International Urology and Nephrology 8 (1), pp. 55--61 (1976)
Congenital Stricture of Male Urethra N. R. NETTO, Jr., R. C. MARTUCCI, E. S. GONCALVES, J. G. C. FREIRE Clinica Urologica, Sag Paulo, Brasil (Received April 12, 1975)
Seven cases of anterior urethral stricture were studied. The diagnosis was made by exclusion of the inflammatory and traumatic causes. In three cases the stricture was located in the bulbar and in four cases in the penile urethra~ The results obtained were good, with a follow-up of 5 to 50 months. Considerations are made about embryogenic, diagnostic, clinical picture and treatment. The verification of extensive lesions of the urethra determined the use of more complex surgical techniques of Johansen's urethroplasty devised for the treatment of this kind of pathology. The urethral stricture is classified in three kinds, according to its etiology: congenital, inflammatory and traumatic [6]. Leadbetter [6] described two cases of congenital stricture of the penile urethra. The lesion would occur in any point of the urethra; it is more common, however, at the bulb-membranous junction or posterior portion of the navicular fossa [4, 8]. Stephens [12] reported on six cases of bulbar urethral stricture. M o o r m a n [l 1] studied 38 males with urethral stricture, whose age varied from 20 to 54 years 21 cases being in "prostatic age" as considered by him. Cobb et al. [3] analysed 26 cases of congenital stricture of bulbar urethra in boys whose age varied from newborn child to thirteen years (average 51/~ years). They believed that by limiting the age of patients up to 16 years, they could be eliminating as much as possible the acquired stricture. Viville et al. [14] described two cases of congenital stricture of the perineal urethra, stressing the rarity of this condition. Tsuji [13] studied six cases of congenital urethral stricture between 1952 and 1968 and concluded that in Japan this pathology would be more frequent than that of the posterior urethral valves. In the Urological Clinic of Hospital das Clinicas of the University Medical School of Sag Paulo, seven cases of congenital urethral stricture were studied in the period from 1953 till 1973. This work was motivated by the low frequency of this pathological entity.
Etiology The anterior and posterior urethra show different embryological origins. The cloaca, by the second month of fetal life, undergoes a division, giving origin to two cavities: a posterior one, the rectum, and an anterior, the urogenital duct. International Urology and Nephrology 8, 1976
56
Netto et al. : Stricture o f male urethra
T h e a n t e r i o r s e g m e n t o f t h e c l o a c a , l a r g e r t h a n t h e o t h e r , will c o n s e c u t i v e l y undergo differentiation into bladder, primitive urethra and urogenital sinus (Fig. 2). F o r s o m e t i m e , t h e r e r e m a i n s a c o m m u n i c a t i o n b e t w e e n t h e r e c t u m and the urogenital canal through the terminal cloacal canal. The cloacal canal disappears completely at the moment the urorectal septum reaches the cloacal
13 11
9
~i i~"~ +
~~
.."~ s
12 #
Fig. 1. Reconstruction of the caudal portion of the h u m a n fetus (two months old) showing the complete septation of the rectus and urogenital sinus, and the relations of the urogenital ducts: 1 -- gonads, 2 -- mesonephros, 4 -- ureter, 5 -- Mullerian duct, 6 -- Wolffian duct, 7 -- rectum, 7' -- carloma deepening with the perineal septum, 8 -- spinal cord, 9 -- anal membrane, 10 -- phallic portion of the urogenital sinus, 10' -- pelvic portion of the urogenital sinus, 10" -- primitive urethra, 11 -- bladder, 12 -- uterovaginal duct, 13 -- mesonephric fold
1
3
2
2
3
4
5
Fig. 2. Model of the genital cord, bladder and primitive urethra, in a 25 m m long h u m a n embryo: 1 -- bladder, 2 -- Mullerian duct, 3 -- Wolffian ducts, 4 -- ureter, 5 -- primitive urethra, 6 -- Muller's eminence, 7 -- Wolffian ducts' endings, 8 -- urogenital sinus International Urology and Nephrology 8, 1976
N e t t o et al.: Stricture o f male urethra
57
membrane and sticks to it. The persistence of this canal causes in the adult the appearance of a fistula between the rectum and urethra [7]. The urorectal septum joining the cloacal membrane divides this structure into two portions: the cranial portion (urogenital membrane) and the distal portion (anal membrane), separated from each other by the primary perineum. In a posterior stage, the ventral segment of the cloaca is incompletely divided into primitive bladder, primitive urethra, pelvic and phallic portions of the urogenital sinus (Fig. 2). The primitive urethra originates in males from the supramontanal urethra (up to the ejaculatory duct openings); in females from the whole urethra. The pelvic segment of the urogenital sinus is responsible for the formation of urethra from the ejaculatory openings till the beginning of the bulbar urethra. The separation point between the urethral segments which derive from the primitive urethra and from the pelvic portion of the urogenital sinus is given by Muller's eminence, which will origin in man the veromontanum. The Mullerian ducts are atrophied in man, except in their terminal portion, where they join to form the prostatic utricle. The Wolffian ducts originate from the epididymis, the vas deferens, the seminal vesicles and the ejaculatory ducts [4]. The mesenchymal penetration between the ectoderm and the endoderm of the most cranial portion of the cloacal membrane determines the formation of a medial prominence, the phallus [7]. As this stricture elongates, there appears a sulcus in its inferior face, which in a secondary stage communicates with the urogenital sinus. This communication is due to the absorption of the urogenital membrane which at that point occluded the inferior face of the urogenital sinus. Later, the sulcus becomes a canal by the approaching and melting of its margins, forming the anterior urethra (bulbar and penile). The stricture would occur due to lack of reabsorption of the urogenital membrane [1] corresponding to the meatal stenosis that occurs in girls [9]. Stephens [12], however, refers to the possibility of congenital urethral stricture not arising by an accurate embryological mechanism, but being a fortuitous stricture, that could happen the same way as in any other tubular structure of the organism. Viville et al. [14] assume that these are urethral valves of Young's type 111 [16, 17]. However, the existence of this type 111 of valve is not accepted by all authors; besides, the secondary lesions to this type of valve are too serious and almost always incompatible with life. Diagnosis and treatment
The diagnosis of the congenital urethral stricture was made by exclusion of the inflammatory and traumatic causes. Seven cases were studied, all of them white, their age varying between 1 and 47 years (average 17.7 years). Most of the congenital urethral lesions result in serious vesical urethral and renal lesions. The alterations of the urinary tract are directly proportional to the grade of obstruction and, when first seen, the patient has generally too International Urology and Nephrology 8, 1976
58
N e t t o et al.: Stricture o f male urethra
m a n y alterations, considering that micturition starts in the fetus by the f o u r t h m o n t h o f intrauterine life. In this way, congenital obstructions o f the u r e t h r a m a y cause serious dilatations and frequently p r o d u c e irreversible lesions t h a t
Fig. 3. Mictional cystourethrography -- penile stenosis
Fig. 4. Same case. Postoperative control International
Urology and Nephrolo,2y 8, 1976
N e t t o et al. : Stricture o f male urethra
59
may be incompatible with life [10]. The symptoms are not different from those of any obstructive process of the inferior urinary tract. They are generally noted later, around two or three years of age [6] and not always receiving attention from children and young men [14]. The opposite of that happens with the anterior and posterior urethral valves [2, 15] urethral catheterisation being very difficult or even impossible in
Fig. 5. Mictional c y s t o u r e t h r o g r a p h y - penile stenosis
these cases. Preoperatively, the patients underwent urethral calibration, intravenous urography and urethrocystography. The stricture was located in the bulbar urethra in three cases, and in the penile urethra in four cases. The damaged segment was long, more than four centimeters in six cases, only one patient having an annular stenosis (opposite to what is described in the literature) [3, 4, 14]. In the cases of stricture in the penile urethra the whole length of the urethra was compromised. All of the patients presented urinary infection in the preoperative phase, on account of having been surgically or instrumentally manipulated. Treatment was surgical: six cases were treated by Johansen's urethroplasty [5] and one with urethrectomy with termino-terminal urethrography (the case of the annular lesion of the bulbar urethra). Two cases were not submitted to the second stage of urethroplasty. In this way, Cobb et al. [3] dilated in 18 cases the urethra with the Kollmann dilator and in eleven cases treated the lesion through internal urethrotomy. Viville et al. [14] obtained success by cutting the urethra longitudinally at the level of the stricture and suturing transversally. Opposite to International Urology and Nephrology 8, 1976
60
Netto et al. : Stricture o f male urethra
what is frequently seen [3, l l , 14], the large extension of the urethral lesion in our cases necessitated the employment of more complex surgical techniques of Johansen's type, or urethrectomy with termino-terminal suture of the urethral stumps. Results The period of follow-up varied between 5 and 50 months (average 18.5 months). The results were evaluated through functional and radiological studies. We consider as functional the evaluation of symptoms and changes of the urinary stream. G o o d results were obtained in those cases which did not show postoperative urinary symptoms and the stream was normal regarding the calibre, intensity, continuity, velocity, presence of effort to micturition and appearance of the urine; in the regular results, one of these items was altered, and in the bad results, both the symptoms and the urinary stream were altered. The results were good in six cases and regular in one case. As regards urethrocystography, we considered a good result when it showed that the urethra was permeable and regular, with no vesical residue and that the urethral calibre at the level of the lesion was normal. A regular result means that the urethra was permeable and irregular with no vesical residue and with a smaller calibre at the level of the operated zone. When there was a dilatation of the urethra proximally to the operated zone, or a stricture, with or without vesical residue, the result was considered bad. The urethrocystography was normal in three cases and regular in two. There were no alterations in the superior urinary tract in cases which were examined by postoperative intravenous urography.
Since this paper was prepared, two other cases of congenital stenosis of the anterior urethra have come to our service. One was treated by Johansen's urethroplasty and the other will be subjected to the same operation.
References 1. Arey, L. B. : Developmental Anatomy. A textbook and laboratory manual of embryology, 7th ed. W. B. Saunders, Philadelphia 1965, pp. 3-12. 2. Cheng, Yang Chang: Anterior urethral valves: a case report. J. Urol. 100, 29 (1968). 3. Cobb, B. G., Wolff, J. A., Ansell, J. S.: Congenital stricture of the proximal urethral bulb. J. Urol. 99, 629 (1968). 4. Grasset, D.: Obstructions au bas appareil urinaire. J. d'Urol, et Ndphrol. 76, 1 (1970). 5. Johansen, B. : Reconstruction of the male urethra in strictures. Acta Chit. Scand. Suppl. 176 (1953). 6. Leadbetter, G. W., Jr., Leadbetter, W. F.: Urethral strictures in male children. J. Urol. 87, 409 (1962). International Urology and Nephrology 8, 1976
Netto et al.: Stricture o f male urethra
61
7. Lordy, C., Oria, J., Aquino, J. T.: Embriologia Humana e Comparada. Cia, Melhoramentos de Sao Paulo, 1940, pp. 572-661. 8. Lowsley, O. S., Kirwin, T. J.: Clinical Urology. 3rd ed. The Williams & Wilkins Co., Baltimore 1956, pp. 280-281. 9. Lyon, R. P., Smith, D. R.: Distal urethral stenosis. J. Urol. 89, 414 (1963). 10. Mogg, R. A.: Congenital anomalies o f the urethra. Brit. J. Urol. 40, 636 (1968). 11. Moorman, J. G.: Congenital bulbar urethral stenosis as a cause of diseases of the urogenital function. Urologie 11, 157 (1972). 12. Stephens, F. D.: Congenital Malformations of the Rectum, Anus and Genitourinary Tracts. E. S. Livingstone Ltd., London 1963, pp. 219-245. 13. Tsuji, cit. Grasset, D. [4]. 14. Viville, Ch., Thomas, M., Jaek, D., Berger, J." Une maladie rare: le st6nose apparemmerit cong6nitale de l'ur6tre p6rineal chez l'homme. A p r o p o s de deux observations. J. d'Urol, et Nkphrol. 77, 715 (1971). 15. Williams, D. I.: Paediatric Urology. Butterworths, London 1968. 16. Young, H. H., Davis, D. M.: Young's Practice of Urology. W. B. Saunders, Philadelphia, Vol. 3, 1926, p. 84. 17. Young, B. W.: Embriologie des valvules urethrales. Rapport au 64 ~ Congr~s Franqais d'Urologie, Paris 1970.
International Urology and Nephrology 8, 1976
Congenital Stricture of Male Urethra N. R. NETTO, Jr., R. C. MARTUCCI, E. S. GONCALVES, J. G. C. FREIRE Clinica Urologica, Sag Paulo, Brasil (Received April 12, 1975)
Seven cases of anterior urethral stricture were studied. The diagnosis was made by exclusion of the inflammatory and traumatic causes. In three cases the stricture was located in the bulbar and in four cases in the penile urethra~ The results obtained were good, with a follow-up of 5 to 50 months. Considerations are made about embryogenic, diagnostic, clinical picture and treatment. The verification of extensive lesions of the urethra determined the use of more complex surgical techniques of Johansen's urethroplasty devised for the treatment of this kind of pathology. The urethral stricture is classified in three kinds, according to its etiology: congenital, inflammatory and traumatic [6]. Leadbetter [6] described two cases of congenital stricture of the penile urethra. The lesion would occur in any point of the urethra; it is more common, however, at the bulb-membranous junction or posterior portion of the navicular fossa [4, 8]. Stephens [12] reported on six cases of bulbar urethral stricture. M o o r m a n [l 1] studied 38 males with urethral stricture, whose age varied from 20 to 54 years 21 cases being in "prostatic age" as considered by him. Cobb et al. [3] analysed 26 cases of congenital stricture of bulbar urethra in boys whose age varied from newborn child to thirteen years (average 51/~ years). They believed that by limiting the age of patients up to 16 years, they could be eliminating as much as possible the acquired stricture. Viville et al. [14] described two cases of congenital stricture of the perineal urethra, stressing the rarity of this condition. Tsuji [13] studied six cases of congenital urethral stricture between 1952 and 1968 and concluded that in Japan this pathology would be more frequent than that of the posterior urethral valves. In the Urological Clinic of Hospital das Clinicas of the University Medical School of Sag Paulo, seven cases of congenital urethral stricture were studied in the period from 1953 till 1973. This work was motivated by the low frequency of this pathological entity.
Etiology The anterior and posterior urethra show different embryological origins. The cloaca, by the second month of fetal life, undergoes a division, giving origin to two cavities: a posterior one, the rectum, and an anterior, the urogenital duct. International Urology and Nephrology 8, 1976
56
Netto et al. : Stricture o f male urethra
T h e a n t e r i o r s e g m e n t o f t h e c l o a c a , l a r g e r t h a n t h e o t h e r , will c o n s e c u t i v e l y undergo differentiation into bladder, primitive urethra and urogenital sinus (Fig. 2). F o r s o m e t i m e , t h e r e r e m a i n s a c o m m u n i c a t i o n b e t w e e n t h e r e c t u m and the urogenital canal through the terminal cloacal canal. The cloacal canal disappears completely at the moment the urorectal septum reaches the cloacal
13 11
9
~i i~"~ +
~~
.."~ s
12 #
Fig. 1. Reconstruction of the caudal portion of the h u m a n fetus (two months old) showing the complete septation of the rectus and urogenital sinus, and the relations of the urogenital ducts: 1 -- gonads, 2 -- mesonephros, 4 -- ureter, 5 -- Mullerian duct, 6 -- Wolffian duct, 7 -- rectum, 7' -- carloma deepening with the perineal septum, 8 -- spinal cord, 9 -- anal membrane, 10 -- phallic portion of the urogenital sinus, 10' -- pelvic portion of the urogenital sinus, 10" -- primitive urethra, 11 -- bladder, 12 -- uterovaginal duct, 13 -- mesonephric fold
1
3
2
2
3
4
5
Fig. 2. Model of the genital cord, bladder and primitive urethra, in a 25 m m long h u m a n embryo: 1 -- bladder, 2 -- Mullerian duct, 3 -- Wolffian ducts, 4 -- ureter, 5 -- primitive urethra, 6 -- Muller's eminence, 7 -- Wolffian ducts' endings, 8 -- urogenital sinus International Urology and Nephrology 8, 1976
N e t t o et al.: Stricture o f male urethra
57
membrane and sticks to it. The persistence of this canal causes in the adult the appearance of a fistula between the rectum and urethra [7]. The urorectal septum joining the cloacal membrane divides this structure into two portions: the cranial portion (urogenital membrane) and the distal portion (anal membrane), separated from each other by the primary perineum. In a posterior stage, the ventral segment of the cloaca is incompletely divided into primitive bladder, primitive urethra, pelvic and phallic portions of the urogenital sinus (Fig. 2). The primitive urethra originates in males from the supramontanal urethra (up to the ejaculatory duct openings); in females from the whole urethra. The pelvic segment of the urogenital sinus is responsible for the formation of urethra from the ejaculatory openings till the beginning of the bulbar urethra. The separation point between the urethral segments which derive from the primitive urethra and from the pelvic portion of the urogenital sinus is given by Muller's eminence, which will origin in man the veromontanum. The Mullerian ducts are atrophied in man, except in their terminal portion, where they join to form the prostatic utricle. The Wolffian ducts originate from the epididymis, the vas deferens, the seminal vesicles and the ejaculatory ducts [4]. The mesenchymal penetration between the ectoderm and the endoderm of the most cranial portion of the cloacal membrane determines the formation of a medial prominence, the phallus [7]. As this stricture elongates, there appears a sulcus in its inferior face, which in a secondary stage communicates with the urogenital sinus. This communication is due to the absorption of the urogenital membrane which at that point occluded the inferior face of the urogenital sinus. Later, the sulcus becomes a canal by the approaching and melting of its margins, forming the anterior urethra (bulbar and penile). The stricture would occur due to lack of reabsorption of the urogenital membrane [1] corresponding to the meatal stenosis that occurs in girls [9]. Stephens [12], however, refers to the possibility of congenital urethral stricture not arising by an accurate embryological mechanism, but being a fortuitous stricture, that could happen the same way as in any other tubular structure of the organism. Viville et al. [14] assume that these are urethral valves of Young's type 111 [16, 17]. However, the existence of this type 111 of valve is not accepted by all authors; besides, the secondary lesions to this type of valve are too serious and almost always incompatible with life. Diagnosis and treatment
The diagnosis of the congenital urethral stricture was made by exclusion of the inflammatory and traumatic causes. Seven cases were studied, all of them white, their age varying between 1 and 47 years (average 17.7 years). Most of the congenital urethral lesions result in serious vesical urethral and renal lesions. The alterations of the urinary tract are directly proportional to the grade of obstruction and, when first seen, the patient has generally too International Urology and Nephrology 8, 1976
58
N e t t o et al.: Stricture o f male urethra
m a n y alterations, considering that micturition starts in the fetus by the f o u r t h m o n t h o f intrauterine life. In this way, congenital obstructions o f the u r e t h r a m a y cause serious dilatations and frequently p r o d u c e irreversible lesions t h a t
Fig. 3. Mictional cystourethrography -- penile stenosis
Fig. 4. Same case. Postoperative control International
Urology and Nephrolo,2y 8, 1976
N e t t o et al. : Stricture o f male urethra
59
may be incompatible with life [10]. The symptoms are not different from those of any obstructive process of the inferior urinary tract. They are generally noted later, around two or three years of age [6] and not always receiving attention from children and young men [14]. The opposite of that happens with the anterior and posterior urethral valves [2, 15] urethral catheterisation being very difficult or even impossible in
Fig. 5. Mictional c y s t o u r e t h r o g r a p h y - penile stenosis
these cases. Preoperatively, the patients underwent urethral calibration, intravenous urography and urethrocystography. The stricture was located in the bulbar urethra in three cases, and in the penile urethra in four cases. The damaged segment was long, more than four centimeters in six cases, only one patient having an annular stenosis (opposite to what is described in the literature) [3, 4, 14]. In the cases of stricture in the penile urethra the whole length of the urethra was compromised. All of the patients presented urinary infection in the preoperative phase, on account of having been surgically or instrumentally manipulated. Treatment was surgical: six cases were treated by Johansen's urethroplasty [5] and one with urethrectomy with termino-terminal urethrography (the case of the annular lesion of the bulbar urethra). Two cases were not submitted to the second stage of urethroplasty. In this way, Cobb et al. [3] dilated in 18 cases the urethra with the Kollmann dilator and in eleven cases treated the lesion through internal urethrotomy. Viville et al. [14] obtained success by cutting the urethra longitudinally at the level of the stricture and suturing transversally. Opposite to International Urology and Nephrology 8, 1976
60
Netto et al. : Stricture o f male urethra
what is frequently seen [3, l l , 14], the large extension of the urethral lesion in our cases necessitated the employment of more complex surgical techniques of Johansen's type, or urethrectomy with termino-terminal suture of the urethral stumps. Results The period of follow-up varied between 5 and 50 months (average 18.5 months). The results were evaluated through functional and radiological studies. We consider as functional the evaluation of symptoms and changes of the urinary stream. G o o d results were obtained in those cases which did not show postoperative urinary symptoms and the stream was normal regarding the calibre, intensity, continuity, velocity, presence of effort to micturition and appearance of the urine; in the regular results, one of these items was altered, and in the bad results, both the symptoms and the urinary stream were altered. The results were good in six cases and regular in one case. As regards urethrocystography, we considered a good result when it showed that the urethra was permeable and regular, with no vesical residue and that the urethral calibre at the level of the lesion was normal. A regular result means that the urethra was permeable and irregular with no vesical residue and with a smaller calibre at the level of the operated zone. When there was a dilatation of the urethra proximally to the operated zone, or a stricture, with or without vesical residue, the result was considered bad. The urethrocystography was normal in three cases and regular in two. There were no alterations in the superior urinary tract in cases which were examined by postoperative intravenous urography.
Since this paper was prepared, two other cases of congenital stenosis of the anterior urethra have come to our service. One was treated by Johansen's urethroplasty and the other will be subjected to the same operation.
References 1. Arey, L. B. : Developmental Anatomy. A textbook and laboratory manual of embryology, 7th ed. W. B. Saunders, Philadelphia 1965, pp. 3-12. 2. Cheng, Yang Chang: Anterior urethral valves: a case report. J. Urol. 100, 29 (1968). 3. Cobb, B. G., Wolff, J. A., Ansell, J. S.: Congenital stricture of the proximal urethral bulb. J. Urol. 99, 629 (1968). 4. Grasset, D.: Obstructions au bas appareil urinaire. J. d'Urol, et Ndphrol. 76, 1 (1970). 5. Johansen, B. : Reconstruction of the male urethra in strictures. Acta Chit. Scand. Suppl. 176 (1953). 6. Leadbetter, G. W., Jr., Leadbetter, W. F.: Urethral strictures in male children. J. Urol. 87, 409 (1962). International Urology and Nephrology 8, 1976
Netto et al.: Stricture o f male urethra
61
7. Lordy, C., Oria, J., Aquino, J. T.: Embriologia Humana e Comparada. Cia, Melhoramentos de Sao Paulo, 1940, pp. 572-661. 8. Lowsley, O. S., Kirwin, T. J.: Clinical Urology. 3rd ed. The Williams & Wilkins Co., Baltimore 1956, pp. 280-281. 9. Lyon, R. P., Smith, D. R.: Distal urethral stenosis. J. Urol. 89, 414 (1963). 10. Mogg, R. A.: Congenital anomalies o f the urethra. Brit. J. Urol. 40, 636 (1968). 11. Moorman, J. G.: Congenital bulbar urethral stenosis as a cause of diseases of the urogenital function. Urologie 11, 157 (1972). 12. Stephens, F. D.: Congenital Malformations of the Rectum, Anus and Genitourinary Tracts. E. S. Livingstone Ltd., London 1963, pp. 219-245. 13. Tsuji, cit. Grasset, D. [4]. 14. Viville, Ch., Thomas, M., Jaek, D., Berger, J." Une maladie rare: le st6nose apparemmerit cong6nitale de l'ur6tre p6rineal chez l'homme. A p r o p o s de deux observations. J. d'Urol, et Nkphrol. 77, 715 (1971). 15. Williams, D. I.: Paediatric Urology. Butterworths, London 1968. 16. Young, H. H., Davis, D. M.: Young's Practice of Urology. W. B. Saunders, Philadelphia, Vol. 3, 1926, p. 84. 17. Young, B. W.: Embriologie des valvules urethrales. Rapport au 64 ~ Congr~s Franqais d'Urologie, Paris 1970.
International Urology and Nephrology 8, 1976
