Laparoscopic versus open pyeloplasty in paediatric pelvi-ureteric junction obstruction.

AIM: Compared to open pyeloplasty (OP), we hypothesised that laparoscopic pyeloplasty (LP) is associated with early recovery, a shorter length of stay (LOS) and less analgesia requirement. METHODS: Between 2011 and 2016, 146 dismembered pyeloplasty cases were reviewed, of which 113 were in the OP group and 33 were in the LP group. We evaluated both groups regarding operative time, LOS, success rate, complications rate and analgesia requirement. Subgroup analysis was done for patients above the age of 5 years, and within the OP group (dorsal lumbotomy (DL) vs. loin incision (LI)). RESULTS: The success rate was 96% in the open group and 97% in the laparoscopic group. The median operative time was significantly shorter in the open group for the entire cohort (127 vs. 200 min; P < 0.05), and in children older than 5 years (n = 41, 134 vs. 225 min; P < 0.05). Other parameters were similar in both groups. The median LOS was significantly shorter (2 vs. 4 days; P < 0.05), and the median analgesia requirement was less (0.44 vs. 0.64 mg/kg morphine; P < 0.05) in the DL (n = 60) compared to LI (n = 53). CONCLUSION: Both OP and LP dismembered approaches are equally effective in treating pelvi-ureteric junction obstruction. Overall, the LOS, complications rate and analgesia requirement were not significantly different; however, the operative time was significantly longer in LP.

Continuous local anaesthetic wound infusion of bupivacaine for postoperative analgesia in neonates: a randomised control trial (CANWIN Study).

OBJECTIVE: To determine the effect of continuous wound infusion of local anaesthetic drug (bupivacaine) on total amount of systemic opioid use in the first 72 hours in newborn infants undergoing laparotomy. DESIGN: A two-arm parallel, open-label randomised controlled trial. SETTING: A quaternary newborn intensive care unit. PATIENTS: Infants>37 weeks of gestation undergoing laparotomy for congenital or acquired abdominal conditions. INTERVENTIONS: Continuous wound infusion of local anaesthetic (bupivacaine) for the first 72 hours along with systemic opioid analgesia (catheter group) or only systemic opioid analgesia (opioid group). MAIN OUTCOME: Total amount of systemic opioid used within the first 72 hours post laparotomy. RESULTS: The study was underpowered as only 30 of the expected sample size of 70 infants were enrolled. 16 were randomised to catheter group and 14 to opioid group. The two groups were similar at baseline. There was no significant difference between the groups for the primary outcome of median total systemic opioid use in the first 72 hours post laparotomy (catheter 431.5 µg/kg vs opioid 771 µg/kg, difference -339.5 µg/kg, 90% CIhigh 109, p value 0.28). There was no significant difference between the groups for any of the secondary outcomes including pain scores, duration of mechanical ventilation, time to reach full feeds and duration of hospital stay. There were no adverse events noted. CONCLUSION: Continuous wound infusion of local anaesthetic along with systemic opioid analgesia is feasible. The lack of a difference in total systemic opioid use in the first 72 hours cannot be reliably interpreted as the study was underpowered. TRIAL REGISTRATION NUMBER: ACTRN12610000633088.

Pediatric transplantation: An international perspective.

The international practice of transplant in the pediatric population is heterogenous. Global trends in pediatric transplant activity are increasing, with diffusion of transplant activities into developing and emerging economies. There have been impacts of the COVID-19 pandemic which have in the earlier part of the pandemic caused a decrease in the number of transplants. While deceased donor programs are well established in advanced economies, emerging and developing countries rely heavily on live donor programs. Prioritization of organs for children exists in different forms throughout the world. Pediatric transplantation as a sub-specialty is young but growing around the world with a need to train surgeons and physicians in this discipline. Outreach efforts with multi-national and multi-institutional partnerships have enabled resource poor countries to establish new transplant programs for children. Further international collaboration, good quality data collection and audit, prospective research and ongoing mentorship and education are needed to further improve outcomes of all children receiving solid organ transplants.

Outcomes for infants with congenital diaphragmatic hernia: A single campus review with low extracorporeal membrane oxygenation utilisation.

AIM: To report the outcome for infants with congenital diaphragmatic hernia (CDH) and identify clinical factors affecting outcome from a tertiary perinatal surgical campus where extracorporeal membrane oxygenation (ECMO) is available however rarely utilised. METHODS: A retrospective cohort study of infants with CDH born in a co-located perinatal neonatal intensive care unit (NICU) or retrieved into and managed at a surgical NICU: 2003-2018. RESULTS: One hundred and fifty-nine infants with CDH were identified. One hundred and twenty were born in the co-located hospital and 39 retrieved from outlying hospitals. Survival of all patients with CDH was 74.8%; The survival for all isolated left CDH was 89% and the survival of post-surgery was 93%. Two patients went onto ECMO and both died. Associated major congenital anomalies were seen in 13.2%. Low birthweight (< 2500 g), 5-min Apgar <5, antenatal diagnosis, right-sided diaphragmatic hernia, herniation of the liver, associated major congenital anomalies, high oxygenation index (>25) on day 1, inotrope use, inhaled nitric oxide and need for high-frequency ventilation were associated with increased mortality on univariate analysis. Neurodevelopmental follow-up was commenced in 2013. Forty-three infants were discharged and developmental data were available for 36 at 4 months (83%) and 32 at 1 year (73%). Outcomes revealed normal motor scores and expressive language with mild delay in the receptive language at 1 year. CONCLUSION: Despite the low use of ECMO, our centre's results reveal excellent survival and neurodevelopmental outcomes consistent with or better than international data.

How should the neonatal retrieval team respond to the neonate referred with bilious vomiting?

AIM: Bilious vomiting (BV) in the neonatal period may herald malrotation with life-threatening volvulus. In New South Wales, contrast fluoroscopy is not available in non-tertiary paediatric centres; therefore, transfer is required. An infant with BV referred to Newborn and Paediatric Emergency Transport Service is prioritised for urgent retrieval to a surgical centre for contrast fluoroscopy and paediatric surgical review. This study analysed how many neonates with BV needed retrieval to prevent bowel loss or to save one life and to identify predictors of malrotation and/or volvulus. METHODS: All neonatal referrals (<29 days) to Newborn and Paediatric Emergency Transport Service between 31 July 2014 and 31 July 2020 with BV or aspirates were examined. Data on time of onset of BV, time of call for retrieval, vital signs, lactate level and blood glucose at referral, time of arrival at the surgical centre and outcome were analysed. RESULTS: Of 391 neonates referred with BV, 113 (28.9%) had a surgical cause and 31 (7.9%) had a time-critical malrotation and/or volvulus. All 31 neonates were well at referral with normal vital signs including three neonates who subsequently died. Lactate levels at referral (1-7.5 mmol/L) were not predictive of outcome. The odds of a time-critical diagnosis increased with every day of age (odds ratio = 1.097), heart rate >140 (P = 0.04) and decreased for each kilogram of birthweight (odds ratio 0.475; confidence interval 0.294-0.768). CONCLUSIONS: Neonates with BV require urgent referral to paediatric surgery and contrast fluoroscopy. Thirteen urgent transfers are required to preserve the bowel integrity and life in one baby.

Surgical management of the acute paediatric scrotum: a three-year single centre experience.

BACKGROUND: Acute scrotal pain is a common emergency presentation in paediatric surgery. Torsion of the testicular appendage (TTA) is the most common cause for pain, with testicular torsion (TT) being the sinister pathology to exclude. Outcomes are time dependent, and a delayed scrotal exploration could result in testicular loss. METHODS: We performed a review on a large retrospective cohort of 449 surgical scrotal explorations at a large referral paediatric surgical centre over three years. RESULTS: Only about a quarter of children with testicular pain presented within 4 h. TT is commonly associated with nausea and an abnormal lie. Two children with a classical 'blue dot' sign were later found to have a testicular torsion. 19% of all children with a TTA were also seen to have Bell clapper anomaly (BCA). Recurrent testicular pain was associated with 84.7% (p < 0.001) of BCA. Intra-operative diagnosis of TTA correlated with histopathology in 84.6% (p=0.021). The sensitivity of intraoperative diagnosis was 90.9% with a specificity of 75.3%. CONCLUSION: Routine histopathology for a classic TTA may not be required especially in resource poor situations. All children presenting with recurrent episodes of testicular pain must be considered for surgical scrotal exploration. And in view of the incidence of BCA in this cohort, all scrotal explorations for acute scrotal pain should include an assessment for BCA.

Liver transplantation in children with inborn errors of metabolism: 30 years experience in NSW, Australia.

BACKGROUND: Inborn errors of metabolism (IEM) are a diverse group of genetic disorders that can result in significant morbidity and sometimes death. Metabolic management can be challenging and burdensome for families. Liver transplantation (LT) is increasingly being considered a treatment option for some IEMs. IEMs are now considered the second most common reason for pediatric LT. AIM: To review the data of all children with an IEM who had LT at The Children's Hospital at Westmead (CHW), NSW, Australia between January 1986 and January 2019. METHODS: Retrospective data collected from the medical records and genetic files included patient demographics, family history, parental consanguinity, method of diagnosis of IEM, hospital and intensive care unit admissions, age at LT, graft type, clinical outcomes and metabolic management pre and post-LT. RESULTS: Twenty-four LT were performed for 21 patients. IEM diagnoses were MSUD (n = 4), UCD (n = 8), OA (n = 6), TYR type I (n = 2) and GSD Ia (n = 1). Three patients had repeat transplants due to complications. Median age at transplant was 6.21 years (MSUD), 0.87 years (UCD), 1.64 years (OA) and 2.2 years (TYR I). Two patients died peri-operatively early in the series, one died 3 months after successful LT due to septicemia. Eighteen LTs have been performed since 2008 in comparison to six LT prior to 2008. Dietary management was liberalized post LT for all patients. CONCLUSIONS: Referral for LT for IEMs has increased over the last 33 years, with the most referrals in the last 10 years. Early LT has resulted in improved clinical outcomes and patient survival.

Pediatric third liver transplantation-A single-center experience.

BACKGROUND: Pediatric retransplantation is an accepted practice for graft failure and complications in Australasia. As 15% of children require a third transplant, this is a growing cohort with limited data in the literature. METHODS: We review nine patients from the commencement of our transplantation program in 1986 up to 2020 assessing demographics, prognosis, and outcome measures. RESULTS: Third transplant patient survival was comparative to first and second transplant patient survival at 5 years. All deaths were within the post-operative period and secondary to sepsis. Operative times and transfusion volumes were increased at third transplant (1.8 and 4.5 times compared to first transplant, respectively). Learning difficulties and psychological disturbances were prevalent (83% and 66.6%, respectively). CONCLUSIONS: While recent mortality outcomes appear comparable to undergoing a second liver transplant, third transplant operations were more complex. Neurological impairment and psychological disturbance appear to be prevalent and need to be considered in pre-transplant counseling.

Acute Macular Neuroretinopathy Related to Alcoholic Hepatitis.

A 33-year-old woman admitted for acute alcoholic hepatitis was referred to the ophthalmology department with an acute onset paracentral scotoma of the left eye. On examination, best-corrected visual acuity was Snellen 6/4 in the right eye and 6/9 in the left eye. Dilated left fundus examination revealed wedge-shaped changes at the macula. Spectral-domain ocular coherence tomography (SD-OCT) initially revealed a small cuff of subfoveal fluid and band-like hyperreflectivity extending outwards from the outer plexiform layer consistent with acute macular neuroretinopathy (AMN). Four days later, repeat SD-OCT was performed and it demonstrated resolution of the subfoveal fluid and disruption of the outer retinal layers. At the 6-week follow-up, the patient had no improvement in her symptoms and OCT angiography demonstrated coarsening and microvascular changes in both the deep vascular plexus and the choriocapillaris. To our knowledge, this is the first case of AMN in association with acute hepatitis. Although the exact pathophysiology of AMN remains obscure, this case highlights the benefits of multimodal retinal imaging and aims to bring attention to the possible association of AMN with alcoholic hepatitis.

The many faces of ocular syphilis: case-based update on recognition, diagnosis, and treatment.

In recent years, syphilis (Treponema pallidum) has become increasingly prevalent in Canada, and as a result, rates of ocular syphilis are also rising. Classically, syphilis was seen primarily in men who have sex with men; now, it is increasingly seen in people of all age groups, sexes, and sexual orientations. We present a series of 26 cases of ocular syphilis from London, Ontario, 5 of which are discussed in detail to illustrate the varied presentations and diagnostic challenges of ocular syphilis. The presentations include uveitis, iris granuloma (gumma), retinitis (acute syphilitic posterior placoid chorioretinitis), vasculitis, optic neuritis, and serous retinal detachment. The 5 cases are mostly middle-aged heterosexual men and women without the typical risk factors that would alert the examiner to suspect syphilis. We emphasize the importance of testing for syphilis when assessing and treating inflammatory eye disease, regardless of demographics and known risk factors, given the increasing prevalence of this disease. Diagnosis of syphilis relies on serologic testing, which is complex and has undergone significant changes from historical reliance on the Venereal Disease Research Laboratory test. We provide an overview of the strategy and rationale for modern serologic testing. The mainstay of treatment remains intravenous penicillin G, with alternative antibiotics (e.g., ceftriaxone) being less effective.

Complications of surgical mesh patches after kidney transplantation in children-A case series.

BACKGROUND: Abdominal compartment syndrome after kidney transplantation in pediatric recipients is a recognized complication relating to size discrepancy requiring abdominal wall closure over a large adult allograft. In order to circumvent this problem, our center implemented use of a surgical mesh, Surgisis® (Cook Surgical, Bloomington, IN), for abdominal wall closure in very small children to increase the surface covering over the organ and prevent compression. In this article, we report on the complications encountered following the use of these mesh patches. METHODS: A retrospective case review was conducted of all pediatric kidney transplants from September 2006 to December 2018 and divided into abdominal wall closure with and without implantation of Surgisis® mesh patch. Review of clinical notes was performed to identify information with respect to clinical course and post-operative outcomes. RESULTS: A surgical mesh patch was used in 7 pediatric recipients, of which 5 (71%) presented with post-operative complications. Three recipients were found to have bowel obstruction related to the surgical patch, necessitating bowel resection in one child. In addition, three children developed large serous fluid collections between the subcutaneous layers and the surgical mesh, requiring surgical drainage in two. CONCLUSIONS: In view of these findings, we recommend close surveillance for potential complications in this cohort. Future research is needed to explore the safety of different approaches to achieve abdominal wall closure in this group.

Portosystemic shunt for portal hypertension after Kasai operation in patients with biliary atresia.

PURPOSE: Many biliary atresia (BA) patients will eventually develop liver failure even after a successful Kasai portoenterostomy. A common complication of long-term BA survivors with their native liver is problematic portal hypertension. The aim of this study was to defend the view that portosystemic shunts can delay or negate the need for transplantation in these children. METHODS: A retrospective single center review of the efficacy of portosystemic shunts in BA patients after a successful Kasai portoenterostomy was conducted. RESULTS: From 1991 to 2017, 11 patients received portosystemic shunts. Median age of Kasai operation was 48 (36-61) days. Shunts were performed at the median age of 6.2 (4.1-6.8) years. Three of these eleven patients required subsequent liver transplantation. OS at 5 and 10 years were 90.9% and 81.8%, respectively. TFS at 5 and 10 years were 90.9% and 72.7%, respectively. Long-term complications included mild encephalopathy in 2 patients, hypersplenism in 3, and cholestasis in 1. CONCLUSION: Portosystemic shunt for the treatment of portal hypertension in carefully selected BA patients is an effective option in delaying or negating the need for liver transplantation.

Double barrel enteroplasty for the management of short bowel syndrome in children.

BACKGROUND: Currently, there are two well-established methods of bowel lengthening in patients with short bowel syndrome (SBS)-longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) [1-4]. Both procedures may carry a high reported morbidity and mortality of 30.2% and 14.4%, respectively [5]. We report the outcomes of a novel technique: double barrel enteroplasty (DBE) for autologous intestinal reconstruction. METHODS: We performed a retrospective review of all ten patients who underwent DBE at our institution since 2011. All patients have SBS and were dependent on parenteral nutrition (PN) at the time of surgery. Etiologies were gastroschisis (n = 4), bowel atresia (n = 3), necrotising enterocolitis (n = 1), volvulus (n = 1), and near-total intestinal aganglionosis (n = 1). Patient survival, complications, and subsequent enteral autonomy were evaluated. RESULTS: All patients are alive with normal liver function. Five children achieved enteral autonomy, while the remaining are on weaning PN. There was no bleeding, anastomotic leak, perforation, infective complications, or intestinal necrosis. No patient has required a liver and/or intestinal transplant. CONCLUSIONS: Double barrel enteroplasty is technically feasible and safe. It has similar efficacy and may have fewer complications when compared with other methods of autologous intestinal reconstruction.

Bilateral periorbital necrotising fasciitis associated with invasive group: a Streptococcus infection.

A previously independent 56-year-old immunocompetent woman presented with septic shock in the setting of periorbital swelling and diffuse infiltrates on chest imaging. Blood cultures were positive for growth of group A Streptococcus (GAS). Broad spectrum antimicrobials were initiated with the inclusion of the antitoxin agent clindamycin. Necrosis of periorbital tissue was noted and surgical consultation was obtained. Débridement of both eyelids with skin grafting was performed. GAS was isolated from wound cultures and also observed on periorbital tissue microscopy. The final diagnosis was bilateral periorbital necrotising fasciitis (PONF) associated with invasive GAS infection. The patient had a prolonged intensive care unit course with input from multiple specialist teams. This case demonstrates the importance of early recognition and treatment of PONF, the profound systemic morbidity caused by these infections, and illustrates successful multidisciplinary teamwork.

Outcomes for children after second liver transplantations are similar to those after first transplantations: a binational registry analysis.

OBJECTIVE: To assess long term graft and patient survival after donor liver retransplantation in children in Australia and New Zealand during 1986-2017; to determine the factors that influence survival. DESIGN: Retrospective cohort analysis (registry data). SETTING, PARTICIPANTS: Australia and New Zealand Liver Transplant Registry data for all liver retransplantations in children (under 18 years of age), 1986-2017, in all four paediatric and six adult liver transplantation centres in the two countries. MAIN OUTCOME MEASURES: Graft and patient survival at one, 5, 10 and 15 years. RESULTS: 142 liver retransplantations were undertaken in children (59 during 1986-2000, 83 during 2001-2017). Kaplan-Meier survival analysis indicated that survival was significantly greater during 2001-2017 than 1986-2000 (P < 0.001). During 2001-2017, graft survival one year after retransplantation was 84%, at 5 years 75%, at 10 years 70%, and at 15 years 54%; patient survival was 89% at one year, 87% at 5 years, 87% at 10 years, and 71% at 15 years. Median time between transplantations was 0.2 years (IQR, 0.03-1.4 years) during 1986-2000, and 1.8 years (IQR, 0.1-6.8 years) during 2001-2017 (P = 0.002). The proportion of graft failures that involved split grafts was larger during 2001-2017 (35 of 83, 42%) than 1986-2000 (10 of 59, 17%). Graft type, cause of graft failure, and number of transplants did not influence survival following retransplantation. CONCLUSION: Survival for children following retransplantation is excellent. Graft survival is similar for split and whole grafts. Children on the liver waiting list requiring retransplantation should have the same access to donor grafts as children requiring a first transplant.

Improved Electrophoretic Deposition of Vertical Single Wall Carbon Nanotubes with Nanoscopic Electrostatic Lenses.

Under certain conditions, electrophoretic deposition (EPD) of single-wall carbon nanotubes (SWCNTs) onto metal at the base of nanoscale insulating windows can result in a single SWCNT per window, bonded at one end to the metal. During EPD charge, buildup on the insulator creates electrostatic lenses at the windows that control the trajectory of the SWCNTs. The aim is to develop a reproducible process for deposition of individual vertically oriented SWCNTs into each window to enable novel devices. The length of the SWCNTs is shown to be the most critical parameter in achieving results that could be used for devices. In particular, single nanotube deposition in windows by EPD was achieved with SWCNTs with lengths on the order of the window depth. By performing current vs voltage (IV) measurements against a platinum wire in a phosphate buffer and by modeling the data, the presence of the nanotube can be detected, the contact interface can be studied, and the nanotube's viability for device applications can be determined. These results provide a basis for process integration of vertical SWCNTs using EPD.

Differences in attitudes to feeding post repair of Gastroschisis and development of a standardized feeding protocol.

BACKGROUND: The purpose of this study was to examine differences in attitudes to feeding in neonates with Gastroschisis between clinical groups and to develop a standardized feeding protocol. Confusion, inconsistencies in practice and lack of evidence could be contributing to avoidable delays in the establishment of enteral feeds resulting in lengthy requirements for central venous access, dependence on total parenteral nutrition (TPN), increased risk of sepsis, TPN related cholestasis and prolongation in length of hospital stay. METHODS: A national survey of clinicians (neonatologists, neonatal intensive care nurses and paediatric surgeons), looking after neonates with gastroschisis was undertaken to determine differences in feeding practice post repair. In addition, an audit of practice in one hospital was undertaken to examine variations in practices between clinicians. A feeding protocol was then developed using inputs from surgeons and neonatologists. RESULTS: Gastric aspirates and residuals were typically used as indicators of feed readiness and feed tolerance; however, there was very little consistency within and between clinical groups in definitions of tolerance or intolerance of feeds and in how to initiate and progress feeds. A feeding protocol with clear definition of feed readiness and a clear pathway to progression of feeds was developed to help overcome these variations in practice with the possibility that this might reduce the length of stay (LOS) and have other secondary benefits. The protocol included early introduction of enteral feeds particularly direct breast or sucking feeds. CONCLUSIONS: Wide differences in attitudes to feeding neonates post Gastroschsis repair exist and the need for a consistent protocolized approach was felt. The feeding protocol we developed requires a change of practice and further clinical trials are needed to evaluate its effectiveness.

Brief Normothermic Machine Perfusion Rejuvenates Discarded Human Kidneys.

Normothermic machine perfusion (NMP) may allow resuscitation and improved assessment of kidneys before transplantation. Using discarded human kidneys, we investigated the mechanistic basis and translational potential of NMP compared with cold static storage (CS). METHODS: Discarded deceased donor kidneys (n = 15) underwent 1-hour NMP following CS. Renal perfusion, biochemical, and histologic parameters were recorded. NMP was directly compared with CS in paired donor kidneys using simulated transplantation with allogeneic whole blood, followed by assessment of the aforementioned parameters, in addition to RNA sequencing. RESULTS: Kidneys were successfully perfused, with improved renal blood flows and resistance over the course of perfusion, and evidence of urine output (median 21 mL), in all but one kidney. NMP completely resolved nonperfused regions in discarded donation after circulatory death kidneys. In paired kidneys (n = 4 pairs), transcriptomic analyses showed induction of stress and inflammatory pathways in NMP kidneys, with upregulation of pathways promoting cell survival and proliferation. Furthermore, the NMP pairs had significantly better renal perfusion (1.5-2 fold improvement) and functional parameters, and amelioration of cell death, oxidative stress, and complement activation. CONCLUSIONS: In this pilot preclinical study using simulated transplantation of paired kidneys, NMP of discarded marginal kidneys demonstrated some significant mechanistic benefits in comparison to CS alone. NMP may have potential to reduce organ discards and enhance early graft function in such kidneys.