Outcomes of one-stage feminizing genitoplasty in children with congenital adrenal hyperplasia and severe virilization.

PURPOSE: To present our surgical experience and outcomes in congenital adrenal hyperplasia (CAH) patients with severe virilization using a combined technique of total urogenital mobilization (TUM) and a modified pull-through vaginoplasty to perform a safe and effective one-stage feminizing genital reconstruction for these children. METHODS: Fourteen CAH patients with severe virilization, defined by a Prader IV and V rating of the external genitalia, underwent TUM followed by a limited vaginal pull-through procedure from June 2016 to December 2020. Postoperative anatomical and cosmetic outcomes, and urinary continence, were evaluated. RESULTS: Out of the 14 cases in this study, 8 were classified as prader IV and 6 as Prader V. The median age at surgery was 11 months (range 6-36 months), and the mean urethral length was 1.4 cm (range 1.2-1.8 cm). The median follow-up period was 4 years. Our cosmetic outcomes were good in 11 (78.5%), satisfactory in 2, and poor in one case. All patients achieved age-appropriate toilet training without urinary incontinence. CONCLUSION: Adopting our surgical approach of TUM with modified pull-through vaginoplasty has simplified feminizing surgical reconstruction in CAH cases with severe genital atypia and a very high vaginal confluence with short urethral length, yielding adequate introitus with good anatomical and cosmetic appearance and adequate urinary continence outcomes.

International Study of the Epidemiology of Paediatric Trauma: PAPSA Research Study.

OBJECTIVES: Trauma is a significant cause of morbidity and mortality worldwide. The literature on paediatric trauma epidemiology in low- and middle-income countries (LMICs) is limited. This study aims to gather epidemiological data on paediatric trauma. METHODS: This is a multicentre prospective cohort study of paediatric trauma admissions, over 1 month, from 15 paediatric surgery centres in 11 countries. Epidemiology, mechanism of injury, injuries sustained, management, morbidity and mortality data were recorded. Statistical analysis compared LMICs and high-income countries (HICs). RESULTS: There were 1377 paediatric trauma admissions over 31 days; 1295 admissions across ten LMIC centres and 84 admissions across five HIC centres. Median number of admissions per centre was 15 in HICs and 43 in LMICs. Mean age was 7 years, and 62% were boys. Common mechanisms included road traffic accidents (41%), falls (41%) and interpersonal violence (11%). Frequent injuries were lacerations, fractures, head injuries and burns. Intra-abdominal and intra-thoracic injuries accounted for 3 and 2% of injuries. The mechanisms and injuries sustained differed significantly between HICs and LMICs. Median length of stay was 1 day and 19% required an operative intervention; this did not differ significantly between HICs and LMICs. No mortality and morbidity was reported from HICs. In LMICs, in-hospital morbidity was 4.0% and mortality was 0.8%. CONCLUSION: The spectrum of paediatric trauma varies significantly, with different injury mechanisms and patterns in LMICs. Healthcare structure, access to paediatric surgery and trauma prevention strategies may account for these differences. Trauma registries are needed in LMICs for future research and to inform local policy.

Neonatal surgery in Africa.

The management of neonatal surgical problems continues to pose considerable challenges, particularly in low-resource settings. The burden of neonatal surgical diseases in Africa is not well documented. The characteristics of some neonatal surgical problems are highlighted. Late presentation coupled with poor understanding of the milieu interior of the neonates by incompetent health care providers and poorly equipped hospitals combine to give rise to the unacceptable high morbidity and mortality in most parts of Africa. Proper training of all staff involved in neonatal health care coupled with community awareness must be vigorously pursued by all stakeholders. Various governments throughout the continent of Africa, in conjunction with international donor agencies, must not only provide an adequate budget for health care services and improve infrastructures, but must also deliberately encourage and provide funding for neonatal surgical care and research across the continent. The well-established pediatric surgical training programs, particularly in North and South Africa, should hold the moral responsibility of training all possible numbers of young surgeons from other African countries that do not have any existing pediatric surgical training programs or those countries suffering from remarkable shortage of trained pediatric surgeons.

Training and practice of pediatric surgery in Africa: past, present, and future.

The evolution and recognition of pediatric surgery as a specialty in Africa can be divided into 4 distinct phases, starting from early 1920s till the present. The pace of development has been quite variable in different parts of Africa. Despite all recent developments, the practice of pediatric surgery in Africa continues to face multiple challenges, including limited facilities, manpower shortages, the large number of sick children, disease patterns specific to the region, late presentation and advanced pathology, lack of pediatric surgeons outside the tertiary hospitals, and inadequate governmental support. Standardization of pediatric surgery training across the continent is advocated. Collaboration with well-established pediatric surgical training centers in Africa and other developed countries is necessary. The problems of delivery of pediatric surgical services need to be addressed urgently, if the African child is to have access to essential pediatric surgical services like his or her counterpart in the high-income parts of the world.

Challenges of training and delivery of pediatric surgical services in Africa.

BACKGROUND: The practice of pediatric surgery in Africa presents multiple challenges. This report presents an overview of problems encountered in the training of pediatric surgeons as well as the delivery of pediatric surgical services in Africa. METHODS: A returned structured self-administered questionnaire sent to pediatric surgeons practicing in Africa was reviewed and analyzed using SPSS version 11.5 (SPSS, Chicago, IL). RESULTS: Forty-nine (57%) of 86 questionnaires were returned from 8 countries. Great variability in the requirements and training of pediatric surgeons, even within the same country, was found. Many surgical colleges are responsible for standardization and board certification of pediatric surgeons across Africa. There were 6 (12%) centers that train middle level manpower. Twenty-six (53%) participants have 1 to 2 trainees, whereas 22 (45%) have irregular or no trainee. A pediatric surgical trainee needs 2 to 4 (median, 2) years of training in general surgery to be accepted for training in pediatric surgery, and it takes a trainee between 2 to 4 (median, 3) years to complete training as a pediatric surgeon in the countries surveyed. The number of pediatric surgeons per million populations is lowest in Malawi (0.06) and highest in Egypt (1.5). Problems facing adequate delivery of pediatric surgical services enumerated by participants included poor facilities, lack of support laboratory facilities, shortage of manpower, late presentation, and poverty. CONCLUSION: The training of pediatric surgical manpower in some African countries revealed great variability in training with multiple challenges. Delivery of pediatric surgical services in Africa presents problems like severe manpower shortage, high pediatric surgeon workload, and poor facilities. Standardization of pediatric surgery training across the continent is advocated, and the problems of delivery of pediatric surgical services need to be addressed urgently, not only by health care planners in Africa but by the international community and donor agencies, if the African child is to have access to essential pediatric surgical services like his or her counterpart in other developed parts of the world.

Delayed presentation of congenital diaphragmatic hernia.

The late-presenting congenital diaphragmatic hernia (CDH) represents a considerable diagnostic challenge. This study was undertaken to define various patterns of delayed presentation and to analyze pitfalls in the diagnosis and treatment of these patients. Thirty-three children with CDH were treated between 1993 and 2000; 15 of these (45.5%) who were diagnosed after the age of 2 months-14 years, median of 2.5 years are reported. Thirteen had a Bochdalek hernia and 2 had a Morgagni hernia. The diaphragmatic defect was right-sided in 6 cases and left-sided in 9. Five patients presented acutely, 3 with respiratory distress and 2 with gastrointestinal (GI) obstruction. The remaining 10 presented with chronic respiratory or GI complaints. Inappropriate insertion of a chest drain occurred in 3 patients misdiagnosed as having pleural effusion (2) and a pneumothorax (1). Two patients had previous chest radiographs reported normal. Plain radiographs were sufficient to make a definitive diagnosis in only 6 patients; GI contrast studies were necessary in the other 9. All patients were treated through an abdominal approach with primary closure of the diaphragmatic defect without a patch. A distinct hernia sac was present in 6 cases, and associated malrotation in 6. All except 1 patient survived the operation with rapid improvement of their GI and respiratory symptoms. We conclude that: (1) late-presenting CDH should be included in the differential diagnosis of any child with persistent GI or respiratory problems associated with an abnormal chest X-ray film; (2) nasogastric tube placement must be considered as an early diagnostic or therapeutic intervention when the diagnosis is suspected; and (3) GI contrast studies should be a part of the diagnostic work-up of these patients.

Transanal one-stage endorectal pull-through for Hirschsprung's disease: a multicenter study.

BACKGROUND/PURPOSE: Transanal endorectal pull-through (TEPT) is the latest development in treatment of Hirschsprung's disease (HD). This prospective study was designed to evaluate the safety and efficacy of 1 stage TEPT technique in the management of patients with HD. METHODS: One hundred forty-nine children (116 boys and 33 girls) aged 8 days to 14 years underwent 1 stage TEPT procedure over an 18-month period at 5 Egyptian academic pediatric surgical centers and affiliated hospitals. Median follow-up was 12 months (range, 3 to 21 months). These patients were evaluated with regard to age, sex, length of the aganglionic segment, intraoperative details, and postoperative functional results or complications. An electromyogram (EMG), endorectal ultrasound scan, and lower gastrointestinal (GI) motility studies were reserved for patients with postoperative problems with bowel control. RESULTS: Mean operating time was 120.2 +/- 27.8 minutes (range, 60 to 210 minutes). The average length of resected bowel was 26.8 +/- 12.4 cm (range, 15 to 45 cm). Thirteen patients required laparotomy because of extension of aganglionic segment beyond the sigmoid colon in 9, tear in the mesenteric vessels in 2, and difficulties in getting to the submucosal plane in 2. Three deaths (2%) occurred 3 days, 4 days, and 4 weeks postoperatively, respectively. Postoperative complications included transient perianal excoriation in 48 patients (30 were <3 months of age), enterocolitis (n = 26), anastomotic stricture (n = 7), recurrent constipation (n = 6), hypoganglionosis at distal end of pulled through segment (n = 2), cuff abscess (n = 3), anastomotic leak (n = 1), adhesive bowel obstruction (n = 1), and rectal prolapse (n = 1). Complete anorectal continence was noted in 35 of 42 (83.3%) children older than 3 years, whereas soiling and frequent accidents still occur in 7, who showed a steady improvement in their continence status. CONCLUSIONS: One-stage TEPT technique is both feasible and safe technique in properly selected children with rectosigmoid HD in all ages. The technique is easily learned and is associated with excellent clinical results.