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.

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.

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.

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.

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.

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.

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.

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.

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.

Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years' experience 1991-2001.

OBJECTIVE: To determine the outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung (CCAM) over a 10 year period. METHODS: This is a retrospective study of all babies diagnosed antenatally in the Prenatal Diagnosis Unit and delivered in Oxford between 1991 and 2001. Data were obtained from the Oxford Congenital Anomaly Register, theatre records, and histopathology reports. RESULTS: Twenty eight cases of CCAM were diagnosed antenatally. Five pregnancies were terminated. Data are available on all 23 of the pregnancies that continued and resulted in two neonatal deaths and 21 surviving babies. Eleven of the 23 cases (48%) showed some regression of the lesion antenatally, and four of these cases appeared to resolve completely on prenatal ultrasound. Three of the 23 babies (13%) were symptomatic in the early neonatal period, and three developed symptoms shortly afterwards. Seventeen of the 23 babies (74%) were asymptomatic, of whom 12 had abnormalities on chest radiograph or computed tomography scan and had elective surgery. Two babies (8%) had completely normal postnatal imaging, and three had abnormalities which resolved in the first year of life. Seventeen of the 23 babies (74%) had surgery. Histology at surgery was heterogeneous. Of the 23 live births, all 21 survivors (91%) are well at follow up or have been discharged. CONCLUSIONS: All babies diagnosed antenatally with CCAM require postnatal imaging with computed tomography irrespective of signs of antenatal resolution. In asymptomatic infants, the recommendations are close follow up and elective surgery for persistent lesions within the first year of life. Histology at surgery was heterogeneous, and this should be considered when counselling parents.

Magnetic resonance imaging of intestinal necrosis in preterm infants.

BACKGROUND AND OBJECTIVE: Noninvasive diagnosis of intestinal necrosis is important in planning surgery in preterm infants with necrotizing enterocolitis (NEC). We aimed to assess the potential of magnetic resonance imaging (MRI) for the diagnosis of intestinal necrosis. STUDY PARTICIPANTS AND METHODS: Abdominal MRI scans were performed in a group of preterm infants with suspected NEC and compared with surgical findings and to MRI results in a group of control infants. In addition, MRI was performed in 2 preterm infants with suspected NEC who did not require surgery. RESULTS: Six infants with a median birth weight of 1220 g (range, 760-1770 g) and median gestational age at birth of 30 weeks (range, 28-34 weeks) were studied at a median postnatal age of 10 days (range, 4-19 days). Four infants had a bubble-like appearance in part of the intestinal wall, intramural gas, and an abnormal fluid level within bowel lumen. At surgery, NEC was found in 5 infants and sigmoid volvulus in 1. The site of the bubble-like appearance corresponded to the site of intestinal necrosis at surgery. Four control infants with a median birth weight of 1500 g (range, 730-2130 g) and a median gestational age of 31 weeks (range, 26-36 weeks) had abdominal MRI at a median postnatal age of 8 days (range, 4-70 days). None of the above findings were seen in any control infant. The bubble-like appearance was not seen in the 2 infants with suspected NEC who did not require surgery. CONCLUSION: Abdominal MRI allows the noninvasive diagnosis of bowel necrosis. This may aid the timing of surgical intervention in preterm infants with a clinical diagnosis of NEC.gangrene, ischemia, MRI, necrotizing enterocolitis.

Anti-reflux surgery for the neonatal intensive care-dependent infant.

AIM: The benefit of anti-reflux surgery for gastro-oesophageal reflux (GOR) in early infancy is uncertain. The aim of this study was to assess the value of Nissen fundoplication in a group of infants with severe GOR. METHODS: 10 infants underwent Nissen fundoplication for gastro-oesophageal reflux following a failure of medical management. All had suffered life-threatening respiratory episodes as a consequence of gastro-oesophageal reflux, and were neonatal intensive care-dependent. Median (range) birth weight was 1.26 kg (0.48-3.8 kg), gestation 30 weeks (25-38 weeks); at surgery, weight was 3.25 kg (2.5-6.1 kg) at a corrected age of 11.5 weeks (term-22) weeks. For each infant, the success of enteral feeding and the level of support required pre- and post-operatively was compared, and where appropriate, the facilitation of palliative feeding was assessed. Median follow-up was 14 (3-36) months. RESULTS: No infant suffered intra-operative morbidity or mortality. One infant died within 1 month of surgery from a collapse unrelated to surgery. Eight of nine surviving infants were discharged from intensive care following extubation and the establishment of enteral feeding. One patient died of severe bronchopulmonary dysplasia 3 months post-surgery. One infant developed a gastrostomy site infection, and two required gastrostomy tube replacement within 6 months of surgery. All were thriving at follow-up. Two infants with a congenital myopathy died as a result of their muscle disease at 9 and 11 months post-operatively. CONCLUSIONS: Nissen fundoplication is a feasible, effective and safe operation in severe gastro-oesophageal reflux unresponsive to medical treatment in term and pre-term infants. It has an additional important role in facilitating safe palliative enteral feeding in infants with a diagnosis incompatible with survival into adulthood.

Immunoreactive epidermal growth factor receptors are present in gastrointestinal epithelial cells of preterm infants with necrotising enterocolitis.

INTRODUCTION: Epidermal growth factor (EGF) affects epithelial cell proliferation, differentiation and migration in the gastrointestinal tract of experimental animals, and increases proliferation when given intravenously to children with congenital microvillous atrophy or necrotising enteritis. The aim of the present study is to determine whether EGF receptors (EGFR) are present in the gut epithelium of preterm infants, and to discover whether neonatal necrotising enterocolitis (NEC) is associated with the absence of EGFR from mucosal cells. METHODS: Tissues were taken from involved colon and small intestine of four preterm infants with NEC, and control tissues were taken from four other neonates who had laparatomies for congenital malformations. Sections of the tissues were examined histopathologically after treatment with a monoclonal antibody against the external domain of the EGFR (Zymed Laboratories, San Francisco, CA, USA). RESULTS: Histopathological examination confirmed diagnosis of NEC in the involved bowel and controls showed appearance within normal limit. Immunoreactive EGFR were present on the epithelial cells of both the colon and small intestine, localised on the basolateral membrane of the cells of both subject and the controls. There was no apparent reduction in expression compared with controls. CONCLUSION: NEC in preterm infants is not associated with absence of EGFR. The presence of EGFR in gut epithelial cells raises the possibility of using EGF for prophylaxis or treatment of NEC.

Mediastinal teratomas: review of 15 pediatric cases.

One hundred fifty-three children with a teratoma presented to one hospital between 1970 and March 1992. The clinical and pathological features of 15 patients with mediastinal teratomas are reviewed; six were newborn and nine aged from infancy to 13 years. Thirteen patients including the six newborns presented with respiratory distress and all 15 patients had a mass on chest radiograph. A definite diagnosis of teratoma was not made preoperatively in any of these patients. At operation, a median sternotomy was used to approach seven anterior tumors and a lateral thoracotomy performed in the other eight patients. Histologically two were mature, 10 had immature elements, and three were malignant teratomas. The patients with malignant tumors were all over 12 years of age and died within 6 months of treatment. All six neonates had immature teratomas. Raised serum alpha-fetoprotein levels provided useful markers in two patients with recurrent tumors. Three conclusions can be drawn: (1) mediastinal teratomas are rare in children and frequently are not diagnosed before operation; (2) in newborns these tumors may be immature and present with respiratory distress; and (3) a median sternotomy gives excellent exposure for anterior mediastinal tumors.

Thymectomy in black children with juvenile myasthenia gravis

Fifteen black children with juvenile myasthenia gravis presented to our institution over a 10-year period at ages ranging from 18 months to 7 years, 4 males and 11 females. Twelve presented with progressive, generalised weakness and 3 had bulbar manifestations. Ocular signs were absent in 2 patients. All 15 patients were placed on pyridostigmine bromide (Mestinon) and 1 also required steroids and plasmapheresis. Medical therapy only was instituted in 5 patients, of whom 2 came to surgery, 2 died, and 1 was lost to follow-up. Thymectomy was performed in 12 children (including the 2 with failed medical therapy) via a median sternotomy with no mortality or morbidity related to the operation. The timing of surgery was less than 3 months in 75% of the children. Thymic hyperplasia was noted in 10 glands and 2 were reported as normal. Of the children in the operative group, 83% are on minimal medication or in total remission. Better results were noted in young patients with early thymectomy and diseased glands.

Pancreatic leiomyosarcoma. A case report.

The pancreas is an unusual site for leiomyosarcoma. Only 12 such cases have previously been reported. A case of pancreatic leiomyosarcoma in a 68-year-old man is presented and the diagnostic dilemma and management of such a tumour are discussed.

Giant submandibular calculus. A case report.

A case of giant mandibular calculus, 6.5 X 5.5 cm, is presented. The management of this condition is reviewed and an explanation offered for the occurrence of these calculi.

Pancreatic injuries in children.

Pancreatic injuries over an 11-year period were reviewed. Pedestrian motor vehicle accidents accounted for 45% of the injuries. Diagnosis was made clinically and on raised serial serum amylase levels. Eighty per cent of the patients were managed conservatively. Two patients required emergency surgery for pancreatic trauma. There were no deaths. It is concluded that a conservative approach is successful in most paediatric patients with pancreatic trauma.