Transition von Jugendlichen mit Blasenekstrophie-Epispadie Komplex in die Erwachsenenmedizin: Implikationen des Langzeitverlaufes für das Management

Zusammenfassung

Menschen mit seltenen kongenitalen Anomalien wie dem Blasenekstrophie-Epispadie Komplex (BEEK) werden heute meist interdisziplinär und in der Kindheit auf hohem Niveau und mit großem Engagement betreut. Werden die Betroffenen jedoch jugendlich oder gar erwachsen, finden sie in unserem Gesundheitssystem oft keinen Ansprechpartner, der mit den Besonderheiten dieser kongenitalen Anomalien betraut ist. Die Überleitung oder Transition der Betroffenen in eine Schwerpunktbehandlung der Erwachsenenmedizin hat in Deutschland leider noch kein konkretes Gesicht.

Seit Jahren beschäftigen wir uns systematisch mit relevanten Langzeitergebnissen nach Rekonstruktion des BEEK im Neugeborenenalter und versuchen ergebnisorientierte Rückschlüsse für die primären Behandlungsstrategien abzuleiten. Thematisiert wurden neben der wichtigen Frage prognostisch relevanter prädiktiver Faktoren der späteren Kontinenzentwicklung und eines langfristigen Blasenerhaltes, die Genitalfunktion und Fertilität, postoperative Beckenbodenmorphologie mit gynäkologischem Outcome, orthopädische Implika­tionen sowie die psychosexuelle und psychosoziale Entwicklung von Individuen mit BEEK.

Als konkrete Konsequenz entwickeln wir derzeit ein strukturiertes, an den Langzeitaspekten orientiertes, die jeweiligen alters- und geschlechtsspezifischen Bedürfnisse der Kinder mit BEEK berücksichtigendes Nachsorgekonzept. Diese Langzeitergebnisse lehren uns aber auch die enorme Wichtigkeit einer interessierten und kooperativen Zusammenarbeit von Kinderurologen/Kinderchi­rurgen, Urologen, (Kinder-) Orthopäden, Gynäkologen, Andrologen, Psychologen und Urotherapeuten von Anfang an, und unterstreichen dringend einen notwendigen Wissenstransfer und damit eine hoffentlich erfolgreiche „Übernahme“ der Individuen mit BEEK in die Erwachsenmedizin.

Abstract

Today, young individuals with rare congenital anomalies as the Exstrophy-Epispadias-Complex (EEC) are mostly monitored interdisciplinary with a high standard of care and enthusiasm during childhood. However, when growing up through adolescence to adulthood adequate care-givers are not available at the moment in adult medicine in Germany and a concrete transition process has yet not been established. Over the past years, we put much effort in systematic evaluation of long-term outcome after reconstruction of the EEC in the newborn period to further improve outcome results. Beside predictive parameters for continence and long-term bladder function, genital function and fertility, as well as postoperative pelvic floor morphology and gynecological outcome, orthopedic results and psychosexual and psychosocial development in EEC were of major interest. As a consequence we currently develop a German-wide follow-up concept in EEC patients regarding age- and gender specific outcome issues. Long-term observations of the EEC outcome however, underline the unrestricted importance of careful long-term follow-up of all EEC patients, as well as the necessity of close cooperation of pediatric urologist, pediatric surgeons, urologists, orthopedic surgeons, gynecologists, andrologists, psychologists and urotherapists from early childhood and the need of knowledge transfer and hopefully a successful transition of the EEC individuals to general medicine.

Schlüsselwörter

Blasenekstrophie-Epispadie Komplex - Langzeitergebnisse - Transition - Erwachsenenalter

Key words

exstrophy-epispadias-­complex - long-term outcome - transition - adulthood

• Literatur

• 1 Ansari MS, Gearhart JP, Cervellione RM et al. The application of pelvic osteotomy in adult female patients with exstrophy: applications and outcomes. BJU 2011; 108 () 908-912
• 2 Baird AD, Gearhart JP, Mathews RI. Applications of the modified Cantwell-Ransley epispadias repair in the exstrophy-epispadias complex. J Pediatr Urol 2005; 1 () 331-336
• 3 Ben-Chaim J, Jeffs RD, Reiner WG et al. The outcome of patients with classical bladder exstrophy in adult life. J Urol 1996; 155: 1251-1252
• 4 Blakely CR, Mills WG. The obstetric and gynaecological complications of bladder exstrophy and epispadias. Br J Obstet Gynaecol 1981; 88: 167-173
• 5 Borer JG, Gargollo PC, Hendren WH et al. Early outcome following complete primary repair of bladder exstrophy in the newborn. J Urol 2005; 174: 1674-1678
• 6 Capolicchio G, McLorie GA, Farhat W et al. A population based analysis of continence outcomes and bladder exstrophy. J Urol 2001; 165 () 2418-2421
• 7 Chan DY, Jeffs RD, Gearhart JP. Determinants of continence in the bladder exstrophy population: predictors of success?. Urology 2001; 57 () 774-777
• 8 Deans R, Banks F, Liao LM et al. Reproductive outcomes in women with classic bladder exstrophy: an observational cross-sectional study. Am J Obstet Gynecol 2012; 206 496 e1-e6
• 9 Ebert A, Scheuering S, Schott G et al. Psychosocial and psychosexual development in childhood and adolescence within the exstrophy-epispadias complex. J Urol 2005; 174 () 1094-1098
• 10 Ebert AK, Bals-Pratsch M, Seifert B et al. Genital and reproductive function in males after functional reconstruction of the exstrophy-epispadias complex – long-term results. Urology 2008; 72 () 566-569
• 11 Ebert AK, Schott G, Bals-Pratsch M et al. Long-term follow-up of male patients after reconstruction of the bladder-exstrophy-epispadias complex: psychosocial status, continence, renal and genital function. J Pediatr Urol 2010; 6 () 6-10
• 12 Ebert AK, Falkert A, Brandl R et al. Pelvic-floor imaging using three-dimensional ultrasonography and magnetic resonance imaging in the long term follow-up of the bladder-exstrophy-epispadias complex. BJU 2010; 105 () 248-253
• 13 Ebert AK, Falkert A, Hofstädter A et al. Pregnancy management in women within the bladder-exstrophy-epispadias complex (BEEC) after continent urinary diversion. Arch Gynecol Obstet 2011; 284 () 1043-1046
• 14 Ender S, Stachow R, Petermann F et al. Verhaltensauffälligkeiten bei körperlich chronisch kranken Jugendlichen: Übereinstimmungen und Unterschiede im Selbst- und Elternurteil. Behavioral Problems of Somatic Chronically Ill Adolescents: Parent-Child-Agreement and Differences. Klin Padiatr 2011; 223: 231-235
• 15 Gargollo PC, Borer JB. Contemporary outcomes in bladder exstrophy. Curr Opin Urol 2007; 17: 272-280
• 16 Gearhart JP. The bladder exstrophy-epispadias-cloacal exstrophy complex. In Pediatric Urology. Edited by Gearhart JP, Rink RC, Moriquand PDE. Philadelphia: W.B. Saunders Co.; 2010. Chapter 30 386-415
• 17 Goldbeck L, Hölling I, Schlack R et al. The Impact of an Inpatient Family-Oriented Rehabilitation Program on Parent-Reported Psychological Symptoms of Chronically Ill Children. Effekte stationärer familienorientierter Rehabilitation auf die von Eltern berichtete psychologische Symptomatik chronisch körperlich kranker Kinder und Jugendlicher. Klin Padiatr 2011; 223: 79-84
• 18 Hampel OA, Schaadt AK, Hasmann SE et al. Evaluation von Stepping Stones Triple P: Zwischenergebnisse der Stepping-Stones-SPZ-MulticenterstudieEvaluation of Stepping Stones Triple P: Interims analysis of the Stepping-Stones-SPC-Multicentric Study. Klin Padiatr 2010; 222: 18-25
• 19 Hensle TW, Bingham JB, Reiley EA et al. The urological care and outcome of pregnancy after urinary tract reconstruction. BJU 2004; 93 () 588-590
• 20 Jenetzky E, Zwink N, Brenner H et al. Nationales Register für kongeni-tale uro-rektale Fehlbildungen (CURE-Net). Klin Pädiatr 2010; 222 – GNPI_PO_54 DOI: 10.1055/s-0030-1261519.
• 21 Kibar Y, Roth C, Frimberger D et al. Long-term results of penile disassembly technique for correction of epispadias. Urology 2009; 73 () 510-514
• 22 Lee C, Reutter HM, Grässer MF et al. Gender-associated differences in the psychosocial and developmental outcome in patients affected with the bladder exstrophy-epispadias complex. BJU 2006; 97 () 349-353
• 23 Matthews R, Sponseller PD, Jeffs RD et al. Bladder neck reconstruction in classic bladder exstrophy: the role of osteotomy in the development of continence. BJU 2000; 85: 498-500
• 24 Mathews RI, Gan M, Gearhart JP. Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU 2001; 91: 845-849
• 25 Mouriquand PDE, Bubanj T, Feyaerts A et al. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias. BJU 2003; 92: 997-1002
• 26 Nakhal RS, Deans R, Creighton SM et al. Genital prolapse in adult women with classical bladder exstrophy. Int Urogynecol J 2012; 23: 1201-1205
• 27 Reiner WG. Psychological and psychiatric aspects of genitourinary conditions. In Pediatric Urology. Edited by Gearhart JP, Rink RC, Moriquand PDE. Philadelphia: W.B. Saunders Co.; 2010. Chapter 39 512-518
• 28 Ricketts S, Hunter-Smith DJ, Coombs CJ. Quality of life after penile reconstruction using the radial forearm flap in adult bladder exstrophy patients – technique and outcomes. ANZ J Surg 2011; 81 () 52-55
• 29 Rösch W, Christl A, Strauß B et al. Comparison of preoperative innervation pattern and postreconstructive urodynamics in extrophy-epispadias-complex. Urol Int 1997; 59: 6-15
• 30 Rubenwolf PC, Eder F, Ebert AK et al. Expression and potential clinical significance of urothelial cytodifferentiation markers in the exstrophic bladder. J Urol 2012; 187 () 1806-1811
• 31 Silver RI, Yang A, Ben-Chaim J et al. Penile length in adulthood after exstrophy reconstruction. J Urol 1997; 157 () 999-1003
• 32 Stein R, Stöckle M, Fisch M et al. The fate of the adult exstrophy patient. J Urol 1994; 152: 1413-1416
• 33 Stein R, Fisch M, Bauer H et al. Operative reconstruction of the external and internal genitalia in female patients with bladder exstrophy or incontinent epispadias. J Urol 1995; 154 () 1002-1007
• 34 Wittmeyer V, Aubry E, Liard-Zmuda A et al. Quality of life in adults with bladder exstrophy-epispadias complex. J Urol 2010; 184 () 2389-2394
• 35 Woodhouse CRJ. Prospects for fertility in patients born with genitourinary anomalies. J Urol 2001; 65: 2354-2360
• 36 Woodhouse CR, North AC, Gearhart JP. Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder. World J Urol 2006; 24 () 244-249
• 37 Woodhouse CRJ, Hinsch R. The anatomy and reconstruction of the adult female genitalia in classical exstrophy. BJU 1997; 79: 618-622