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.

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.

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.