Outcomes of patients with exomphalos and associated congenital heart diseases.

INTRODUCTION: Exomphalos is an anterior abdominal wall defect resulting in herniation of contents into the umbilical cord. Severe associated chromosomal anomalies and congenital heart disease (CHD) are known to influence mortality, but it is not clear which cardiac anomalies have the greatest impact on survival. METHODS: We performed a retrospective review of the treatment and outcome of patients with exomphalos over a 30-year period (1990-2020), with a focus on those with the combination of exomphalos major and major CHD (EMCHD). RESULTS: There were 123 patients with exomphalos identified, 59 (48%) had exomphalos major (ExoMaj) (defect > 5 cm or containing liver), and 64 (52%) exomphalos minor (ExoMin). In the ExoMaj group; 17% had major CHD (10/59), M:F 28:31, 29% premature (< 37 weeks, 17/59) and 14% had low birth-weight (< 2.5 kg, 8/59). In the ExoMin group; 9% had major CHD (6/64), M:F 42:22, 18% premature and 10% had low birth-weight. The 5-year survival was 20% in the EMCHD group versus 90% in the ExoMaj with minor or no CHD [p < 0.0001]. Deaths in the EMCHD had mainly right heart anomalies and all of them required mechanical ventilation (MV) for pulmonary hypoplasia prior to cardiac intervention. In contrast, survivors did not require mechanical ventilation prior to cardiac intervention. CONCLUSION: EMCHD is associated with high mortality. The most significant finding was high mortality in those with right heart anomalies in combination with pulmonary hypoplasia, especially if pre-intervention mechanical ventilation is required.

Getting it right first time: implementation of laparoscopic pyloromyotomy without a learning curve.

INTRODUCTION: Laparoscopic pyloromyotomy is now an accepted procedure for the treatment of pyloric stenosis. However, it is clear that during the implementation period there are significantly higher incidences of mucosal perforation and incomplete pyloromyotomy. We describe how we introduced a new laparoscopic procedure without the complications associated with the learning curve. MATERIALS AND METHODS: Five consultants tasked one surgeon to pilot and establish laparoscopic pyloromyotomy before mentoring the others until they were performing the procedure independently; all agreed to use exactly the same instruments and operative technique. This involved a 5mm 30-degree infra-umbilical telescope with two 3mm instruments. Data were collected prospectively. RESULTS: Between 1 January 2013 and 31 December 2017, 140 laparoscopic pyloromyotomies were performed (median age 27 days, range 13-133 days, male to female ratio 121:19). Fifty-five per cent of procedures were performed by trainees. Complications were one mucosal perforation and one inadequate pyloromyotomy. There were no injuries to other organs, problems with wound dehiscence or other significant complications. The median time of discharge was one day (range one to six days). CONCLUSION: Our rate of perforation and incomplete pyloromyotomy was 1.4%, which is equivalent to the best published series of either open or laparoscopic pyloromyotomy. We believe that this resulted from the coordinated implementation of the procedure using a single technique to reduce clinical variability, increase mentoring and improve training. This approach appears self-evident but is rarely described in the literature of learning curves. In this age of increased accountability, new technologies should be incorporated into routine practice without an increase in morbidity to patients.

Congenital mesoblastic nephroma: a single-centre series.

BACKGROUND: Congenital mesoblastic nephroma is a rare disease. Treatment is surgical in the first instance. Chemotherapy has traditionally been thought not to have a role. Recent literature suggests a 50% mortality rate for recurrent/metastatic disease. MATERIALS AND METHODS: This study is a retrospective case review of prospectively collected data. Demographics, histopathology, treatment, outcomes and follow up were reviewed. RESULTS: Nine patients, 6 male and 3 female, were included. The median age at presentation was one month (range 0-7 months); follow-up was for a median of 21.5 months (range 16-79 months). Two patients had mixed and classical subtypes and the other five had the cellular subtype. Surgery was completed by an open procedure in eight patients and laparoscopically in one. There were three recurrences; two were local and one was pulmonary. Recurrences were treated with a combination of chemotherapy, radiotherapy and surgery. One patient with recurrent disease died from acute-on-chronic respiratory failure secondary to lung irradiation but was disease free. The other eight are disease free, alive and well with no sequelae at latest follow-up. CONCLUSIONS: Surgery remains the mainstay of management with chemo- and radiotherapy reserved for unresectable tumours or adjuvant management of recurrent disease. Specimen-positive margins are not an indication for instituting chemotherapy. The tyrosine kinase pathway seems to be a potential target for future chemotherapeutic agents although it is too early to assess how that will impact on the management of congenital mesoblastic nephroma.

Human factors in decision making in major trauma in Camp Bastion, Afghanistan.

INTRODUCTION: The concentration of major trauma experience at Camp Bastion has allowed continuous improvements to occur in the patient pathway from the point of wounding to surgical treatment. These changes have involved clinical management as well as alterations to the physical layout of the hospital, training and decision making. Consideration of the human factors has been a major part of these improvements. METHODS: We describe the Camp Bastion patient pathway with the communication template that focused decision making at various key moments during damage control resuscitation and damage control surgery (DCR-DCS). This system identifies four key stages: 'command huddle', 'snap brief', 'sit-reps' (situation reports) and 'sign-out/debrief'. The attitude of staff to communication and decision making is also evaluated. RESULTS: Twenty cases admitted to Camp Bastion with battlefield injuries were studied from 6 September to 6 October 2012. Qualitative responses from 115 members of staff were collected. All patients were haemodynamically shocked with a median pH of 7.25 (range: 6.83-7.40) and a median of 18 units of mixed red cells and plasma were transfused. In 89% of instances, theatre staff were aware of what was required of them at the beginning of the case, 86% felt there were regular updates and 93% understood what was required of them as the case progressed. CONCLUSIONS: The evolution of the hospital at Camp Bastion has been a unique learning experience in the field of major trauma. The Defence Medical Services have responded with continuous innovation to optimise DCR-DCS for seriously injured patients. Together with the improvements in clinical care, a communication and decision making matrix was developed. Staff evaluation showed a high degree of satisfaction with the quality of communication.

Performance improvement through best practice team management: human factors in complex trauma.

Human factors or non-technical skills are now commonplace in the medical literature, having taken the lead from the airline and nuclear industries and more recently Formula One motor racing. They have been suggested as playing a vital role in the success of the trauma teams in recent conflicts. This article outlines the background to human factors, referring to early papers and reports and also outlines high profile cases that highlight their importance. We then describe the importance of human factors in the deployed setting and some of the lessons that have been learnt from current conflicts.

Patency of neck veins following ultrasound-guided percutaneous Hickman line insertion.

PURPOSE: Venous occlusion following permanent central venous catheter (CVC) insertion by open cutdown or the landmark percutaneous technique has been reported between up to 25 %. However, there are no published data on the equivalent rate following ultrasound-guided percutaneous CVC insertion. The purpose of this study was to document the rate of venous occlusion associated with ultrasound-guided percutaneous CVC insertion in children. METHOD: From 1 April 2010 to 1 December 2011, all children having elective or emergency removal of a Hickman line by the vascular access team had a Doppler ultrasound of their neck veins. Only Hickman lines inserted by the ultrasound-guided percutaneous route were included. Internal jugular, innominate and subclavian veins were scanned and recorded as patent, reduced or absent. RESULTS: We identified 100 consecutive children. Median age was 6 years (range 21 days to 16 years). Indication for insertion was chemotherapy (60), parenteral nutrition (15), blood products (12), renal replacement (3) and other indications (10). Three children had absent flow at the time of line removal (median age 4 months, range 3-6 months), with 2 out of 3 requiring removal for infection. The venous occlusion rate following ultrasound-guided insertion of CVC is 3 % in our study. CONCLUSIONS: We conclude that (1) complete venous occlusion is associated with younger age and CVC infection. (2) In our study, the venous occlusion rate of 3 % is significantly lower than the published series of either open cutdown or the landmark technique.

Management of children in the deployed intensive care unit at Camp Bastion, Afghanistan.

BACKGROUND: The deployed Intensive Therapy Unit (ITU) in the British military field hospital in Camp Bastion, Afghanistan, admits both adults and children. The purpose of this paper is to review the paediatric workload in the deployed ITU and to describe how the unit copes with the challenge of looking after critically injured and ill children. METHODS: Retrospective review of patients <16 years of age admitted to the ITU in the British military field hospital in Camp Bastion, Afghanistan, over a 1-year period from April 2011 to April 2012. RESULTS: 112/811 (14%) admissions to the ITU were paediatric (median age 8 years, IQR 6-12, range 1-16). 80/112 were trauma admissions, 13 were burns, four were non-trauma admissions and 15 were readmissions. Mechanism of injury in trauma was blunt in 12, blast (improvised explosive device) in 45, blast (indirect fire) in seven and gunshot wound in 16. Median length of stay was 0.92 days (IQR 0.45-2.65). 82/112 admissions (73%) were mechanically ventilated, 16/112 (14%) required inotropic support. 12/112 (11%) died before unit discharge. Trauma scoring was available in 65 of the 80 trauma admissions. Eight had Injury Severity Score or New Injury Severity Score >60, none of whom survived. However, of the 16 patients with predicted mortality >50% by Trauma Injury Severity Score, seven survived. Seven cases required specialist advice and were discussed with the Birmingham Children's Hospital paediatric intensive care retrieval service. The mechanisms by which the Defence Medical Services support children admitted to the deployed adult ITU are described, including staff training in clinical, ethical and child protection issues, equipment, guidelines and clinical governance and rapid access to specialist advice in the UK. CONCLUSIONS: With appropriate support, it is possible to provide intensive care to children in a deployed military ITU.

The full spectrum of handlebar injuries in children: a decade of experience.

BACKGROUND: Traumatic paediatric handlebar injury (HBI) is known to occur with different vehicles, affect different body regions, and have substantial associated morbidity. However, previous handlebar injury research has focused on the specific combination of abdominal injury and bicycle riding. Our aim was to fully describe the epidemiology and resultant spectrum of injuries caused by a HBI. METHODS: Retrospective data analysis of all paediatric patients (<18 years) in a prospectively identified trauma registry over a 10-year period. Primary outcome was the HBI, its location and management. The effects of patient age, vehicle type, the impact region, and Injury Severity Score (ISS) were also evaluated. HBI patients were compared against a cohort injured while riding similar vehicles, but not having sustained a HBI. RESULTS: 1990 patients were admitted with a handlebar-equipped vehicle trauma; 236 (11.9%) having sustained a HBI. HBI patients were twice as likely to be aged between 6 and 14 years old compared with non-HBI patients (OR 2.2; 95% CI 1.5-3.2). 88.6% of the HBI patients sustained an isolated injury, and 45.3% had non-abdominal handlebar impact. There were no significant differences in median ISS (p=0.4) or need for operative intervention (OR 1.1; 95% CI 0.9-1.5) between HBI and non-HBI patients. HBI patients had a significantly longer LOS (1.8 days vs. 1.2 days; p=0.001), and more frequently required a major operation (OR 3.4; 95% CI 2.2-5.4). The majority of splenic, renal and hepatic injuries were managed conservatively. CONCLUSIONS: Although the majority of paediatric HBI is associated with both intra-abdominal injury and bicycle riding, it produces a spectrum of potentially serious injuries and patients are more likely to undergo major surgery. Therefore these patients should always be treated with a high degree of suspicion.

Optimising communication in the damage control resuscitation -- Damage Control Surgery sequence in major trauma management.

Damage Control Resuscitation and Damage Control Surgery (DCR-DCS) is an approach to managing severely injured patients according to their physiological needs, in order to optimise outcome. Key to delivering DCR-DCS is effective communication between members of the clinical team and in particular between the surgeon and anaesthetist, in order to sequence and prioritise interventions. Although the requirement for effective communication is self-evident, the principles to achieving this can be forgotten and sub-optimal when unexpected problems arise at critical points during management of challenging cases. A system is described which builds on the 'World Health Organisation (WHO) safer surgery checklist' and formalises certain stages of communication in order to assure the effective passage of key points. We have identified 3 distinct phases: (i) The Command Huddle, once the patient has been assessed in the Emergency room; (ii) The Snap Brief, once the patient has arrived in the Operating Room but before the start of surgery; and (iii) The Sit-Reps, every 10 minutes for the entire theatre team to maintain situational awareness and allow effective anticipation and planning.

Paediatric admissions to the British military hospital at Camp Bastion, Afghanistan.

INTRODUCTION: International humanitarian law requires emergency medical support for both military personnel and civilians, including children. Here we present a detailed review of paediatric admissions with the pattern of injury and the resources they consume. METHODS: All paediatric admissions to the hospital at Camp Bastion between 1 January and 29 April 2011 were analysed prospectively. Data collected included time and date of admission, patient age and weight, mechanism of injury, extent of wounding, treatment, length of hospital stay and discharge destination. RESULTS: Eighty-five children (65 boys and 17 girls, median age: 8 years, median weight: 20 kg) were admitted. In 63% of cases the indication for admission was battle related trauma and in 31% non-battle trauma. Of the blast injuries, 51% were due to improvised explosive devices. Non-battle emergencies were mainly due to domestic burns (46%) and road traffic accidents (29%). The most affected anatomical area was the extremities (44% of injuries). Over 30% of patients had critical injuries. Operative intervention was required in 74% of cases. The median time to theatre for all patients was 52 minutes; 3 patients with critical injuries went straight to theatre in a median of 7 minutes. A blood transfusion was required in 27 patients; 6 patients needed a massive transfusion. Computed tomography was performed on 62% of all trauma admissions and 40% of patients went to the intensive care unit. The mean length of stay was 2 days (range: 1-26 days) and there were 7 deaths. CONCLUSIONS: Paediatric admissions make up a small but significant part of admissions to the hospital at Camp Bastion. The proportion of serious injuries is very high in comparison with admissions to a UK paediatric emergency department. The concentration of major injuries means that lessons learnt in terms of teamwork, the speed of transfer to theatre and massive transfusion protocols could be applied to UK paediatric practice.

The use of Celox gauze as an adjunct to pelvic Packing in otherwise uncontrollable pelvic haemorrhage secondary to penetrating trauma.

Haemorrhage from severe pelvic fractures can be associated with significant mortality. Modern civilian trauma centres may manage these injuries with a combination of external pelvic fixation, extra-peritoneal packing and/or selective angiography; however, military patterns of wounding are different and deployed medical facilities may be resource constrained. We report two successful instances of pelvic packing using chitosan impregnated gauze (Celox) when conventional surgical attempts at vascular control had failed. We conclude that pelvic packing should be considered early in patients with military pelvic trauma and major haemorrhage, as part of damage control surgery and that Celox gauze may be a useful adjunct. In our cases, the Celox gauze was easily removed after 24-48 hours without significant bowel adhesions and did not leave a residual phelgmon (of exudate or gel) that may predispose to infection.

Ultrasound-guided percutaneous insertion of 2.7 Fr tunnelled Broviac lines in neonates and small infants.

BACKGROUND/PURPOSE: Insertion of permanent central venous access (Broviac line) can be a challenge in neonates especially when sites for peripherally inserted central catheters (PICC) have been exhausted. The landmark technique (LT) has been well described for the percutaneous insertion of central lines in neonates but can be associated with significant complications including death. The use of the ultrasound-guided approach for temporary central line access has been reported but as yet there are no reports of the adaptation of the technique for Broviac line insertion in neonates. METHOD: A prospective database records all procedures carried out by the vascular access team and any complications which occur; this database was reviewed from November 2004 to January 2008. RESULTS: A consecutive series of 34 neonates underwent insertion of 36 Broviac lines using the ultrasound-guided percutaneous technique with a 2.7 Fr silastic line and a 3 Fr peel-apart sheath. Median gestational age was 34 weeks (range 24-40), chronological age was 102 days (14-209 days), weight 2.9 kg (0.63-4.1). Successful cannulation occurred in 100% of patients. There were no cases of arterial puncture or perioperative complications due to surgery. CONCLUSION: The ultrasound-guided percutaneous approach for insertion of tunnelled permanent vascular access is safe in neonates with no surgical complications in our series. However, it is a technically demanding procedure to do in neonates and should not be attempted without significant prior experience.

Oesophageal replacement in children.

INTRODUCTION: The usual indications for oesophageal replacement in childhood are intractable corrosive strictures and long-gap oesophageal atresia. Generally, paediatric surgeons attempt to preserve the native oesophagus with repeat dilatations. However, when this is not successful, an appropriate conduit must be fashioned to replace the oesophagus. The neo-oesophagus should allow normal oral feeding, not have gastro-oesophageal reflux, and be able to function well for the life-time of the patient. PATIENTS AND METHODS: A Medline search for oesophageal replacement, oesophageal atresia, gastric transposition, colon transposition, gastric tube, caustic stricture was conducted. The commonest conduits including whole stomach, gastric tube, colon and jejunum are all discussed. RESULTS: No randomised controlled studies exist comparing the different types of conduits available for children. The techniques used tend to be based on personal preference and local experience rather than on any discernible objective data. The biggest series with long-term outcome are reported for gastric transposition and colon replacement. Comparison of a number of studies shows no significant difference in early or late complications. Early operative complications include graft necrosis, anastomotic leaks and sepsis. Late problems include strictures, poor feeding, gastro-oesophageal reflux, tortuosity of the graft and the development of Barrett's oesophagus. The biggest series, however, seem to have lower complications than small series probably reflecting the experience, built up over years, in their respective centres. CONCLUSIONS: Long-term follow-up is recommended because of the risks of late strictures, excessive tortuosity of the neo-oesophagus and the development of Barrett's oesophagus.

Mydriasis in association with MMIHS in a female infant: evidence for involvement of the neuronal nicotinic acetylcholine receptor.

We report a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), occurring in association with mydriasis, in a female infant born to consanguineous Asian parents. This association has not previously been reported and is of interest because mydriasis has been found in a murine MMIHS model produced by knockout of the genes coding for the alpha3 subunit or the beta2 and beta4 subunits of the neuronal nicotinic acetylcholine receptor. This may provide an important clue to the genetic basis of MMIHS in humans.

Intestinal complications associated with twin-twin transfusion syndrome after antenatal laser treatment: Report of two cases.

Two infants found to have ileal atresia after birth and who had intrauterine laser treatment to interupt twin to twin transfusion are presented. The donor twin in each pregnancy died in utero.

Ecthyma gangraenosum--a trap for the unwary.

Neutropaenic patients are at particular risk of developing a pseudomonal fasciitis known as ecthyma gangraenosum. Despite the similarities with necrotising fasciitis, Fournier's gangrene has a very different aetiology and management.

Mucin gene expression in Barrett's oesophagus: an in situ hybridisation and immunohistochemical study.

BACKGROUND AND AIMS: Mucin genes are expressed in a site specific manner throughout the gastrointestinal tract. Little is known about the expression pattern in the oesophagus. In this study we have investigated MUC gene expression in both the normal oesophagus and specialised intestinal metaplasia (Barrett's oesophagus). PATIENTS: Archived paraffin embedded material from eight specimens of normal oesophagus, 18 Barrett's oesophagus, eight gastric metaplasia, six high grade dysplasia, and six cases of adenocarcinoma were examined for expression of the mucin genes MUC1-6. METHODS: Mucin mRNA was detected by in situ hybridisation using [(35)S] dATP labelled oligonucleotide probes. Mucin core protein was detected by immunohistochemistry. RESULTS: Normal oesophagus expressed MUC5B in the submucosal glands and MUC1 and MUC4 in the stratified squamous epithelium. Barrett's oesophagus strongly expressed MUC5AC and MUC3 in the superficial columnar epithelium, MUC2 in the goblet cells, and MUC6 in the glands. In high grade dysplasia and adenocarcinoma there was downregulation of MUC2, MUC3, MUC5AC, and MUC6, but upregulation of MUC1 and MUC4 in half of the specimens examined. CONCLUSIONS: Normal oesophagus and Barrett's oesophagus have a novel pattern of mucin gene expression. Barrett's oesophagus expressed the mucins associated with normal gastric epithelium and normal intestinal epithelium. While most mucin genes were downregulated in severely dysplastic and neoplastic tissues, there was upregulation of the membrane bound mucins MUC1 and MUC4. This may prove useful in detecting early signs of progression to adenocarcinoma of the oesophagus.