is increasing scientific interest and research into non-operative treatment of appendicitis in adults and children. While a number of studies have investigated non-operative treatment of appendicitis in adults, this research cannot be applied to the paediatric population. Ultimately, we aim to perform a UK-based multicentre randomised controlled trial (RCT) to test the clinical and cost effectiveness of non-operative treatment of acute uncomplicated appendicitis in children, as compared with appendicectomy. First, we will undertake a feasibility study to assess the feasibility of performing such a trial.Methods/designThe study involves a feasibility RCT with a nested qualitative research to optimise recruitment as well as a health economic substudy. Children (aged 4–15 years inclusive) diagnosed with acute uncomplicated appendicitis that would normally be treated with an appendicectomy are eligible for the RCT. Exclusion criteria include clinical/radiological suspicion of perforated appendicitis, appendix mass or previous non-operative treatment of appendicitis. Participants will be randomised into one of two arms. Participants in the intervention arm are treated with antibiotics and regular clinical assessment to ensure clinical improvement. Participants in the control arm will receive appendicectomy. Randomisation will be minimised by age, sex, duration of symptoms and centre. Children and families who are approached for the RCT will be invited to participate in the embedded qualitative substudy, which includes recording of recruitment consultants and subsequent interviews with participants and non-participants and their families and recruiters. Analyses of these will inform interventions to optimise recruitment. The main study outcomes include recruitment rate (primary outcome), identification of strategies to optimise recruitment, performance of trial treatment pathways, clinical outcomes and safety of non-operative treatment. We have involved children, young people and parents in study design and delivery.DiscussionIn this study we will explore the feasibility of performing a full efficacy RCT comparing non-operative treatment with appendicectomy in children with acute uncomplicated appendicitis. Factors determining success of the present study include recruitment rate, safety of non-operative treatment and adequate interest in the future RCT. Ultimately this feasibility study will form the foundation of the main RCT and reinforce its design.Trial registrationISRCTN15830435. Registered on 8 February 2017.

Screening for complete androgen insensitivity syndrome in girls with inguinal hernia: parental insight

Complete androgen insensitivity syndrome (CAIS) is an X-linked recessive disorder resulting from an androgen receptor mutation. Affected individuals have 46,XY karyotype but female phenotype are raised as girls.1 A short vagina and absent uterus result in infertility, likely psychosexual problems and a risk of later gonadal malignancy such that gonadectomy is recommended.2 Presentation may be in adolescence with primary amenorrhoea, but 1% of premenstrual phenotypic girls with inguinal hernia (IH) will have CAIS.3 Despite current recommendations4 ,5 that all premenstrual girls with IH should be screened for CAIS, a survey6 of 32 UK paediatric surgeons found that 63% do not mention CAIS to their parents at any stage and 43% made no …