History of the Pan African Paediatric Surgery Association (PAPSA).

The Pan African Paediatric Surgery Association (PAPSA) was formed in 1994. The need for an organisation in Africa to voice children's surgery and the trials and tribulations in forming this organisation was covered in this journal 2 years ago (Heinz R, Kyambi J, Lakhoo K. Surg Int 34(5):499-504, 2018). This article covers the history of the organisation post inception in 1994 to date. The near disbanding of the organisation due to political unrest and wars in Africa, to its success in the recent decade is highlighted in this manuscript.

Primary paediatric chest wall tumours necessitating surgical management.

INTRODUCTION: Limited literature exists on oncological chest wall reconstruction in the paediatric population, with the field still largely undecided on the best surgical reconstructive techniques to employ. The use of biological grafts/meshes is gaining popularity in certain adult surgical procedures but their use in paediatric procedures is rarely reported in the literature. We present the outcomes of our institution's multidisciplinary approach to managing paediatric chest wall tumours as well as our experience with the use of biological grafts for chest wall reconstruction following oncological resections. METHODS: Data were analysed retrospectively from eight paediatric patients who were treated for primary chest wall tumours between 2010 and 2018. RESULTS: The tumours comprised two lipoblastomas, three Ewing's sarcomas, an undifferentiated sarcoma with osteosarcomatous differentiation, a high grade undifferentiated sarcoma and a myofibroma. Seven of the eight patients underwent chest wall reconstruction with a biological graft. There were no postoperative mortalities and no evidence of recurrence in any of the patients in the series. No further chest wall operations were required and there were no postoperative infection related complications. CONCLUSIONS: We support the use of biological grafts for chest wall reconstruction after oncological resections and maintain that a multidisciplinary approach is essential for the management of paediatric chest wall tumours.

Correction to: Development of a new reproductive tissue cryopreservation clinical service for children: the Oxford programme.

In the published version, the Acknowledgements section was missing a funding note of co-author Dr C Verrill. The corrected version should read as follows.

Reducing Gastroschisis Mortality: A Quality Improvement Initiative at a Ugandan Pediatric Surgery Unit.

INTRODUCTION: With modern treatment, survival of gastroschisis exceeds 90% in high-income countries. Survival in these countries has been largely attributed to prenatal diagnosis, delivery at tertiary facilities with timely resuscitation, timely intervention, parenteral nutrition and intensive care facilities. In sub-Saharan Africa, due to lack of these facilities, mortality rates are still alarmingly high ranging from 75 to 100%. In Uganda the mortality is 98%. AIM: The aim of this study was to reduce gastroschisis mortality in a feasible, sustainable way using a locally derived gastroschisis care protocol at a referring hospital in Western Uganda. METHODS: Data collection was performed from January to October 2018. Nursing staff were interviewed regarding the survival and management of gastroschisis babies. A locally derived protocol was created with staff input and commitment from all the team members. RESULTS: Four mothers absconded and 17 babies were cared for using the newly designed protocol. Seven survived and were well at one month post discharge follow-up, reducing the mortality for this condition from 98 to 59%. CONCLUSION: A dedicated team with minimal resources can significantly reduce the mortality in gastroschisis by almost 40% using a locally derived protocol.

Investigating Wilms' Tumours Worldwide: A Report of the OxPLORE Collaboration-A Cross-Sectional Observational Study.

BACKGROUND: Childhood cancer is neglected within global health. Oxford Pediatrics Linking Oncology Research with Electives describes early outcomes following collaboration between low- and high-income paediatric surgery and oncology centres. The aim of this paper is twofold: to describe the development of a medical student-led research collaboration; and to report on the experience of Wilms' tumour (WT). METHODS: This cross-sectional observational study is reported as per STROBE guidelines. Collaborating centres included three tertiary hospitals in Tanzania, Rwanda and the UK. Data were submitted by medical students following retrospective patient note review of 2 years using a standardised data collection tool. Primary outcome was survival (point of discharge/death). RESULTS: There were 104 patients with WT reported across all centres over the study period (Tanzania n = 71, Rwanda n = 26, UK n = 7). Survival was higher in the high-income institution [87% in Tanzania, 92% in Rwanda, 100% in the UK (X2 36.19, p < 0.0001)]. Given the short-term follow-up and retrospective study design, this likely underestimates the true discrepancy. Age at presentation was comparable at the two African sites but lower in the UK (one-way ANOVA, F = 0.2997, p = 0.74). Disease was more advanced in Tanzania at presentation (84% stage III-IV cf. 60% and 57% in Rwanda and UK, respectively, X2 7.57, p = 0.02). All patients had pre-operative chemotherapy, and a majority had nephrectomy. Post-operative morbidity was higher in lower resourced settings (X2 33.72, p < 0.0001). Methodology involving medical students and junior doctors proved time- and cost-effective. This collaboration was a valuable learning experience for students about global research networks. CONCLUSIONS: This study demonstrates novel research methodology involving medical students collaborating across the global south and global north. The comparison of outcomes advocates, on an institutional level, for development in access to services and multidisciplinary treatment of WT.

Development of a new reproductive tissue cryopreservation clinical service for children: the Oxford programme.

PURPOSE: This article describes the development of a new reproductive tissue cryopreservation clinical service for children at high risk of infertility in the NHS during times of severe financial constraints in the health service. METHOD: A development plan with two phases was drawn up. Phase 1 restricted the service to childhood cancer patients referred to the Oxford Paediatric Oncology and Haematology Principle Treatment Centre. It was estimated that there would be 10 patients/year and used existing staff and facilities from paediatric oncology, surgery, anaesthetics radiology, pathology, psychology, teenage-young adult gynaecology, and an existing Human Tissue Authority tissue bank with a licence for storage of tissue under a Human Sector Licence. Phase 2 extended the service to include children and young adults across England, Wales and Ireland-patients from Scotland having access to a research programme in Edinburgh. The main challenge in phase 2 being resources and the need for patients to be able to be treated as close to home as safely as possible. RESULTS: The Oxford team developed information resources and eligibility criteria based on published best practice, referral and treatment pathways, multidisciplinary team meetings, a network of third party sites, and a dedicated case management and database. As the programme expanded, the Oxford team was able to justify to management the need for a dedicated theatre list. Patient feedback through questionnaires, qualitative work conducted as part of a Ph.D. thesis as well as direct patient stories and interviews in TV, and radio features underpins the positive impact the programme has on patients and their families. CONCLUSION: The Oxford Reproductive Cryopreservation programme delivers fertility preservation treatment to children and young adults at high risk of infertility safely, effectively and as close to home as possible. The onward view is to apply for national funding for this programme for recognition and sustainability.

Low-fidelity Paediatric Surgical Simulation: Description of Models in Low-Resource Settings.

BACKGROUND: Surgical simulation is an important aspect of competency-based training. Recent trends in paediatric surgical simulations have migrated towards high-fidelity simulation with advanced technology resulting in models which are expensive and largely inaccessible in low- and middle-income countries. METHODS: This article describes four wet simulation models of common surgical procedures in paediatric population created with animal tissue from local abattoir. The models are designed to provide a framework for others to make the models and benefit from the training opportunity they provide especially in low-middle-income countries. RESULTS: The models created in the wet laboratory are neonatal bowel anastomosis, duodenoduodenostomy for discrepancy anastomosis, gastrostomy and pyeloplasty. These models are easily reproducible in resource-challenged healthcare setting as they are low cost, utilise locally available resources and require only a basic set of surgical instruments with which to perform the procedures. CONCLUSION: These models provide locally accessible material for sustainable training programmes which are fundamental in developing safe and affordable surgical care worldwide.

Post-natal outcomes of antenatally diagnosed intra-abdominal cysts: a 22-year single-institution series.

PURPOSE: The aim of this study was to establish the post-natal diagnosis and outcome of antenatally diagnosed intra-abdominal cysts between 1991 and 2013 at our institution. METHODS: All antenatally diagnosed intra-abdominal cysts between 1991 and 2013 were identified using a foetal anomaly database. The cysts were monitored for resolution. In all cases where the cyst had not resolved antenatally, additional post-natal scans were conducted. Antenatal diagnosis, post-natal diagnosis and outcomes were also recorded. RESULTS: 118 cases of antenatal intra-abdominal cysts were identified over the 22-year study period with a 98 % live birth rate. The overall accuracy of an antenatal diagnosis at our institution was 92 %. 26 cases (22 %) resolved spontaneously in utero, the majority of which (77 %) were ovarian in nature. Four tumour cases were identified in the series, which included two neuroblastomas, one yolk sac tumour and one teratoma. 90 cysts persisted post-natally with 52 % requiring surgery. These primarily included choledochal and enteric duplication cysts as well as symptomatic solid organ cysts. Diagnostic revision was limited to 8 % of cases over the study period with an overall improvement over the last decade. Overall, 40 % of all antenatally diagnosed cysts required surgical intervention. In those cysts that persisted post-natally, 52 % required surgery. CONCLUSIONS: A fifth of prenatally diagnosed intra-abdominal cysts will resolve with most ovarian cysts regressing in utero. Half of all persistent cysts will, however, require surgical intervention. These data are useful for prenatal counselling and demonstrates the important role played by the paediatric surgeon in the overall management of intra-abdominal cysts.

Anorectal atresia with gross terminal colonic distension in Africa.

UNLABELLED: Anorectal malformation (ARM) is a group of significant birth defects with geographic variation in incidence, individual phenotypes and regional geographic subtypes occurring in approximately 1:5,000 live births. Anorectal atresia with gross terminal colonic distension in the presence of an absent anal canal has also been known as 'pouch colon' which is mainly associated with the Indian subcontinent. Its prevalence is unknown but it is rare, possibly representing a fraction of 1 % of ARM. The problem seems to revolve around the gross terminal distension of the distal pouch which remains a significant surgical challenge to surgeons resulting in poorer postoperative continence in many instances. AIM: This study set out to collate data on ARM patients with gross terminal distension of the distal pouch from Africa to evaluate its epidemiology and outcome in African patients. METHODS: Fifteen African paediatric surgical centres (7 South African and 8 African centres) were polled on the occurrence of anorectal atresia with gross terminal distension of the terminal bowel, an ARM variant. Data included ethnic group, age, gender as well as the anatomical pathology, classification and presence or absence of associated anomalies. RESULTS: Of 12 respondents, 8 (67 %) responded and sufficient data to classify and analyse were obtained from six of the eight positive replies (7 new cases). Abdominal X-ray showed a grossly dilated terminal portion of the colon in the presence of an imperforate anus. A colovesical fistula was observed in four (three males and one female cloaca). Three were associated with a colonic atresia, of which two were in the transverse colon and one in the sigmoid colon. Surgical corrective procedures were carried out in six, but one patient, with a cloaca, died prior to surgical correction. CONCLUSIONS: In this survey of 15 African centres, we have attempted to document the occurrence and presentation of anorectal atresia with gross terminal distension in Africa and report seven additional new cases. A possible association with associated conditions like colonic atresia requires further investigation.

Rectal biopsy for Hirschsprung's disease--are we performing too many?

BACKGROUND: Rectal biopsy is considered the gold standard for the diagnosis of Hirschsprung's disease. The aim of this study was to evaluate the outcome of rectal biopsies performed in our institution, and to determine whether we are performing an adequate number of biopsies in patients presenting with features suggestive of this disease. METHODS: A retrospective analysis was conducted of patients who underwent rectal biopsy to exclude Hirschsprung's disease over a seven year period between 2000 and 2006. The histological diagnosis of Hirschsprung's disease was made using haematoxylin and eosin as well as acetylcholinesterase on frozen section. Patients were grouped into three age categories: neonates (group A), infants (group B) and those over 1 year of age (group C). The results of the biopsies were compared between groups. RESULTS: A total of 668 patients underwent rectal biopsy. 18 samples were insufficient. Based on the histological studies of 650 suitable samples, 73 (11%) were positive and 577 (89%) were negative for Hirschsprung's disease. Of the 73 positive biopsies, 34 (47%) were from group A, 20 (27%) from group B and 19 (26%) from group C. The percentage of positive biopsies was much higher in group A with 29% (34 out of 118) compared to group B with 15% (20 out of 135) and group C with 5% (19 out of 395). Three complications of minor rectal bleeding occurred. CONCLUSIONS: With 3 complications and 18 insufficient samples out of 668, rectal biopsy is a safe procedure and remains the gold standard for the diagnosis of Hirschsprung's disease, despite the large number of negative biopsies. Contrary to some reports in the literature which question the need for rectal biopsy in those presenting after the neonatal period, 53% of our positive diagnoses were made in children presenting after this period, with 19 positive biopsies out of 395 (5%) performed in children above the age of 1 year.

Finding vessels--accuracy of CT for identifying anomalous feeding vessels in patients with congenital cystic lung lesions.

INTRODUCTION: Contrast computer tomography (CT) scanning is the investigation of choice for the further assessment of suspected cystic congenital lung lesions (CCLL). Its use to identify the presence of anomalous feeding vessels supplying the lesion is well documented, but data regarding its accuracy is limited. This study compares CT results to operative and pathological findings to determine the accuracy of CT in identifying these anomalous vessels. METHODS: 51 consecutive cases of cystic congenital lung lesions managed in one hospital by a single consultant were reviewed. All cases had contrast CT scans performed preoperatively, as standard practice in this institution. We compared the results of these CT scans to the macroscopic appearance at surgery and histological findings postoperatively. We also compared the results of 2 CT protocols used in our institution between 1999-2007 and 2007-2009, respectively. RESULTS: Anomalous vessels were reported on CT in 9 cases. All but 1 had concordant operative and pathological findings. In the remaining 42 cases, no anomalous vessels were seen on CT. Of these, 9 cases were found to have an anomalous blood supply at surgery, 6 of which were hybrid lesions and 3 isolated sequestrations. The specificity of CT in identifying feeding vessels in the study was 97% (95% CI: 0.83-0.99) and the sensitivity was 47% (95% CI: 0.23-0.71). The positive predictive value was 89% (95% CI: 0.50-0.99) and negative predictive value 79% (95% CI: 0.62-0.89). The most recent protocol yielded an improved sensitivity of 75% (95% CI: 0.22-0.98) and a specificity of 100% (95% CI: 0.46-1.0) with a 100% (95% CI: 0.31-1.0) positive and 83% (95% CI: 0.36-0.99) negative predictive value. CONCLUSION: CT is a specific investigation for identifying anomalous vessels in CCLL but lacks sensitivity, leading to a relatively low negative predictive value. This emphasises the need in every case to look for anomalous vessels at surgery to avoid morbidity and potential mortality. An improved protocol for CT scans leads to improved specificity and sensitivity and predictive values.

An unusual cause of caecal perforation in a child.

Faecolith impaction leading to caecal perforation is a rare cause of acute abdominal pain in children. We present a case of an 11-year-old boy who was admitted to our department with a perforated caecum caused by faecolith impaction. Histology demonstrated a normal appendix with ganglion cells present. No mechanism of action could be detected for the faecolith impaction. This is a previously unreported cause of caecal perforation in children.

Patent processus vaginalis: a window to the abdomen.

A patent processus vaginalis (PPV) allows a communication between the peritoneum and scrotum. Hydrocoele is the usual presentation of a PPV in children. However, with intraabdominal pathology the patent PPV may provide the first clue to the mischief within the abdomen. We present here two unusual cases associated with a PPV and migration of intraabdominal contents from the abdomen to the scrotum.

Duodenal atresia: associated anomalies, prenatal diagnosis and outcome.

BACKGROUND: The diagnosis of duodenal atresia is commonly made prenatally, either as an isolated lesion or due to its association with other chromosomal abnormalities (Robertson et al. in Semin Perinatol 18:182-195, 1994; Hemming and Rankin in J Prenat Diagn 27:1205-1211, 2007). The aim of this study was to describe the prevalence of associated anomalies, prenatal diagnostic accuracy and survival of cases of congenital duodenal atresia in our institution. METHODS: All cases of duodenal atresia registered with our local congenital anomaly register over a 10-year period, 1995-2004 inclusive, were studied, including those resulting in termination of pregnancies, stillbirths, intrauterine deaths and neonatal deaths. To ensure high-case ascertainment, data were cross checked with prenatal ultrasound, cytogenetic laboratory, pathology department and neonatal surgical data base. Data were analysed for associated anomalies, accuracy of prenatal diagnosis and neonatal outcomes. RESULTS: A total of 65 patients were initially diagnosed as having duodenal atresia, of these 4 were subsequently excluded (1 postnatal normal bowel and 3 high jejunal atresias). In the remaining 61 cases, 35 (57%) had an association with other congenital abnormalities and 26 (43%) were isolated anomalies. Thirty-five were male and 26 female (M:F = 1.4:1). Twenty-one out of 29 (72%) patients prenatally diagnosed, compared with 14 out of 32 (44%) patients diagnosed postnatally had associated anomalies. Duodenal atresia was suspected on routine prenatal ultrasonography at 20-week gestation in 33 cases and confirmed in 29 (48%) cases with 4 false-positive diagnoses (1 normal bowel and 3 high jejunal atresias). No prenatal diagnosis was made in 32 (52%) babies. Of the 61 cases, 53 were live births with 2 early neonatal deaths (1 cardiac and 1 VACTERL), 5 terminations, 2 intrauterine deaths and 1 stillbirth (Fig. 3). Overall neonatal survival was 96% (51 cases). Mortality in the group diagnosed prenatally was 34 % (10 cases). CONCLUSION: This study shows an overall increased association of duodenal atresia with Down's syndrome. In the group diagnosed prenatally, mortality as well as the association with other congenital anomalies was found to be higher. We have demonstrated a greater prenatal diagnostic accuracy, but confirm postnatal outcomes similar to previous studies.

Management of congenital cystic adenomatous malformations of the lung.

Congenital cystic adenomatous malformation of the lung (CCAM) is a rare lung lesion easily diagnosed on prenatal scan. The pathology of fetal lesions differs from postnatal lesion, hence the need for separate classifications during the different stages of development. Fetuses with CCAMs and hydrops have a poor prognosis and may be candidates for prenatal intervention where available. Most prenatally diagnosed CCAMs have a favourable outcome. Early surgery is required for symptomatic babies. Management of prenatally diagnosed asymptomatic lesions remains controversial, with the options of conservative management with CT scan surveillance or surgical excision. Surgical excision is favoured by many centres because of the risk of infection and malignant transformation if the CCAM remains in situ. Surgical outcomes are excellent.

Manejo de la malformación congénita adenomatosa quística del pulmón / Management of cystic adenomatous malformation of the lung

La malformación congénita adenomatosa quística del pulmón es una alteración rara dentro de las malformaciones broncopulmonares, con una incidencia de 1/10000 a1/35000; pero son las lesiones quísticas más comunes detectadas en las ecografías de control prenatal.

Congenital lung lesions: classification and concordance of radiological appearance and surgical pathology.

Congenital lung lesions are diagnosed antenatally in the majority of cases. Postnatal management includes chest radiography and CT-scanning, followed by either surgical resection or CT surveillance. Pre-operatively, lesions are often "labelled" as CCAM (and the Stocker classification incorrectly applied), sequestration, or lobar emphysema, and their frequent "hybrid" nature sometimes missed. The aim of our study was to correlate antenatal and postnatal radiological diagnoses and classification of congenital lung lesions with surgical and pathological outcome. Twenty-six consecutive cases of antenatally-diagnosed cystic lung lesions managed at our centre between January 2003 and April 2007 were reviewed. Diagnoses were: cystic adenomatoid malformations (CCAM) in 13 cases (50.0%), three bronchopulmonary sequestrations (11.5%), "hybrid" lesions in eight cases (30.8%), one isolated lobar emphysema (3.8%) and a normal lung segment with anomalous systemic supply (3.8%). Overall concordance rate between prenatal ultrasound and resection pathology was 61.5%, whereas CT findings correlated with pathology in 65.4% of cases. Incorrect radiological classification resulted in one morbidity, when an attempted thoracoscopic resection of a presumed CCAM had to be converted to an open procedure due to bleeding from an unsuspected anomalous vessel. The common embryological origin of these lesions, and the need for standardisation of reporting is discussed. We propose that lesions should be classified anatomically according to their nature, arterial supply and venous drainage in order to reduce discrepancies between radiological, intra-operative and pathological reporting.

Epidermal growth factor in neonatal saliva.

AIMS OF STUDY: Epidermal growth factor (EGF) is an amino acid polypeptide that has been shown to promote mucosal healing and intestinal growth in experimental models and has a potential role in the aetiology and treatment of NEC. The aim of this study was to determine the normal levels of salivary EGF in a cohort of healthy neonatal infants. MATERIAL AND METHODS: With appropriate ethical approval and informed consent, saliva was collected using a suction catheter from all normal neonatal infants admitted to our unit over a 9-month period. The samples were immediately frozen at - 20 degrees C and analysed using an ELISA assay (R & D Systems, Oxford, UK). Samples taken a week prior to and two weeks following a septic episode (as diagnosed by clinical or biochemical evidence) were excluded. Patients with congenital malformations and chromosomal anomalies were excluded. RESULTS: A total of 65 samples were collected and analysed from 27 babies. The mean gestation and weight of the patients was 32.2 (+/- 5.50) weeks and 1954.44 (+/- 1045.7) grams, respectively. Five samples from 2 infants were discarded. None of the patients in whom the samples were analysed had clinical or serum markers of sepsis or NEC. All infants were given maternal breast milk starting at the mean age of 5 (+/- 3.47) days. The mean EGF values were 676.95 (+/- 168.47) pg/ml. There was a significant rise in serial salivary EGF values in patients (p = 0.0019). There was no correlation between the EGF values and gestation (R = 0.35, p = 0.08). The birth weight, sex or timing of initiating enteral feeds did not correlate with EGF values. CONCLUSION: In conclusion, our study shows a rise in salivary EGF values in serial samples in a clearly defined group of healthy infants.

A comparison of neonatal surgical admissions between two linked surgical departments in Africa and Europe.

Resources for neonatal surgery vary hugely between the developed countries and Africa. Due to the burden of other childhood diseases, neonatal surgery is of low priority to health-care budget holders in the developing countries. Admissions to two linked neonatal surgical institutions in Africa and Europe are compared to draw attention to the need of newborn care in developing countries. In a 3-year period, there have been 528 neonatal surgical admissions to the Kilimanjaro Christian Medical Centre (KCMC), Tanzania. During the same period, 707 surgical neonates were admitted to the John Radcliffe Hospital, UK (JRH). Conditions more commonly seen in the JRH include necrotising enterocolitis (NEC) (10.2 vs 0%), gastrochisis (5.5 vs 0.9%), congenital diaphragmatic hernia (2.4 vs 0.4%), congenital lung cysts (1.6 vs 0.4%), meconium ileus (2.4 vs 0%), malrotation (2.7 vs 0.6%) and abdominal/pelvic cysts (1.1 vs 0%). Conditions more commonly seen in KCMC include anorectal malformation (9.5 vs 4.7%), sacrococcygeal teratoma (2.3 vs 0.3%), Hirschsprung's disease (10.2 vs 4.5%), branchial fistula (0.9 vs 0%), haemangioma (2.1 vs 0%) and cystic hygroma (2.1 vs 1.0%). The spectrum of neonatal surgical admissions in our two institutions varied. We believe this is attributable to availability of antenatal diagnosis, primary health-care, transport facilities, genetics as well as survival of delayed presentation. This study demonstrates the extent and spectrum of neonatal surgical pathology in this part of Africa and highlights the need for newborn surgical care in developing countries.