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Radiological findings of the urinary tract in hypospadias: A report of 110 cases
Clin. Radiol. (1979) 30, 471-476
Radiological Findings of the Urinary Tract in Hypospadias: A Report of 110 Cases j. ROZENMAN,* M. HERTZ* and H, BOICHISt
From the Departments o f *Diagnostic Radiology and ~Pediatrics, The Chaim Sheba Medical Centre, Tel-Hashomer, Israel, affiliated to the Tel-Aviv University Sackler School of Medicine One hundred and ten patients with hypospadias underwent excretory urography and micturating cystourethrography before surgical correction. Urographic studies revealed abnormalities in 50 patients (45%), mainly anomalies of ascent and rotation of the kidneys. None of these patients required corrective surgery. Voiding cysto-urethrography disclosed disease of the urinary tract in 65 of 110 patients (59%). Meatal stenosis was found in 38 patients and a rudimentary vagina or a prostatic utricle was revealed in 11 others. Vesico. ureteral reflux was present in 14 patients, seven of whom had meatal stenosis as well. Cystitis was found in eight and a stricture of the membraneous urethra in one patient. The voiding cysto-urethrogram is able to demonstrate functionally significant meatal stenosis, genital abnormalities and vesico-ureteral reflux. It can also serve as a useful baseline for comparison with postoperative studies. We therefore consider that it should be included in the preoperative work-up of every patient with hypospadias. Excretory urography, however, is perhaps indicated only in those with urinary tract infection, or in patients otherwise symptomatic.
INTRODUCTION Hypospadias is a congenital anomaly of defective closure of the urethra in which the meatus is situated proximal and ventral to its normal location. It may be classified as glandular, penile, penoscrotal and perineal hypospadias according to the location of the meatus (Aarskog, 1970; Gray and Skandalakis, 1972). The anomaly represents incomplete masculinisation of the external genitalia (Horton and Devine, 1972) and it may be accompanied by other genital as well as renal anomalies (Campbell and Harrison, 1970). In most cases the defect will require surgical correction. In order to assess the urinary tract prior to surgery in patients with hypospadias, both excretory urography (EU) and micturating cysto-urethrography (MCU) have been routinely carried out at our hospital. Conflicting reports have been published questioning the necessity of urography in patients with hypospadias (Felton, 1959; Neyman and Schirmer, 1965; McArdle and Lebowitz, 1975; Fallon et al., 1976; Lutzker et al., 1977). No large series of MCU examinations has yet been reported. We have reviewed our material and records, and present the radiological Findings of the upper and lower urinary tract in 110 patients with hypospadias.
MATERIAL AND METHODS The records of all patients with hypospadias diagnosed at the Chaim Sheba Medical Centre from
1969 to 1977 were reviewed. One hundred and ten patients who underwent both excretory urography (EU) and micturating cysto-urethrography (MCU) after being referred to our centre, and before correction of their hypospadias, are included in this study. On reviewing the urograms, note was made of associated congenital anomalies and malfunction of the urinary tract, such as hydronephrosis and reflux nephropathy. The MCU was studied to detect either any possible dilatation of the urethra or the filling of a rudimentary vagina or prostatic utricle. We also looked for signs of vesico-ureteral reflux.
RESULTS The age distribution of the patients is set forth in Table 1. Ninety were below five years of age, while the complete age range was from one week to 45 years old. In most of the patients hypospadias was the only indication for the radiological examinations. However, 20 had symptoms and signs, including voiding difficulties in 11, urinary tract infection in eight and enuresis in one patient. Five others with a varying degree of hypospadias had additional congenital anomalies in other organ systems. Table 2 shows the findings on EU. Abnormalities were discovered in 50 patients (45%) with mild malrotation of the kidney in 32 (29%). In no case was it severe enough to cause hydronephrosis. The left kidney was more frequently involved than the right one. An
472
CLINICAL RADIOLOGY Table 1 -- Ages of 110 patients with hypospadias
Table 3 -- Cysto-urethrography in 110 patients with hypospadias
Years
Normal Meatal stenosis Meatal stenosis with reflux Vesico-ureteral reflux Vagina or utriculus prostaticus Cystitis Urethral stricture
0 1 1-2
2 5 5 15 15+
49 15 26 8 12
Table 2 - Excretory urography in 110 patients with hypospadias Normal Malrotation Left 19 Right 3 Bilateral 10 Ectopic kidney Uretero-pelvic junction obstruction Double collecting system Dilated collecting system
60 32
4 5 3 6
ectopic kidney was seen in four patients (3.6%). A complete double-collecting system was present in three patients, two of whom had vesico-ureteral reflux on MCU. Hydronephrosis was present in six patients two had vesico-ureteral reflux and one uretero-vesical obstruction.
45 31 7 7 11 8 1
The cysto-urethrographic findings are listed in Table 3. Sixty-five patients (59%) had an abnormal cystogram. The most frequent finding was meatal stenosis in 38 patients (45%), shown as slowing of the stream and dilatation of the urethra over its entire length. Occasionally a trabeculated bladder was also seen. Irregularity of the bladder wall as the only pathological finding was present in eight cases, while vesico-ureteral reflux was demonstrated in 14 patients. Only four of these patients had documented urinary tract infection, while seven in the group of 14 had meatal stenosis as well. The filling of a rudimentary vagina or a prostatic utricle was seen in 11 patients. Hypospadias was severe in all of them, the meatus being situated in the perineal or scrotal area, and the urethra was short.
i2 i<
'!!!,
Fig. 1 (!eft) Glandular hypospadias. Downward deflection of urinary stream, Fig. 2 (right) Glandular hypospadias with meatal stenosis. Dilated urethra.
R A D I O L O G I C A L F I N D I N G S OF THE U R I N A R Y T R A C T IN H Y P O S P A D I A S
473
Case 3. A three-month-old infant with glandular hypospadias. The urethra is of normal length and width, and a prostatic utricle is opacified (arrow) (Fig. 3). Case 4. A two-month-old premature infant with other congenital anomalies (deformed ears, hydrocephalus) as well as glandular hypospadias. The bladder wall is irregular and the posterior urethra is somewhat narrow. A cyst-like cavity is filled during voiding through a small communication with the posterior urethra. This is a cyst of the prostatic utricle (Fig. 4). Case 5. A 4½-year-old boy with penile hypospadias, cryptorchism and a bifid scrotum. During voiding there is backfiow into a rudimentary vagina, which appears as an elongated cavity continuous with the bulbar urethra and parallel to the posterior urethra (Fig. 5). Case 6. A six-month-old infant with penoscrotal hypospadias. Normal cysto-urethrogram, except for a short urethra (Fig. 6a). The same patient at the age of four years following correction of the hypospadias, with the creation of a new distal urethral canal, and with the meatus at the glans of the penis. Examined because of marked voiding difficulties resulting in urinary retention. MCU shows a very dilated and irregular urethra with a fistula at its distal end and a sinus tract at the site of the former diverting urethrostomy. A large prostatic utricle is opacified (Fig. 6b). Schematic drawing (Fig. 6c).
Case 1. A 21-year-old male, otherwise well. Glandular hypospadias with chordee. The urethra is of normal width with the meatus located on the ventral aspect of the glans penis and the stream deflected downward (Fig. 1). Case 2. A 2½-year-old boy. Straining on voiding. Glandular hypospadias. Dilatation of the urethra over its entire length due to meatal stenosis (Fig_ 2).
DISCUSSION Embryological development in the e i g h t h w e e k o f e m b y r o n a l life the labioscrotal swellings lateral to the p h a l l u s m o v e caudally. T h e y s t a r t to fuse f r o m the base t o w a r d s the tip o f the phallus, a n d in this way the u r e t h r a l groove is covered. S h o u l d the urethral folds be a r r e s t e d during this process o f closure h y p o s p a d i a s will ensue. T h e severity o f this will d e p e n d o n the time o f arrest. In penile h y p o s p a d i a s a f i b r o u s b a n d b e t w e e n the m e a t u s and the b l i n d d i m p l e o n the penis causes s h o r t e n i n g a n d a d o w n ward curving o f the penis -- a c o n d i t i o n k n o w n as
Fig. 4 - Glandular hypospadias with cyst of prostatic utricle. Large cyst-like cavity filled from posterior urethra compressing the bladder neck,
Fig. 5 Penile hypospadias and rudimentary vagina. Elongated cavity continuous with bulbar urethra filled during voiding: vagina.
Fig. 3 - Glandular hypospadias. Small prostatic utricle 0pacified (arrow).
ILLUSTRATIVE CASE R E P O R T S
474
S
CLINICAL
3
chordee (Paul and Kanagasuntheram, 1956). In scrotal and perineal hypospadias there is often associated maldevelopment of the gonads (Campbell and Harrison, 1979). Due to a deficiency of masculinising influence the Mullerian duct structures fail to regress, while the Wolffian duct fails to develop completely. Howard (1948) has shown a correlation between the size of the Mullerian duct remnant and the severity of the hypospadias. He found that in penile hypospadias the prostatic utricle is small (Fig. 3) while in penoscrotal and perineal hypospadias the utricle is markedly enlarged and may even take the form of a well-developed vagina. The ascent and rotation of the kidneys occurs during the sixth and seventh week, somewhat earlier than the development of the urethra. However there is some overlap of maturation and it is possible that the
RADIOLOGY
Fig. 6 - Penoscrotal hypospadias. (a, top left) Urethra short, otherwise normal (b, right) After correction markedly dilated urethra and ffiling of a large prostatic utricle. (c, bottom left) Schematic drawing of b, in which 1 is bladder; 2, prostatic utricle; 3, urethra; 4, sinus tract; 5, fistula; 6, stenosed meatus.
development of the kidneys and the urethra is influ. enced by a common factor (Gray and Skandalakis, 1972). According to the literature abnormalities of the upper urinary tract are more frequently found in patients with hypospadias than in the general population (Felton, 1959; Kennedy, 1961; Neyman and Schirmer, 1965; Lutzker e t al., 1977). Similarly, a high incidence of anomalies was found in our series, mainly those of malrotation and ectopia of the kidneys (32 and 4 patients, respectively). The overall incidence of malrotated kidneys in the population is reported to be between 1:400 .and 1:2000 (Campbell and Harrison, 1970; Gray and Skandalakis, 1972; Lutzker et al., 1977). Such differences depend on the degree of malrotation considered to be anomalous. In our patients it was mostly of a minor degree
RADIOLOGICAL FINDINGS OF THE URINARY TRACT IN HYPOSPADIAS and did not cause pelvi-ureteral obstruction. Renal ectopia occurs in the general population in a frequency of 0.12% (Gray and Skandalakis, 1972), and the incidence of 3.6% in our series is significantly greater. Duplication was found in three cases, an i~cidence that might be expected in the general population ( 0 . 6 - 4 % ) (Campbell, 1970; Gray and Skandalakis, 1972). In contrast to the upper urinary tract tire bladder and urethra have not been extensively studied radiologically in patients with hypospadias, and to date no large series has been reported. Avellan and Johanson (1973) perform cystourethrography in all patients ~vith hypospadias prior to surgical correction. Shopfner (1969) stresses the importance of adequately demonstrating the lower urogenital tract in cases of intersex (including hypospadias) early in life to allow the assignment of a practical sex. A Mullerian duct remnant, i.e. a utriculus or rudimentary vagina was demonstrated in 11 of our patients. In all of them the meatus was located in the penoscrotal or perineal region. In some other cases the Mullerian duct remnant may not have been demonstrated because no compression of the external urethral orifice was applied (Fig. 6a). Compression increases the intra-urethral pressure and may result in a backflow of contrast material into a prostatic utricle or vagina (Marcinski and Grzbowska, 1969). A normal utricle according to Howard (1948) may extend up to the base of the prostate. It may become enlarged due to obstruction by valves or desquamated epithelium, and may then present as a fluid-filled mass. When the Mullerian duct remnant is situated more distally and is elongated in form it is termed a vagina. The patient with the cyst of the prostatic utricle (Fig. 4) had signs of bladder outlet obstruction due to compression by the cyst, which filled with contrast material during voiding. Obstruction of this type has been reported in a few cases (Polse and Edlebrock, 1968; Neustein and Schutte, 1968), and in some calculi had formed in the cyst proper (Spence and Chenoweth, 1957). Cysto-urethrography as well as demonstrating possible Mullerian duct remnants shows the calibre of the urethra. Thus a wide urethra indicates a functionally significant meatal stenosis (Fig. 2). Conversely a normal urethrogram in the presence of a meatus which on physical examination seemed narrow excludes functional stenosis. In our series meatal stenosis was present in one-third of all patients regardless of the location of the meatus. Early meatomy was performed in many patients, even when the definitive correction of the anomaly was postponed. Vesico-ureteral reflux is another possible finding in these patients. Such reflux was present in 14 patients, 10 of whom were asymptomatic while
475
four had proven urinary tract infection. These 10 patients may have had previous infections and 50% of them had meatal stenosis, which is known to cause infection (Campbell and Harrison, 1970). The EU was normal in 10 o f the 14 patients and reflux would not have been suspected in them had we not carried out MCU. As a result of this finding all these patients are now receiving appropriate treatment including antibacterial coverage in the immediate postoperative period. Patients who undergo surgical correction for hypospadias often develop complications (Shafir et al., 1978) such as fistulae, strictures and saccular dilatations (Fig. 6b). It is therefore very useful to have a preoperative urethrogram for comparison. The results of this study lead us to conclude that MCU is indicated in all patients with hypospadias, since this study may reveal a significant degree of meatal stenosis and vesico-ureteral reflux, which may require early specific surgical and medical treatment. Cases with urinary tract infection or who are otherwise symptomatic should be investigated fully by adding EU to the above examination.
REFERENCES
Aarskog, D. (1970). Clinical and cytogenetic studies in hypospadias. Acta paediatrica scandinavia, Suppl. 203, 51, 52, 54, 55. Avellan, L. & Johanson, B. (1973). Preoperative and Postoperative Evaluation in Plastic and Reconstructive Surgery of the Genital Area, ed. Horton, C. E., pp. 370-
381. Little, Brown and Co., Boston. Campbell, M. F. & Harrison, J. H. (1970). Urology, 3rd edn, Vol. 2, pp. 1465, 1598-1606. W. B_ Saunders Co., Philadelphia. Fallon, B., Devine, C. J. & Horton, C. E. (1976). Congenital anomalies associated with hypospadias. Journal of" Urology, 116,585 586. Felton, L. M. (1959). Should intravenous pyelography be a routine procedure for children with cryptorchism or hypospadias? Journal of Urology, 81,335 338. Gray, W. & Skandalakis, J. E. (1972). Embryology COt Surgeons, pp. 443, 595, 625. W. B. Saunders Co., Philadelphia. Horton, C. E. & Devine, C. J. (1972). Hypospadias and epispadias. Clinical Symposia, ed. Roberts, R. H. 24, pp. 2-21. Ciba-Geiger Corp., Summit, New Jersey. Howard, F. S. (1948). Hypospadias with enlargement of the prostatic utricle. Surgery, Gynecology and Obstetrics, 86, 307-315. Kennedy, P. A. J. (1961). Hypospadias: a twenty-year review of 489 cases. Journal of Urology, 85, 814 -817. Lutzker, L. G., Kogan, S. J. & Levitt, S. B. (1977). Is routine intravenous urography indicated in patients with hypospadias? Pediatrics, 59, 630-633. Marcinski, A. & Gryzbowska, B. (1969). Cysto-urethrovaginography in male pseudohermaphroditism. Annals of. Radiology, 13,277 282. McArdle, R. & Lebowitz, R. (1975). Uncomplicated hypospadias and anomalies of upper urinary tract. Need for screening? Urology, 5,712-716.
476
CLINICAL RADIOLOGY
Neyman, M. A. & Schirmer, H. K. A, (1965), Urinary tract evaluation in bypospadias. Journal of Urology, 94,439. Neustein, D_ H. & Schutte, H. (1968)- Mullerian duct cyst with report of a case. British Journal of Urology, 40, 72-78. Paul, M. & Kanagasuntheram, R. (1956). The congenital anomalies of the lower urinary tract. Part II: Hypospadias. British Journal of Urology, 28, 118-122. Poise, S. & Edelbrock, H. (1968). Prostatic utricular enlargement as a cause of vesical outlet obstruction in children. Journal of Urology, 100, 329-332.
Shafir, R. Hertz, M. Bornstein, L. & Tsur, H. (1978). Cysto urethrography as an aid in the evaluation of hypgspadia I and its complications. Journal of Plastic and Recon structive Surgery, 62,722 -726. Shopfner, C. E. (1969). Gynecologic roentgenology i~ children. Seminars in Roen tgenology, 4, 218-234. Spence, H. M. & Chenoweth, V. C. (1957). Cysts of the P~0, static utricle (Mullerian duct cysts): report of two cases in children, each containing calculi, cured by retropuhic operation. Transactions of" the American Association of Genito-urinary Surgeons, 49, 85-91.
Radiological Findings of the Urinary Tract in Hypospadias: A Report of 110 Cases j. ROZENMAN,* M. HERTZ* and H, BOICHISt
From the Departments o f *Diagnostic Radiology and ~Pediatrics, The Chaim Sheba Medical Centre, Tel-Hashomer, Israel, affiliated to the Tel-Aviv University Sackler School of Medicine One hundred and ten patients with hypospadias underwent excretory urography and micturating cystourethrography before surgical correction. Urographic studies revealed abnormalities in 50 patients (45%), mainly anomalies of ascent and rotation of the kidneys. None of these patients required corrective surgery. Voiding cysto-urethrography disclosed disease of the urinary tract in 65 of 110 patients (59%). Meatal stenosis was found in 38 patients and a rudimentary vagina or a prostatic utricle was revealed in 11 others. Vesico. ureteral reflux was present in 14 patients, seven of whom had meatal stenosis as well. Cystitis was found in eight and a stricture of the membraneous urethra in one patient. The voiding cysto-urethrogram is able to demonstrate functionally significant meatal stenosis, genital abnormalities and vesico-ureteral reflux. It can also serve as a useful baseline for comparison with postoperative studies. We therefore consider that it should be included in the preoperative work-up of every patient with hypospadias. Excretory urography, however, is perhaps indicated only in those with urinary tract infection, or in patients otherwise symptomatic.
INTRODUCTION Hypospadias is a congenital anomaly of defective closure of the urethra in which the meatus is situated proximal and ventral to its normal location. It may be classified as glandular, penile, penoscrotal and perineal hypospadias according to the location of the meatus (Aarskog, 1970; Gray and Skandalakis, 1972). The anomaly represents incomplete masculinisation of the external genitalia (Horton and Devine, 1972) and it may be accompanied by other genital as well as renal anomalies (Campbell and Harrison, 1970). In most cases the defect will require surgical correction. In order to assess the urinary tract prior to surgery in patients with hypospadias, both excretory urography (EU) and micturating cysto-urethrography (MCU) have been routinely carried out at our hospital. Conflicting reports have been published questioning the necessity of urography in patients with hypospadias (Felton, 1959; Neyman and Schirmer, 1965; McArdle and Lebowitz, 1975; Fallon et al., 1976; Lutzker et al., 1977). No large series of MCU examinations has yet been reported. We have reviewed our material and records, and present the radiological Findings of the upper and lower urinary tract in 110 patients with hypospadias.
MATERIAL AND METHODS The records of all patients with hypospadias diagnosed at the Chaim Sheba Medical Centre from
1969 to 1977 were reviewed. One hundred and ten patients who underwent both excretory urography (EU) and micturating cysto-urethrography (MCU) after being referred to our centre, and before correction of their hypospadias, are included in this study. On reviewing the urograms, note was made of associated congenital anomalies and malfunction of the urinary tract, such as hydronephrosis and reflux nephropathy. The MCU was studied to detect either any possible dilatation of the urethra or the filling of a rudimentary vagina or prostatic utricle. We also looked for signs of vesico-ureteral reflux.
RESULTS The age distribution of the patients is set forth in Table 1. Ninety were below five years of age, while the complete age range was from one week to 45 years old. In most of the patients hypospadias was the only indication for the radiological examinations. However, 20 had symptoms and signs, including voiding difficulties in 11, urinary tract infection in eight and enuresis in one patient. Five others with a varying degree of hypospadias had additional congenital anomalies in other organ systems. Table 2 shows the findings on EU. Abnormalities were discovered in 50 patients (45%) with mild malrotation of the kidney in 32 (29%). In no case was it severe enough to cause hydronephrosis. The left kidney was more frequently involved than the right one. An
472
CLINICAL RADIOLOGY Table 1 -- Ages of 110 patients with hypospadias
Table 3 -- Cysto-urethrography in 110 patients with hypospadias
Years
Normal Meatal stenosis Meatal stenosis with reflux Vesico-ureteral reflux Vagina or utriculus prostaticus Cystitis Urethral stricture
0 1 1-2
2 5 5 15 15+
49 15 26 8 12
Table 2 - Excretory urography in 110 patients with hypospadias Normal Malrotation Left 19 Right 3 Bilateral 10 Ectopic kidney Uretero-pelvic junction obstruction Double collecting system Dilated collecting system
60 32
4 5 3 6
ectopic kidney was seen in four patients (3.6%). A complete double-collecting system was present in three patients, two of whom had vesico-ureteral reflux on MCU. Hydronephrosis was present in six patients two had vesico-ureteral reflux and one uretero-vesical obstruction.
45 31 7 7 11 8 1
The cysto-urethrographic findings are listed in Table 3. Sixty-five patients (59%) had an abnormal cystogram. The most frequent finding was meatal stenosis in 38 patients (45%), shown as slowing of the stream and dilatation of the urethra over its entire length. Occasionally a trabeculated bladder was also seen. Irregularity of the bladder wall as the only pathological finding was present in eight cases, while vesico-ureteral reflux was demonstrated in 14 patients. Only four of these patients had documented urinary tract infection, while seven in the group of 14 had meatal stenosis as well. The filling of a rudimentary vagina or a prostatic utricle was seen in 11 patients. Hypospadias was severe in all of them, the meatus being situated in the perineal or scrotal area, and the urethra was short.
i2 i<
'!!!,
Fig. 1 (!eft) Glandular hypospadias. Downward deflection of urinary stream, Fig. 2 (right) Glandular hypospadias with meatal stenosis. Dilated urethra.
R A D I O L O G I C A L F I N D I N G S OF THE U R I N A R Y T R A C T IN H Y P O S P A D I A S
473
Case 3. A three-month-old infant with glandular hypospadias. The urethra is of normal length and width, and a prostatic utricle is opacified (arrow) (Fig. 3). Case 4. A two-month-old premature infant with other congenital anomalies (deformed ears, hydrocephalus) as well as glandular hypospadias. The bladder wall is irregular and the posterior urethra is somewhat narrow. A cyst-like cavity is filled during voiding through a small communication with the posterior urethra. This is a cyst of the prostatic utricle (Fig. 4). Case 5. A 4½-year-old boy with penile hypospadias, cryptorchism and a bifid scrotum. During voiding there is backfiow into a rudimentary vagina, which appears as an elongated cavity continuous with the bulbar urethra and parallel to the posterior urethra (Fig. 5). Case 6. A six-month-old infant with penoscrotal hypospadias. Normal cysto-urethrogram, except for a short urethra (Fig. 6a). The same patient at the age of four years following correction of the hypospadias, with the creation of a new distal urethral canal, and with the meatus at the glans of the penis. Examined because of marked voiding difficulties resulting in urinary retention. MCU shows a very dilated and irregular urethra with a fistula at its distal end and a sinus tract at the site of the former diverting urethrostomy. A large prostatic utricle is opacified (Fig. 6b). Schematic drawing (Fig. 6c).
Case 1. A 21-year-old male, otherwise well. Glandular hypospadias with chordee. The urethra is of normal width with the meatus located on the ventral aspect of the glans penis and the stream deflected downward (Fig. 1). Case 2. A 2½-year-old boy. Straining on voiding. Glandular hypospadias. Dilatation of the urethra over its entire length due to meatal stenosis (Fig_ 2).
DISCUSSION Embryological development in the e i g h t h w e e k o f e m b y r o n a l life the labioscrotal swellings lateral to the p h a l l u s m o v e caudally. T h e y s t a r t to fuse f r o m the base t o w a r d s the tip o f the phallus, a n d in this way the u r e t h r a l groove is covered. S h o u l d the urethral folds be a r r e s t e d during this process o f closure h y p o s p a d i a s will ensue. T h e severity o f this will d e p e n d o n the time o f arrest. In penile h y p o s p a d i a s a f i b r o u s b a n d b e t w e e n the m e a t u s and the b l i n d d i m p l e o n the penis causes s h o r t e n i n g a n d a d o w n ward curving o f the penis -- a c o n d i t i o n k n o w n as
Fig. 4 - Glandular hypospadias with cyst of prostatic utricle. Large cyst-like cavity filled from posterior urethra compressing the bladder neck,
Fig. 5 Penile hypospadias and rudimentary vagina. Elongated cavity continuous with bulbar urethra filled during voiding: vagina.
Fig. 3 - Glandular hypospadias. Small prostatic utricle 0pacified (arrow).
ILLUSTRATIVE CASE R E P O R T S
474
S
CLINICAL
3
chordee (Paul and Kanagasuntheram, 1956). In scrotal and perineal hypospadias there is often associated maldevelopment of the gonads (Campbell and Harrison, 1979). Due to a deficiency of masculinising influence the Mullerian duct structures fail to regress, while the Wolffian duct fails to develop completely. Howard (1948) has shown a correlation between the size of the Mullerian duct remnant and the severity of the hypospadias. He found that in penile hypospadias the prostatic utricle is small (Fig. 3) while in penoscrotal and perineal hypospadias the utricle is markedly enlarged and may even take the form of a well-developed vagina. The ascent and rotation of the kidneys occurs during the sixth and seventh week, somewhat earlier than the development of the urethra. However there is some overlap of maturation and it is possible that the
RADIOLOGY
Fig. 6 - Penoscrotal hypospadias. (a, top left) Urethra short, otherwise normal (b, right) After correction markedly dilated urethra and ffiling of a large prostatic utricle. (c, bottom left) Schematic drawing of b, in which 1 is bladder; 2, prostatic utricle; 3, urethra; 4, sinus tract; 5, fistula; 6, stenosed meatus.
development of the kidneys and the urethra is influ. enced by a common factor (Gray and Skandalakis, 1972). According to the literature abnormalities of the upper urinary tract are more frequently found in patients with hypospadias than in the general population (Felton, 1959; Kennedy, 1961; Neyman and Schirmer, 1965; Lutzker e t al., 1977). Similarly, a high incidence of anomalies was found in our series, mainly those of malrotation and ectopia of the kidneys (32 and 4 patients, respectively). The overall incidence of malrotated kidneys in the population is reported to be between 1:400 .and 1:2000 (Campbell and Harrison, 1970; Gray and Skandalakis, 1972; Lutzker et al., 1977). Such differences depend on the degree of malrotation considered to be anomalous. In our patients it was mostly of a minor degree
RADIOLOGICAL FINDINGS OF THE URINARY TRACT IN HYPOSPADIAS and did not cause pelvi-ureteral obstruction. Renal ectopia occurs in the general population in a frequency of 0.12% (Gray and Skandalakis, 1972), and the incidence of 3.6% in our series is significantly greater. Duplication was found in three cases, an i~cidence that might be expected in the general population ( 0 . 6 - 4 % ) (Campbell, 1970; Gray and Skandalakis, 1972). In contrast to the upper urinary tract tire bladder and urethra have not been extensively studied radiologically in patients with hypospadias, and to date no large series has been reported. Avellan and Johanson (1973) perform cystourethrography in all patients ~vith hypospadias prior to surgical correction. Shopfner (1969) stresses the importance of adequately demonstrating the lower urogenital tract in cases of intersex (including hypospadias) early in life to allow the assignment of a practical sex. A Mullerian duct remnant, i.e. a utriculus or rudimentary vagina was demonstrated in 11 of our patients. In all of them the meatus was located in the penoscrotal or perineal region. In some other cases the Mullerian duct remnant may not have been demonstrated because no compression of the external urethral orifice was applied (Fig. 6a). Compression increases the intra-urethral pressure and may result in a backflow of contrast material into a prostatic utricle or vagina (Marcinski and Grzbowska, 1969). A normal utricle according to Howard (1948) may extend up to the base of the prostate. It may become enlarged due to obstruction by valves or desquamated epithelium, and may then present as a fluid-filled mass. When the Mullerian duct remnant is situated more distally and is elongated in form it is termed a vagina. The patient with the cyst of the prostatic utricle (Fig. 4) had signs of bladder outlet obstruction due to compression by the cyst, which filled with contrast material during voiding. Obstruction of this type has been reported in a few cases (Polse and Edlebrock, 1968; Neustein and Schutte, 1968), and in some calculi had formed in the cyst proper (Spence and Chenoweth, 1957). Cysto-urethrography as well as demonstrating possible Mullerian duct remnants shows the calibre of the urethra. Thus a wide urethra indicates a functionally significant meatal stenosis (Fig. 2). Conversely a normal urethrogram in the presence of a meatus which on physical examination seemed narrow excludes functional stenosis. In our series meatal stenosis was present in one-third of all patients regardless of the location of the meatus. Early meatomy was performed in many patients, even when the definitive correction of the anomaly was postponed. Vesico-ureteral reflux is another possible finding in these patients. Such reflux was present in 14 patients, 10 of whom were asymptomatic while
475
four had proven urinary tract infection. These 10 patients may have had previous infections and 50% of them had meatal stenosis, which is known to cause infection (Campbell and Harrison, 1970). The EU was normal in 10 o f the 14 patients and reflux would not have been suspected in them had we not carried out MCU. As a result of this finding all these patients are now receiving appropriate treatment including antibacterial coverage in the immediate postoperative period. Patients who undergo surgical correction for hypospadias often develop complications (Shafir et al., 1978) such as fistulae, strictures and saccular dilatations (Fig. 6b). It is therefore very useful to have a preoperative urethrogram for comparison. The results of this study lead us to conclude that MCU is indicated in all patients with hypospadias, since this study may reveal a significant degree of meatal stenosis and vesico-ureteral reflux, which may require early specific surgical and medical treatment. Cases with urinary tract infection or who are otherwise symptomatic should be investigated fully by adding EU to the above examination.
REFERENCES
Aarskog, D. (1970). Clinical and cytogenetic studies in hypospadias. Acta paediatrica scandinavia, Suppl. 203, 51, 52, 54, 55. Avellan, L. & Johanson, B. (1973). Preoperative and Postoperative Evaluation in Plastic and Reconstructive Surgery of the Genital Area, ed. Horton, C. E., pp. 370-
381. Little, Brown and Co., Boston. Campbell, M. F. & Harrison, J. H. (1970). Urology, 3rd edn, Vol. 2, pp. 1465, 1598-1606. W. B_ Saunders Co., Philadelphia. Fallon, B., Devine, C. J. & Horton, C. E. (1976). Congenital anomalies associated with hypospadias. Journal of" Urology, 116,585 586. Felton, L. M. (1959). Should intravenous pyelography be a routine procedure for children with cryptorchism or hypospadias? Journal of Urology, 81,335 338. Gray, W. & Skandalakis, J. E. (1972). Embryology COt Surgeons, pp. 443, 595, 625. W. B. Saunders Co., Philadelphia. Horton, C. E. & Devine, C. J. (1972). Hypospadias and epispadias. Clinical Symposia, ed. Roberts, R. H. 24, pp. 2-21. Ciba-Geiger Corp., Summit, New Jersey. Howard, F. S. (1948). Hypospadias with enlargement of the prostatic utricle. Surgery, Gynecology and Obstetrics, 86, 307-315. Kennedy, P. A. J. (1961). Hypospadias: a twenty-year review of 489 cases. Journal of Urology, 85, 814 -817. Lutzker, L. G., Kogan, S. J. & Levitt, S. B. (1977). Is routine intravenous urography indicated in patients with hypospadias? Pediatrics, 59, 630-633. Marcinski, A. & Gryzbowska, B. (1969). Cysto-urethrovaginography in male pseudohermaphroditism. Annals of. Radiology, 13,277 282. McArdle, R. & Lebowitz, R. (1975). Uncomplicated hypospadias and anomalies of upper urinary tract. Need for screening? Urology, 5,712-716.
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CLINICAL RADIOLOGY
Neyman, M. A. & Schirmer, H. K. A, (1965), Urinary tract evaluation in bypospadias. Journal of Urology, 94,439. Neustein, D_ H. & Schutte, H. (1968)- Mullerian duct cyst with report of a case. British Journal of Urology, 40, 72-78. Paul, M. & Kanagasuntheram, R. (1956). The congenital anomalies of the lower urinary tract. Part II: Hypospadias. British Journal of Urology, 28, 118-122. Poise, S. & Edelbrock, H. (1968). Prostatic utricular enlargement as a cause of vesical outlet obstruction in children. Journal of Urology, 100, 329-332.
Shafir, R. Hertz, M. Bornstein, L. & Tsur, H. (1978). Cysto urethrography as an aid in the evaluation of hypgspadia I and its complications. Journal of Plastic and Recon structive Surgery, 62,722 -726. Shopfner, C. E. (1969). Gynecologic roentgenology i~ children. Seminars in Roen tgenology, 4, 218-234. Spence, H. M. & Chenoweth, V. C. (1957). Cysts of the P~0, static utricle (Mullerian duct cysts): report of two cases in children, each containing calculi, cured by retropuhic operation. Transactions of" the American Association of Genito-urinary Surgeons, 49, 85-91.