Presentations of bladder exstrophy in a resource-limited setting and the role of Mainz II continent diversion for late referrals or failed primary closures: a multicentric report.

Primary closure techniques that have been updated and longterm follow-up for CBE (classic bladder exstrophy) may be out of reach for many patients living in resource-limited settings. Late referrals to medical care and primary closures that lack the necessary skills and facilities for comprehensive treatment are still common. Alternative and long-term surgical solutions may improve the lives of these unfortunate patients. During surgical outreach missions, patients with CBE, either non-operated or with a previous unsuccessful bladder closure, who were referred from vast under-resourced rural areas to three Eastern African hospitals, were studied. The following information is provided: mode of presentation, clinical history, diagnostic workout, management, and outcome. There were 25 cases (M/F ratio 17/8) ranging in age from two days to twenty years. Five of the seventeen patients who were not treated (35%) were under 120 days old and eligible for primary closure in a qualified tertiary center when one was available in the country. There were twelve late referred cases (ranging from 120 days to 20 years). Between the ages of ten months and twelve years, eight children arrived following a failed primary closure. In all of them, the bladder plate was too altered to allow closure. Following a preoperative diagnostic workout, a Mainz II continent internal diversion was proposed to fourteen patients with acceptable bowel control and postponed in the other three. Three cases were lost before treatment because parents refused the procedure. Twelve cases ranging in age from three to twenty years (mean seven years) were operated on. Eight people were followed for a total of 53.87 months (range: 36-120). Except for three people who complained of occasional night soiling, day and night continence were good. The average voiding frequency during the day was four and 1.3 at night. There was no evidence of a metabolic imbalance, urinary infection, or significant upper urinary tract dilatation. Two fatalities could not be linked to urinary diversion. Four patients were not followed up on. Due to the limited number of specialist surgical facilities, CBE late referral or failed closure is to be expected in a resource-limited context. In lieu of the primary closure, a continent internal diversion will be proposed and encouraged even at the level of a non-specialist hospital to improve the quality of life of these unfortunate patients. It is recommended that patients be warned about the procedure's potential long-term risks, which will necessitate a limited but regular follow-up.

Development of a template for operative reporting of pediatric cancer surgery in limited-resource settings by using a modified Delphi method.

PURPOSE: A comprehensive operative report for cancer surgery is crucial for accurate disease staging, risk stratification, and therapy escalation/de-escalation, which affects the outcome. Narrative operative reports may fail to include some critical findings. Furthermore, standardized operative reports can form the basis of a local registry, which is often lacking in limited-resource settings (LRSs). In adult literature, synoptic operative reports (SOR) contain more key findings than narrative operative reports. In the LRSs, where the capacity of diagnostic pathology services is typically suboptimal, the value of a thorough operative report is even greater. The aim of this study was to develop a SOR template to help standardize childhood cancer surgery reporting in LRSs. METHODS: Twenty-three experts in pediatric cancer with extensive experience practicing in LRSs were invited to participate in a modified Delphi procedure. SOR domains for pediatric oncology surgery were drafted based on a literature search and then modified based on experts' opinions. The experts anonymously answered multiple rounds of online questionnaires until all domains and subdomains reached a consensus, which was predefined as 70% agreement. RESULTS: Sixteen experts participated in the study, and two rounds of the survey were completed. Twenty-one domains were considered relevant, including demographics, diagnosis, primary site, preoperative disease stage, previous tumor biopsy or surgery, preoperative tumor rupture, neoadjuvant therapy, surgical access, type of resection, completeness of resection, tumor margin assessment, locoregional tumor extension, organ resection, intraoperative tumor spillage, vascular involvement, lymph node sampling, estimated blood loss, intraoperative complications and interventions to address them, specimen names, and specimen orientation. CONCLUSION: We developed a SOR template for pediatric oncology surgery in LRSs. Consensus for all 21 domains and associated subdomains was achieved using a modified Delphi procedure.

Pitfalls and technical errors in the first approach to neonates with anorectal malformations in a non-specialist context: can we do any better? A review from three Eastern African Centres.

INTRODUCTION: In sub-Saharan Africa, Anorectal malformations (ARM) are the most frequent cause of neonatal obstruction. Referral to a Pediatric Surgeon is frequently delayed. The first treatment is often delivered at not specialist level and mismanagement may result. AIM: To study ARM patients referred beyond neonatal period and managed at a non-specialist level. MATERIALS AND METHODS: One hundred and thirty patients were included (M/F ratio 63/67) among 144 admitted to three Eastern African Hospitals with Pediatric Surgical facilities. Demographics, type of anomaly, delay on referral, previous management, most commonly observed errors are reported. RESULTS: The Mean age at referral was 23 months (range five weeks - 23 years). Colostomy was the most frequent surgery (92 cases). Stomas often did not follow the recommended criteria. Ten per cent were not on the sigmoid, and 35% were not divided. "Loop" or "double-barrel" colostomies did not exclude the distal loop. Inverted (10,5%), prolapsed stomas (7,5%), short distal loop (16%) were observed. Twenty-four cases (26%) needed redo. Primary perineal exploration in eight patients resulted in incontinence. CONCLUSIONS: Investments on training practitioners, acting at District/Rural level, and closer links with tertiary centres are recommended to avoid ARM mismanagement and delayed referral to a Specialist.

Challenges, constraints and failures that are related to the posterior sagittal anorectoplasty approach to anorectal malformations in a low-resource context: An experience from a sudanese tertiary referral centre.

BACKGROUND: Anorectal malformations (ARMs) in the sub-Saharan Africa are a common cause of neonatal referral for intestinal obstruction, and the posterior sagittal anorectoplasty (PSARP) approach is rapidly spreading. The small number of paediatric surgeons and the low-resource context limit children's access to care and constrain the quality of results. A retrospective, observational study has been done on a consecutive series of ARM cases admitted to a Sudanese tertiary paediatric surgical centre within the framework of a partnership between Italian and Sudanese academic institutions addressed to review and upgrade the standard of care of major congenital anomalies. MATERIALS AND METHODS: The authors collected 94 ARM cases in a 3 years' period. Conditions on referral, operative procedures, post-operative course and follow-up were recorded and examined. Their correlations with complications and outcome were analysed. RESULTS: The male/female ratio was 47/47. Eighty patients presented with an untreated ARM; 66 had a divided stoma and 14 had already a PSARP procedure, followed by a poor outcome or sequelae. In 25% of the cases, colostomy required re-doing. In 57 cases, a staged PSARP (primary or re-do) was done. Surgical-site infections occurred in nine patients. Some patients were lost to follow-up after preliminary colostomy. Post-operative dilatation programme suffered from the lack of systematic follow-up, and colostomy closure was possible in 46% of the cases due to problems in travelling and accessing hospital care. Anal stenosis was frequently observed among unfollowed patients. CONCLUSION: Despite PSARP's widespread adoption in Africa, the risk of complications and failures is high. Primary management is often inappropriate, and a high rate of colostomy-related complications is observed. Poverty and lack of transportation reduce attendance to follow-up, hampering the final results. Investments in healthcare facilities and retention of trained health providers are needed to improve the standard of care.

Neuroblastoma in Sudan: experience of a single institute.

Neuroblastoma (NBL) is the most common malignant solid extracranial tumour in children. It accounts for about 8%-10% of overall childhood malignancies in developed countries and only 1%-3% in developing countries. We aim to study the clinical presentation and outcome of NBL in children treated at the National Cancer Institute (NCI), University of Gezira, Wad Madani, Sudan. This is a retrospective, descriptive, hospital-based study, where patients' records were reviewed from the years 2004-2015. During this period, 38 paediatric patients were treated in the Paediatric Oncology Unit at the NCI, accounting for about 4% of the overall childhood malignancies. Of them, 24 (63%) were males and 14 (37%) were females with a ratio of 1.7: 1. The age at presentation ranged between 2 months and 7 years with a mean age at the presentation of 3 years. Twenty-nine (76%) of them were classified as high-risk disease, no one received high-dose chemotherapy, 33(87%) died and only 5 (13%) achieved complete remission. This study reflects the poor outcome of NBL among Sudanese children which can be explained by the late presentation of the patients, lack of the diagnostic modalities and lack of the sophisticated treatment modalities for high-risk NBL.

Improving standard of pediatric surgical care in a low resource setting: the key role of academic partnership.

BACKGROUND: An epidemiological transition is interesting Sub-Saharan Africa increasing the burden of non-communicable diseases most of which are of surgical interest. Local resources are far from meeting needs and, considering that 50% of the population is less than 14 years of age, Pediatric surgical coverage is specially affected. Efforts are made to improve standards of care and to increase the number of Pediatric surgeons through short-term specialist surgical Missions, facilities supported by humanitarian organization, academic Partnership, training abroad of local surgeons. This study is a half term report about three-years Partnership between the University of Chieti- Pescara, Italy and the University of Gezira, Sudan to upgrade standard of care at the Gezira National Centre for Pediatric Surgery (GNCPS) of Wad Medani. Four surgical Teams per year visited GNCPS. The Program was financed by the Italian Agency for Development Cooperation. METHODS: The state of local infrastructure, current standard of care, analysis of caseload, surgical activity and results are reported. Methods utilized to assess local needs and to develop Partnership activities are described. RESULTS: Main surgical task of the visiting Team were advancements in Colorectal procedures, Epispadias/Exstrophy Complex management and Hypospadias surgery (20% of major surgical procedures at the GNCPS). Intensive care facilities and staff to assist more complex cases (i.e. neonates) are still defective. Proctoring, training on the job of junior surgeons, anaesthetists and nurses, collaboration in educational programs, advisorship in hospital management, clinical governance, maintenance of infrastructure together with training opportunities in Italy were included by the Program. Despite on-going efforts, actions have not yet been followed by the expected results. More investments are needed on Healthcare infrastructures to increase health workers motivation and prevent brain drain. CONCLUSIONS: The key role that an Academic Partnership can play, acting through expatriated Teams working in the same constrained contest with the local workforce, must be emphasized. Besides clinical objectives, these types of Global Health Initiatives address improvement in management and clinical governance. The main obstacles to upgrade standard of care and level of surgery met by the Visiting Team are scarce investments on health infrastructure and a weak staff retention policy, reflecting in poor motivation and low performance.

Wilms tumour in Sudan.

BACKGROUND: Wilms tumour is one of the commonest childhood solid tumours which has an excellent outlook in the developed world with 5-year overall survival exceeding 90%. There is little information from Sudan regarding Wilms tumour. PROCEDURE: Records of patients with Wilms tumour diagnosed and treated at Institute of Nuclear Medicine, Molecular Biology and Oncology (INMO) in the University of Gezira from May 1999 to June 2007 were reviewed. RESULTS: Thirty-seven children presented at a mean age of 4.1 years (range 2 months-13 years). The male to female ratio was 0.9-1. Abdominal swelling or mass was the commonest symptom. There was 1 child with Stage I (2.7%), 7 with stage II (18.9%), 25 with Stage III (67.6%) and 4 with Stage IV (10.8%). Following diagnosis 27% of children did not receive further treatment (5.4% died prior to treatment, 5.4% were not able to finance treatment and for the rest 16.2% no cause was identified). More than half of the children did not have a nephrectomy and only 4 (11%) completed treatment. CONCLUSIONS: The poor outlook is related to several factors. Delayed presentation, poor awareness of treatment options, lack of finances, no provision of food, lodging and transport, absence of paediatric trained staff are the obstacles to better outcomes. Empowering parents with information, giving chemotherapy prior to nephrectomy, training staff and establishing links with a tertiary cancer centre in the developed world are some of the options to improve survival.

Evaluation of ureteral reimplantation in 65 sudanese patients.

The evaluation of the complications of uretero-neocystostomy is very scant in the literature. The objectives of this study were to determine the indications for uretero-neocystostomy and to evaluate the complications of different techniques. This retrospective descriptive study was conducted in the Gezira Hospital for Renal Diseases and Surgery, Sudan between January 2001 and January 2004. A total of 65 patients were enrolled in this study. All of them underwent reimplantation of either one or both ureters for various indications. The methods of reimplantation were direct, Lich Gregoire, Cohen, Boari's and ileum substitution. The mean follow-up period was18 months. The 65 patients underwent a total of 77 reimplanted ureters. Of them, 39 ureters were gynecological ureters, 21 were renal transplant and 17 others had miscellaneous indications. The direct method was used for 25 transplanted cases (27.5%), Lich Gergoire for 40 patients (51.9%), Cohen for eight patients (10.4%) and Boari's for seven (9.0%). Leakage and lower urinary tract symptoms (LUTS) were diagnosed in four patients each (4.8%), two had hydronephrosis while one each had obstruction and necrosis. Our study indicates that gynecological ureter was the leading indication for ureteral reimplantation and the incidence of complications was comparable to the literature.