Aktuelle Defizite in der Therapie des Maldeszensus testis – Analyse eines Pädiaters

Zusammenfassung

Maldeszensus testis (MT) stellt das häufigste kinderchirurgische Krankheitsbild der westlichen Welt und einen der größten Risikofaktoren für maligne Entartung und Subfertilität dar. 2009 wurde die Empfehlung bzgl. des Zeitpunktes, zu dem die Therapie bei MT spätestens abgeschlossen sein soll, vom zweiten auf den ersten Geburtstag abgesenkt. Der Anteil von im ersten Lebensjahr durchgeführten Orchidopexien bei MT ist nach Änderung der AWMF-Leitlinie nur gering angestiegen. Sowohl in Deutschland als auch international werden zu wenige Kinder im ersten Lebensjahr operiert. Einem Großteil der Kinder behandelnden Ärzte scheint diese Diskrepanz zwischen Leitlinienempfehlung und tatsächlichem Alter zum Operationszeitpunkt nicht bewusst zu sein. Außerdem scheint ein nicht unerheblicher Teil der Fälle von Hodenhochstand durch sekundären Hodenhochstand bedingt zu sein, der oft erst im Schulkindesalter auftritt. Schlussfolgernd müssen eine Optimierung des Operationszeitpunktes bei kongenitalem MT und regelmäßige Untersuchungen des Hodens auch jenseits des Kleinkindesalters konsequenter angestrebt werden. Eine kontinuierliche Aufklärung für Kinder behandelnde Ärzte, Eltern und Patienten ist erforderlich, um die Prognose bei Hodenhochstand hinsichtlich Subfertilität und maligner Entartung zu verbessern. Die hohe Anzahl später Operationen bedarf weiterer Aufklärung.

Abstract

Cryptorchidism is the most common disorder in paediatric surgery in the Western world and a major risk factor for subfertility and malignancy. In 2009, German national guidelines were changed to recommend that treatment for undescended testes should be completed by the age of 1 instead of 2. However, the share of boys undergoing orchidopexy for cryptorchidism within the first year of life has only marginally increased in response to the guideline of the Association of the Scientific Medical Societies in Germany (AWMF). The number of orchidopexies performed in children below the age of 1 is too low both in Germany as well as internationally. The majority of primary care physicians treating children do not seem to be aware of this discrepancy between guideline recommendations and average actual age at orchidopexy. Moreover, a considerable number of cryptorchidism cases seem to be due to secondary ascent of the testis – an underappreciated condition that usually occurs at school age. Consequently, the timing of orchidopexy in primary undescended testes must be optimised. Therefore, education of parents and primary care physicians regarding the necessity of early orchidopexy and frequent testicular examinations even beyond infancy is mandatory to improve the prognosis of cryptorchidism regarding subfertility and malignancy. Further studies are needed to clarify the reasons for the large number of late orchidopexies.

Schlüsselwörter

Hodenhochstand - Kryptorchismus - Leitlinien - Orchidopexie - Versorgungsforschung

Key words

undescended testis - cryptorchidism - guidelines - orchidopexy - health services research

• Literatur

• 1 Barthold JS, Gonzalez R. The epidemiology of congenital cryptorchidism, testicular ascent and orchiopexy. The Journal of urology 2003; 170: 2396-2401
• 2 Virtanen HE, Bjerknes R, Cortes D et al. Cryptorchidism: classification, prevalence and long-term consequences. Acta paediatrica (Oslo, Norway: 1992) 2007; 96: 611-616
• 3 John Radcliffe Hospital Cryptorchidism Study Group. Cryptorchidism: a prospective study of 7500 consecutive male births, 1984-1988. Archives of disease in childhood 1992; 67: 892-899
• 4 Murphy F, Paran TS, Puri P. Orchidopexy and its impact on fertility. Pediatric surgery international 2007; 23: 625-632
• 5 Hutson JM, Hasthorpe S, Heyns CF. Anatomical and functional aspects of testicular descent and cryptorchidism. Endocrine reviews 1997; 18: 259-280
• 6 Bay K, Main KM, Toppari J et al. Testicular descent: INSL3, testosterone, genes and the intrauterine milieu. Nature reviews Urology 2011; 8: 187-196
• 7 Hutson JM. Journal of Pediatric Surgery-Sponsored Fred McLoed Lecture. Undescended testis: the underlying mechanisms and the effects on germ cells that cause infertility and cancer. Journal of pediatric surgery 2013; 48: 903-908
• 8 Zakaria M, Azab S, Elbaz M et al. Cryptorchidism in Egyptian neonates. Journal of pediatric urology 2013; 9: 815-819
• 9 Nah SA, Yeo CS, How GY et al. Undescended testis: 513 patients’ characteristics, age at orchidopexy and patterns of referral. Archives of disease in childhood 2014; 99: 401-406
• 10 Foresta C, Ferlin A. Role of INSL3 and LGR8 in cryptorchidism and testicular functions. Reproductive biomedicine online 2004; 9: 294-298
• 11 Kohler B, Schumacher V, Schulte-Overberg U et al. Bilateral Wilms tumor in a boy with severe hypospadias and cryptochidism due to a heterozygous mutation in the WT1 gene. Pediatric research 1999; 45: 187-190
• 12 Kirsch AJ, Escala J, Duckett JW et al. Surgical management of the nonpalpable testis: the Children’s Hospital of Philadelphia experience. The Journal of urology 1998; 159: 1340-1343
• 13 Berkowitz GS, Lapinski RH, Dolgin SE et al. Prevalence and natural history of cryptorchidism. Pediatrics 1993; 92: 44-49
• 14 Ritzen EM, Kollin C. Management of undescended testes: how and when?. Pediatric endocrinology reviews: PER 2009; 7: 32-37
• 15 Wenzler DL, Bloom DA, Park JM. What is the rate of spontaneous testicular descent in infants with cryptorchidism?. The Journal of urology 2004; 171: 849-851
• 16 Pyorala S, Huttunen NP, Uhari M. A review and meta-analysis of hormonal treatment of cryptorchidism. The Journal of clinical endocrinology and metabolism 1995; 80: 2795-2799
• 17 Henna MR, Del Nero RG, Sampaio CZ et al. Hormonal cryptorchidism therapy: systematic review with metanalysis of randomized clinical trials. Pediatric surgery international 2004; 20: 357-359
• 18 Ong C, Hasthorpe S, Hutson JM. Germ cell development in the descended and cryptorchid testis and the effects of hormonal manipulation. Pediatric surgery international 2005; 21: 240-254
• 19 Ludwikowski B, Gonzalez R. The controversy regarding the need for hormonal treatment in boys with unilateral cryptorchidism goes on: a review of the literature. European journal of pediatrics 2013; 172: 5-8
• 20 Spinelli C, Strambi S, Busetto M et al. Effects on normalized testicular atrophy index (TAIn) in cryptorchid infants treated with GnRHa pre and post-operative vs. surgery alone: a prospective randomized trial and long-term follow-up on 62 cases. Pediatric surgery international 2014; 30: 1061-1067
• 21 Jallouli M, Rebai T, Abid N et al. Neoadjuvant gonadotropin-releasing hormone therapy before surgery and effect on fertility index in unilateral undescended testes: a prospective randomized trial. Urology 2009; 73: 1251-1254
• 22 Al-Mandil M, Khoury AE, El-Hout Y et al. Potential complications with the prescrotal approach for the palpable undescended testis? A comparison of single prescrotal incision to the traditional inguinal approach. The Journal of urology 2008; 180: 686-689
• 23 Na SW, Kim SO, Hwang EC et al. Single scrotal incision orchiopexy for children with palpable low-lying undescended testis: early outcome of a prospective randomized controlled study. Korean journal of urology 2011; 52: 637-641
• 24 Penson D, Krishnaswami S, Jules A et al. Effectiveness of hormonal and surgical therapies for cryptorchidism: a systematic review. Pediatrics 2013; 131: e1897-e1907
• 25 Merguerian PA, Mevorach RA, Shortliffe LD et al. Laparoscopy for the evaluation and management of the nonpalpable testicle. Urology 1998; 51: 3-6
• 26 Kollin C, Karpe B, Hesser U et al. Surgical treatment of unilaterally undescended testes: testicular growth after randomization to orchiopexy at age 9 months or 3 years. The Journal of urology 2007; 178: 1589-1593 discussion 1593
• 27 Cortes D, Thorup JM, Visfeldt J. Cryptorchidism: aspects of fertility and neoplasms. A study including data of 1,335 consecutive boys who underwent testicular biopsy simultaneously with surgery for cryptorchidism. Hormone research 2001; 55: 21-27
• 28 Bruijnen CJ, Vogels HD, Beasley SW. Age at orchidopexy as an indicator of the quality of regional child health services. Journal of paediatrics and child health 2012; 48: 556-559
• 29 Kinderchirurgie DGf . Leitlinien Kinderchirurgie: Hodenhochstand – Maldeszensus testis, 2009. AWMF 2009; Register-Nr. 006-022 www.awmf.org/leitlinien/detail/ll/006-022.html
• 30 Kinderchirurgie DGf . Leitlinien Kinderchirurgie: Hodenhochstand – Maldeszensus testis, 2013. AWMF 2013; Register-Nr. 006-022 www.awmf.org/leitlinien/detail/ll/006-022.html
• 31 Hensel KO, Caspers T, Jenke AC et al. Operative Management of Crypt-orchidism: Guidelines and Reality – a 10-Year Observational Analysis of 3587 Cases. BMC Pediatrics. [im Druck]
• 32 Hoefling K, Sperling P, Meyer T. Time of Operative Treatment of Maldescensus Testis in Childhood – Wishes and Reality. Zentralblatt fur Chirurgie 2012;
• 33 Springer A, Huber C, Reck CA et al. Delayed referral despite appropriate knowledge in cryptorchidism as a cause of delayed orchidopexies in Austria. Klinische Padiatrie 2010; 222: 248-251
• 34 Hrivatakis G, Astfalk W, Schmidt A et al. The timing of surgery for undescended testis – a retrospective multicenter analysis. Deutsches Arzteblatt international 2014; 111: 649-657
• 35 Kokorowski PJ, Routh JC, Graham DA et al. Variations in timing of surgery among boys who underwent orchidopexy for cryptorchidism. Pediatrics 2010; 126: e576-e582
• 36 Chen YF, Huang WY, Huang KH et al. Factors related to the time to cryptorchidism surgery–a nationwide, population-based study in Taiwan. Journal of the Formosan Medical Association=Taiwan yi zhi 2014; 113: 915-920
• 37 Jensen MS, Olsen LH, Thulstrup AM et al. Age at cryptorchidism diagnosis and orchiopexy in Denmark: a population based study of 508,964 boys born from 1995 to 2009. The Journal of urology 2011; 186: 1595-1600
• 38 Marchetti F, Bua J, Tornese G et al. Management of cryptorchidism: a survey of clinical practice in Italy. BMC pediatrics 2012; 12: 4
• 39 Steckler RE, Zaontz MR, Skoog SJ et al. Cryptorchidism, pediatricians, and family practitioners: patterns of practice and referral. The Journal of pediatrics 1995; 127: 948-951
• 40 Herrinton LJ, Zhao W, Husson G. Management of cryptorchism and risk of testicular cancer. American journal of epidemiology 2003; 157: 602-605
• 41 Williams EV, Appanna T, Foster ME. Management of the impalpable testis: a six year review together with a national experience. Postgraduate medical journal 2001; 77: 320-322
• 42 Hack WW, Meijer RW, Van Der Voort-Doedens LM et al. Previous testicular position in boys referred for an undescended testis: further explanation of the late orchidopexy enigma?. BJU international 2003; 92: 293-296
• 43 Atwell JD. Ascent of the testis: fact or fiction. British journal of urology 1985; 57: 474-477
• 44 Schiffer KA, Kogan SJ, Reda EF et al. Acquired undescended testes. American journal of diseases of children (1960) 1987; 141: 106-107
• 45 Lamah M, McCaughey ES, Finlay FO et al. The ascending testis: is late orchidopexy due to failure of screening or late ascent?. Pediatric surgery international 2001; 17: 421-423
• 46 van der Plas E, Zijp G, Froeling F et al. Orchidopexy in late childhood often associated with previously normal testicular position. European journal of pediatric surgery: official journal of Austrian Association of Pediatric Surgery [et al.]=Zeitschrift fur Kinderchirurgie 2013; 23: 276-282
• 47 Bonney T, Southwell B, Donnath S et al. Orchidopexy trends in the paediatric population of Victoria, 1999-2006. Journal of pediatric surgery 2009; 44: 427-431
• 48 Guven A, Kogan BA. Undescended testis in older boys: further evidence that ascending testes are common. Journal of pediatric surgery 2008; 43: 1700-1704
• 49 Agarwal PK, Diaz M, Elder JS. Retractile testis--is it really a normal variant?. The Journal of urology 2006; 175: 1496-1499
• 50 Rusnack SL, Wu HY, Huff DS et al. The ascending testis and the testis undescended since birth share the same histopathology. The Journal of urology 2002; 168: 2590-2591
• 51 Yoshida T, Ohno K, Morotomi Y et al. Clinical and pathological features of ascending testis. Osaka city medical journal 2009; 55: 81-87
• 52 Hutson JM, Balic A, Nation T et al. Cryptorchidism. Seminars in pediatric surgery 2010; 19: 215-224
• 53 Smith JA, Hutson JM, Beasley SW et al. The relationship between cerebral palsy and cryptorchidism. Journal of pediatric surgery 1989; 24: 1303-1305
• 54 Docimo SG. Testicular descent and ascent in the first year of life. Urology 1996; 48: 458-460
• 55 Stec AA, Thomas JC, DeMarco RT et al. Incidence of testicular ascent in boys with retractile testes. The Journal of urology 2007; 178: 1722-1724 discussion 1724-1725
• 56 Davey RB. Undescended testes: early versus late maldescent. Pediatric surgery international 1997; 12: 165-167
• 57 Shapiro E. The risk of retractile testes becoming ascending testes. Reviews in urology 2006; 8: 231-232
• 58 Hack WW, Meijer RW, Bos SD et al. A new clinical classification for undescended testis. Scandinavian journal of urology and nephrology 2003; 37: 43-47