Age at Primary Cleft Lip Repair

Background: The bellwether procedures described by the Lancet Commission on Global Surgery represent the ability to deliver adult surgical services after there is a clear and easily made diagnosis. There is a need for pediatric surgery bellwether indicators. A pediatric bellwether indicator would ideally be a routinely performed procedure, for a relatively common condition that, in itself, is rarely lethal at birth, but that should ideally be treated with surgery by a standard age. Additionally, the condition should be easy to diagnose, to minimize the confounding effects of delays or failures in diagnosis. In this study, we propose the age at primary cleft lip
(CL) repair as a bellwether indicator for pediatric surgery.
Method: We reviewed the surgical records of 71,346 primary cleft surgery patients and ultimately studied age at CL repair in 40,179 patients from 73 countries, treated by Smile Train partners for 2019. Data from Smile Train’s database were correlated with World Bank and WHO indicators.
Results: Countries with a higher average age at CL repair (delayed access to surgery) had higher maternal, infant, and child mortality rates as well as a greater risk of catastrophic health expenditure for surgery. There was also a negative correlation between delayed CL repair and specialist surgical workforce numbers, life expectancy, percentage of deliveries by C-section, total health expenditure per capita, and Lancet Commission on Global Surgery procedure rates.
Conclusion: These findings suggest that age at CL repair has potential to serve as a bellwether indicator for pediatric surgical capacity in Lower- and Middleincome Countr

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Background
Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality.

Methods
We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis.

Findings
We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in low-income countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality.

Interpretation
Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between low-income, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030.

Predictors of Rehabilitation Service Utilisation among Children with Cerebral Palsy (CP) in Low- and Middle-Income Countries (LMIC): Findings from the Global LMIC CP Register

Background: We assessed the rehabilitation status and predictors of rehabilitation service utilisation among children with cerebral palsy (CP) in selected low- and middle-income countries (LMICs). Methods: Data from the Global LMIC CP Register (GLM-CPR), a multi-country register of children with CP aged <18 years in selected countries, were used. Descriptive and inferential statistics (e.g., adjusted odds ratios) were reported. Results: Between January 2015 and December 2019, 3441 children were registered from Bangladesh (n = 2852), Indonesia (n = 130), Nepal (n = 182), and Ghana (n = 277). The proportion of children who never received rehabilitation was 49.8% (n = 1411) in Bangladesh, 45.8% (n = 82) in Nepal, 66.2% (n = 86) in Indonesia, and 26.7% (n = 74) in Ghana. The mean (Standard Deviation) age of commencing rehabilitation services was relatively delayed in Nepal (3.9 (3.1) year). Lack of awareness was the most frequently reported reason for not receiving rehabilitation in all four countries. Common predictors of not receiving rehabilitation were older age at assessment (i.e., age of children at the time of the data collection), low parental education and family income, mild functional limitation, and associated impairments (i.e., hearing and/or intellectual impairments). Additionally, gender of the children significantly influenced rehabilitation service utilisation in Bangladesh. Conclusions: Child’s age, functional limitation and associated impairments, and parental education and economic status influenced the rehabilitation utilisation among children with CP in LMICs. Policymakers and service providers could use these findings to increase access to rehabilitation and improve equity in rehabilitation service utilisation for better functional outcome of children with CP

Evaluation of a surgical treatment algorithm for neglected clubfoot in low-resource settings

Purpose
Idiopathic clubfoot affects approximately 1/1000 alive-born infants, of whom 80–91% are born in low- or middle-income countries (LMICs). This retrospective study aimed to evaluate the morphological, functional, and social outcomes in patients with neglected clubfoot in rural Bangladesh, after receiving surgical treatment.

Methods
Patients received a posteromedial release (PMR) with or without an additional soft tissue intervention (group 1), a PMR with an additional bony intervention (group 2), or a triple arthrodesis (group 3) according to our surgical algorithm. Patients were followed until two year post-intervention. Evaluation was done using a modified International Clubfoot Study Group Outcome evaluation score and the Laaveg-Ponseti score.

Results
Twenty-two patients with 32 neglected clubfeet (ages 2–24 years) received surgical treatment. Nineteen patients with 29 clubfeet attended follow-up. At two year follow-up an excellent, good, or fair Laaveg-Ponseti score was obtained in 81% (group 1), 80% (group 2), and 0% (group 3) of the patients (p value 0.0038). Age at intervention is inversely correlated with the Laaveg-Ponseti score at two year follow-up (p < 0.0001). All patients attended school or work and were able to wear normal shoes.

Conclusion
Our treatment algorithm is in line with other surgical algorithms used in LMICs. Our data reconfirms that excellent results can be obtained with a PMR regardless of age. Our algorithm follows a pragmatic approach that takes into account the reality on the ground in many LMICs. Good functional outcomes can be achieved with PMR for neglected clubfoot. Further research is needed to investigate the possible role of triple arthrodesis.

Feasibility of establishing an infant hearing screening program and measuring hearing loss among infants at a regional referral hospital in south western Uganda

Introduction
Despite the high burden of hearing loss (HL) globaly, most countries in resource limited settings lack infant hearing screening programs(IHS) for early HL detection. We examined the feasibility of establishing an IHS program in this setting, and in this pilot program measured the prevalence of infant hearing loss (IHL) and described the characteristics of the infants with HL.

Methods
We assessed feasibility of establishing an IHS program at a regional referral hospital in south-western Uganda. We recruited infants aged 1 day to 3 months and performed a three-staged screening. At stage 1, we used Transient Evoked Oto-acoustic Emissions (TEOAEs), at stage 2 we repeated TEOAEs for infants who failed TEOAEs at stage 1 and at stage 3, we conducted Automated brainstem responses(ABRs) for those who failed stage 2. IHL was present if they failed an ABR at 35dBHL.

Results
We screened 401 infants, mean age was 7.2 days (SD = 7.1). 74.6% (299 of 401) passed stage 1, the rest (25.4% or 102 of 401) were referred for stage 2. Of those referred (n = 102), only 34.3% (35 of 102) returned for stage 2 screening. About 14.3% (5/35) failed the repeat TEOAEs in at least one ear. At stage 3, 80% (4 of 5) failed the ABR screening in at least one ear, while 25% (n = 1) failed the test bilaterally. Among the 334 infants that completed the staged screening, the prevalence of IHL was 4/334 or 12 per 1000. Risk factors to IHL were Newborn Special Care Unit (NSCU) admission, gentamycin or oxygen therapy and prematurity.

Conclusions
IHS program establishment in a resource limited setting is feasible. Preliminary data indicate a high prevalence of IHL. Targeted screening of infants at high risk may be a more realistic and sustainable initial step towards establishing IHS program s in a developing country like Uganda.

A critical threshold for global pediatric surgical workforce density

Purpose
1.7 billion children lack access to surgical care, particularly in low- and middle-income countries (LMIC). The pediatric surgical workforce density (PSWD), an indicator of surgical access, correlates with survival of complex pediatric surgical problems. To determine if PSWD also correlates with population-level health outcomes for children, we compared PSWD with pediatric-specific mortality rates and determined the PSWD associated with improved survival.

Methods
Using medical licensing registries, pediatric surgeons practicing in 26 countries between 2015 and 2019 were identified. Countries’ PSWD was calculated as the ratio of pediatric surgeons per 100,000 children. The correlation between neonatal, infant and under 5 mortality rates and PSWD was assessed using Spearman’s correlations and piecewise linear regression models.

Results
Four LIC, eight L-MIC, ten UMIC and four HIC countries, containing 420 million children, were analyzed. The median PSWD by income group was 0.03 (LIC), 0.12 (L-MIC), 1.34 (UMIC) and 2.13 (HIC). PSWD strongly correlated with neonatal (0.78, p < 0.001), infant (0.82, p < 0.001) and under 5 (0.83, p  0.37. Currently, PSWD in LMICs is inadequate to meet UN Sustainable Development Goal 3.2 for child mortality.

Access to pediatric surgery delivered by general surgeons and anesthesia providers in Uganda: Results from 2 rural regional hospitals

Background
Significant limitations in pediatric surgical capacity exist in low- and middle-income countries, especially in rural regions. Recent global children’s surgical guidelines suggest training and support of general surgeons in rural regional hospitals as an effective approach to increasing pediatric surgical capacity.

Methods
Two years of a prospective clinical database of children’s surgery admissions at 2 regional referral hospitals in Uganda were reviewed. Primary outcomes included case volume and clinical outcomes of children at each hospital. Additionally, the disability-adjusted life-years averted by delivery of pediatric surgical services at these hospitals were calculated. Using a value of statistical life calculation, we also estimated the economic benefit of the pediatric surgical care currently being delivered.

Results
From 2016 to 2019, more than 300 surgical procedures were performed at each hospital per year. The majority of cases were standard general surgery cases including hernia repairs and intussusception as well as procedures for surgical infections and trauma. In-hospital mortality was 2.4% in Soroti and 1% in Lacor. Pediatric surgical capacity at these hospitals resulted in over 12,400 disability-adjusted life-years averted/year. This represents an estimated economic benefit of 10.2 million US dollars/year to the Ugandan society.

Conclusion
This investigation demonstrates that lifesaving pediatric procedures are safely performed by general surgeons in Uganda. General surgeons who perform pediatric surgery significantly increase surgical access to rural regions of the country and add a large economic benefit to Ugandan society. Overall, the results of the study support increasing pediatric surgical capacity in rural areas of low- and middle-income countries through support and training of general surgeons and anesthesia providers.

Gender-role behaviour and gender identity in girls with classical congenital adrenal hyperplasia

Introduction
Girls with classical congenital adrenal hyperplasia (CAH) are exposed to excess fetal adrenal androgens in-utero, and often born with masculinised genitalia. They are conventionally reared as females, but show more “boyish” gender-role behaviour (GRB) and gender-identity (GI) issues in childhood and adolescence. Male-rearing is also reported mainly due to delayed treatment and/or socio-cultural factors. We compared GRB/GI in girls with CAH with healthy age matched children, and explored for associations with socio-demographic and diagnosis/treatment related factors.

Methods
GRB and GI were assessed using the Gender Identity Questionnaire for children (GIQC) in 27 girls with classical CAH at a specialised clinic, and compared with 50 age-matched healthy controls, with exploratory-analysis based on socio-demographic and diagnosis/treatment-related factors.

Results
Girls with CAH had lower total GIQC scores compared to healthy children (3.29 vs. 4.04, p = < 0.001) with lower GRB score (3.39 vs. 4.23, p < 0.001), and tendency for lower GI score (3.19 vs. 3.5, p = 0.08). Exploratory analysis showed no differences based on diagnosis/treatment factors including age, degree of virilisation at diagnosis and surgical procedures. and only subtle changes based on ethnicity and maternal education.

Discussion/conclusion
Girls with CAH managed at a specialised centre showed more masculinised GRB and tendency for ambiguous GI, which did not vary upon diagnosis/treatment related factors, suggesting that prenatal androgen exposure was the likely contributor. Clinicians should be vigilant about the increased risk of gender-related problems in girls with CAH, irrespective of sociocultural background and despite early treatment.

Taking Paediatrics Abroad: Working with low- and middle-income countries in a global pandemic

Children and young people around the world face challenges to their health and wellbeing. In particular, in low- and middle-income countries they experience a higher burden of disease, exacerbated by global inequity limiting access to quality health care. According to the inverse care law, the availability of quality health care varies inversely to the need of the population, and hardworking health-care professionals in under-resourced countries may face impediments to continued education or subspecialty training. In line with the Sustainable Development Goals, collaborations have been developed between high-income and low- and-middle-income countries to address global disparities in health. These collaborations face challenges of high financial costs, difficulties creating long-term sustainable change, and with the emergence of the COVID-19 pandemic, border closures preventing fly-in volunteers. In this paper, we describe the development of an innovative, paediatric-specific model of care for training and support between high- and low-income countries – Taking Paediatrics Abroad Ltd. Taking Paediatrics Abroad supports the development of mutually beneficial relationships between Australian paediatric health-care professionals and paediatric health-care professionals in developing countries and remote, underserved Australian Aboriginal communities. Since May 2020, there have been over 100 sessions covering a vast array of paediatric specialties. This article explores Taking Paediatrics Abroad’s model of care, its implementation and challenges, and opportunities for the futur

Impact of the COVID-19 pandemic on paediatric patients with cancer in low-income, middle-income and high-income countries: protocol for a multicentre, international, observational cohort study

Introduction
Childhood cancers are a leading cause of non-communicable disease deaths for children around the world. The COVID-19 pandemic may have impacted on global children’s cancer services, which can have consequences for childhood cancer outcomes. The Global Health Research Group on Children’s Non-Communicable Diseases is currently undertaking the first international cohort study to determine the variation in paediatric cancer management during the COVID-19 pandemic, and the short-term to medium-term impacts on childhood cancer outcomes.

Methods and analysis
This is a multicentre, international cohort study that will use routinely collected hospital data in a deidentified and anonymised form. Patients will be recruited consecutively into the study, with a 12-month follow-up period. Patients will be included if they are below the age of 18 years and undergoing anticancer treatment for the following cancers: acute lymphoblastic leukaemia, Burkitt lymphoma, Hodgkin lymphoma, Wilms tumour, sarcoma, retinoblastoma, gliomas, medulloblastomas and neuroblastomas. Patients must be newly presented or must be undergoing active anticancer treatment from 12 March 2020 to 12 December 2020. The primary objective of the study was to determine all-cause mortality rates of 30 days, 90 days and 12 months. This study will examine the factors that influenced these outcomes. χ2 analysis will be used to compare mortality between low-income and middle-income countries and high-income countries. Multilevel, multivariable logistic regression analysis will be undertaken to identify patient-level and hospital-level factors affecting outcomes with adjustment for confounding factors.

Ethics and dissemination
At the host centre, this study was deemed to be exempt from ethical committee approval due to the use of anonymised registry data. At other centres, participating collaborators have gained local approvals in accordance with their institutional ethical regulations. Collaborators will be encouraged to present the results locally, nationally and internationally. The results will be submitted for publication in a peer-reviewed journal.