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Patent Urachus: With a Report of Two Additional Cases
PATENT URACHUS: WITH A REPORT OF TWO ADDITIONAL CASES DONALD
w.
ATCHESON, CAPTAIN, M.C.
From the Station Hospital, March Field, California
A surgeon may go a lifetime and never see a patent urachus or any of the variations thereof. Herbst, in 1936, reported only 148 published cases in the last 400 years. Such is the perversity of fate that recently 2 such cases reported to the Urological Service, this Station Hospital, within a period of 1 month. Probably the first mention of this condition in medical literature is Bartholomaeus Cabrolius' report, in his Alphabete Anatomique, of a case in a female, age 18. This was in 1550. His treatment consisted of ligation of the umbilical end of the fistula and in dilation of the urethra. It was not until 360 years later that another case was reported: this one by Littre, in 1810. Fifty years later, the third was published, and, since then, reports have been coming in slowly until, in 1936, 148 had been found and described. Opinions as to the etiological development of the urachus generally fall into 2 groups. Some investigators (Cross, Ginsberg) maintain that the allantois, which is an embryonic tube-like structure running from the placenta to the cloaca, plays the most important part in the formation of the urachus. The theory is that when the cloaca divides to form the bladder and the rectum this tube-like structure ordinarily closes and that when it fails to do this the result is a patent urachus which drains urine to the umbilicus. The most generally accepted theory today, however (Begg, Hammond, Herbst, Garvin) is that the urachus is the result of the bladder developing in the region of the umbilicus and later withdrawing deep into the pelvis. The result is a cone-shaped outgrowth from the dome of the bladder with its apex at the umbilicus. Eventually the entire tube becomes smaller until it finally closes off from the bladder. When it does not close, it is known as a patent urachus. There are 4 possible anomalies which may result from this process going astray: 1) the tube may open into the bladder but not reach the umbilicus; 2) it may open at the umbilicus and not reach the bladder; 3) it may extend from the bladder to the umbilicus as a fistula; 4) it may close off in sections and form small cysts. Most of the knowledge of the anatomy of the urachus is due to the work of Hammond and of R. C. Begg, who independently dissected out the tracts in many cadavers and fetuses. Their reports agree in most respects. They found that the urachus was made up of essentially the same layers as the bladder; namely, the transitional epithelium, loose and dense connective tissue, and a muscular layer. Practically all the tracts were found to have a lumen present at some point. Thirty-three per cent of them open into the bladder. Ninetyfive per cent fray out near the umbilical end. The average length is 5.5 cm to 15 cm, and it lies between the fascia transversalis and the peritoneum, with an obliterated umbilical artery lying to either side. The diagnosis of patent urachus of the fistulous type is not difficult, as the 424
PATENT URACHUS
425
leaking of the urine at the umbilicus usually calls attention to the anomaly. The drainage usually is intermittent, and may disappear for weeks at a time, only to reappear again. While it is draining, the patient usually experiences no symptoms and suffers only from the inconvenience of wet clothing. When the fistula closes off, the patient has pain which may be felt in the neighborhood of the cyst or be distributed along the nerve pressed upon. In one of the cases to be reported in this article the cyst became infected and the distribution of pain was to the right lower quadrant so that the problem of acute appendicitis arose. Sometimes these cysts become of such size that they may be confused with the various abdominal tumors. When a urachal cyst becomes infected it is called a pyourachus. In these cases one usually will find a tender triangular mass in the hypochondrium. There may, or may not be pyuria, hematuria, fever, dysuria, or pus and urine present at the umbilicus. Cystoscopic examination may possibly reveal a redness and infection near the dome of the bladder. Pressure upon the cyst while observing with a cystoscope may cause a ribbon of pus to appear inside the bladder. In some cases one might even pass a ureteral catheter into the internal urachal opening. Should one of these pyo-urachuses rupture, the pus may go into the peritoneal cavity, may appear at the umbilicus, or may burrow down through the deeper muscular fascia! planes and appear in the groin. Often there will be associated with these draining urachuses some sort of obstruction in the bladder neck, the removal of which is an essential part of the treatment. Begg has reported 44 tumors occurring in the urachus. These were typed as follows: fibro-adenomata, 3; adenomata, 4; colloid carcinomata, 19; mixed tumors, 8; myxomata, 4; and sarcomata, 6. There have been some cases where calculi have formed within the urachus and appear in x-rays. In some instances (as in one of the cases herein reported) one can outline the cyst or tract by means of dye injected into it. The treatment of patent urachus depends upon the type of anomaly present, upon the degree of incapacity it is producing, and upon whether or not it is infected. In any case of pyo-urachus it is much better to use all means of clearing up the infection before any very extensive surgery is attempted. The reason for this is the anatomical location of the tract as its position makes it almost impossible of resection without opening the peritoneum. Usually one can clear up the pyo-urachus sufficiently by merely dilating the opening and obtaining improved drainage. Washing out the tract by means of a catheter and a solution such as azochloramide or Dakin's solution will facilitate the subsiding of the infection. When the drainage has ceased one can then consider resection of the tract. This will prove to be less troublesome and s::ifor than blindly attempting to cauterize the sinus. Opening the sinus and allowing it to granulate up usually will be a long tedious process because of the deep anatomical position and will result in a wide scar with danger of future herniation. When resecting the tract it will be necessary to make an eliptic1al incision around the umbilicus and remove this in toto. As one is almost certain to open the peritoneum anyway, this author believes that it is a good policy to open it below the umbilicus so that a finger can be inserted, and then, by palpation, one may be certain that
426
DONALD W. ATCHESON
none of the intra-abdominal structures are adherent to the under surface of the umbilicus. When this ~s determined, one may then proceed to fearlessly remove the tract and adherent peritoneum. During the operation the obliterated umbilical arteries will be found lying to either side of the urachus and probably fusing with the urachus near the umbilical end. It is usually sufficient to carry out the dissection of the lower end of the tract as far as possible and then to clamp it, cut it, phenolize it, and ligature it. If desired, one may invert the dome of the bladder over the stump by means of Lembert mattress sutures. A postoperative inlying catheter will give one added protection. The age best suited for the surgery is any time after the bladder has reached its normal position in the bony pelvis. CASE REPORTS
Case 1. Private C. H., a white male, aged 20, was admitted February 11, 1943, complaining of drainage of thin fluid from the umbilicus as long as he can remember, and an undescended left testicle. He gave no history of congenital defects in family. He had had a yellow-colored discharge drainage from the umbilicus for as long as he could remember. Occasionally, he stated, it had a bloody tinge. He had not noticed any increase in this discharge when he has retained his urine. He had had no difficulty in urination, and there was no history of acute retention. The drainage was sufficient to keep his underclothing wet constantly. The drainage never became blocked, nor had the abdomen below the umbilicus become painful or tender. There was no history of nausea, vomiting, hematuria, pain in the back, or urinary calculi. The discharge had not become worse recently. The patient was a well developed, well nourished white male. The temperature, pulse and respirations were normal. The examination was essentially negative except for 1 carious tooth, some bilateral, firm, non-tender inguinal adenopathy, a normal left testicle which lay in the mid-portion of the left inguinal canal, and a small moist, red, granulomatous lesion in the center of the umbilicus. Draining from this was a sero-sanguinous exudate which had a slight ammoniacal odor. Attempts to pass a probe into this area without anesthesia were unsuccessful. Deep palpation suggested a fibrous-like cord deep to or within the rectus muscle sheath in mid-line. It was not tender. Pressure upon this "cord" caused fluid to be extruded at the umbilicus. The diagnosis was 1) patent urachus and 2) left cryptorchidism. The urinalysis, blood count, Kahn test and urine culture were negative. On cystoscopic examination the bladder capacity was 540 cc, no residual urine. The actual opening of the urachus could not be seen but a suspicious area where there was considerable roughening and dimpling at the dome· of the bladder in mid-line could be visualized. There was no evidence of bladder-neck obstruction. A ureteral catheter could not be passed into this area. Catheters were passed up both ureters 26 cm. with ease, and clear urine obtained. There were 8 cc and 7 cc of stasis in the right and left kidneys, respectively. Indigo carmine took 5 minutes to appear from each kidney and concentrated well. The
PATENT URACHUS
427
retrograde pyelograms revealed normal ureteral and pelvic outlines. Cystograms showed no evidence of a sinus extending from the dome of the bladder. Attempts to pass a probe into the sinus at the umbilical area were unsuccessful. On February 16, 1943, with the patient under spinal anesthesia, a patent urachus removed. The incision was made in the mid-line and enclosed the umbilicus, through the skin, subcutaneous areas and sheaths of the recti muscles. The urachus was found to be lying between the fascia transversalis and the peritoneum and appeared as a pinkish-white, fibrous, cord-like structure which became funnel shaped at both ends and into which a probe could be passed downward approximately to, but not into, the bladder. The peritoneum was opened 2 inches below the umbilicus and a finger inserted into the peritoneal cavity. It was ascertained that none of the abdominal contents were connected with the umbilicus. The entire cord and tract were then dissected out, the end of the urachus next to the bladder being doubly clamped, cut, and ligated with a fixation No. 1 chromic catgut suture. The peritoneum, recti sheaths, and skin were then closed in the usual way. An inlying No. 20 two-way Foley catheter was placed in the bladder by way of the urethra. The gross specimen was a mass of tissue which was exceedingly hard and appeared to have been placed in too strong fixative. It measured 4 by 2.5 by 2.3 cm. Along one edge there was a triangular wedge of epithelium which terminated in a depressed area having the appearance of an umbilicus. On the inferior surface of the umbilicus there was attached a long cord-like structure approximately 5 cm. in length. The remainder of the specimen consists of fatty subcutaneous tissue." On section through the subcutaneous tissue immediately beneath the umbilicus there was seen a minute, pinpoint sized opening which apparently represented a duct or fistulous tract. A microscopic section through the base of the umbilicus revealed connective tissue and fat with a few thick-walled vessels in the center. This particular section did not reveal any evidence of a duct or sinus. A section through the cord-like structure attached to the specimen revealed it to consist of fibrous connective tissue, blood vessels and nerves. Two days after operation the patient had 1 attack of vomiting and Wangensteen suction was started. Fluid by mouth was discontinued and patient given 3000 cc of 5 per cent glucose in saline over a period of 24 hours. Peristaltic sounds could be heard in the abdomen but the patient had passed nothing by rectum. The next day the abdomen was still distended. The patient complained of abdominal cramping pain but passed nothing by rectum. The Wan gensteen suction worked well. On February 20, 1943 the abdomen was soft. The patient had been given 2 enemas with good results. On February 22, 1943 the urethral catheter was removed and also the skin sutures. Four days later the retention sutures were removed. The patient was discharged from the hospital on March 10, 1943. Case 2. Private E. P., a white male, aged 21, was admitted January 22, 1943, complaining of pain in the rightlower quadrant of 3 days' duration. The patient had had pneumonia at the age of 3 years, complicated by empyema and re-
428
DONALD W. ATCHESON
section of rib. The family history was negative. The patient was last well on January 19, 1943, at which time he was seized with pain in the right lower quad~ rant, with tenderness in the same area, and he became nauseated and vomited. Physical examination at that time revealed spasm of the right rectus muscle, with tenderness around the umbilicus and extending downward into the right lower quadrant. Rebound tenderness was present on both sides of the abdomen. No mass could be felt. There was no rectal tenderness. No mention was made in the history as to whether or not pus was seen at the umbilicus; hence, it is assumed it was not. The appendix was removed through a right McBurney incision on January 22, 1943. No pathological report was available. Grossly, it was described as slightly inflamed and edematous. The postoperative course was smooth, but on the seventh postoperative day a purulent discharge was noticed from the umbilicus. Coincident with the appearance of this discharge, pain and tenderness which had been present in the lower abdomen since the operation disappeared. At this time the patient was further questioned and a history obtained of his having had discharge from the umbilicus on many previous occasions since childhood. The discharge was described as varying in character from serous to muco-purulent. The physical examination was essentially negative except for a 1) mucopurulent exudate appearing at the umbilicus, and 2) tenderness in the mid-line below the umbilicus, at which point a deep induration was felt in the abdominal wall for a distance of four inches below the umbilicus. A probe was passed into a sinus at the umbilicus and down into this area for approximately 3 inches. The red blood cells were normal; white blood cells, 16,000, which, after the urachal sinus drained, fell to 9,650, with a normal differential count. Urinalyses on many occasions were reported as having 3 to 6 white blood cells, and on 1 occasion a moderate growth of Staphylococcus albus. X-rays were made with dye injected into the sinus cavity and were reported as follows: "Antero-posterior film of the abdomen presents a pear-shaped mass of dye measuring 11 by 4 cm., extending from the lower border of the third lumbar to the upper border of the first sacral, lying essentially in the mid-line. It is relatively smooth in outline and apparently does not communicate with the bladder, which is also filled with dye." Cystoscopy revealed no opening into the dome of the bladder and no suspicious area of roughening. No pus could be made to appear in the bladder by pressure over the sinus. The upper urinary tract was normal. A cannula also was passed into the sinus tract, and it was noted that this passed only approximately 3 inches below the umbilicus and appeared to lie deep to the rectus abdominal muscles and fascia. The sinus tract was dilated with a hemostat and daily irrigations of the tract, using 1 : 3300 azochloramide, instituted. A suspension of sulfathiazole was left in the sinus tract after each irrigation. This treatment was continued from February 5 until February 20, 1943. By this time all discharge had ceased and it was believed that the infection had subsided. On February 21, 1943 under spinal anesthesia, a linear incision, which in-
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eluded the umbilicus at the upper end, was carried through the skin and subcutaneous tissues and through the point of fusion of the recti sheaths. Lying between the fascia transversalis and the peritoneum, and practically incorporated in the peritoneum, was a thick-walled indurated sinus. This extended approximately 4 inches below the umbilicus and was continuous at its upper end with the umbilicus. Methylene blue, which was injected into the sinus, passed below this structure into a long cord-like tube which went to the dome of the bladder. The dye, however, did not appear in the urine. A similar cord-like structure ran from the lower region of the sinus but angulated off to the right. No dye appeared in this second structure. The entire umbilicus, the indurated sinus tract and the cord-like structure below the sinus, were excised. The cord was clamped, cut and ligated just above the dome of the bladder. The dome of the bladder was closed over the stump. The wound was closed in layers in the usual manner. The gross specimen consisted of a nearly square piece of skin measuring 3 by 3.2 by 2.7 cm. Near one end was a deep dimple in the skin measuring 1.5 cm. long and in this dimple at a point 2 mm. from the skin margin there was an opening measuring approximately 1 mm. in diameter which extended into a tract which had been opened on the superficial surface. This tract was stained with a blue dye and was surrounded by dense fibrous tissue. Attached to the piece of epithelium there was an elongated mass of fibrous tissue measuring 7 cm. in length. The first half of it measured 2.5 by 2 cm. and the distal half was a small cord measuring from 0.4 to 0.7 cm. in diameter. The long cord-like attachment on the distal end of the specimen showed a small central sinus tract which was stained with blue dye. Microscopic sections showed the sinus tract to be lined by richly vascularized granulation tissue which was markedly infiltrated with round cells, macrophages, polymorphs and fairly numerous multinucleated giant cells. The surrounding fibrous stroma showed perivascular round cell infiltration but no other noteworthy changes. No epithelial lining remained in the tract. The postoperative course was normal. The patient took a regular diet on the third day. The skin sutures were removed on the fifth day, and the retention sutures on the ninth. The wound healed by primary intention and the patient was allowed out of bed on the fourteenth day and went on furlough on the twentyfirst day. SUM:MARY
A discussion of the history, types, development, diagnosis and treatment of patent urachus, together with the clinical records of 2 additional cases, has been presented. Patent urachus is a congenital condition which may go a long time before being diagnosed and treatment instituted. The diagnosis of patent urachus may easily be confused with the diagnosis of intra-abdominal conditions, such as acute appendicitis and cystic tumors if a history of drainage from the umbilicus is not obtained.
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DONALD W. ATCHESON
In some cases the urachus will change from one type to another. In the 2 cases presented they both existed as fistulous types, as evidenced by the history of urine draining in early life, but as inflammatory changes occurred the fistula sealed off and the sinus type resulted. It is almost a technical impossibility to excise .the tract without opening the peritoneum, especially in those cases where inflammation has existed. It is suggested that the peritoneum be routinely opened at a point below the umbilicus and then the attached tract and umbilicus be excised in toto. The treatment of the infection in the tract before surgical treatment is carried out is an important part of the therapy. Establishing of drainage and irrigation of the tract with some solution such as azochloramide are satisfactory for the treatment of the infection. Inflammation may have changed the structures of the urachal area to such an extent that the pathological sections do not always reveal the lumen and epithelial lining of the tract. REFERENCES ANDERSON, H. E.: Urachal abscess complicating puerperium. Neb. Med. J., 22: 390-391, 1937.
BEGG, R. C.: Urachus, columnar, tumor of, invading the bladder. Brit. J. Surg., 23: 769-772, 1936.
CRoss, J.B.: Urachus, cysts of. J. Urol., 33: 408-410, 1935. DEWAARD, T.: Cancer. Two rare cases in a patent urachus. J. Urol., 42: 554-558, 1939. DREYFUSS, M. L. AND FLIEss, M. N.: Patent urachus with stone formation. J. Urol., 46: 77-81, 1941.
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DUDGEON, H., JR.: Tumor of patent urachus. Surg., Gynec. and Obst., 71: 302-306, 1940. FERRI~~, P.A., CRAIG;i_L. G. AND FooRD, A.G.: Cancer, tumors of bladder of urachal origm. Urol. and uutan. Rev., 40: 457--464, 1936. GINSBERG, H. M. AND NIXON, C. E.: Urachus, infected cysts of. Calif. and West. Med., 43: 153-154, 1935.
GIVEN, E. J.: Patent urachus. J. Urol., 42: 463--469, 1939. HAMMOND, G., YGLESIAS, I. AND DAVIS, J.E.: Anatomy and associated fasciae of patent urachus. J. Anat. Rec., 80: 271-287, 1941. HAMM, F. C.: Tumors, cyst adenoma of bladder. J. Urol., 44: 227-233, 1940. HERBST, W. P.: Patent urachus. South. Med. J., 30: 711-719, 1937. IVEs, J. D.: Urachus. U.S. Naval Bull., 32: 205-207, 1934. KANTOR, H. I.: Cysts of patent urachus, two cases. Ann. Surg:, 109: 277-283 1939. LAZARUS, J. A. AND RosENTHAL, A. A.: Urachus, pyo-urachus, complicated by urethral stricture. Ann. Surg., 102: 49-54, 1935. MAHONEY, P. J. AND ENNIS, D.: Congenital patent urachus. New Eng. J. Med., 215: 193-195, 1936.
MEADE, H. S.: Urachal cysts complicating prostatectomy. Irish J. Med. Sci., pp. 32-33, 1941.
MosT, W. H., STREAMER, C. W; AND UNFUG, G. A.: Patent urachus. Am. J. Surg., 22: 210-214, 1933.
STEVENS, A. R.: Pyo-urachus. J. Urol., 30: 319-325, 1933. WYATT, G. M. AND LAMMAN, T. H.: Calculus of patent urachus with enuresis. Am. J. Roent., 43: 673-675, 1940. WILMOTH, C, L.: Persistent urachus in adult. J. A. M. A., 106: 526-529, 1936.
w.
ATCHESON, CAPTAIN, M.C.
From the Station Hospital, March Field, California
A surgeon may go a lifetime and never see a patent urachus or any of the variations thereof. Herbst, in 1936, reported only 148 published cases in the last 400 years. Such is the perversity of fate that recently 2 such cases reported to the Urological Service, this Station Hospital, within a period of 1 month. Probably the first mention of this condition in medical literature is Bartholomaeus Cabrolius' report, in his Alphabete Anatomique, of a case in a female, age 18. This was in 1550. His treatment consisted of ligation of the umbilical end of the fistula and in dilation of the urethra. It was not until 360 years later that another case was reported: this one by Littre, in 1810. Fifty years later, the third was published, and, since then, reports have been coming in slowly until, in 1936, 148 had been found and described. Opinions as to the etiological development of the urachus generally fall into 2 groups. Some investigators (Cross, Ginsberg) maintain that the allantois, which is an embryonic tube-like structure running from the placenta to the cloaca, plays the most important part in the formation of the urachus. The theory is that when the cloaca divides to form the bladder and the rectum this tube-like structure ordinarily closes and that when it fails to do this the result is a patent urachus which drains urine to the umbilicus. The most generally accepted theory today, however (Begg, Hammond, Herbst, Garvin) is that the urachus is the result of the bladder developing in the region of the umbilicus and later withdrawing deep into the pelvis. The result is a cone-shaped outgrowth from the dome of the bladder with its apex at the umbilicus. Eventually the entire tube becomes smaller until it finally closes off from the bladder. When it does not close, it is known as a patent urachus. There are 4 possible anomalies which may result from this process going astray: 1) the tube may open into the bladder but not reach the umbilicus; 2) it may open at the umbilicus and not reach the bladder; 3) it may extend from the bladder to the umbilicus as a fistula; 4) it may close off in sections and form small cysts. Most of the knowledge of the anatomy of the urachus is due to the work of Hammond and of R. C. Begg, who independently dissected out the tracts in many cadavers and fetuses. Their reports agree in most respects. They found that the urachus was made up of essentially the same layers as the bladder; namely, the transitional epithelium, loose and dense connective tissue, and a muscular layer. Practically all the tracts were found to have a lumen present at some point. Thirty-three per cent of them open into the bladder. Ninetyfive per cent fray out near the umbilical end. The average length is 5.5 cm to 15 cm, and it lies between the fascia transversalis and the peritoneum, with an obliterated umbilical artery lying to either side. The diagnosis of patent urachus of the fistulous type is not difficult, as the 424
PATENT URACHUS
425
leaking of the urine at the umbilicus usually calls attention to the anomaly. The drainage usually is intermittent, and may disappear for weeks at a time, only to reappear again. While it is draining, the patient usually experiences no symptoms and suffers only from the inconvenience of wet clothing. When the fistula closes off, the patient has pain which may be felt in the neighborhood of the cyst or be distributed along the nerve pressed upon. In one of the cases to be reported in this article the cyst became infected and the distribution of pain was to the right lower quadrant so that the problem of acute appendicitis arose. Sometimes these cysts become of such size that they may be confused with the various abdominal tumors. When a urachal cyst becomes infected it is called a pyourachus. In these cases one usually will find a tender triangular mass in the hypochondrium. There may, or may not be pyuria, hematuria, fever, dysuria, or pus and urine present at the umbilicus. Cystoscopic examination may possibly reveal a redness and infection near the dome of the bladder. Pressure upon the cyst while observing with a cystoscope may cause a ribbon of pus to appear inside the bladder. In some cases one might even pass a ureteral catheter into the internal urachal opening. Should one of these pyo-urachuses rupture, the pus may go into the peritoneal cavity, may appear at the umbilicus, or may burrow down through the deeper muscular fascia! planes and appear in the groin. Often there will be associated with these draining urachuses some sort of obstruction in the bladder neck, the removal of which is an essential part of the treatment. Begg has reported 44 tumors occurring in the urachus. These were typed as follows: fibro-adenomata, 3; adenomata, 4; colloid carcinomata, 19; mixed tumors, 8; myxomata, 4; and sarcomata, 6. There have been some cases where calculi have formed within the urachus and appear in x-rays. In some instances (as in one of the cases herein reported) one can outline the cyst or tract by means of dye injected into it. The treatment of patent urachus depends upon the type of anomaly present, upon the degree of incapacity it is producing, and upon whether or not it is infected. In any case of pyo-urachus it is much better to use all means of clearing up the infection before any very extensive surgery is attempted. The reason for this is the anatomical location of the tract as its position makes it almost impossible of resection without opening the peritoneum. Usually one can clear up the pyo-urachus sufficiently by merely dilating the opening and obtaining improved drainage. Washing out the tract by means of a catheter and a solution such as azochloramide or Dakin's solution will facilitate the subsiding of the infection. When the drainage has ceased one can then consider resection of the tract. This will prove to be less troublesome and s::ifor than blindly attempting to cauterize the sinus. Opening the sinus and allowing it to granulate up usually will be a long tedious process because of the deep anatomical position and will result in a wide scar with danger of future herniation. When resecting the tract it will be necessary to make an eliptic1al incision around the umbilicus and remove this in toto. As one is almost certain to open the peritoneum anyway, this author believes that it is a good policy to open it below the umbilicus so that a finger can be inserted, and then, by palpation, one may be certain that
426
DONALD W. ATCHESON
none of the intra-abdominal structures are adherent to the under surface of the umbilicus. When this ~s determined, one may then proceed to fearlessly remove the tract and adherent peritoneum. During the operation the obliterated umbilical arteries will be found lying to either side of the urachus and probably fusing with the urachus near the umbilical end. It is usually sufficient to carry out the dissection of the lower end of the tract as far as possible and then to clamp it, cut it, phenolize it, and ligature it. If desired, one may invert the dome of the bladder over the stump by means of Lembert mattress sutures. A postoperative inlying catheter will give one added protection. The age best suited for the surgery is any time after the bladder has reached its normal position in the bony pelvis. CASE REPORTS
Case 1. Private C. H., a white male, aged 20, was admitted February 11, 1943, complaining of drainage of thin fluid from the umbilicus as long as he can remember, and an undescended left testicle. He gave no history of congenital defects in family. He had had a yellow-colored discharge drainage from the umbilicus for as long as he could remember. Occasionally, he stated, it had a bloody tinge. He had not noticed any increase in this discharge when he has retained his urine. He had had no difficulty in urination, and there was no history of acute retention. The drainage was sufficient to keep his underclothing wet constantly. The drainage never became blocked, nor had the abdomen below the umbilicus become painful or tender. There was no history of nausea, vomiting, hematuria, pain in the back, or urinary calculi. The discharge had not become worse recently. The patient was a well developed, well nourished white male. The temperature, pulse and respirations were normal. The examination was essentially negative except for 1 carious tooth, some bilateral, firm, non-tender inguinal adenopathy, a normal left testicle which lay in the mid-portion of the left inguinal canal, and a small moist, red, granulomatous lesion in the center of the umbilicus. Draining from this was a sero-sanguinous exudate which had a slight ammoniacal odor. Attempts to pass a probe into this area without anesthesia were unsuccessful. Deep palpation suggested a fibrous-like cord deep to or within the rectus muscle sheath in mid-line. It was not tender. Pressure upon this "cord" caused fluid to be extruded at the umbilicus. The diagnosis was 1) patent urachus and 2) left cryptorchidism. The urinalysis, blood count, Kahn test and urine culture were negative. On cystoscopic examination the bladder capacity was 540 cc, no residual urine. The actual opening of the urachus could not be seen but a suspicious area where there was considerable roughening and dimpling at the dome· of the bladder in mid-line could be visualized. There was no evidence of bladder-neck obstruction. A ureteral catheter could not be passed into this area. Catheters were passed up both ureters 26 cm. with ease, and clear urine obtained. There were 8 cc and 7 cc of stasis in the right and left kidneys, respectively. Indigo carmine took 5 minutes to appear from each kidney and concentrated well. The
PATENT URACHUS
427
retrograde pyelograms revealed normal ureteral and pelvic outlines. Cystograms showed no evidence of a sinus extending from the dome of the bladder. Attempts to pass a probe into the sinus at the umbilical area were unsuccessful. On February 16, 1943, with the patient under spinal anesthesia, a patent urachus removed. The incision was made in the mid-line and enclosed the umbilicus, through the skin, subcutaneous areas and sheaths of the recti muscles. The urachus was found to be lying between the fascia transversalis and the peritoneum and appeared as a pinkish-white, fibrous, cord-like structure which became funnel shaped at both ends and into which a probe could be passed downward approximately to, but not into, the bladder. The peritoneum was opened 2 inches below the umbilicus and a finger inserted into the peritoneal cavity. It was ascertained that none of the abdominal contents were connected with the umbilicus. The entire cord and tract were then dissected out, the end of the urachus next to the bladder being doubly clamped, cut, and ligated with a fixation No. 1 chromic catgut suture. The peritoneum, recti sheaths, and skin were then closed in the usual way. An inlying No. 20 two-way Foley catheter was placed in the bladder by way of the urethra. The gross specimen was a mass of tissue which was exceedingly hard and appeared to have been placed in too strong fixative. It measured 4 by 2.5 by 2.3 cm. Along one edge there was a triangular wedge of epithelium which terminated in a depressed area having the appearance of an umbilicus. On the inferior surface of the umbilicus there was attached a long cord-like structure approximately 5 cm. in length. The remainder of the specimen consists of fatty subcutaneous tissue." On section through the subcutaneous tissue immediately beneath the umbilicus there was seen a minute, pinpoint sized opening which apparently represented a duct or fistulous tract. A microscopic section through the base of the umbilicus revealed connective tissue and fat with a few thick-walled vessels in the center. This particular section did not reveal any evidence of a duct or sinus. A section through the cord-like structure attached to the specimen revealed it to consist of fibrous connective tissue, blood vessels and nerves. Two days after operation the patient had 1 attack of vomiting and Wangensteen suction was started. Fluid by mouth was discontinued and patient given 3000 cc of 5 per cent glucose in saline over a period of 24 hours. Peristaltic sounds could be heard in the abdomen but the patient had passed nothing by rectum. The next day the abdomen was still distended. The patient complained of abdominal cramping pain but passed nothing by rectum. The Wan gensteen suction worked well. On February 20, 1943 the abdomen was soft. The patient had been given 2 enemas with good results. On February 22, 1943 the urethral catheter was removed and also the skin sutures. Four days later the retention sutures were removed. The patient was discharged from the hospital on March 10, 1943. Case 2. Private E. P., a white male, aged 21, was admitted January 22, 1943, complaining of pain in the rightlower quadrant of 3 days' duration. The patient had had pneumonia at the age of 3 years, complicated by empyema and re-
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DONALD W. ATCHESON
section of rib. The family history was negative. The patient was last well on January 19, 1943, at which time he was seized with pain in the right lower quad~ rant, with tenderness in the same area, and he became nauseated and vomited. Physical examination at that time revealed spasm of the right rectus muscle, with tenderness around the umbilicus and extending downward into the right lower quadrant. Rebound tenderness was present on both sides of the abdomen. No mass could be felt. There was no rectal tenderness. No mention was made in the history as to whether or not pus was seen at the umbilicus; hence, it is assumed it was not. The appendix was removed through a right McBurney incision on January 22, 1943. No pathological report was available. Grossly, it was described as slightly inflamed and edematous. The postoperative course was smooth, but on the seventh postoperative day a purulent discharge was noticed from the umbilicus. Coincident with the appearance of this discharge, pain and tenderness which had been present in the lower abdomen since the operation disappeared. At this time the patient was further questioned and a history obtained of his having had discharge from the umbilicus on many previous occasions since childhood. The discharge was described as varying in character from serous to muco-purulent. The physical examination was essentially negative except for a 1) mucopurulent exudate appearing at the umbilicus, and 2) tenderness in the mid-line below the umbilicus, at which point a deep induration was felt in the abdominal wall for a distance of four inches below the umbilicus. A probe was passed into a sinus at the umbilicus and down into this area for approximately 3 inches. The red blood cells were normal; white blood cells, 16,000, which, after the urachal sinus drained, fell to 9,650, with a normal differential count. Urinalyses on many occasions were reported as having 3 to 6 white blood cells, and on 1 occasion a moderate growth of Staphylococcus albus. X-rays were made with dye injected into the sinus cavity and were reported as follows: "Antero-posterior film of the abdomen presents a pear-shaped mass of dye measuring 11 by 4 cm., extending from the lower border of the third lumbar to the upper border of the first sacral, lying essentially in the mid-line. It is relatively smooth in outline and apparently does not communicate with the bladder, which is also filled with dye." Cystoscopy revealed no opening into the dome of the bladder and no suspicious area of roughening. No pus could be made to appear in the bladder by pressure over the sinus. The upper urinary tract was normal. A cannula also was passed into the sinus tract, and it was noted that this passed only approximately 3 inches below the umbilicus and appeared to lie deep to the rectus abdominal muscles and fascia. The sinus tract was dilated with a hemostat and daily irrigations of the tract, using 1 : 3300 azochloramide, instituted. A suspension of sulfathiazole was left in the sinus tract after each irrigation. This treatment was continued from February 5 until February 20, 1943. By this time all discharge had ceased and it was believed that the infection had subsided. On February 21, 1943 under spinal anesthesia, a linear incision, which in-
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eluded the umbilicus at the upper end, was carried through the skin and subcutaneous tissues and through the point of fusion of the recti sheaths. Lying between the fascia transversalis and the peritoneum, and practically incorporated in the peritoneum, was a thick-walled indurated sinus. This extended approximately 4 inches below the umbilicus and was continuous at its upper end with the umbilicus. Methylene blue, which was injected into the sinus, passed below this structure into a long cord-like tube which went to the dome of the bladder. The dye, however, did not appear in the urine. A similar cord-like structure ran from the lower region of the sinus but angulated off to the right. No dye appeared in this second structure. The entire umbilicus, the indurated sinus tract and the cord-like structure below the sinus, were excised. The cord was clamped, cut and ligated just above the dome of the bladder. The dome of the bladder was closed over the stump. The wound was closed in layers in the usual manner. The gross specimen consisted of a nearly square piece of skin measuring 3 by 3.2 by 2.7 cm. Near one end was a deep dimple in the skin measuring 1.5 cm. long and in this dimple at a point 2 mm. from the skin margin there was an opening measuring approximately 1 mm. in diameter which extended into a tract which had been opened on the superficial surface. This tract was stained with a blue dye and was surrounded by dense fibrous tissue. Attached to the piece of epithelium there was an elongated mass of fibrous tissue measuring 7 cm. in length. The first half of it measured 2.5 by 2 cm. and the distal half was a small cord measuring from 0.4 to 0.7 cm. in diameter. The long cord-like attachment on the distal end of the specimen showed a small central sinus tract which was stained with blue dye. Microscopic sections showed the sinus tract to be lined by richly vascularized granulation tissue which was markedly infiltrated with round cells, macrophages, polymorphs and fairly numerous multinucleated giant cells. The surrounding fibrous stroma showed perivascular round cell infiltration but no other noteworthy changes. No epithelial lining remained in the tract. The postoperative course was normal. The patient took a regular diet on the third day. The skin sutures were removed on the fifth day, and the retention sutures on the ninth. The wound healed by primary intention and the patient was allowed out of bed on the fourteenth day and went on furlough on the twentyfirst day. SUM:MARY
A discussion of the history, types, development, diagnosis and treatment of patent urachus, together with the clinical records of 2 additional cases, has been presented. Patent urachus is a congenital condition which may go a long time before being diagnosed and treatment instituted. The diagnosis of patent urachus may easily be confused with the diagnosis of intra-abdominal conditions, such as acute appendicitis and cystic tumors if a history of drainage from the umbilicus is not obtained.
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In some cases the urachus will change from one type to another. In the 2 cases presented they both existed as fistulous types, as evidenced by the history of urine draining in early life, but as inflammatory changes occurred the fistula sealed off and the sinus type resulted. It is almost a technical impossibility to excise .the tract without opening the peritoneum, especially in those cases where inflammation has existed. It is suggested that the peritoneum be routinely opened at a point below the umbilicus and then the attached tract and umbilicus be excised in toto. The treatment of the infection in the tract before surgical treatment is carried out is an important part of the therapy. Establishing of drainage and irrigation of the tract with some solution such as azochloramide are satisfactory for the treatment of the infection. Inflammation may have changed the structures of the urachal area to such an extent that the pathological sections do not always reveal the lumen and epithelial lining of the tract. REFERENCES ANDERSON, H. E.: Urachal abscess complicating puerperium. Neb. Med. J., 22: 390-391, 1937.
BEGG, R. C.: Urachus, columnar, tumor of, invading the bladder. Brit. J. Surg., 23: 769-772, 1936.
CRoss, J.B.: Urachus, cysts of. J. Urol., 33: 408-410, 1935. DEWAARD, T.: Cancer. Two rare cases in a patent urachus. J. Urol., 42: 554-558, 1939. DREYFUSS, M. L. AND FLIEss, M. N.: Patent urachus with stone formation. J. Urol., 46: 77-81, 1941.
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DUDGEON, H., JR.: Tumor of patent urachus. Surg., Gynec. and Obst., 71: 302-306, 1940. FERRI~~, P.A., CRAIG;i_L. G. AND FooRD, A.G.: Cancer, tumors of bladder of urachal origm. Urol. and uutan. Rev., 40: 457--464, 1936. GINSBERG, H. M. AND NIXON, C. E.: Urachus, infected cysts of. Calif. and West. Med., 43: 153-154, 1935.
GIVEN, E. J.: Patent urachus. J. Urol., 42: 463--469, 1939. HAMMOND, G., YGLESIAS, I. AND DAVIS, J.E.: Anatomy and associated fasciae of patent urachus. J. Anat. Rec., 80: 271-287, 1941. HAMM, F. C.: Tumors, cyst adenoma of bladder. J. Urol., 44: 227-233, 1940. HERBST, W. P.: Patent urachus. South. Med. J., 30: 711-719, 1937. IVEs, J. D.: Urachus. U.S. Naval Bull., 32: 205-207, 1934. KANTOR, H. I.: Cysts of patent urachus, two cases. Ann. Surg:, 109: 277-283 1939. LAZARUS, J. A. AND RosENTHAL, A. A.: Urachus, pyo-urachus, complicated by urethral stricture. Ann. Surg., 102: 49-54, 1935. MAHONEY, P. J. AND ENNIS, D.: Congenital patent urachus. New Eng. J. Med., 215: 193-195, 1936.
MEADE, H. S.: Urachal cysts complicating prostatectomy. Irish J. Med. Sci., pp. 32-33, 1941.
MosT, W. H., STREAMER, C. W; AND UNFUG, G. A.: Patent urachus. Am. J. Surg., 22: 210-214, 1933.
STEVENS, A. R.: Pyo-urachus. J. Urol., 30: 319-325, 1933. WYATT, G. M. AND LAMMAN, T. H.: Calculus of patent urachus with enuresis. Am. J. Roent., 43: 673-675, 1940. WILMOTH, C, L.: Persistent urachus in adult. J. A. M. A., 106: 526-529, 1936.