Congenital Anomalies Associated with Cryptorchidism

Congenital Anomalies Associated with Cryptorchidism

0022 5347/82/1271-0091$02.00/0 THE JOURNAL OF UROLOG"\' Copyright© 1982 by The ~,liliI]iarns & 1;ViJ.ki1t~, ('.o. beforn and of cases. abnorrnaliti...

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0022 5347/82/1271-0091$02.00/0 THE JOURNAL OF UROLOG"\'

Copyright© 1982 by The ~,liliI]iarns & 1;ViJ.ki1t~, ('.o.

beforn and

of cases. abnorrnalities function'- or maldescent even 1n c"""~"~'-"'"-''"'' also may be a factor. The

RRSUL'I'S

abnorm_alities vvith bilateral

n1ental associated with MATERIALS AND IVIETHOD.S DISCUSSION

discovered toms. Of the 205 IVPs done 64 anomalies and 141 were Accepted for pubhcation Decembe.r 19, 1980. 9I

92

FALLON, WELTON AND HAWTREY

TABLE

1. Other congenital anomalies in 126 patients with bilateral

cryptorchidism 13 1 1

2 7 1 2 1 1

2. Other congenital anomalies in 208 patients with unilateral cryptorchidism

Horseshoe kidney Complete It. duplex

Significant Minor

* Of these

Meningomyelocele Polycystic kidneys Complete It. duplex, with upper segment obstruction Solitary kidney TABLE

4 2 2

Unilat. reflux Unilat. duplex, reflux lower pole Solitary rt. kidney Complete It. duplex

1 1 1 1 1 1 1 17 42*

3. Summary of IVP findings No. Pts.

No. IVPs

No. Abnorma!

126

87

16

61 57

4 5

334

205

25

Asymptomatic cryptorchidism

232

141

4

4. IVP findings in 87 studies of bilateral cryptorchidism

Prune belly syndrome Meningomyelocele Posterior urethral valves Ureteropelvic junction obstruction Malrotation Partial duplex

No. Cases 10

2 1 1

Class Majorl MajorJ Major Major

No. Cases

Class Major} Major Major

2

Comments Previously documented Patient had ventricular septa! defect, reimplant done

Significant

No. Cases

Class

Comment

1 1

Major Major

Reim plant Reimplant

1 1

Significant Minor

No pathology

Of the 141 asymptomatic patients 43 had bilateral cryptorchidism, none of whom had an abnormal IVP, and 98 had unilateral cryptorchidism, 4 of whom had abnormal IVPs.

111 97

Abnormality

No pathology

cryptorchidism

Bilat. cryptorchidism Rt. cryptorchidism Lt. cryptorchidism Totals

TABLE

Reimplant done Lower pole atrophy, reimplant done

7. IVP findings in 141 studies of asymptomatic

Abnormality

42 patients 5 had hypospadias and 22 were retarded. TABLE

Comments

6. IVP findings in 57 studies of left cryptorchidism

No. Pts.

7 1 1 1

Class Major Major

Abnormality

1

30 60*

Mental retardation Macrocephaly plus retardation Hydrocephalus plus retardation Retardation plus hypospadias Hypospadias Meningomyelocele Ventricular septa! defect Congenital hydrocele Diaphragmatic hernia Hirschsprung' s disease Aphakia Deaf Gigantism Duodenal atresia Multiple anomaly syndromes Total

No. Cases

Unilat. reflux Unilat. duplex with reflux

TABLE

* Of these 60 patients 11 had hypospadias and 28 were retarded. TABLE

5. IVP findings in 61 studies of right cryptorchidism

Abnormality

No. Pts. Mental retardation Retardation plus hypospadias Cleft palate plus hypospadias Multiple anomalies plus hypospadias Hypospadias Micro penis Meningomyelocele Cardiac anomalies Hypopituitary hypogonadism Biliary duct agenesis Multiple anomaly syndromes Total

TABLE

Comments Previously documented upper tract damage Required pyeloplasty, pt. had ventricular septa! defect

Minor Minor

belly syndrome. 8 • 9 Serum testosterone levels in adult patients with unilateral cryptorchidism are no different from a control group, although sperm density is only 27 million per cc compared to 73 million per cc in controls. 19 Of the patients who have married 76 per cent with unilateral cryptorchidism and 44 per cent with bilateral cryptorchidism have had children. 20 Recommendations regarding timing of an operation for cryptorchidism are based more on psychological reasons than any other, although it is believed that an operation should be done in patients ~4 years old because of possible germinal cell failure, if it is delayed. 21 ' 22 The question of greatest interest to us in this review is whether there are any other congenital anomalies associated with cryptorchidism, particularly upper urinary tract anomalies. It is apparent from our patient series that cryptorchidism often is found as part of multiple anomaly syndromes, especially

bilateral cryptorchidism. The finding of cryptorchidism in association with hypospadias in 16 cases is not surprising, since both probably reflect disturbances in androgen-mediated events, occurring at about the same time of intrauterine development. The association of mental retardation with cryptorchidism was described in a series of 100 idiots, of whom 6 had bilateral and 12 had unilateral undescended testes. 23 There is little discussion about this point in the literature, although we found that 50 of our 334 patients were retarded and that this was the only associated problem in 20 patients. In 1910 Anders reviewed the literature and found that 94 per cent of 135 patients with anomalous genitalia had upper tract anomalies. 24 In 1951 Campbell reported that 33 per cent of 292 autopsied boys with cryptorchidism had associated genitourinary anomalies. 25 Based on these data it generally has been recommended that children with cryptorchidism should have an IVP performed in case of silent upper urinary tract disease. However, Campbell's data should not be used as a base line for several reasons: all of the children had died, 218 of the 292 patients had bilateral cryptorchidism and 175 had other nonurologic anomalies, which does not constitute a representative series. Also, no information was given regarding the 92 patients who had other urologic problems. Certainly, this series should not be used as a comparison to patients with asymptomatic cryptorchidism. There have been many reports regarding findings on IVPs in asymptomatic cryptorchid boys and most recommend that an IVP should be done routinely. The reports that provide sufficient information to classify the upper tract anomalies into major, significant or minor are summarized in table 8. Including our own data on asymptomatic patients 902 cryptorchid boys had an IVP and abnormalities were discovered in the upper tract in 90 cases. Only 5 of the abnormalities were major: 1 case of ureteropelvic junction obstruction and 4 cases of reflux. Two patients with reflux had associated complete ureteral duplication. Seventeen patients were found to have significant anomalies, mostly consisting of solitary or horseshoe kidneys. Sixtyeight patients had minor anomalies, most often partial duplex or complete duplex without associated pathology.

CONGENITAL ANOMALIES ASSOCIATED WITH CRYPTORCHIDISM TABLE

8. Literature incidence of abnormal IVPs in cryptorchidism

Reference Kelly, D. G. and Hyland, J.: Birth Defects, 13: 287, 1977 Grossman, H. and Ririe, D. G.: Amer. J. Roentgen., 103: 210, 1968 Watson, R. A., Lennox, K. W. and Gangai, M. P.: J. Urol., 111: 789, 1974 Noe and Patterson 26 Donohue, R. E., Utley, W. L. F. and Maling, T. M.: J. Urol., 109: 912, 1973 Felton, L. M.: J. Urol., 81: 335, 1959 Fa1Tington, G. H. and Kerr, I. H.: Brit. J. Urol., 41: 77, 1969 Tveter, K. J. and Fjaerli, J.: Scand. J. Ural. Nephrol., 9: 171, 1975 Current series Totals

No. Pts.

No. Abnorma! IVPs

Abnormalities Major

Significant

Minor

No. Needing Operation

154 100 84

0 0 0

3 2 2

7

0

12 17

10

0

15

0

140 100

11 14

0 2

4 2

7

0

10

2

61 45 77 141

12

3

8

l 0 0

___±

1

902

90

5

0 0 _! 17

6

4

1 0 0

Recognizing that some 10 per cent of the general population will have upper tract anomalies of varying degrees found on an IVP 26 ' 27 we believe that there is no significant increase in the number of upper tract anomalies discovered in asymptomatic boys and, therefore, we recommend that an IVP not be performed routinely in these patients. REFERENCES 1. Pinch, L., Aceto, T., Jr. and Meyer-Bahlburg, H. F.: Cryptorchidism. A pediatric review. Urol. Clin. N. Amer., 1: 573, 1974.

2. Rajfer, J. and Walsh, P. C.: The incidence of intersexuality in patients with hypospadias and cryptorchidism. J. Urol., 116: 769, 1976. 3. Laurance, B. M., Darby, C. W. and Vanderschueren-Lodeweyckx, M.: Two XX males diagnosed in childhood. Endocrine, renal, and laboratory findings. Arch. Dis. Child., 51: 144, 1976. 4. Hadziselimovic, F. and Herzog, B.: The meaning of the Leydig cell in relation to the etiology of cryptorchidism: an experimental elect:ron-microscopic study. J. Ped. Surg., 11: 1, 1976. 5. Rajfer, J. and Walsh, P. C.: Testicular descent. Normal and abnormal. Symposium on congenital anomalies of the lower genitourinary tract. Urol. Clin. N. Amer., 5: 223, 1978. S. Schechter, J.: An investigation of the anatomical mechanisms of testicular descent. Thesis for Master of Arts Degree, Johns Hopkins University, Baltimore, Maryland, 1963. 7. Mininberg, D. T. and Bingol, N.: Chromosomal abnormalities in undescended testes. Urology, 1: 98, 1973. 8. Gibbons, M. D., Cromie, W. J. and Duckett, J. W., Jr.: Management of the abdominal undescended testicle. J. Urol., 122: 76, 1979. 9. Goulding, F. J. and Garrett, R. A: Twenty-five-year experience with prune belly syndrome. Urology, 12: 329, 1978. 10. Cawthem, T. H., Bottene, C. A. and Grant, D.: Prune belly syndrome associated with Hirschsprung's disease. Amer. J. Dis. Child., 133: 652, 1979. 11. Puchne:r, P. J., Santulli, T. V. and Lattimer, J. K.: Urology problems associated with imperforate anus. Urology, 6: 205, 1975. 12. Raghavaiah, N. G.: Noonan's syndrome associated with cake kidney. Urology, 5: 640, 1975. 13. Turne.r's and Noonan's syndromes. Editorial. Brit. Med. J., 1: 470, 1974. 14. Bond, J. V.: Bilateral Wilms' tumour and urinary-tract anomalies. Letter to the Editor. Lancet, 2: 721, 1975. 15. Aarskog, D.: Intersex masquerading as simple hypospadias. Birth Defects, 7: 122, 1971. 16. Marshall, F. F. and Shermeta, D. W.: Epididymal abnormalities associated with undescended testis. J. Urol., 121: 341, 1979.

10

6

4

_! 68

1

IVP Recommended No Yes Yes No Yes Yes Yes No No

5

17. Martin, D. C. and Menck, H. R.: The undescended testis: management after puberty. J. Urol., 114: 77, 1975. 18. Hinman, F., Jr.: Unilateral abdominal cryptorchidism. J. Urol., 122: 71, 1979. 19. Lipshultz, L. I., Caminos-Torres, R., Greenspan, C. S. and Synder, P. J.: Testicular function after orchiopexy for unilaterally unde-scended testis. New Engl. J. Med., 295: 15, 1976. 20. Atkinson, P. M.: A follow-up study of surgically treated cryptorchid patients. J. Ped. Surg., 10: 115, 1975. 21. Kelalis, P. P. and the Action Committee on Surgery on the Genitalia of Male Children: The timing of elective surgery on the genitalia of male children with particular reference to undescended testes and hypospadias. Pediatrics, 56: 479, 1975. 22. Lattimer, J. K., Smith, A. M., Dougherty, L. J. and Beck, L.: The optimum time to operate for cryptorchidism. Pediatrics, 53: 96, 1974. 23. Uffreduzzi, cited by Coley, W. B.: Operative treatment of undescended or maldescended testis with especial reference to end results. A report of 415 cases. Surg., Gynec. & Obst., 28: 452, 1919. 24. Anders, J. M.: Congenital single kidney with report of a case. The practical significance of the condition with statistics. Amer. J. Med. Sci., 139: 313, 1910. 25. Campbell, M.: Clinical Pediatric Urology. Philadelphia: W. B. Sa.unders Co., p. 324, 1951. 26. Noe, H. N. and Patterson, T. H.: Screening urography in asymptomatic cryptorchid patients. J. Urol., 119: 669, 1978. 27. Leary, F. J., Myers, R. P., Greene, L. F. and Hartman, G. W.: The value of excretory urography as a screening test in asymptomatic patients. J. Urol., 10'1: 850, 1972. EDITORIAL COMMENT These authors review the incidence of upper urinary tract abnormalities associated with cryptorchidism. Since surgically curable abnormalities are found rarely they do not advise the routine use of IVP in patients with undescended testes. Their conclusion is ,uuuu11,,eu by a number of other authors who have made similar oh,serv,,ti,ans It is noteworthy that the most frequent operation performed on the urinary tract in these patients with undescended testes was an antireflux operation. The indication for this procedure was infrequent and I do not suggest that voiding cystourethrography become a routine procedure in patients with undescended testes. Donald C. Martin Department University of Irvine, California