Magnetic resonance quantification of skeletal muscle lipid infiltration in a humanized mouse model of Duchenne muscular dystrophy.

Rodent models of Duchenne muscular dystrophy (DMD) often do not recapitulate the severity of muscle wasting and resultant fibro-fatty infiltration observed in DMD patients. Having recently documented severe muscle wasting and fatty deposition in two preclinical models of muscular dystrophy (Dysferlin-null and mdx mice) through apolipoprotein E (ApoE) gene deletion without and with cholesterol-, triglyceride-rich Western diet supplementation, we sought to determine whether magnetic resonance imaging and spectroscopy (MRI and MRS, respectively) could be used to detect, characterize, and compare lipid deposition in mdx-ApoE knockout with mdx mice in a diet-dependent manner. MRI revealed that both mdx and mdx-ApoE mice exhibited elevated proton relaxation time constants (T2 ) in their lower hindlimbs irrespective of diet, indicating both chronic muscle damage and fatty tissue deposition. The mdx-ApoE mice on a Western diet (mdx-ApoEW ) presented with greatest fatty tissue infiltration in the posterior compartment of the hindlimb compared with other groups, as detected by MRI/MRS. High-resolution magic angle spinning confirmed elevated lipid deposition in the posterior compartments of mdx-ApoEW mice in vivo and ex vivo, respectively. In conclusion, the mdx-ApoEW model recapitulates some of the extreme fatty tissue deposition observed clinically in DMD muscle but typically absent in mdx mice. This preclinical model will help facilitate the development of new imaging modalities directly relevant to the image contrast generated in DMD, and help to refine MR-based biomarkers and their relationship to tissue structure and disease progression.

Access to emergency paediatric surgery for testicular torsion or intestinal volvulus in New Zealand: A system perspective.

AIM: The risk of organ loss is increased in children with testicular torsion or intestinal volvulus if surgical management is not expedient. The current retrospective study aims to review the time-course from first symptom to 'knife to skin' in these conditions, to determine where delays occur and facilitate a systems approach to better manage these children. METHODS: One hundred consecutive paediatric cases of scrotal exploration for presumed testicular torsion, and 100 neonatal cases presenting with possible malrotation/volvulus were analysed to evaluate the exact time-course of events from admission to surgery. RESULTS: (i) Scrotal exploration: the median time from onset of symptoms to presentation was 12 h (interquartile range (IQR): 5-48 h). In children over 5 years of age, 36% (33/93) were transferred from an external district service area. (ii) Malrotation/volvulus: the median duration of symptoms prior to arrival/assessment was 12 h (IQR: 4-24 h). The median cumulative in-hospital time was over 6 h (368 min, IQR: 247-634 min). CONCLUSIONS: Time to presentation contributes significantly to testicular ischaemic time. This delay to timely surgical intervention is multi-factorial, and must be addressed at a public health level. Support and training in the management of testicular torsion should be provided to all adult surgeons/trainees that may care for these children. In general, this condition is best managed at the presenting hospital whenever appropriate expertise is available. Novel pathways that streamline care may improve efficiency at an institutional level. Addressing issues of access to specialised neonatal surgery is more vexed on account of the tyranny of distance, and the pre-requisite level of surgical expertise required.

Umbilicus and its extensive clinical repertoire.

The umbilicus is involved in a wide range of abnormalities in infants and children. The most severe are evident at birth and include exomphalos (omphalocele) and gastroschisis, both of which can be life-threatening but are easy to diagnose. Exomphalos is often associated with other congenital abnormalities, whereas the associated problems in gastroschisis are largely confined to the gut. Infection of the umbilicus in the neonate presents as omphalitis. The causes of a moist umbilicus following separation of the umbilical stump are multiple, from the relatively minor umbilical granuloma or ectopic bowel mucosa to the more significant patent urachus that leaks urine. Patency of the entire vitello-intestinal (omphalomesenteric) tract allows air and faecal fluid to drain through the umbilicus. The clinical manifestations of persistence of the vitello-intestinal tract vary markedly according to which part remains: clinical presentations include melaena and anaemia, closed-loop bowel obstruction and Meckel diverticulitis. An umbilical hernia occurs when the umbilical cicatrix fails to close. On the other hand, the umbilicus has its uses, which range from being a route for intravenous access in the neonate to being a convenient point of access in laparoscopic surgery.

The 'ins' and 'outs' of intussusception: Where best practice reduces the need for surgery.

Intussusception may be difficult to diagnose because the classical triad of symptoms is evident at the time of presentation in only about 20% of cases. This may lead to delays in making the correct diagnosis. The most common presenting features are colicky abdominal pain, vomiting, pallor, lethargy and listlessness. In recent years, the algorithms of management of intussusception have changed significantly: a routine plain abdominal X-ray has been replaced by ultrasonography, previously perceived contraindications to attempting an enema reduction no longer apply, gas has largely replaced barium for enema reduction, incomplete initial reduction is an indication for a delayed repeat enema if the child is stable clinically and partial reduction has been achieved, the indications for surgery have been tightened and laparoscopy affords an alternative surgical approach. This commentary expands on the rationale behind some of the recent advances in the management of children who present with suspected intussusception.

The ex utero intrapartum treatment (EXIT) procedure: application of a new therapeutic paradigm.

The ex utero intrapartum treatment (EXIT) procedure is a term given to a technique that can transform a potentially fatal neonatal emergency to a controlled intervention with an improved outcome. It has revolutionised the care of prenatally diagnosed congenital malformations in which severe upper airway obstruction is anticipated. An extended period of utero-placental circulation can be utilised to avoid profound cardiopulmonary compromise. Its therapeutic applications have been broadened to include fetuses with congenital diaphragmatic hernia after tracheal plugging, high-risk intrathoracic masses, severe cardiac malformations and conjoined twins. It requires the co-ordination of a highly skilled and experienced multidisciplinary team. The recent enthusiasm for the EXIT procedure needs to be balanced against maternal morbidity. Specific indications and guidelines are likely to be refined as a consequence of ongoing advances in fetal intervention and antenatal imaging.

Longitudinal Force Measurement and its Relationship to Technical Competence for Esophageal Anastomosis in a Thoracoscopic Esophageal Atresia/Tracheo-Esophageal Fistula Simulator.

PURPOSE: Thoracoscopic esophageal atresia with tracheo-esophageal fistula (EA/TEF) repair requires the gentle manipulation of delicate tissue. Force sensors were attached to the upper and lower esophagus of a 3D-printed EA/TEF simulator to explore force parameters as markers of performance. METHODS: Participants completed one intracorporeal suture between the anterior walls of upper and lower esophageal ends. Longitudinal force data were recorded at each end. A blinded pediatric surgeon marked attempt videos. Excessive force events, maximum tension, and force interquartile range (IQR) were measured. Data were reported as median (range) significance of p < 0.05. RESULTS: 17 participants of varying levels of experience performed the task. OSATS scores showed significant differences between experts and novices. Experts completed the task in a median time of 4 min. They used lower maximum tension, higher force IQR, and had fewer excess force events compared to the intermediate and novice groups. DISCUSSION: The application of force was dependent on expertise with more skilled participants having fewer excess force events. The higher expert force IQR likely reflects the consistent tension needed for task completion. Analysis of force data may be an indicator of competence, and trainees may benefit from a thoracoscopic simulator which provides force data feedback. LEVEL OF EVIDENCE: Not applicable.

Testicular torsion: potential pitfalls in its diagnosis and management.

Torsion of the testis is a surgical emergency. Within the paediatric population, peaks in incidence occur in the perinatal and adolescent age groups. There exist traps that can make torsion of the testis a difficult diagnosis to make and once made a tricky condition to manage, in some instances. It is becoming clear that perinatal torsion should serve as an umbrella term for what is increasingly being regarded as two separate entities: prenatal and post-natal torsion. Evidence suggests that the management for each of these may be different. While management of testicular torsion in the adolescent is universally agreed upon, the diagnosis can sometimes be difficult to make. This notwithstanding, early diagnosis is imperative for ensuring the best possible outcome.

Age at orchidopexy as an indicator of the quality of regional child health services.

Early treatment for primary undescended testis (UDT) is necessary to prevent testicular degeneration and improve fertility. The general recommended age for orchidopexy is before 2 years of age. Comparing the age distribution of orchidopexy between different services may give an indirect indication of the quality of the child health services, since timely diagnosis and referral will lead to boys undergoing orchidopexy at the optimal age. This paper reviews the age distribution of boys having orchidopexies for UDT between 1997 and 2006 in the South Island of New Zealand and the state of Victoria, Australia: 64.2% of boys on the South Island and 48.0% of boys in Victoria had orchidopexies before the age of 5. There was a considerable difference in the proportions of children from 0 to 4 years and from 10 to 14 years between the two regions. No distinct trend in age was seen in either population during the period reviewed. These results show that many boys do not receive treatment for UDT at the optimal age. Age at orchidopexy may be an indicator of the quality (in terms of diagnosis, access and timeliness) of a region's paediatric surgical service and may identify where attention should be paid if earlier diagnosis and referral is to be achieved.

The role of steroids in children with multiple recurrences of intussusception.

We describe the case of a boy who had 9 recurrences of intussusception, for which no pathological lesion at the leadpoint was identified. A contrast follow-through study revealed a follicular/nodular mucosal pattern, particularly prominent in the terminal ileum and caecum. Patients with multiple recurrences usually have an identifiable lesion at the leadpoint, but sometimes recurrences may be due to lymphoid hyperplasia, as presumed in our case. No further recurrences occurred after a two month tapering course of oral prednisolone.

The role of motion tracking in assessing technical skill acquisition using a neonatal 3D-printed thoracoscopic esophageal atresia/tracheo-esophageal fistula simulator.

INTRODUCTION: Acquiring the technical skills required for thoracoscopic repair of esophageal atresia with tracheo-esophageal fistula (EA/TEF) is challenging. A high-fidelity 3D-printed pediatric thoracoscopic EA/TEF simulator has been developed to address this issue. This study explored motion-tracking as an assessment tool to distinguish between surgeons of different expertise using the simulator. METHODS: Participants performed a single intracorporeal suture between the esophageal ends in EA with TEF. Total relative path lengths of the right and left surgical instruments were recorded during the task. Each video-recorded attempt was assessed by a blinded pediatric surgeon using a modified Objective Structured Assessment of Technical Skills (OSATS) score. Data recorded as median (range) and statistical significance as p<0.05. RESULTS: The task was performed by 17 participants. The median OSATS scores identified a significant difference between experts and novices. A difference between left- and right-hands was only found in the mid-skill level group. Right-hand path length was greatest in novices and lowest in experts. Left-hand path length was greatest in novices and the mid-skill level group compared to experts. CONCLUSION: Experts had the lowest total path length for either hand, suggesting they had the greatest efficiency of movement. The similar high path lengths in both hands for novices indicate their relatively low level of skill with either hand. The difference between right- and left-hand path lengths in the mid-skill level group likely reflects the improved right-handed technical skills in contrast to the still developing left hand. Further focus on the left hand during simulation training may improve left-handed economy of movement.

Learning and teaching stage 4 clinical decision making: progression from novice to expert.

BACKGROUND: This paper describes the development of learning from novice to expert in Stage 4: Clinical Decision Making (CDM) in surgery: Postoperative reflection and review. It also outlines some or the assessment and teaching approaches suitable to facilitate that transition in skill level. METHODS: This paper is drawn from a much broader study of learning and teaching CDM, that used qualitative methodology based on Constructivist and Grounded Theory. Data was collected in individual interviews and focus groups. Using thematic analysis the data were analysed to identify key ideas. All participants worked in the Department of Surgery at one large regional hospital in Victoria. RESULTS: For each stage there is a sequence of learning beginning from relying on external resources, gradually developing internal resources to guide and direct the learner's CDM. Those internal resources built through experience include multisensory and kinaesthetic memories that expand to facilitate the ability to cope with complexity. DISCUSSION: Armed with the mind-map and rubric table included in this paper it should be possible for any senior clinician or teacher to diagnose their trainees' progression in Stage 4 CDM. This will enable them to tailor their teaching to best match the capabilities of the trainee and to enable to be more effectively targeted. CONCLUSION: CDM can be taught and both trainees and senior clinicians can benefit from understanding the processes involved.

Use of the operative logbook to monitor trainee progress, and evaluate operative supervision provided by accredited training posts.

The Surgical Education and Training (SET) program of the Royal Australasian College of Surgeons (RACS) represents a change from a time-based program to a competency-based program and much greater emphasis is placed on formative assessment and timely feedback (to Surgical Training Boards - STBs - as well as to the trainee). It demands early recognition of the trainee who is struggling to progress, the so-called "marginal trainee". Many of these are simply failing to acquire the desired competencies at the desired or expected rate, although some have more profound underlying deficiencies. SET also places a demand on training posts--with the expectation that they are providing an environment that is conducive to learning and that the trainees are getting adequate learning opportunities. In the domain of operative skills, this largely implies that levels of operative teaching and supervision should be appropriate to the trainee's stage and ability.

Out of tragedy can emerge the finest human qualities.

In March 2019 the Pacific Association of Pediatric Surgeons held its annual conference in Christchurch, New Zealand, a normally peaceful city that was in the process of rebuilding following the devastating earthquakes of 2010 and 2011. Then the day after the conference concluded a horrendous atrocity was committed in two nearby mosques which presented the hospital with 25 years of major gunshot injuries in just one hour. The remarkable response of the hospital and its staff, the victims and the broader community are outlined. Although the surgical response was impressive, lessons could still be learnt. These related to the consequences of the lack of warning as the ED was flooded with casualties, the effects of scoop and run when a disaster occurs near a hospital, limitations around record keeping, the rapid arrival of expert support into ED, pre-empting pressure points, designation of two key destinations and avoiding patient return to ED after imaging, the importance of flexibility and collaboration between services, the consequences on normal hospital activity in the weeks following the incident and the behaviour of victims. The response of PAPS to the event was no less impressive: consistent with its longstanding commitment to the care and welfare of children around the globe, our organisation, donated over $40,000 through the Christchurch Foundation towards scholarships for the children of the victims to support their higher education. Out of tragedy can emerge the finest human qualities.

Academic paediatric surgery in Australia and New Zealand: Its governance, drivers, successes and challenges.

Traditionally, academic surgeons have been expected to excel in research, administration, teaching and clinical work. For many, to be strong in all of these areas is aspirational rather than a reality - and it may not always be a desirable expectation. It is more likely that future academic surgeons will have exceptional ability in several of these domains, but probably not all. Clinical expertise (even if it is within a narrow field) is critical to gaining credibility with non-academic surgical colleagues; and research leadership and substantial ongoing academic output is critical to maintaining credibility among academic surgical colleagues - and facilitates funding success. The Board of Paediatric Surgery is the specialty training board of the Royal Australasian College of Surgeons (RACS) that is responsible for the training program in paediatric surgery for both Australia and New Zealand. "Scholarship and teaching" is designated as being one of the nine competencies RACS expects of all surgeons. Expertise in the domain of scholarship (and research) occurs at two levels: (1) A working knowledge of scientific method, having a critical and curious mind matched with an ability to formulate a research question and contribute to research studies, and an ability to analyse research data and to use it to inform clinical practice. This is expected of all surgeons; and (2) A career academic surgeon with a formal commitment to research which becomes a major component of their work, with the requisite expertise in scientific method to be able to design, set up and complete research studies. The RACS provides support for academia in surgery to flourish in multiple ways and at various stages in the surgeons' career, as described in this chapter. Increasingly, the academic surgeon has to forge links and to collaborate with other research groups. At least in Australia and New Zealand, departments should work to ensure that their academic surgeons are not excessively burdened with departmental leadership and governance roles that do not require specific academic expertise. Arguably, future academic paediatric surgeons will expect to have a better balance in their lives than some of their predecessors!

How electronically available referral guidelines for primary medical practitioners can improve the timeliness of orchidopexy.

AIM: This study determined whether easily used guidelines and an electronic referral process could decrease the age of referral of suspected undescended testes (UDT). An online resource for primary medical practitioners was introduced for which the UDT guideline advises referral to paediatric surgery for testes not sitting spontaneously in the scrotum at three-months corrected age. METHOD: Data were collected prospectively for boys referred with UDT over a seven-year period (2012-2018), during which time agreed GP guidelines on the Community HealthPathways website for referral were introduced. Trends in the age at referral and age at orchidopexy were analysed. RESULTS: Complete data were obtained for 212 boys. Referral before age six months increased from 13% to 61%, and before 12 months from 48% to 78%. Orchidopexy by 12 months increased from 16% to 39%, and by 18 months from 48% to 74%, during the same period. Median age at orchidopexy for this 2012-2018 cohort was 21.6 months compared with 31.1 months from 1997-2007. DISCUSSION: These data demonstrate earlier referral of boys with UDT and earlier orchidopexy corresponded to the introduction of the GP Community HealthPathways website. A similar resource available in other regions or countries also might be expected to reduce the age of referral of suspected UDT from primary care providers.