Bladder Exstrophy

Author(s):  
Jason Bryant

Bladder exstrophy is a rare condition in which there is an error in fetal development leaving the bladder and pelvic structure malformed. The bladder, pelvic girdle, and external genitalia are often split, requiring repair. The goals of this repair are to improve quality of life in terms of urinary continence, aesthetics, pelvic stabilization, and sexual function. This repair often requires prolonged traction and external fixation to adequately fuse the pelvic girdle. To aid in the healing and tolerance of this, a prolonged epidural catheter is often used. The pharmokinetics and risk of infection are major considerations during the prolonged use of a caudal catheter.

2021 ◽  
Vol 31 (06) ◽  
pp. 468-471
Author(s):  
Heiko Reutter ◽  
Gundela Holmdahl

AbstractBladder exstrophy-epispadias complex (BEEC) represents the severe end of the uro-rectal malformation spectrum and has profound impact on continence, sexual, and renal function. Treatment of BEEC is primarily surgical, and the main goals are safe closure of the abdominal wall, urinary continence while preserving renal function, and adequate cosmetic and functional genital reconstruction. Psychosocial and psychosexual outcomes and adequate health-related quality of life depend on long-term multidisciplinary care. The overall outcome is now considered very positive and affected individuals usually lead self-determined and independent lives with the desire to start their own families later in life. Certainty about the risk of recurrence and the provision of information about the current state of knowledge about the identified genetic causes with high penetrance will have an impact on family planning for healthy parents with an affected child and for affected individuals themselves. This review addresses this information and presents the current state of knowledge.


2014 ◽  
Vol 2014 ◽  
pp. 1-9 ◽  
Author(s):  
Brian M. Inouye ◽  
Ali Tourchi ◽  
Heather N. Di Carlo ◽  
Ezekiel E. Young ◽  
John P. Gearhart

The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-epispadias complex and potential surgical complications.


2020 ◽  
Vol 30 (03) ◽  
pp. 251-260
Author(s):  
Gundela Holmdahl ◽  
Michaela Dellenmark-Blom ◽  
Agneta Nordenskjöld ◽  
Sofia Sjöström

AbstractThe bladder exstrophy-epispadia complex (BEEC) includes malformations with midline closing defects of the lower abdomen and external genitalia. Long-term consequences with urinary incontinence and sexual dysfunction, in spite of multiple surgical interventions, are common and expected to affect the patient's health-related quality of life (HRQOL). The extent and the predictive factors are, however, not known. New patient-reported outcome research is emerging, but valid and reliable condition-specific HRQOL are still missing. The aim of this review is to summarize and discuss the latest published reports (2015–2019) on HRQOL in patients with the BEEC and its relationship to incontinence and sexual factors.


2021 ◽  
pp. 47-52
Author(s):  
M. A. Agapov ◽  
D. R. Markaryan ◽  
V. V. Kakotkin ◽  
A. M. Lukyanov ◽  
V. A. Kubyshkin

Introduction: Hirschsprung's disease (HD) is a relatively rare condition, in which the intermuscular (Auerbach) and submucosal (Meissner) plexus are absent in the colon wall at various lengths. In the literature, HD is described mainly in relation to the pediatric surgery area of interests because up to 90% of cases of this disease are detected before the age of 5 years 6. However, in some patients, the symptoms of the disease are not severe, so patients survive to adulthood without an established diagnosis.Clinical case: we demonstrate our experience of surgical treatment of Hirschsprung's disease in a 18-year-old patient.Conclusion: Although Hirschsprung's disease is in most cases a disease of childhood, it is necessary to keep in mind its typical symptoms when an adult patient complains of constipation. Proper diagnosis and correctly selected surgical treatment can not only eliminate symptoms and improve quality of life, but also reduce the likelihood of serious complications.


Author(s):  
E. V. Shevchuk ◽  
A. V. Shpak

The article describes experience of creating and implementing information-managing educational environment at university. The model of creating information-managing educational environment of university with elements of artificial intelligence and indicative management is described. This environment contributes to improve quality of training and management of educational processes and resources. The stages of creating and implementing information-managing educational environment are considered systemically, as continuous process focused on a consumer. The inhibitory and facilitating conditions for introduction of the model at university are described. To provide subject-oriented approach to the use of information resources of environment, recommended clusters of information subsystems for each category of users are described.Practically implemented scientific and methodological recommendations for subjects of educational process to overcome resistance to innovations introduced in educational organizations are proposed.Features of adaptation of the developed information-managing educational environment for schools are presented.


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