Management Of Congenital Malformations Research Articles

Paediatric urology in Sub-Saharan Africa: challenges and opportunities.

The need for paediatric urological care in low- and middle-income countries in sub-Saharan Africa (SSA) is enormous due to a burgeoning paediatric-aged population and a disproportionate burden of congenital malformations. There are formidable challenges in the provision of a skilled workforce and appropriate infrastructure, resulting in a huge unmet need with consequent effects on the long-term health and prosperity of the population. Constraints of funding, geography, culture, surgical and anaesthetic skills, and instrumentation means that many conditions present late and with complications that could have been avoided by an earlier attendance. It also means that the management of congenital malformations, e.g., bladder exstrophy and congenital obstructive posterior urethral membrane, differ substantially from that seen in the developed world, with the outlook for children with renal failure being particularly bleak. Collaborations between paediatric urologists from high- and low-income countries are beginning to help with the development of a surgical infrastructure customised to paediatric care, and with the training of specific paediatric urological knowledge and skills. These collaborations, whilst welcome, still require substantial expansion to achieve more equitable access to appropriate paediatric urological care for children in SSA. Future efforts have to focus on the creation of sustainable and equal partnerships between urologists from low- and high-income healthcare environments, with an emphasis on providing sustainable management, appropriate to local need and available resources. The provision of shared learning, utilising the benefits of global digital communication, will improve mutual understanding of needs in a resource-poor environment and the involvement of trainees from both income settings can help perpetuate long-term collaborations.

Challenges in the Management of Congenital Malformations in Infants: A Single Centre Experience

Background: Congenital malformations are structural anomalies that occur during intra-uterine life that may be identified before or at birth or later in life. Materials and Methods: This was a retrospective evaluation of the challenges encountered during the management of children with congenital anomalies at the pediatric surgery unit of Enugu State University Teaching Hospital (ESUTH), Enugu, Nigeria. Diagnosis of congenital anomalies was made based on clinical and radiological findings. Results: A total of 104 infants with congenital anomalies were evaluated during the study period. There was male predominance and majority of the patients were neonates. Seven days and 3 days were the mean age at diagnosis and median interval before presentation respectively. Hypospadias was the most common congenital anomaly and surgical site infection was the most common post-operative complication. Mortality occurred in about one-third of the patients. The general challenges encountered in the management of these patients could categorized as patient related, surgeon related or facility related. Conclusion:In developing countries, management of congenital anomalies is challenging and is fraught with difficulties. Modifiable factors such as late presentation and provision of facilities will improve outcome.

1699 Distribution of Congenital Malformation in a Neonatal Intensive Care Unit in Turkey

Background and AimCongenital malformations are one of the important reasons of mortality and morbidity in newborns. The aim of this study is to give the frequency and distribution of the...

Applying a knowledge‐to‐action framework for primary prevention of spina bifida in tropical Africa

Maternal periconceptual folate supplementation reduces the incidence of neural tube defects; however, in settings where population-level food fortification is not available, it is not clear how best to promote this prevention strategy. Guided by a knowledge-to-action methodology, we used mixed quantitative and qualitative methods to define the local disease burden, then designed, implemented and evaluated a culturally tailored educational intervention in eastern Democratic Republic of Congo, where resource limitations and threats to human security contribute to restricted capacity for the prevention and management of congenital malformations. A descriptive case series of 27 patients undergoing surgery for spina bifida demonstrated a short-term mortality of 15% and long-term disability in survivors. A survey of knowledge, attitudes and practices demonstrated a low level of folate awareness (53%) among women of reproductive age. Focus group discussions revealed exotic aetiologic views, significant gender issues and several barriers to folate use. A culturally tailored radio broadcast and an educational video were designed and produced locally based on qualitative and quantitative findings. Evaluation of the video documented high levels of viewer satisfaction and unequivocal knowledge gain (P ≤ 0.001). We conclude that spina bifida poses a significant burden on affected patients and their families in the African context, but folate is underutilized as a prevention strategy. Patient education through video media results in increased awareness and understanding of spina bifida and folate, a first step in empowering women to reduce the risk of spina bifida in their children in the absence of population-wide food fortification.

Update in the Diagnosis and Management of Congenital Malformations of the Airway and Lung

Update in the Diagnosis and Management of Congenital Malformations of the Airway and Lung

End-Stage Renal Disease in Children

The pediatrician should be able to identify chronic renal failure and to determine a specific etiobogic diagnosis for most patients with CRF, based upon age and mode of presentation, examination of the urine, anatomic evaluation of the urinary tract, and, if indicated, relatively simple laboratory tests, such as antinuclear antibody and serum complement. While referral to a pediatric urologist is indicated for diagnosis and management of congenital malformations of the urinary tract, consultation with the pediatric nephrologist is advisable for the child who requires a renal biopsy for definitive diagnosis, as well as to plan future management, including diet, drug therapy, dialysis, and transplantation. The primary care pediatrician should retain responsibility for implementing the medical recommendations of the pediatric urologist or nephrologist and should resume his full role for the child who has undergone successful renal transplantation.

End-Stage Renal Disease in Children

The pediatrician should be able to identify chronic renal failure and to determine a specific etiobogic diagnosis for most patients with CRF, based upon age and mode of presentation, examination of the urine, anatomic evaluation of the urinary tract, and, if indicated, relatively simple laboratory tests, such as antinuclear antibody and serum complement. While referral to a pediatric urologist is indicated for diagnosis and management of congenital malformations of the urinary tract, consultation with the pediatric nephrologist is advisable for the child who requires a renal biopsy for definitive diagnosis, as well as to plan future management, including diet, drug therapy, dialysis, and transplantation. The primary care pediatrician should retain responsibility for implementing the medical recommendations of the pediatric urologist or nephrologist and should resume his full role for the child who has undergone successful renal transplantation.

Congenital Deformities of the Hand and Forearm

The last two decades have witnessed a burst of interest in the etiology, classification, and management of congenital malformations. This volume is the culmination of the efforts of one man's dedication to this subject. The author of this book, Emeritus Prof Kelikian, is a former professor of orthopedic surgery at Northwestern University Medical School in Chicago. The objective of the book, as stated in the preface, is to examine the past and to present the author's own experience in this challenging field of surgery. The book is divided into 29 main chapters. At the beginning of each chapter the author traces the historical evolvement by citing the various authors in chronological order. This chronology helps to provide the novice with the needed background and broadens the reader's perception of this complicated field. The first three chapters are devoted to the outlining of the definition and scope of congenital anomalies and

Surgical management of congenital malformations of the lymphatic system

Surgical management of congenital malformations of the lymphatic system