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Patent Urachus: Review and Report of a Case1
THE JOURNAL OF UROLOGY
Vol. 63, No. 4, April, 1950
Printed in U. S. A.
PATENT URACHUS: REVIEW AND REPORT OF A CASE 1 J. W. CHERRY From the Department of Surgery, The Clinic, Honolulu, Territory of Hawaii
Among the rarer and more interesting congenital anomalies are those occurring as a result of failure of some step in the usual transformation of the fetal to the adult type of urinary bladder. An anomalous condition of the urachus attracted attention and was reported in the literature in 1550 by Cabral whose case was that of a teen-aged girl who voided from an umbilical opening as well as through the urethra. His attempt to cure the patent urachus surgically was successful. Herbst, in 1937, was able to collect in an exhaustive survey of the literature about 150 cases stated to be patent urachi. Under this heading, however, were included uracho-umbilical fistulas, umbilico-cystic fistulas, acquired and congenital, urachal cysts, true patent urachi, and urachal abscesses. In testimony to the rarity of this group of conditions is the fact that in 15,000 admissions to the Brady Urological Institute only 3 had conditions involving the urachus; in 200,000 admissions to the Children's and Infant's Hospital in Boston, only 3 cases of patent urachus were recorded; and in spite of Doctors Ladd and Gross' extensive experience with congenital abnormalities, they reported only 3 cases in the chapter on umbilical conditions in their classic text. EMBRYOLOGY AND ANATOMY
In order to appreciate the sequence of events leading to the development of anomalies in this region, the embryology and normal adult anatomy of the involved structures should be considered. The work of Begg in 1927 and 1930 stands as the authority on this subject, and he emphasized the work of earlier authors when he stated that the bladder and urachus arise from the early urogenital sinus which in turn arises from the ventral cloaca. He stated that the allantois, contrary to some authorities' opinions, contributes nothing to the formation of either the bladder or the urachus. The urachus itself is formed by constriction and elongation of the apex of the bladder. At or shortly after birth, the bladder begins its descent into the pelvis from the fetal position and drags the urachus down with it. The urachus in turn drags the ends of the obliterated umbilical arteries with it, forming the median umbilical ligament and the two lateral umbilical ligaments, respectively. These structures lie in the space between the peritoneum and the transversalis fascia. If the bladder fails to descend, there results a non-formation of the urachus and a persistent fetal type bladder remains. This may or may not be associated with a persistent umbilico-cystic fistula. If there is no fistula, the condition may go undetected throughout life since it does not give rise to symp1
Read before the staff meetings of The Clinic, Honolulu, July 1949. 693
694
J. W. CHERRY
toms. If, however, a fistula is present with urinary leakage, the condition is at once apparent. In the average normal adult, the urachus arises from the dome of the bladder anterior to the bladder apex and extends upward toward the umbilicus for a distance of 3 to 10 cm. It is tapered from 8 mm. in diameter at its base to approximately 1 mm. at its extremity. Ordinarily, it does not reach to within 15 cm. of the umbilicus. In one third of the subjects whom Begg examined, the uracha1 cavity was separated from the bladder by a thin membrane and in the remaining two thirds there was no such membrane, the bladder and urachus having lumens communicating one with another. It is easily seen that the former situation might give rise to cyst formations since the urachus is an epithelial-lined structure. The wall of the urachus is musculotendinous and it is reported to have the function of taking up slack in distention and evacuation of the bladder.
Fm. 1. A, ureteral catheter ,vas passed through umbilical opening and into bladder. Quite unintentionally, it was passed out through external urethral meatus. Xote granulation tissue tumor surrounding urachal opening. B, roentgenogram following introduction of a radiopaque solution into sinus tract resulted in above cystogram. :'\ ote air in intestine in umbilical hernia. PATHOLOGY
Brief mention has already been made as to the types of abnormality that may occur in maldevelopment of these structures. The umbilico-uracho-cystic fistula develops as a result of non-descent of the bladder and is frequently associated (as it was in our case) with a tumor consisting of granulation tissue in the center of the umbilicus. This granulation tissue is formed from the base of the umbilical cord and is nourished by the urachal artery, a branch of the superior vesical artery. This vessel normally descends with the urachus and the cord is sharply demarcated by a dry gangrene. However, when the urachus fails to descend, the blood supply from the urachal artery is adequate to prevent the dry gangrene of the base of the cord and the granulation tissue persists (fig. 1, A). Urachal cysts are the result of closure of both ends of the urachus and are usually initiated by trauma. They may assume enormous size. One such ·vrns reported to contain 52 liters of liquid. Uracho-umbilical fistula may occur when the urachus communicates with the umbilicus but not with the bladder. These are characterized by intermittent discharges of mucus and pus from the umbilicus.
,
',
i
PATENT URACHUS
695
There is a marked difference in pathogenesis between a congenital and an acquired umbilico-cystic fistula.The congenital fistula is the result of the previously described series of developmental events, whereas the acquired fistula occurs when, due to increased intravesical pressure, urine leaks from a weak area in the normally developed bladder, usually at the junction of the urachus and bladder. The escaped urine is confined by natural boundaries between the transversalis fascia and peritoneum and the lateral umbilical ligaments and finds its way along the plexus of fibers left as a result of the descent of urachus (Luschka's plexus), to the umbilicus and eventually ruptures through the floor of the umbilicus. The acquired umbilico-cystic fistulas occur at later ages and as the result of urethral obstruction such as might occur in stricture or prostatic hypertrophy. DIAGNOSIS
Patent urachus is naturally suspected when liquid drains from an umbilical fistula. Roentgenograms follo,ving the introduction of an opaque medium into the sinus tract may establish or demonstrate the origin of the fistulous tract. The umbilical opening may be large enough to permit the insertion of a cystoscope, or cystoscopic examination via the urethra may reveal the presence of a fetal type of bladder, or a urachal opening may be seen. In our own case, the nature of the tract was established when a ureteral catheter introduced into the umbilical opening protruded from the external urethral meatus (fig. 1, A). Subsequent withdrawal of the catheter into the bladder and the introduction of a solution of sodium iodide allowed us to obtain an excellent cystogram (fig. 1, B). TREATMENT
Congenital umbilico-cystic fistulas may close spontaneously provided there is no urethral obstruction. Many simple methods of treatment have been reported to be successful. Ligation of the umbilical opening may result in a cure. Cauterization of the upper end of the tract may result in obliteration of the sinus. Mast, Streamer and Unfug, in 1933, felt that if these simpler methods failed, more radical surgery should be postponed until the infant was at least 1 year of age. In recent years, however, J\l[ahoney, Ladd and Gross, and others have recommended immediate surgical correction by dissection and plastic closure of the abnormal tract, provided: 1) infection can be eradicated; 2) there is no urethral obstruction; and 3) renal function is adequate. They believe that the more radical approach is actually better because the tract, if left in situ, may be subject to neoplastic and inflammatory changes at later dates. All authors recommend extraperitoneal procedures if such can be carried out since the risk of pertonitis is necessarily increased if the peritoneum is opened. Adequate attention to pre-existing infection and the administration of antibiotics have rendered this risk a much less formidable one. A somewhat simpler procedure has also been recommended. This consists of converting the umbilico-
696
J. W. CHERRY
cystic tract to a suprapubic cystostomy and allowing a spontaneous closure of the cystostomy or closing it at a later date if spontaneous closure fails to occur. Following resection of the tract and closure of the opening into the bladder, it is desirable to maintain drainage via an indwelling urethral catheter for a period of five to seven days. We selected the one-stage plastic closure of the urachal tract since our patient was in all other respects a normal healthy child and with the single exception of her age seemed a good operative risk. Following is a brief case presentation. CASE REPORT
C. Y., a one-month old Chinese female infant, was brought to The Clinic on January 14, 1949, by the mother, who complained that the child had a tumor of the umbilicus which emitted spurts of clear fluid. On examination, the child did have a large, red, granulomatous mass associated with an umbilical hernia, and from the center of this mass a small fountain of clear fluid emitted when the
Fm. 2. Healed scar after resection of umbilicus, closure of fistulous tract, and repair of umbilical hernia.
child cried. A tentative diagnosis of omphalitis, umbilical hernia and patent urachus was made and the child was admitted to The Queen's Hospital for the treatment of the omphalitis, which subsided rapidly with penicillin injections and wet dressings. A definite diagnosis of patent urachus was made by introducing a No. 8 ureteral catheter through the umbilical sinus into the bladder. A cystogram was made by the introduction of a solution of sodium iodide into the bladder through this transurachal catheter. There was no obstruction of the urethra, as is so commonly the case with patent urachus, and a 16 F rubber catheter could be inserted into the bladder from the urethra without difficulty. The patient urinated freely both from the urethra and the umbilical tract. Careful examination revealed no other abnormalities. On January 25, 1949, under drop ether anesthesia, the urachal tract was dissected free, ligated and excised, and the stump inverted into the bladder, and the umbilical hernia was repaired without entering the peritoneum. The postoperative course was uneventful. An indwelling urethral catheter was kept in the bladder for the first 5 days. The child continued to gain weight and was
PATENT URACHUS
697
discharged from the hospital on the eighth postoperative day with the incision well healed (fig. 2). Six months postoperatively, the child was normal from a pediatric viewpoint,, showed no evidence of any other urinary abnormality, and at 8 months of age 'weighed 16 pounds, 7 ounces. SUMMARY
The history, incidence and clinical features of patent urachi have been briefly discussed and a case of patent urachus successfully treated by surgery has been reported, REFERENCES BEGG, R. C.: Surg., Gynec. & Obst., 45: 165-178, 1927. BEGG, R. C.: J. Urol., 59: 870, 1947. CULLEN, T. S.: The Umbilicus and Its DiEeases. Philadelphia: W. B. Saunders Co., 1916. DouGLAS, M.: Am. J. Surg., 22: 557, 1933. DUDGEON, H.: Surg., Gynec. & Obst., 71: 302-306, 1940. HERBST, W. P.: South. Med. J., 30: 711-719, 1937. MAHONE, P. J. AND ENNIS, D.: Kew Eng. J. Med., 215: 193-195, 1936. MAST, W. H., STREAMER, C. W. AND UNFUG, G. A.: Am. J. Surg., 22: 210, 1933. McCLELLAND, J.C. AND DAVIS, K. R.: J. Urol., 57: 270, 1947. RANSON, H.K.: Am. J. Surg., 22: 187, 1933. SIMON, H. E. AND BRANDEBERRY, N. A.: J. Urol., 55: 401, 1946. WILMOTH. C. L.: .T. A. 2VI. A .. 106: 526, 1936.
Vol. 63, No. 4, April, 1950
Printed in U. S. A.
PATENT URACHUS: REVIEW AND REPORT OF A CASE 1 J. W. CHERRY From the Department of Surgery, The Clinic, Honolulu, Territory of Hawaii
Among the rarer and more interesting congenital anomalies are those occurring as a result of failure of some step in the usual transformation of the fetal to the adult type of urinary bladder. An anomalous condition of the urachus attracted attention and was reported in the literature in 1550 by Cabral whose case was that of a teen-aged girl who voided from an umbilical opening as well as through the urethra. His attempt to cure the patent urachus surgically was successful. Herbst, in 1937, was able to collect in an exhaustive survey of the literature about 150 cases stated to be patent urachi. Under this heading, however, were included uracho-umbilical fistulas, umbilico-cystic fistulas, acquired and congenital, urachal cysts, true patent urachi, and urachal abscesses. In testimony to the rarity of this group of conditions is the fact that in 15,000 admissions to the Brady Urological Institute only 3 had conditions involving the urachus; in 200,000 admissions to the Children's and Infant's Hospital in Boston, only 3 cases of patent urachus were recorded; and in spite of Doctors Ladd and Gross' extensive experience with congenital abnormalities, they reported only 3 cases in the chapter on umbilical conditions in their classic text. EMBRYOLOGY AND ANATOMY
In order to appreciate the sequence of events leading to the development of anomalies in this region, the embryology and normal adult anatomy of the involved structures should be considered. The work of Begg in 1927 and 1930 stands as the authority on this subject, and he emphasized the work of earlier authors when he stated that the bladder and urachus arise from the early urogenital sinus which in turn arises from the ventral cloaca. He stated that the allantois, contrary to some authorities' opinions, contributes nothing to the formation of either the bladder or the urachus. The urachus itself is formed by constriction and elongation of the apex of the bladder. At or shortly after birth, the bladder begins its descent into the pelvis from the fetal position and drags the urachus down with it. The urachus in turn drags the ends of the obliterated umbilical arteries with it, forming the median umbilical ligament and the two lateral umbilical ligaments, respectively. These structures lie in the space between the peritoneum and the transversalis fascia. If the bladder fails to descend, there results a non-formation of the urachus and a persistent fetal type bladder remains. This may or may not be associated with a persistent umbilico-cystic fistula. If there is no fistula, the condition may go undetected throughout life since it does not give rise to symp1
Read before the staff meetings of The Clinic, Honolulu, July 1949. 693
694
J. W. CHERRY
toms. If, however, a fistula is present with urinary leakage, the condition is at once apparent. In the average normal adult, the urachus arises from the dome of the bladder anterior to the bladder apex and extends upward toward the umbilicus for a distance of 3 to 10 cm. It is tapered from 8 mm. in diameter at its base to approximately 1 mm. at its extremity. Ordinarily, it does not reach to within 15 cm. of the umbilicus. In one third of the subjects whom Begg examined, the uracha1 cavity was separated from the bladder by a thin membrane and in the remaining two thirds there was no such membrane, the bladder and urachus having lumens communicating one with another. It is easily seen that the former situation might give rise to cyst formations since the urachus is an epithelial-lined structure. The wall of the urachus is musculotendinous and it is reported to have the function of taking up slack in distention and evacuation of the bladder.
Fm. 1. A, ureteral catheter ,vas passed through umbilical opening and into bladder. Quite unintentionally, it was passed out through external urethral meatus. Xote granulation tissue tumor surrounding urachal opening. B, roentgenogram following introduction of a radiopaque solution into sinus tract resulted in above cystogram. :'\ ote air in intestine in umbilical hernia. PATHOLOGY
Brief mention has already been made as to the types of abnormality that may occur in maldevelopment of these structures. The umbilico-uracho-cystic fistula develops as a result of non-descent of the bladder and is frequently associated (as it was in our case) with a tumor consisting of granulation tissue in the center of the umbilicus. This granulation tissue is formed from the base of the umbilical cord and is nourished by the urachal artery, a branch of the superior vesical artery. This vessel normally descends with the urachus and the cord is sharply demarcated by a dry gangrene. However, when the urachus fails to descend, the blood supply from the urachal artery is adequate to prevent the dry gangrene of the base of the cord and the granulation tissue persists (fig. 1, A). Urachal cysts are the result of closure of both ends of the urachus and are usually initiated by trauma. They may assume enormous size. One such ·vrns reported to contain 52 liters of liquid. Uracho-umbilical fistula may occur when the urachus communicates with the umbilicus but not with the bladder. These are characterized by intermittent discharges of mucus and pus from the umbilicus.
,
',
i
PATENT URACHUS
695
There is a marked difference in pathogenesis between a congenital and an acquired umbilico-cystic fistula.The congenital fistula is the result of the previously described series of developmental events, whereas the acquired fistula occurs when, due to increased intravesical pressure, urine leaks from a weak area in the normally developed bladder, usually at the junction of the urachus and bladder. The escaped urine is confined by natural boundaries between the transversalis fascia and peritoneum and the lateral umbilical ligaments and finds its way along the plexus of fibers left as a result of the descent of urachus (Luschka's plexus), to the umbilicus and eventually ruptures through the floor of the umbilicus. The acquired umbilico-cystic fistulas occur at later ages and as the result of urethral obstruction such as might occur in stricture or prostatic hypertrophy. DIAGNOSIS
Patent urachus is naturally suspected when liquid drains from an umbilical fistula. Roentgenograms follo,ving the introduction of an opaque medium into the sinus tract may establish or demonstrate the origin of the fistulous tract. The umbilical opening may be large enough to permit the insertion of a cystoscope, or cystoscopic examination via the urethra may reveal the presence of a fetal type of bladder, or a urachal opening may be seen. In our own case, the nature of the tract was established when a ureteral catheter introduced into the umbilical opening protruded from the external urethral meatus (fig. 1, A). Subsequent withdrawal of the catheter into the bladder and the introduction of a solution of sodium iodide allowed us to obtain an excellent cystogram (fig. 1, B). TREATMENT
Congenital umbilico-cystic fistulas may close spontaneously provided there is no urethral obstruction. Many simple methods of treatment have been reported to be successful. Ligation of the umbilical opening may result in a cure. Cauterization of the upper end of the tract may result in obliteration of the sinus. Mast, Streamer and Unfug, in 1933, felt that if these simpler methods failed, more radical surgery should be postponed until the infant was at least 1 year of age. In recent years, however, J\l[ahoney, Ladd and Gross, and others have recommended immediate surgical correction by dissection and plastic closure of the abnormal tract, provided: 1) infection can be eradicated; 2) there is no urethral obstruction; and 3) renal function is adequate. They believe that the more radical approach is actually better because the tract, if left in situ, may be subject to neoplastic and inflammatory changes at later dates. All authors recommend extraperitoneal procedures if such can be carried out since the risk of pertonitis is necessarily increased if the peritoneum is opened. Adequate attention to pre-existing infection and the administration of antibiotics have rendered this risk a much less formidable one. A somewhat simpler procedure has also been recommended. This consists of converting the umbilico-
696
J. W. CHERRY
cystic tract to a suprapubic cystostomy and allowing a spontaneous closure of the cystostomy or closing it at a later date if spontaneous closure fails to occur. Following resection of the tract and closure of the opening into the bladder, it is desirable to maintain drainage via an indwelling urethral catheter for a period of five to seven days. We selected the one-stage plastic closure of the urachal tract since our patient was in all other respects a normal healthy child and with the single exception of her age seemed a good operative risk. Following is a brief case presentation. CASE REPORT
C. Y., a one-month old Chinese female infant, was brought to The Clinic on January 14, 1949, by the mother, who complained that the child had a tumor of the umbilicus which emitted spurts of clear fluid. On examination, the child did have a large, red, granulomatous mass associated with an umbilical hernia, and from the center of this mass a small fountain of clear fluid emitted when the
Fm. 2. Healed scar after resection of umbilicus, closure of fistulous tract, and repair of umbilical hernia.
child cried. A tentative diagnosis of omphalitis, umbilical hernia and patent urachus was made and the child was admitted to The Queen's Hospital for the treatment of the omphalitis, which subsided rapidly with penicillin injections and wet dressings. A definite diagnosis of patent urachus was made by introducing a No. 8 ureteral catheter through the umbilical sinus into the bladder. A cystogram was made by the introduction of a solution of sodium iodide into the bladder through this transurachal catheter. There was no obstruction of the urethra, as is so commonly the case with patent urachus, and a 16 F rubber catheter could be inserted into the bladder from the urethra without difficulty. The patient urinated freely both from the urethra and the umbilical tract. Careful examination revealed no other abnormalities. On January 25, 1949, under drop ether anesthesia, the urachal tract was dissected free, ligated and excised, and the stump inverted into the bladder, and the umbilical hernia was repaired without entering the peritoneum. The postoperative course was uneventful. An indwelling urethral catheter was kept in the bladder for the first 5 days. The child continued to gain weight and was
PATENT URACHUS
697
discharged from the hospital on the eighth postoperative day with the incision well healed (fig. 2). Six months postoperatively, the child was normal from a pediatric viewpoint,, showed no evidence of any other urinary abnormality, and at 8 months of age 'weighed 16 pounds, 7 ounces. SUMMARY
The history, incidence and clinical features of patent urachi have been briefly discussed and a case of patent urachus successfully treated by surgery has been reported, REFERENCES BEGG, R. C.: Surg., Gynec. & Obst., 45: 165-178, 1927. BEGG, R. C.: J. Urol., 59: 870, 1947. CULLEN, T. S.: The Umbilicus and Its DiEeases. Philadelphia: W. B. Saunders Co., 1916. DouGLAS, M.: Am. J. Surg., 22: 557, 1933. DUDGEON, H.: Surg., Gynec. & Obst., 71: 302-306, 1940. HERBST, W. P.: South. Med. J., 30: 711-719, 1937. MAHONE, P. J. AND ENNIS, D.: Kew Eng. J. Med., 215: 193-195, 1936. MAST, W. H., STREAMER, C. W. AND UNFUG, G. A.: Am. J. Surg., 22: 210, 1933. McCLELLAND, J.C. AND DAVIS, K. R.: J. Urol., 57: 270, 1947. RANSON, H.K.: Am. J. Surg., 22: 187, 1933. SIMON, H. E. AND BRANDEBERRY, N. A.: J. Urol., 55: 401, 1946. WILMOTH. C. L.: .T. A. 2VI. A .. 106: 526, 1936.