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The Kelly Operation for Epispadias
ESPU Programme 2008 RESULTS Bladder volume estimated from the X-ray films (big, normal, small), correlated well to bladder volumes obtained from the urodynamic investigations, with significant difference between the groups (p<0.001). Regression formulas have been constructed for the respective bladder size on X-ray in ml versus age. Small bladders were characteristic for the infant group and big bladders for children after age 1 year.
S75 Low age (<1 year) was significantly correlated to pathology of the bladder wall and the presence of PUF both during the filling and voiding phase. Abnormalities of the bladder wall also correlated to low bladder capacity and overactive contractions during filling. PUF during filling significantly correlated to high pressure overactive contractions and PUF during voiding to high voiding pressure.
CONCLUSIONS Our observations indicate that bladder capacity could roughly be estimated from xray films by using the landmarks suggested in this study. The typical urodynamic pattern for small infants: low bladder capacity, overactivity, high voiding pressure and dyscoordination at voiding was recognised in X-ray films from VCUG.
S17: EXSTROPHY-EPISPADIAS COMPLEX 2 From 12:45 to 12:48 # S17-1 (PP) ORIGINAL INSIGHTS INTO SURGICAL ANATOMY AND HISTOLOGY OF CORPORAL BODIES OF THE EPISPADIAC PENIS WITH IMPLICATIONS FOR SURGERY Sava PEROVIC, Rados DJINOVIC, Yuriy RUDIN* University Children’s Hospital, Urology, Belgrade, YUGOSLAVIA - *St Vladimir Children Hospital, Urology, Moscow, YUGOSLAVIA
PURPOSE Surgical anatomy and histology of corporal bodies of the epispadiac penis is still not well described. Using our complete disassembly technique we revealed specific anatomical and histological features of the epispadiac penis which have a significant impact on surgical outcome.
mobilization of NVB, urethral plate and corporal bodies was performed. Free corporeal bodies are evaluated during surgery in the flaccid and erect state. In 8 patients a histological specimen was harvested from ventral, medial and wedgeshaped dorsal side.
examination revealed a normal ventral side of albuginea with two layers while medial side have only an underdeveloped circular layer. Dorsal, wedge-shaped side consists only of thick fibrous tissue. These features are compared to normal peers’ cavernosal bodies.
RESULTS CONCLUSIONS
MATERIAL AND METHODS From October 1996 till August 2007, 58 patients age between 2 days and 23 years (mean 48 months) underwent primary repair of the epispadiac penis. Nineteen patients presented with an isolated epispadias while the remaning 39 had epispadias eextrophy complex. Complete penile disassembly with
Investigating anatomical and histological features of the epispadiac penis we revealed several important particularities. Corporal bodies have a triangular shape: ventral convex side, medial and lateral. Dorsal side is concave and narrowed into a wedge shaped streak. The main substrate of dorsal curvature is disproportion between longer ventral and shorter dorsal side. Histological
A new insight into anatomical and histological features of epispadiac corporal bodies have significant impact on surgical repair. Only equalizing the shorter dorsal and longer ventral side by minimum of two transversal dorsal incisions with grafting can correct curvature and this way straighten and lengthen the penis.
From 12:48 to 12:51 # S17-2 (PP) THE KELLY OPERATION FOR EPISPADIAS Aruna ABHYANKAR, Divyesh DESAI, Peter CUCKOW Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM
PURPOSE To review the results of Kelly soft tissue reconstruction in incontinent primary epispadias.
MATERIAL AND METHODS 22 patients are reviewed from our database at least 1 year after their Kelly operation.
Non-invasive bladder function assessments and clinical histories were used to evaluate continence. Half (11 patients -10m, 1f) had a primary Kelly operation at a median age of 2 years (10 m - 8 y, 3 m). Half (11 patients 9m, 2f) had a secondary Kelly procedure following previous epispadias repair, at a median age of 10 years (3 y, 3 m - 16 y).
RESULTS In the Primary group 2 patients remain too young to assess reliably. Of the remainder 4/ 9 are dry by day and 2 of these also at night. 5/9 are judged to have no control. In the secondary group 8/11 patients are dry by day and 5 of these are also dry at night. 3 remain wet with inadequate control and
S76 capacity. All females are dry by day, 1 also at night.
CONCLUSIONS As in patients with bladder exstrophy, continence relies on a combination of bladder outlet resistance and capacity.
ESPU Programme 2008 Whilst the results from secondary cases are comparable with our data in classic exstrophy (70% daytime continence) those in primary cases are inferior. Reasons for this may include: 1. patients with primary operations are younger and so their continence may continue to improve. 2. radical primary dissection may have
a negative effect on native continence mechanisms in epispadias. 3. primary epispadias is not simply a more minor variant of the exstrophy-epispadias complex. (supported by anatomical observations of our cases).
From 12:51 to 12:54 # S17-3 (PP) PHALLOPLASTY IN BOYS WITH CLOACAL EXSTROPHY. Piet HOEBEKE, Stanislas MONSTREY*, Peter CEULEMANS*, Nicolaas LUMENy, Katrien KLOCKAERTSz, Erik VAN LAECKE{ Ghent University Hospital, Paediatric Urology & Urogenital reconstruction, Gent, BELGIUM - *Ghent University Hospital, Plastic Surgery, Gent, BELGIUM - yGhent Univesrity Hospital, Urology, Gent, BELGIUM - zGhent University Hospital, Paeditric Urology, Gent, BELGIUM - {Ghent University Hospital, Paediatric Urology, Gent, BELGIUM
PURPOSE
MATERIAL AND METHODS
CONCLUSIONS
Cloacal exstrophy is considered one of the most serious congenital anomalies. Boys present with insufficient penile development and formerly female gender reassignment was considered the treatment of choice. Recently bad outcome was reported in female assigned patients, so male gender assignment is preferred (1-2). In male gender assignment subsequent phalloplasty will be necessary. Due to former surgery in the pelvic region the gold standard phalloplasty using free tissue transfer is not preferred. We therefore offered a pedicled tissue transfer (anterolateral tigh flap ALT) to 2 boys with cloacal exstrophy and raised as boys.
Two boys, age 17 and 18 years were seen after multiple reconstructions for cloacal exstrophy. They both had an augmented bladder with closed bladder neck and continent vesicostomy. In both boys a phalloplasty was done using an ALT flap. No urethral reconstruction was done as it was uneccesary. The urethra was left open at the base of the phallus for eventual sperm evacuation.
With male gender assignment as gold standard approach to boys with cloacal exstrophy, we present the ALT flap as the method of choice for penile reconstruction.
RESULTS Both procedures were uneventfull and in both patients a satisfactory result could be obtained. Both boys expressed their extreme hapiness with the result. Erectile devices will be implanted after 1 year.
REFERENCES Reiner WG: Psychosexual development in genetic males assigned female: the cloacal exstrophy experience. Child Adolesc Psychiatr Clin N Am. 2004 Jul;13(3):657-74 Meyer-Bahlburg HF: Gender identity outcome in female-raised 46, XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation. Arch Sex Behav. 2005 Aug;34(4):423-38.
From 12:54 to 12:59# S17-4 (O) SELF-ESTEEM AND SATISFACTION WITH PENILE APPEARANCE IN ADOLESCENT MALES WITH BLADDER EXSTROPHY: A PILOT STUDY Raimondo CERVELLIONE, Ruth HURRELL*, Sarah GASKELL*, Janet FISHWICK, A BIANCHI, Alan DICKSON Royal Manchester Childrens Hospital, Urology, Manchester, UNITED KINGDOM - *Booth Hall Childrens Hospital, Clinical Psychology, Manchester, UNITED KINGDOM
PURPOSE
MATERIAL AND METHODS
Self-esteem can play an important role in satisfaction with body appearance. The present pilot study was designed to examine the relationship between self-esteem and satisfaction with penile appearance in adolescent males with bladder exstrophy and/or epispadisas.
Nine adolescent males aged 11-16 with bladder exstrophy or primary epispadias were identified in the Manchester Bladder Exstophy database. Six took part in the study completing a ‘satisfaction with penile appearance’ questionnaire and the Harter Self-Perception Profile to provide a global measure of self-worth. Standarised
photographs were taken of each participant’s genital area. Two Paediatric Urologists independently rated participants’ penile appearance based on the anonymised photographs using a penile appearance rating scale. Each participant’s genital developmental stage was assessed using the Tanner Rating Scale.
S75 Low age (<1 year) was significantly correlated to pathology of the bladder wall and the presence of PUF both during the filling and voiding phase. Abnormalities of the bladder wall also correlated to low bladder capacity and overactive contractions during filling. PUF during filling significantly correlated to high pressure overactive contractions and PUF during voiding to high voiding pressure.
CONCLUSIONS Our observations indicate that bladder capacity could roughly be estimated from xray films by using the landmarks suggested in this study. The typical urodynamic pattern for small infants: low bladder capacity, overactivity, high voiding pressure and dyscoordination at voiding was recognised in X-ray films from VCUG.
S17: EXSTROPHY-EPISPADIAS COMPLEX 2 From 12:45 to 12:48 # S17-1 (PP) ORIGINAL INSIGHTS INTO SURGICAL ANATOMY AND HISTOLOGY OF CORPORAL BODIES OF THE EPISPADIAC PENIS WITH IMPLICATIONS FOR SURGERY Sava PEROVIC, Rados DJINOVIC, Yuriy RUDIN* University Children’s Hospital, Urology, Belgrade, YUGOSLAVIA - *St Vladimir Children Hospital, Urology, Moscow, YUGOSLAVIA
PURPOSE Surgical anatomy and histology of corporal bodies of the epispadiac penis is still not well described. Using our complete disassembly technique we revealed specific anatomical and histological features of the epispadiac penis which have a significant impact on surgical outcome.
mobilization of NVB, urethral plate and corporal bodies was performed. Free corporeal bodies are evaluated during surgery in the flaccid and erect state. In 8 patients a histological specimen was harvested from ventral, medial and wedgeshaped dorsal side.
examination revealed a normal ventral side of albuginea with two layers while medial side have only an underdeveloped circular layer. Dorsal, wedge-shaped side consists only of thick fibrous tissue. These features are compared to normal peers’ cavernosal bodies.
RESULTS CONCLUSIONS
MATERIAL AND METHODS From October 1996 till August 2007, 58 patients age between 2 days and 23 years (mean 48 months) underwent primary repair of the epispadiac penis. Nineteen patients presented with an isolated epispadias while the remaning 39 had epispadias eextrophy complex. Complete penile disassembly with
Investigating anatomical and histological features of the epispadiac penis we revealed several important particularities. Corporal bodies have a triangular shape: ventral convex side, medial and lateral. Dorsal side is concave and narrowed into a wedge shaped streak. The main substrate of dorsal curvature is disproportion between longer ventral and shorter dorsal side. Histological
A new insight into anatomical and histological features of epispadiac corporal bodies have significant impact on surgical repair. Only equalizing the shorter dorsal and longer ventral side by minimum of two transversal dorsal incisions with grafting can correct curvature and this way straighten and lengthen the penis.
From 12:48 to 12:51 # S17-2 (PP) THE KELLY OPERATION FOR EPISPADIAS Aruna ABHYANKAR, Divyesh DESAI, Peter CUCKOW Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM
PURPOSE To review the results of Kelly soft tissue reconstruction in incontinent primary epispadias.
MATERIAL AND METHODS 22 patients are reviewed from our database at least 1 year after their Kelly operation.
Non-invasive bladder function assessments and clinical histories were used to evaluate continence. Half (11 patients -10m, 1f) had a primary Kelly operation at a median age of 2 years (10 m - 8 y, 3 m). Half (11 patients 9m, 2f) had a secondary Kelly procedure following previous epispadias repair, at a median age of 10 years (3 y, 3 m - 16 y).
RESULTS In the Primary group 2 patients remain too young to assess reliably. Of the remainder 4/ 9 are dry by day and 2 of these also at night. 5/9 are judged to have no control. In the secondary group 8/11 patients are dry by day and 5 of these are also dry at night. 3 remain wet with inadequate control and
S76 capacity. All females are dry by day, 1 also at night.
CONCLUSIONS As in patients with bladder exstrophy, continence relies on a combination of bladder outlet resistance and capacity.
ESPU Programme 2008 Whilst the results from secondary cases are comparable with our data in classic exstrophy (70% daytime continence) those in primary cases are inferior. Reasons for this may include: 1. patients with primary operations are younger and so their continence may continue to improve. 2. radical primary dissection may have
a negative effect on native continence mechanisms in epispadias. 3. primary epispadias is not simply a more minor variant of the exstrophy-epispadias complex. (supported by anatomical observations of our cases).
From 12:51 to 12:54 # S17-3 (PP) PHALLOPLASTY IN BOYS WITH CLOACAL EXSTROPHY. Piet HOEBEKE, Stanislas MONSTREY*, Peter CEULEMANS*, Nicolaas LUMENy, Katrien KLOCKAERTSz, Erik VAN LAECKE{ Ghent University Hospital, Paediatric Urology & Urogenital reconstruction, Gent, BELGIUM - *Ghent University Hospital, Plastic Surgery, Gent, BELGIUM - yGhent Univesrity Hospital, Urology, Gent, BELGIUM - zGhent University Hospital, Paeditric Urology, Gent, BELGIUM - {Ghent University Hospital, Paediatric Urology, Gent, BELGIUM
PURPOSE
MATERIAL AND METHODS
CONCLUSIONS
Cloacal exstrophy is considered one of the most serious congenital anomalies. Boys present with insufficient penile development and formerly female gender reassignment was considered the treatment of choice. Recently bad outcome was reported in female assigned patients, so male gender assignment is preferred (1-2). In male gender assignment subsequent phalloplasty will be necessary. Due to former surgery in the pelvic region the gold standard phalloplasty using free tissue transfer is not preferred. We therefore offered a pedicled tissue transfer (anterolateral tigh flap ALT) to 2 boys with cloacal exstrophy and raised as boys.
Two boys, age 17 and 18 years were seen after multiple reconstructions for cloacal exstrophy. They both had an augmented bladder with closed bladder neck and continent vesicostomy. In both boys a phalloplasty was done using an ALT flap. No urethral reconstruction was done as it was uneccesary. The urethra was left open at the base of the phallus for eventual sperm evacuation.
With male gender assignment as gold standard approach to boys with cloacal exstrophy, we present the ALT flap as the method of choice for penile reconstruction.
RESULTS Both procedures were uneventfull and in both patients a satisfactory result could be obtained. Both boys expressed their extreme hapiness with the result. Erectile devices will be implanted after 1 year.
REFERENCES Reiner WG: Psychosexual development in genetic males assigned female: the cloacal exstrophy experience. Child Adolesc Psychiatr Clin N Am. 2004 Jul;13(3):657-74 Meyer-Bahlburg HF: Gender identity outcome in female-raised 46, XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation. Arch Sex Behav. 2005 Aug;34(4):423-38.
From 12:54 to 12:59# S17-4 (O) SELF-ESTEEM AND SATISFACTION WITH PENILE APPEARANCE IN ADOLESCENT MALES WITH BLADDER EXSTROPHY: A PILOT STUDY Raimondo CERVELLIONE, Ruth HURRELL*, Sarah GASKELL*, Janet FISHWICK, A BIANCHI, Alan DICKSON Royal Manchester Childrens Hospital, Urology, Manchester, UNITED KINGDOM - *Booth Hall Childrens Hospital, Clinical Psychology, Manchester, UNITED KINGDOM
PURPOSE
MATERIAL AND METHODS
Self-esteem can play an important role in satisfaction with body appearance. The present pilot study was designed to examine the relationship between self-esteem and satisfaction with penile appearance in adolescent males with bladder exstrophy and/or epispadisas.
Nine adolescent males aged 11-16 with bladder exstrophy or primary epispadias were identified in the Manchester Bladder Exstophy database. Six took part in the study completing a ‘satisfaction with penile appearance’ questionnaire and the Harter Self-Perception Profile to provide a global measure of self-worth. Standarised
photographs were taken of each participant’s genital area. Two Paediatric Urologists independently rated participants’ penile appearance based on the anonymised photographs using a penile appearance rating scale. Each participant’s genital developmental stage was assessed using the Tanner Rating Scale.