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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Health website editors Do you have your own website? 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.

Case report

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis extophie diagnosis or treatment. It does not collect any personal information and contains no spyware or other hidden functions.

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. 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.

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. Check this box if you wish to receive a copy of your message.

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! Urine drips from the ureteric orifices, visible on the bladder surface or per urethram. Detailed information Professionals Review article English Though spontaneous voiding is the main issue, additional surgery might be needed to optimize bladder storage and emptying function. Other search option s Alphabetical list.

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Does your site need to be reassessed? Summary and related texts. In cases of definite reconstruction failure, urinary diversion should be undertaken. Data di rassegna iniziale: Providing you with extropie health information. 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.

Specialised Social Services Eurordis directory. In puberty, genital and reproductive functions constitute increasingly important issues for both sexes. Etiology The underlying cause remains unknown: The HONcode toolbar is easy to download and add to your Web browser. The documents contained in this web site are presented for information purposes only. It connects in real time to the HON server to verify the certification status of health and wxtrophie websites.

This principle is not applicable to this site. Depending on severity, the EEC may involve the urinary system, the musculoskeletal system, the pelvis, the pelvic floor, the abdominal wall, the genitalia and sometimes the spine and the anus.

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The data displayed correspond to only one of the selected samples. NeonatalAntenatal ICD Atypical forms of the EEC duplicated exstrophy, covered exstrophy and pseudo-exstrophy have also been described.

Orphanet: Exstrophie vesicale epispadias

For all other comments, please send your remarks via contact us. Health care resources for this disease Expert centres Diagnostic tests 3 Patient organisations 30 Orphan drug s 0.

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. In the rare cases of CE, two exstrophied hemibladders, as well as omphalocele, an imperforate anus and spinal defects, can be seen after birth. The timing of the rupture determines the severity of the malformation.

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

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

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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.

Removal of the bladder template with complete urinary diversion to a rectal reservoir can be an alternative. The underlying cause remains unknown: Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.