EXTROPHIE VESICALE PDF

Request PDF on ResearchGate | On Jun 1, , S. Jochault-Ritz and others published CL – Qualité de vie dans l’exstrophie vésicale, résultats de l’étude. Malformation: extrophie vésicale has members. Parents Group. Website; Head of organisation: Mr Philip ANDERMATT; Vereinigung Blasenexstrophie – Association Exstrophie Vésicale – Associazione Estrofia Vesicale.

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Case report

Disease definition Exstrophy-Epispadias Complex EEC represents a spectrum of genitourinary malformations ranging in severity from epispadias E and classical bladder exstrophy CEB to exstrophy of the cloaca EC as the most severe form see these terms. Psychosocial and psychosexual outcome reflect the importance of long-term care from birth into adulthood from a multidisciplinary team of experts for parents and children with EEC to facilitate an adequate quality of life.

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The underlying cause remains unknown: Specialised Social Services Eurordis directory. NeonatalAntenatal ICD Urine drips from the ureteric orifices, visible on the bladder surface or per urethram. Etiology The underlying cause remains unknown: Health website editors Do you have your own website?

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Management is primarily surgical, with the main aims of obtaining secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital vesicalf.

Does your site need to be reassessed? This principle is not applicable to this site. Check this box if you wish to receive a copy of your message. Depending on severity, the EEC may involve the urinary system, the musculoskeletal system, the pelvis, the pelvic veicale, the abdominal wall, the genitalia and sometimes the veeicale and the anus. It connects in real time to the HON server to verify the certification status of health and medical websites.

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Removal of the bladder template with complete urinary diversion to a rectal reservoir can be an alternative.

Clinical description EEC is characterized by a visible defect of the lower abdominal wall, either with an evaginated bladder plate CEBor with an open urethral plate in males or a cleft in females E.

Extensive counseling should be provided to parents but due to a generally favorable outcome with appropriate surgical management, termination of the vesivale should no longer be systematically recommended.

In puberty, genital and reproductive functions constitute increasingly important issues for both sexes. Genetic counseling Extensive counseling should be provided to parents but due to a generally favorable outcome with appropriate surgical management, termination of the pregnancy should no longer be systematically recommended. Management and treatment Management is primarily surgical, with the main aims of obtaining secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital reconstruction.

The persons responsible for the site commit to maintaining its compliance with the HONcode principles for the duration of the site’s certification. Health information you can trust! Other search option s Alphabetical list.

Health care resources for this disease Expert centres Diagnostic tests 3 Patient organisations 30 Orphan drug s 0. The timing of the rupture determines the severity of the malformation. The documents contained in this web site are presented for information purposes only.

EEC is characterized by a visible defect of the lower abdominal wall, either with an veskcale bladder plate CEBor with an open urethral plate in males or a cleft in extrlphie E.

Orphanet: Exstrophie vesicale epispadias

Providing you with trustworthy veiscale information. Diagnosis at birth is made on the basis of the clinical presentation but EEC may be detected prenatally during careful ultrasound examinations with the index finding of repeated non-visualization of a normally filled fetal bladder.

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Journal de la Recherche Scientifique de l’Université de Lomé

Only comments seeking to improve the quality and accuracy of information dxtrophie the Orphanet website are accepted. Data di rassegna iniziale: For all other comments, please send your remarks via contact us. Though spontaneous voiding is the main issue, additional surgery might be needed to optimize bladder storage and emptying function.

Diagnostic methods Diagnosis at birth is made on the basis of the clinical presentation but EEC may be detected prenatally during careful ultrasound examinations with the index finding of repeated non-visualization of a normally filled fetal bladder.

EEC results from mechanical disruption or enlargement of the cloacal membrane, which prevents the invasion of mesodermal cells along the infraumbilical midline and thereby results in exstrophy. The data displayed correspond to only one of the selected samples.

Exstrophy-Epispadias Complex EEC represents a spectrum of genitourinary malformations ranging in severity from epispadias E and classical bladder exstrophy CEB extropihe exstrophy of the cloaca EC as the most severe form see these terms.

In the rare cases of CE, two exstrophied hemibladders, as well as omphalocele, an imperforate anus and spinal defects, can be seen after birth. Download the free HONcode toolbar.

Detailed information Professionals Review article English In cases of definite reconstruction failure, urinary diversion should be undertaken.