Book - Umbilicus (1916) 32
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Cullen TS. Embryology, anatomy, and diseases of the umbilicus together with diseases of the urachus. (1916) W. B. Saunders Company, Philadelphia And London.
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Chapter XXXII. Large Urachal Cysts
Differential diagnosis; personal observations on a large diffuse neuroma of the bladder.
Detailed report of large, non-infected urachal cysts.
The small urachal cysts that we have considered rarely reached 1 cm. in diameter, and were naturally readily overlooked clinically. Probably one of the first urachal cysts ever opened was the one observed by Peu in 1648, and recorded in his Pratique des Accouchements, 1694, p. 38, and recently referred to by Wutz. The patient was a child two hours old. Situated at the umbilicus was a tumor the size of a pigeon's egg. It was opened, and a serum-like fluid escaped. This proved to be urine, and on the following morning urine escaped in a jet from the umbilicus..
Atlee, in 1873, in his treatise on Ovarian Tumors, reported the case of a girl eighteen years old. When opening the abdomen for the removal of an ovarian tumor he accidentally incised a urachal cyst containing an ounce of fluid resembling ordinary ascitic fluid.
Von Recklinghausen in 1902 demonstrated a polycystic tumor the size of a walnut which had been excised from a man thirty years old.
E. R. LeCount found a urachal cyst the size of an orange while making an autopsy on a man fifty-two years of age.
Interesting articles on urachal cysts have been written by Rippmann (1872), Wolff (1873), Scholz (1878), Schaad (1886), Tait (1886), Dossekker (1893), Douglas (1897), and others, and in 1906 the splendid monograph of Weiser appeared.
These cysts are naturally first noted in the mid-line between the umbilicus and pubes. They lie in the anterior abdominal wall just external to the peritoneum.
Size. — In the beginning they are relatively small, as in von Recklinghausen's, Atlee's, and LeCount's cases. As a rule, the increase in size is only gradual, but in a few instances the growth has been very rapid. They rarely extend above the umbilicus, but in some instances have reached as far as the xiphoid. Among the largest cysts are those recorded by Pratt and Bond, Macdonald, Rippmann, and Tait. In Pratt and Bond's case the cyst reached upward beneath the liver. Macdonald' s patient had a markedly distended abdomen; it was firm and rather flat as far as the ensiform cartilage. In Tait's Case 1, 30 pints of fluid were evacuated at operation. Rippmann's was probably the largest on record. At autopsy the cyst was found to contain 52 liters of fluid weighing 100 pounds.
The cyst may or may not burrow beneath the bladder, and encroach on the vaginal vault. It is sometimes attached to the bladder by the urachal cord, and where the tumor has reached large proportions, it is usually adherent to the umbilicus.
The cyst-walls vary considerably in thickness. Some are verj^ thin, others may be from 1 to 4 mm. thick.
540 THE UMBILICUS AND ITS DISEASES.
The inner surface of the cyst is usually smooth. Sometimes coagulated cyst fluid clings to its walls. In Macdonald's case papillary masses were found springing from the inner surface of the cyst (Fig. 240, p. 559).
As these cysts are due to dilatations of the urachus, we should naturally expect to find them lined with transitional epithelium. When the cysts are small, the lining with transitional epithelium is often found, but in the large cysts, there not being enough to cover the whole surface, remnants of this transitional epithelium are often found only over certain areas on the cyst-wall. The walls are composed of fibrous tissue and contain a varying quantity of non-striped muscle. In Tait's Case XI calcareous particles were found scattered throughout the wall of the cyst.
Cyst Fluid. — The character of the fluid contained in urachal cysts varies considerably. Sometimes it is pale yellow and limpid, closely resembling ascitic fluid. In other cysts it is yellow and transparent or tenacious and ropy. The fluid may be of a pale-green color. In some cysts it is brown or of a chocolate color; or it may be thin and with a hemorrhagic tint. Whether the fluid be thin and clear, or dark and turbid, it often contains large clumps of coagulated lymph or fibrin. Such masses have been referred to by some writers as "necrotic lymph " or cheesy masses. They are strongly suggestive of the coagulated material often noted in ovarian cysts. The cyst fluid contains albumin and mucus. On histologic examination exfoliated squamous epithelium, fat-droplets, and cholesterin crystals are often noted.
Sex. — Of the cases of simple uncomplicated and non-infected urachal cysts here recorded, and in which we were able to obtain definite data as to the sex, 16 were in women and 5 in men.
Age. — The youngest patient was six years and the oldest fifty-four. The accompanying table furnishes the following data:
Six years of age 1 case
Between ten and twenty years 1 "
" twenty and thirty years 1 "
" thirty and forty years 7 cases
" forty and fifty years 3 "
" fifty and sixty years 2 "
The first symptom is usually enlargement of the lower part of the abdomen. This, as a rule, is in the mid-line, but the swelling, sometimes accompanied by pain, may first be noticed in the right iliac fossa, and the picture may strongly suggest an appendicitis.
With the increase in abdominal girth there may be a moderate degree of indigestion, and where the cyst has reached large proportions, there has been dyspnea. Some of the patients have become progressively emaciated and have lost in strength.
Micturition has been normal in some, frequent in others. It is but natural that the bladder should be markedly encroached upon in some cases, particularly as the excursus of the tumor is limited, on the one side by the peritoneum, and on the other by the anterior abdominal wall.
Pain has been a marked feature in some cases, absent in others. The pain is probably in a measure due to pressure on the terminal sensory nerve-trunks, owing
LARGE URACHAL CYSTS. 541
to the tension under which the cyst develops, confined, as it is, between the layers of the abdominal wall. But it must also be remembered that the cyst is separated from the abdominal contents only by a thin peritoneum, and consequently the slightest inflammation of the cyst-wall must readily extend to the peritoneum and not only produce pain, but also cause the omentum or some other abdominal structure to become adherent to the abdominal wall over the cyst. Such a condition was noted in Carroll's case, and also in one recorded by Doran.
On physical examination an abdominal swelling is noted. This may extend over the entire abdomen, or be limited to the lower portion. Although the tumor may be exceedingly large, there exists a certain amount of repression of the abdominal wall, due to the tonic contraction of the recti muscles. When the patient is anesthetized and the recti muscles are relaxed, instead of being board-like, the abdomen may become quite soft, and the cystic tumor can then be readily detected. If the abdominal walls are naturally tense, the difficulties in making an accurate diagnosis are augmented. In some cases definite fluctuation can be elicited.
Urachal cysts have been diagnosed as a distended bladder, as ascites, as an appendicitis with abscess formation, as a cyst with or -without twisting of the pedicle, as a localized peritonitis with a serous exudate under the anterior abdominal wall, and as a tuberculous peritonitis.
The distended bladder is readily emptied, and the ascites relieved by paracentesis. With the patient asleep, it is relatively easy to outline the cyst and to differentiate it by the absence of the induration, usually associated with an appendix abscess. Furthermore, with the abscess there is likely to be a history of an elevation of temperature and of a definite leukocytosis.
An ovarian cyst, whether mobile or twisted, lies much farther back in the abdomen and can be separated from the anterior abdominal wall, particularly when the patient is under narcosis. The differentiation from a localized peritonitis or from a tuberculous peritonitis is not so easy, particularly when the patient has become emaciated. Even in these cases, however, when the patient is asleep, the sharp outlines of the urachal cyst are readily distinguishable from the rather diffuse cystic accumulation occurring with a peritonitis. Again, in the case of a urachal cyst, moving it from side to side is likely to produce traction on the umbilicus. With an aspirating needle one can readily remove some of the cyst fluid and thus usually settle the diagnosis.
The following case that recently came under my notice is of such interest in connection with the differential diagnosis of urachal cysts that I shall report its salient features.
A Tremendously Thickened B 1 a d d e r - w a 1 1 Producing a Tumor Reaching Almost to the Umbilicus and Simulating a Urachal Cyst. — The great thickening of the vesical wall was due to a diffuse neuroma. I shall refer to this case very briefly, as Dr. Welch and I will report it in detail elsewhere.
Surg. No. 34093. P. B., a colored boy three years and seven months old. was admitted to the surgical service of the Johns Hopkins Hospital on March 9, 1914. for an ununited fracture of the left tibia and fibula. Dr. Heuer wired the ununited
THE UMBILICUS AND ITS DISEASES.
fracture, and the boy made an uneventful recovery. When he entered the hospital it was noted that he had a firm mass extending upward from the symphysis to within 2 cm. of the umbilicus. This mass was broad below and rather narrow near
« 3 1 5
Fig. 234. — A Diffuse Neuroma of the Bladder. (After William H. Welch and Thomas S. Cullen.) The picture shows the appearance of the bladder when the abdomen was opened. The contracted viscus extended almost to the umbilicus, was large and exceedingly hard, and even after it had been brought out of the abdomen, it was almost impossible to realize that it was the bladder. When the bladder was lifted up, it was found that the right ureter was 8 mm. in diameter. The left ureter was slightly enlarged. The surface of the bladder was covered with great congeries of what appeared to be small and tortuous vessels. These were noted at once, but were particularly well seen when the peritoneum was stripped back. Subsequent histologic examination showed that most of these tortuous cords were nerves. The remnant of the urachus was larger than usual. Not knowing at the time the unusual character of the growth, I cut into it and found that the tumor was caused by a tremendous thickening of the bladderwall. For the appearance of the cut bladder-wall see Fig. 235; for the histologic picture see Fig. 236.
the umbilicus. Through the lax abdominal walls it could be readily grasped with the hand. Micturition was normal, and when the bladder was empty, this tumor diminished little, if any, in size.
It seemed to be a urachal tumor of some kind, and Professor Halsted, knowing
LARGE URACHAL CYSTS.
that I was much interested in urachal remains, kindly transferred the case to the Gynecologic Department.
Operation (March 28, 1914). — Feeling confident that we were dealing with a
Fig. 235. — Cut Surface of the Bladder Showing a Diffuse Neuroma of its Walls. (After William H. Welch and Thomas S. Cullen.) The figure shows the lower part of the bladder seen in Fig. 234, after the top had been removed. The bladderwalls protruded into the cavity, rendering it very small. The inner surface at this point was covered over with only.a single layer of epithelium, which stained very faintly. All trace of the transitional epithelium was wanting in the sections examined. The bladder-walls in the portion removed varied from 1 to 3 cm. in thickness, and everywhere this coarse and tortuous texture was the striking characteristic. A low-power section through the bladder-wall showed an abundance of nerves on the outer surface. There was a muscular zone with nerve-bundles scattered throughout it, and an inner zone, varying from 1 to 2 cm. broad, consisting almost entirely of nerve elements. (See Fig. 236.)
urachal tumor, I made a median incision from the umbilicus to the symphysis, and at once encountered the tumor seen in Fig. 234. It was very firm, and over a large area was covered with peritoneum. Attached to its upper end was what appeared to be the urachal cord. Immediately beneath the peritoneum of the tumor were
THE UMBILICUS AND ITS DISEASES.
Fig. 236. — A Diffuse Neuroma Forming a Mantle Abound the Cavity of the Bladder. (After William H. Welch and Thomas S. Cullen.) Surg. No. 34093. Service of Professor William S. Halsted, Johns Hopkins Hospital. The section has been taken through the top of the bladder seen in Fig. 234. It embraces both walls of the bladder, and near the center the slit-like vesical lumen is visible. This photomicrograph shows numerous nerve-trunks on the outer surface of the bladder. The white areas scattered throughout the bladder muscle are also nerves. Surrounding the bladder cavity is a mantle composed almost entirely of nerves. This nerve zone varied from 1 to 2 cm. in thickness. The mucosa of the bladder in this vicinity was in most places reduced to one layer of epithelial cells that were cuboid or flat. (Iron. hematoxylin. Photomicrograph by Mr. Herman Schapiro.)
LARGE URACHAL CYSTS. 545
numerous small, tortuous cords. The obliterated hypogastric remains were unusually large'.
The ureter on the left side was normal in size; that on the right, fully 8 mm. in diameter. It was evident that this tumor either lay as a cap on the top of the bladder or that it formed an integral part of the bladder-wall. After carefully walling it off, I cut into it and found that we were dealing with a greatly thickened bladderwall. Fig. 235 shows the proximal portion of the wall on section. The inner surface of the bladder was thrown into folds, and its mucosa was exceedingly thin. The bladder-wall was markedly changed, being coarse in texture, due to the crosssection of many cords which emerged from the surface. Only near the peritoneal surface was there any semblance of normal bladder muscle. The walls of the bladder were approximated with considerable difficulty, and sutured, and a drain was laid down to the peritoneum. After the operation the boy did well for several hours; he then developed nausea, vomiting, abdominal distention, and tenderness; his temperature ranged from 100.4° to 103.8° F. and his pulse was very rapid.
On April 1st it was deemed advisable to do an enterostomy. He was given a few whiffs of gas, but died before any operative procedure could be carried out. Much to our regret no autopsy could be obtained, but the abdomen was sufficiently opened to see that peritonitis existed.
Examination of the portion of the bladder removed showed that its walls varied from 1 to 3 cm. in thickness, the extreme degree of thickening being more marked in the posterior vesical wall and at the top of the bladder. Wherever the thickening was marked, this very unusual and coarse appearance was noted.
Fig. 236 is a photomicrograph of a section taken through the top of the bladder. It embraces both walls and the lumen of the bladder. On the outer surface of the bladder are a large number of nerves. These represent the tortuous cords noted at operation. The muscular walls of the bladder are still well preserved, but penetrating here and there are large nerves. Separating the muscle from the bladder mucosa is a zone consisting entirely of nerve elements. In other words, surrounding the bladder cavity in this region is a mantle of nerve tissue varying from 1 to 2 cm. in thickness. We are indebted to Mr. Charles Miller, the technician in Professor Mall's department, for preparing many exquisite sections showing the appearances with the various nerve-stains. These findings will be reported in detail at a later date.
The bladder mucosa in the portion removed was in some places composed of several layers of transitional cells, but in most places the epithelium was but one layer thick and almost flat, and the nerves came up to and encroached upon the epithelium.
Had I, prior to operation, for a moment dreamed that this was not a urachal tumor, 'the bladder would have been at once filled with thorium and x-rayed. Knowing what we do now, we are not in the least surprised that such a bladder would be very slow to heal after being incised. The broad inner zone consisted almost entirely of nerves, and in addition had a very meager blood-supply.
This is the only bladder tumor of this character with which we are familiar; a mistake in diagnosis of this kind will rarely occur.
546 THE UMBILICUS AND ITS DISEASES.
TREATMENT OF URACHAL CYSTS.
A median incision, commencing just below the umbilicus and extending to the pubes, will be sufficient to expose a urachal cyst of moderate size. As soon as the recti muscles are separated, the cyst will come into view. Sometimes it is infected and shows signs of inflammation. It is usually loosely adherent to the peritoneum, and can be readily shelled out. Sometimes it is rather firmly adherent to the posterior surface of the bladder. In those cases in which the urachus is rather thick and passes directly into the cyst, it is well to treat it as a pervious cord and to ligate it with Pagenstecher thread and cover this in turn with catgut, to prevent the possible development of a urinary fistula in the lower angle of the abdominal wound.
If the urachal cyst extends upward beyond the umbilicus, it is wise, when making the abdominal incision, to encircle the umbilicus, as this is often adherent to the cyst and should be removed with it.
In some cases it has been found possible to remove the cyst without opening the abdominal cavity. In others the cyst had become adherent to the omentum, and it was necessary to liberate the omental adhesions before the tumor could be removed.
When the cyst is exceptionally large, the peritoneum has of necessity been widely separated from the anterior abdominal wall. After operation the normal intimate relation is usually restored, but that this does not always happen is evident from Douglas's case. After drawing off 25 pints of clear fluid, Douglas readily separated the cyst-wall. The area of peritoneum separated from the parietes extended from about three inches above the umbilicus to the symphysis. It was observed that the peritoneum sank away from the parietes, but, thinking that when the abdominal wound was closed the intra-abdominal pressure would bring it into apposition with the abdominal wall, Douglas made no effort to stitch it there. The abdominal wound was closed in the usual manner and a firm compress was applied. The patient left the operating room in a remarkably good condition. Twenty-four hours later her temperature was 99.4° F., her pulse 136, respirations, 30. She was nauseated, vomited slightly, and there was some epigastric distention. She became dull and roused only when vomiting. Her condition rapidly grew worse, and she died forty-six hours after operation.
At autopsy the entire detached peritoneum on the right side was found to be gangrenous. There had been no hemorrhage, but there' was a little effusion between the peritoneum and abdominal wall. The peritoneal cavity contained a little brown serous effusion, but no pus or lymph.
Tait also reported a death in one of his large cyst cases. The cause could not beascertained, as no autopsy was obtainable.
As a rule, non-infected urachal cysts can be removed with little clanger. If very large, it may in rare instances be advisable merely to drain them and allow the sac to contract down gradually. It can then be removed with less danger of injury to the peritoneum. On the other hand, the adhesions at the second operation are liable to be much denser.
Where the peritoneum has been widely denuded, it may be tacked to the abdominal wall with several delicate catgut sutures; or one or two delicate protective drains may be carried down to the peritoneum, not only providing for the escape of any slight amount of fluid that may accumulate, but also allowing the air to escape and tending to make the abdominal walls flatten down on the peritoneum.
LARGE URACHAL CYSTS. 547
DETAILED REPORT OF LARGE, NON-INFECTED URACHAL CYSTS.
This list includes those cases in which little or no infection existed. Tait, in his article published in 1886, recorded a relatively large number of cases. The majority of these and some others were rather indefinite and have purposely been omitted.
The cyst in Schaad's case was probably urachal in origin, but it was lined with high cylindric epithelium; and as glands opened into it, its origin from remnants of the omphalomesenteric duct cannot be absolutely excluded.
A Urachal Cyst. — Atlee,* on opening the abdomen for the removal of an ovarian tumor in a girl eighteen years of age, found a urinary pouch in the linea alba. This he accidentally divided with the knife. The abdominal walls were very thick, vascular, and remarkably muscular. Between the muscle and the peritoneum he opened a small cyst from which about one ounce of yellowish liquid, resembling ordinary ascitic fluid, escaped. The posterior wall of the sac was cut through and the peritoneum opened. There were no adhesions. The bladder occupied the normal position. On the sixth day the dressings were moist, and by the end of a month Dr. Fay, who looked after the case, felt sure that the fluid was urine. The patient was advised to empty the bladder frequently, and the discharge soon ceased.
"The only conclusion possible was that we were dealing with a dilated urachus, which, although closed at the umbilicus, had from birth maintained a communication with the bladder."
A Urachal C y s t . f — "I. F., aged six years; Newcomerstown, Ohio. Physician, Dr. Hosick. The patient had been taken suddenly sick about three weeks before. The pain seemed to be in the neighborhood of the appendix, but somewhat below McBurney's point. Slight elevation of temperature. Thighs flexed. Amount of pain quite variable. Bowels regular. No appetite. A little before she came to the hospital the abdomen became much distended and painful. Pulse more rapid. Temperature, 100° F. The presumptive diagnosis had been appendicitis with enormous abscess formation. When the patient reached the hospital (May 7, 1911), the abdomen was considerably distended and tender throughout, and with distinct fluctuation. There was perhaps a little more tenderness in the appendix region than elsewhere, but this was not marked. Diagnosis, very doubtful, but the case clearly one for exploration.
"When the patient was under the anesthetic I could determine nothing more about the case. No lump in the region of the appendix. Made the usual median incision. As soon as the incision was made there was an escape of a large amountof rather thin, yellow, odorless fluid. The opening was enlarged, and the cavity thoroughly flushed out, the water bringing out a large amount of what seemed to be necrotic lymph. The cavity was found to be bounded below by the pelvis, above by probably the transverse colon and the stomach. It extended on each side clear to the flanks. The intestines were crowded back by the posterior wall of the cyst. The uterus in this case could be readily felt, though infantile in size, below the membrane. Introduced drainage, with partial closure of the incision. The patient made a smooth convalescence and returned home in the usual time, with distinct warning as to the probability of a hernia.
- Atlee, Washington L. : Ovarian Tumors, Philadelphia, Lippincott, 1873, 50.
t Baldwin: Large Cysts of the Urachus. Surg., Gyn. and Obst., 1912, xiv, 636.
548 THE UMBILICUS AND ITS DISEASES.
" September 3, 1911, patient returned with her mother because they had noticed a beginning hernia. The hernia was operated upon the next day. I made an incision directly through the old scar, dissecting down very cautiously, as I expected to find extensive adhesions. On finally opening the peritoneum I found that the abdominal contents were in every respect absolutely normal, except for two cobweb adhesions of the omentum to the anterior abdominal wall. The appendix was brought up and found to be entirely normal; was removed on general principles. Pelvic organs normal. In fact, had one not familiar with the previous history of the case made the operation, he would have found nothing whatever to suggest any previous trouble in the abdomen. In other words, the sac had absolutely disappeared. The bladder, however, seemed to be a little higher up than usual, though even that was not positive."
Large Urachal Cysts. — Dr. Bantock* said he was sure he was expressing the sentiments of every one present when he desired to offer the thanks of the Society to their President [Lawson Tait] for the very remarkable and interesting paper which he had just read. The cases were of remarkable interest, but he feared there was no one who could discuss the subject from experience. The paper was one for future perusal and careful study. He at least was not prepared to discuss it, but he thought he might refer to two cases of which he was reminded by some of the cases related by the President.
The first case was that of a married woman, aged thirty, the mother of two children. On dividing the parietes, Bantock opened into a cyst containing 25 pints of a thick, grumous fluid, with a very decided biliary tinge. When the whole of the fluid was removed, the cyst was found to be unilocular, and looking down into the pelvis was like looking into one's hat, so completely did the walls of the cyst line the pelvic cavity. After separating what appeared to be cyst-wall from the parietes on each side, and cutting away what was thus separated, recognizing the hopelessness of proceeding further, he washed out the cyst with a solution of iodin and closed the wound, leaving a drainage-tube passing down to the bottom of the pouch. Although the separation of what was taken as cyst-wall was carried beyond the umbilicus, the peritoneal cavity was not opened. A thick, pultaceous fluid of the color of mustard came from the cavity for many weeks, but the patient was discharged quite well at the end of about two months. Bantock had lately seen this patient in perfect health. He adds that the source of the brilliant yellow color of the discharge was still a puzzle to him.
The second case was that of a married woman, thirty-seven years of age, the mother of three children. The history told that she was taken ill on January 10th with violent sickness and pain all over the stomach. She was laid up and became feverish; the pain being severe for five days and the sickness for two days. The abdomen gradually got larger, and about the end of February she was tapped of rather more than half a gallon of a thickish, pale-yellowish fluid. In about a month more she was tapped again to the extent of three pints of a thicker fluid, and recommended to apply poultices. Shortly after this the puncture-hole opened and discharge came away. She then presented herself at the out-patient department of the Samaritan Hospital, under the care of Dr. Amand Routh, with whom Bantock saw her. There was then a fistulous opening about two inches below the umbilicus, in the middle line, and an ordinary surgical probe passed in for its whole length. She
- Bantock: From Tait's article, Brit. Gyn. Jour., 1886-87, ii, 348.
LARGE URACHAL CYSTS. 549
was admitted into the hospital on July 20th, and Bantock thought he had to deal with a multilocular tumor of which a central cyst had suppurated, as on withdrawing the probe no discharge followed. On July 27th he divided the parietes by a double elliptic incision, with the view of cutting out the fistulous tract, and was not a little surprised to find, on completing the division on one side, that he had opened directly into a unilocular cyst containing from three to four pints of a purulent-looking fluid. On further examination he found the same condition of things as in the first case, and, recognizing the inadvisability of proceeding further, he thoroughly washed out the cavity with plain warm water and closed the wound, leaving in a glass drainagetube. The patient presented herself at the hospital two or three weeks before the meeting of the society and was in perfect health. In this case the uterus was low down, pressed forward, and fixed. Bantock said that he was as much at a loss to explain the relations and origin of this cyst as in the first instance, but he thought they were worthy of being related in connection with the very remarkable cases read by the President.
Probably a Urachal Cyst. — Bryant,* in discussing Doran's paper, reported two cases. In Case 1, on operating on what had been diagnosed as an ovarian cyst, he suddenly opened into a cyst from which serosanguineous fluid escaped. This was in front of the peritoneum, and was with difficulty separated from the bladder. When this had been done, the cyst came away in his hand, and it was clear that it had no pedicle nor any connection with the broad ligament.
A Cystic Urachus. — Carroll's! patient was a woman thirty-four years old. She had been well until twenty-three. After that she had had attacks of abdominal pain, loss of weight, and on one occasion inflammation of the bladder.
On examination an induration was found extending from the umbilicus two to three inches to the right, and downward for three or four inches. The tumor was apparently too near the umbilicus to be of appendiceal origin.
Roswell Park made a median incision below the umbilicus. The tissues were very dense and difficult to cut. A sac was opened and fluid escaped. The incision was enlarged, and a finger introduced. The tumor was found to be a cystic urachus. A connection with the bladder could be traced, but a probe could not be passed. The connection was tied off and the cyst dissected out. There were a number of adhesions between the tumor and the omentum. The patient made a good recovery. "The probable explanation of the attacks seemed to be an oozing of urine into the upper or cystic part of the urachus, and as there was no egress for the fluid once gathered, it was absorbed into the system, causing a toxemia."
A Large Cyst of the Urachus. £ — The patient was a girl, twenty years of age. The tumor had first been noticed a year before admission. It had increased greatly in size in the last four months. It had commenced as a painful point in the right iliac fossa. On account of the patient's emaciation and the increase in abdominal girth the physician had diagnosed tuberculous peritonitis. On admission there was great abdominal distention, evidently due to fluid.
Operation. — An incision was first made as far as the umbilicus, and was extended upward to the xiphoid. The tumor was adherent at the umbilicus. The pedicle was attached to the summit of the bladder. It had no lumen and did not open into
- Bryant, T. : Brit, Med. Jour., 1898, i, 1390.
f Carroll, Jane W.: Buffalo Med. Jour., 1895-96, xxxv, 869.
1 Cotte et Delore: Gros kyste de l'ouraque. Lyon med., 1905,, cv, 373.
550 THE UMBILICUS AND ITS DISEASES.
the bladder. The uterus, tubes, and ovaries were normal. The cyst was unilocular and contained between eight and nine liters of brown, hemorrhagic fluid. This was not examined microscopically. The inner lining of the cyst was made up of inflammatory tissue. On the cut surface the urachus was recognized as a cord. The authors say that the cyst had developed from the urachus. The patient made a good recovery.
A Urachal Cyst Simulating an Appendicular Abscess.* — "The patient, aged seventeen and a half years, unmarried, applied to Dr. R. Drummond Maxwell at the out-patient department of the Samaritan Free Hospital on July 16, 1908. She complained of tenderness and swelling in the right iliac fossa, associated with a history of a sudden attack of pain in that region a month previously, and she was admitted into my ward at once. After admission I found that the relations of the swelling to adjacent organs could not well be defined until I examined the patient with the aid of anesthesia, under circumstances presently to be explained. The patient's mother informed me that the catamenia were established at the age of fourteen years, without pain or constitutional disturbance. The periods were always scanty and attended with very little pain, and the interval was about five weeks. The patient had never suffered from any neurosis before, at, or after puberty. On June 16th, one calendar month before admission, the menstrual flow appeared as usual, but was accompanied by violent pain never experienced before. The pain continued for two days and then it abated. The patient at once resumed her work, but the pain returned two days later and obliged her to take to her bed again. During the whole of the week before admission she was quite incapable of attending to her duties. Roughly speaking, as regards what could be made out before anesthesia was employed, there was a fairly defined, almost spheric swelling in the right iliac fossa, slightly movable and tender to the touch. There was resonance on percussion over its outer aspect. The lower part of the swelling could be defined on rectal examination. I refrained from making a vaginal exploration until a consultation was held. Then it was found that the vagina was barely two inches deep. A kind of dimple could be defined at the blind extremity toward the right. The tumor did not bulge into the vagina. At the lower limits of the swelling was a tuberosity which lay behind the vagina and in front of the rectum. The temperature and pulse were low. The patient had never been laid up with any severe illness. Before the arrested development of the vagina had been detected, appendicular abscess was suspected, but after the examination, hematometra or hematosalpinx seemed equally probable. On July 21st the period began, as usual, about five weeks after that which had preceded it. I found that there was no palpable increase of pain or tenderness in the tumor nor any appreciable increase or decrease in size. The flow was unusually free. I decided to examine the patient under anesthesia during the period in order to discover the channel which transmitted the menstrual blood into the vagina, and for other manifest reasons.
"Examination under Anesthesia. — The perineum was markedly deep, so that the anterior commissure lay far forward. The labia, clitoris, and meatus urinarius were normally developed. There appeared, on the other hand, to be Ao hymen nor was there the least trace of carunculse."
"The vagina formed a blind pouch about two inches deep. The rugae were prominent.
- Doran, Alban H. G.: The Lancet, 1909, i, 1304.
LARGE URACHAL CYSTS.
"The vaginal pouch was distinctly deeper on the right side, whence dark menstrual blood was seen to issue. On stretching the adjacent mucosa with the fingers, a crescentic fold with the concavity toward the left was detected. It covered the aperture whence proceeded the blood. A uterine sound could be passed into this aperture and pushed onward for three inches upward, backward, and a little to the right, closely following the outer limits of the lower pole of the swelling, as could easily be defined on digital exploration from the rectum (Fig. 237). On bimanual palpation the swelling was found to be a well-circumscribed tumor, firm, pushed a little downward, yet even then its lower pole did not bulge into the vagina, but passed behind it. The tuberosity in the rectovaginal septum, discovered at the previous examination, lay to the left of the menstruating tract. It felt like a small cervix. The nature of the case remained obscure. I kept the patient at rest for a week. The period ceased, and the tumor remained stationary. There was one sharp attack of local pain on July 28th, without any rise of pulse or temperature."
"Operation. — On July 29th I operated with the assistance of Dr. R. V. G. Monckton, Dr. S. H. Belfrage administering ether and chloroform. I made an incision in the middle line. The parietes were unusually vascular. After separating the recti I came across a thick membrane of doubtful character, and lower clown I exposed the wall of the bladder, which extended for quite two inches above the pubes. The membrane was cut through, and about half a pint of a perfectly clear fluid was removed; unfortunately, none was preserved. The fluid lay in a cyst behind the recti and anterior to the parietal peritoneum, the membrane through which I had made
the incision being the anterior portion of the cyst-wall. The cyst was connected with the bladder by a thick cord half an inch in length. The upper limits of the cyst lay close below the umbilicus. In exploring the upper end of the tumor I laid open the peritoneal cavity. The omentum adhered to the peritoneum, investing the back of the cyst in this region. The intestines seemed healthy; there was no evidence of tuberculous disease, no free fluid, and no intraperitoneal tumor. Lower down some coils of ileum adhered to the parietal peritoneum behind the tumor. "I endeavored to define the relations of the cyst to the genito-urinary tract. A catheter was passed into the bladder, and a few ounces of urine were drawn off. There was no communication between the cavity of the bladder and the cavity of the cyst; the thick cord between the two was clearly a portion of the urachus, and I observed that it ran into and not over the cyst-wall.
"As might have been suspected from what could be defined before the operation, the cyst lay to the right of the middle line. On pressing against its wall on the right interiorly, from the inner side I detected a fusiform body like a uterine cornu or a small but entire virgin uterus, lying in the position of the menstruating tract along
Fig. 237. — Diagram Showing the Arrested Development of the Genital Tract and the Relation of the Malformed Parts to the Cyst of
the Urachus. (After A. Doran.)
Vg, vagina, its blind end rising higher on the right
side than on the left; VI, valvular fold, through which
a sound (
) passes into Rt. Ut., the right cornu; Ov, right
ovary; Lft. Ut., solid body, probably left cornu; the dotted lines indicate a band, not clearly definable, connecting it with the right cornu.
THE UMBILICUS AND ITS DISEASES.
which a sound had been passed a week before. Above this body thickened tissue could be felt — apparently a small ovary. The tuberous, cervix-like body already mentioned could be plainly defined through the walls of the lowest part of the cyst. When thus explored, it was found to be a distinct, fairly movable structure — the left ovary or uterine cornu. On further palpation through the cyst-wall the pelvic cavity felt quite free from any tumor or deposit. There certainly was no such thing as a collection of retained menstrual blood.
" At this stage of the operation it became evident that the swelling, which disappeared entirely when I opened the cavity full of fluid, was a urachal cyst. The swelling — in other words, the cyst — had been the cause of all the patient's recent trouble. As there was no trace of a hematometra or hematosalpinx, I did not feel justified in dissecting in the dark behind the cyst, amid deformed structures, in
very uncertain relations to ureters, blood-vessels, etc., merely to make out the extent of arrested development of the uterus and appendages. It was with the cyst, therefore, alone that I had to deal. I knew of several objections to the draining of a urachal cyst, nor could I dissect away its outer wall, since, as I have just observed, its positive relations to malformed structures were very uncertain. For these reasons I simply trimmed away as much of the lining membrane as could be safely removed. Then I cautiously passed several fine catgut sutures along the substance of the outer wall and tied them, so that the cyst cavity was closed in. This outer wall was the muscular sheath of the urachus abnormally thickened, so that the manceuver just described was easy and nothing was caught up behind the cyst. I transfixed the segment of the urachus, which ran between the lower limits of the cyst and the bladder, with a fine linen suture and tied it on both sides. It was then divided between the cyst and the ligature. As will be explained presently, it is fortunate that I transfixed the urachus instead of tying a single ligature around it as though it were an artery. I kept the portion attached to the cyst for microscopic examination. Lastly, the sheaths of the recti were united with interrupted fine linen sutures and the integuments closed with interrupted silkworm-gut.
"During the summer vacation Dr. Maxwell took charge of the patient in my absence. He reported that up to the day of her discharge at the end of August there was no sign of leakage of urine through the wound nor any show of blood."
Microscopic Examination of the Cord Between the Cyst and the Bladder. — A section of the cord-like structure which ran on the surface of the parietal peritoneum
Fig. 238. — Section of the Segment op Urachus which Passed Between the Bladder and the Cyst-wall, as Seen Under a Low Power. (After A. Doran.) The canal is quite unobstructed and lined with transitional epithelium; the muscular coat is very thick. (In our reproduction part of the detail has been lost — T. S. C.)
LARGE URACHAL CYSTS. 553
between the fundus of the bladder and the cyst was made at the Royal College of Surgeons of England. There could be no doubt that it was a portion of the urachus. Mr. S. G. Shattock reported that the canal was quite patulous and lined with perfect transitional epithelium of the bladder type. The lumen was free from catarrhal or other morbid products. The muscular coat was abnormally thick, but showed no evidence of inflammation or edema. Its inner portion was mostly made up of circular, and its outer portion of longitudinal, fibers, but there was some irregularity in the direction of the fibers in both portions. Some subperitoneal fat was intimately connected with the periphery of the urachus. The appended reproduction of a photomicrograph (Fig. 238) shows the above-described appearance of the urachus as seen under the microscope.
On p. 635 I have recorded another interesting case of Doran's — a cystic sarcoma of the urachus.
A Large Cyst of the Urachus. — Dossekker* reports the case of a woman, born in 1850. When forty years of age a tumor the size of a small fist was found to the right of the uterus. She had various abdominal symptoms, and finally was sent to a sanitarium. When forty-two years of age she was admitted under the care of Kronlein. She looked very pale. The abdomen was markedly distended, as with a pregnancy at the ninth month. There was, in addition, a distention at the umbilical region, with definite fluctuation. The diagnosis made was ovarian cyst, possibly from the right side, with hemorrhage into the cyst, and probably torsion of the pedicle.
Operation. — An incision was made from the umbilicus to the symphysis. As soon as the abdominal walls were cut through the knife entered a cyst cavity. The wall of the cyst was intimately attached to the abdominal wall, and a large quantity of thin, hemorrhagic fluid escaped. This was not sticky and had no odor. It amounted to between three and four liters. The tumor was gradually shelled out, with little or no hemorrhage, and the abdominal cavity proper was not opened. The cyst did not extend into the pelvis, but reached as far as the top of the bladder. At no point was the peritoneum opened. In other words, the large cyst with its contents lay between the abdominal wall and the parietal peritoneum. The patient made a splendid recovery. Examination later showed that the uterus and left ovary were normal. The right ovary could not be outlined.
Dossekker, after discussing the various points of interest, says that on histologic examination the wall was found to consist chiefly of dense connective tissue. The inner surface in most places was without any epithelial lining, but at some points this was intact. It consisted of a high, many-layered, so-called transitional epithelium. The basal nuclei were elongate or oval ; the peripheral were more roundish or flat in form. The epithelium corresponded in character to that of the bladder, and agreed with the description given by Luschka of the epithelium lining the canal of the urachus.
A Cyst of the Urachus. — On page 182 Douglasf describes the case of " Mrs. C, aged thirty-six, married eleven years, but sterile. The family and personal history is good; she has always enjoyed good health, but has never been robust. Menstruation has been scanty and painful, but regular; she has suffered with con
- Dossekker: Klin. Beitrag zur Lehre von den Urachuscysten. Beitrage z. klin. Chir.,
1893, x, 102.
t Douglas, Richard: Trans. Amer. Assoc, of Obstet. and Gynecologists, 1897, x, 177.
554 THE UMBILICUS AND ITS DISEASES.
stipation, but the kidneys have acted freely and normally until recently. About eighteen months ago she observed a swelling in the lower portion of the abdomen, rather more prominent on the right side. The enlargement was soft and painless. It grew slowly and did not materially show until the last four months, within which time its growth has been rapid, chiefly to the right side. She has suffered from backache, some loss of flesh, slight cough, and decided digestive disorders. There has been but little pain or tenderness from the tumor, and no history indicating local peritoneal inflammation. The bladder has been somewhat disturbed, its action frequent, but the urine normal. She now complains more particularly of vomiting after eating and a sense of weight and heaviness in the epigastric region. Of late she has grown nervous and suffers from insomnia."
"Physical Examination. — -The abdomen presented a very peculiar appearance. It was symmetrically distended to about the size of a seven months' pregnancy, the greatest enlargement being on the right side; the veins were not enlarged, the skin was white and anemic-looking. By palpation the irregular swelling could be outlined. The tumor seemed to lie in the lower zone and the right half of the abdomen. It was soft, elastic, fluctuant and compressible. It was not movable; there were no irregularities or bosses upon it ; its surface was smooth; palpation was painless; the abdominal walls did not appear to glide freely over the surface of the tumor. There was dulness upon percussion over the entire tumor, yet that dulness, as was repeatedly remarked during examination, was not the characteristic flatness noted in ovarian cystoma. The dulness was absolute low down, but in the region of the umbilicus and beyond, the note became more resonant. Auscultation negative. Vaginal examination showed the uterus small, retroflexed, and rather low in the pelvis ; vaginal vault encroached upon by an elastic, fluctuant swelling. The weight of the evidence was in favor of the diagnosis of ovarian cystoma. The following peculiarities, however, were remarked upon, and were of such importance in our judgment as to render questionable the nature of the case. The appearance of the abdomen was not such as is usually noted in ovarian cystoma. While, of course, we appreciate that the shape of the abdomen varies greatly, yet in a cyst so distinctly unilocular as this appeared to be, and lying so superficially, one would expect to find the abdomen rising abruptly from the symphysis; that is, the tumor forming a distinct angle with the abdominal plane. In this case the abdomen looked more like one distended by ascitic fluid, rather flat upon the upper surface, and widely bulging upon the right flank. The next peculiar physical sign was the character of the percussion dulness.
" Operation. — An incision was made in the middle line, and in going through the linea alba and transversalis fascia I came upon the red, congested cyst-wall, which I at first thought was the peritoneum inflamed. I now aspirated the cyst and drew off 25 pints of clear fluid. An examination of the collapsed sac soon convinced me that I was not in the peritoneal cavity, and that I was dealing with a cyst of the urachus. Its attachment was not very intimate, and its enucleation was readily accomplished. Only slight hemorrhage attended its separation. As I removed the sac I recognized that I was working entirely outside of the peritoneum. The viscera could be felt through the peritoneum. The sac dipped down into the true pelvis in front of the uterus, depressing and retroflexing it. There was no apparent attachment of the sac of a ligamentous character to the bladder. Indeed, the cyst lay between the peritoneum and the transversalis fascia, with no special attachment
LARGE URACHAL CYSTS. 555
beyond a universal adhesion to all surrounding parts. The area of the peritoneum separated from the parietes extended from about three inches above the umbilicus to the symphysis, and from two inches to the left of the linea alba and through the lumbar and iliac regions of the right side. As there was no bleeding of consequence, we now prepared to close the abdominal wound. It was observed that the peritoneum sank away from the parietes, but thinking that, when the abdominal wound was closed, the force of intra-abdominal pressure would bring it in apposition with the wall, no effort was made to stitch it there. The abdominal wound was closed in the ordinary way. A good compress was applied over the abdomen, and a snuglyfitting bandage adjusted.
" The patient sustained but little shock from the operation and was placed in bed in remarkably good condition. The fluid removed measured 25 pints, was of a pale green color, and a few flocculi were observed in it. I regret to say that it was carelessly thrown away without being submitted to chemical and microscopic tests. The sac was composed of a thin, fibrous material, showing no evidence of muscular structure, and almost transparent; it was removed without tearing.
"The patient was operated upon on June 20th at 1 1 o'clock. Twenty-four hours after the operation the pulse was 136, respiration 30, temperature 99.4° F. She was nauseated and had vomited slightly; there was some epigastric distention; she had slept but little; the bowels had not moved, although active efforts were employed; the kidneys had acted sufficiently, 36 ounces of urine having been voided since the operation. The patient now became very dull, inclined to sleep, was roused only when vomiting; the vomiting was of regurgitant character, without apparent effort; the matter ejected had that ugly green color that we so much dislike to see. Her condition grew rapidly worse, the pulse became more frequent, the temperature reached 102° F. She died at 10 a. m., forty-six hours after operation.
"Autopsy. — The entire detached peritoneum on the right side was gangrenous. There was no hemorrhage, and but very little effusion between the peritoneum and wall. There was a little brown, serous effusion in the peritoneal cavity, no pus nor lymph. Death was due undoubtedly to sapremia. The detached peritoneum was not forced against the abdominal wall, as I had supposed it would be, but hung loosely, leaving quite a space between. This peritoneum was deprived of its nutrition, and had simply died from starvation."
Cysts of the Urachus.* — Ferguson says: " I do not feel, however, as has been stated by Tait, that extraperitoneal tumors in that region are all derived from the urachus. Tait's dictum was based on two cases submitted to operation, both of which resulted in death, in neither of which was there a postmortem examination, and in both of which the reported character of the cystic contents would justify the hypothesis entertained by some that cysts originating in the pelvic region may develop upward and forward in such a manner and way as to separate the peritoneum from the anterior abdominal wall, and thus become extraperitoneal. It is my conviction that I have seen at least one case of that character — one which grew to great dimensions and was cured over twenty-five years ago by excision of some of the anterior portion of the sac, and 'suture puckering' of the opening thus made, with drainage of the remainder, enucleation of the entire sac seeming too large an undertaking.
"In June, 1898, the patient, a man aged about forty-seven years, was brought to
- Ferguson, E. D.: Phil. Med. Jour., 1899, iii, 830.
556 THE UMBILICUS AND ITS DISEASES.
my office by Dr. M. B. Hutton, of Valley Falls, New York. He had lost notably in flesh and strength, though he was not anemic. He was inconvenienced by frequent urination, and complained of considerable pain in the lower portion of the abdomen. Dr. Hutton had satisfied himself that notable abdominal enlargement had been developing lately, which he ascribed to a tumor in the hypogastric region. The first recognition of the tumor was about a month earlier, but the first sense or discomfort was felt in July, 1897, nearly a year before the discovery of the tumor.
"On examination a flat tumor was found extending from the pubes to about two inches above the umbilicus, and from near each anterior superior spine of the ilium to its opposite fellow. The upper border was slightly irregular near the umbilical region, but elsewhere the contour was quite regular. The sense of resistance was that of a very firm, solid tumor, and at no point could fluctuation or diminished hardness be found. There was, however, a sense of nearness of the mass to the surface, which led me to state that it seemed to me to be in the abdominal wall, but its flattened shape and hardness, together with some irregularity of the upper border, led me to conclude that it was probabry a malignant disease of the omentum. Though such a growth as a primary trouble must be exceedingly rare, the shape and hardness led me to that working hypothesis, while the freedom from evidence of bowel involvement, and the yet moderate constitutional effects, led me to advise an exploratory operation, the final decision as to what could and should be done with the mass being left to a consideration of the conditions found on section.
"I heard nothing further of the patient until in July, when his increasing size and discomfort led him to accept my somewhat gloomy, or at least to him unsatisfactory, view of his case, and he decided to submit to an operation. Of course, the absence of renal or other contraindication had been established. The operation was undertaken July 26, 1898, and the first surprise occurred when, on moving the antiseptic dressing after he was under the anesthesia, I found the mass to be then of a globular form. To this was added a great diminution in the sense of resistance and a manifest fluctuation, showing the cystic character of the tumor. This change in the tumor was undoubtedly due to the relaxation produced by the anesthetic in recti muscles of unusual development. My first impression now was that I was dealing with a distended bladder, for the sac evidently extended into the pelvis and seemed more remote than formerly. Having satisfied myself that it was not a distended bladder, I proceeded with the operation until I came to the wall of the cyst just under the deep fascia of the abdominal wall. At this juncture the nature of the case flashed upon me, and I was able to state to those present that we were dealing with a cyst of the urachus. This conclusion was strengthened by the water-like appearance of the fluid which was removed by an exploring syringe. It being apparent that the lower portion of the cyst extended deeply into the pelvis and was probably intimately associated with the bladder-wall, a condition that would explain the frequent urination, I exposed the wall of the cyst before opening it, from as near the umbilicus as the mergence of structures would allow, to near the pubes. This I did in order to further a plan which I had quickly formed for the management of the case. In the first place, I had determined not to try to finieleate the entire cyst, bu1 to remove the posterior portion with the underlying peritoneum so far as I could, and allow the reclosure of the peritoneum, dealing with the remainder according to circumstances. Such a procedure would require free access to the deep portions of the cyst, hence my long incision. The cyst was
LARGE URACHAL CYSTS. 557
then opened the entire extent of the overlying incision, and an unknown quantity of water-like fluid escaped. The quantity, from absence of convenience for collection (the operation occurring in a private house), could only be estimated, but it was evidently more than two quarts, and probably less than four quarts.
"It was now practicable to investigate the relation of the wall of the cyst to contiguous parts; it was found to be intimately related to the bladder over a considerable extent of the surface of that organ, for it extended deeply into the pelvis. The posterior wall of the cyst was free from evidence of adhesion or other connection with the abdominal organs, and I was about to excise that portion of the sac when it occurred to me to ascertain whether the inner and secreting layer could be removed, thereby securing a surface which would unite. Beginning at the inner edge of my incision in the wall of the sac, and near the lower end of the opening of the belly, I was surprised and gratified to find that a layer of tissue, so thin as to be diaphanous in moderate light, and so strong as to allow of considerable traction without tearing, could be removed without much trouble and with practically no hemorrhage. In that manner the entire lining of the cyst was removed except at the umbilical region, where quite a surface existed, in which digitations penetrated the abdominal wall, and a blending of the tissues prevented the removal of the lining. This surface seemed rather large for complete excision with subsequent easy closure of the belly at that point, hence it was allowed to remain while attention was given to the denuded portion of the cyst. A single deep skin suture was placed to divide the unclosed umbilical area from the subcutaneous suturing below that point. Some iodoform gauze was then placed in the pocket left at the umbilicus, where the lining layer could not be removed, and the whole was sealed with a collodion seal, except over the gauze packing, with the request that it be left for several days unless indications arose showing inflammatory processes.
"It had closed in September, and the area showed in December a perfectly normal state of affairs aside from the scar at the umbilicus."
Cyst of the Urachus.* — The patient from whom this specimen was obtained was admitted to the Cook County Hospital February 27, 1895. He was a man fifty-two years of age, white, and single. He was admitted for an illness which had begun four weeks previously, with frequent micturition and pain in the region of the kidneys. Examination revealed an enlarged prostate. He had symptoms of cystitis with retention of urine. Hydronephrosis was present, and uremia ensued. He died on April 9th.
Autopsy Abstract. — " The bladder is large, with markedly thickened walls. Each lateral lobe of the prostate is the size of an English walnut. At the summit of the bladder, and separated from the bladder cavity by a thin membrane, is a cyst, the size of an average orange. It contains a thick, turbid, viscid, brownish fluid. The lining of the cyst presents an irregular surface, but there are no distinct rugae. The irregularities of the cyst lining are present on the upper surface of the interveningseptum, between it and the bladder cavity. The rugae of the bladder are continued upon its inferior surface. The ureters are dilated, as are also the pelves of both kidneys. Careful dissection fails to reveal further urachus remains in the abdominal wall or about the navel. Microscopic examination of the septum between cyst and bladder cavities disclosed the fact that the muscular coats of the bladder-wall were
- Le Count, E. R.: Transactions of the Chicago Pathological Society, Dec, 1895, to April,
1897, ii, 215.
THE UMBILICUS AXD ITS DISEASES.
not continued into the septum. This fact, taken in conjunction with the position of the cyst and the fact that the peritoneum of the abdominal wall was reflected upon the back of the cyst, and thence upon the back of the bladder, leaves no doubt that the cyst represents the obliterated and dilated lower end of the urachus."
An Enormous Cyst of the Urachus.* — - The following case is cited on account of some unusual features, and because it should be added to the list reviewed by W. R. Weiser in a most interesting and instructive article published in the Annals of Surgery for October. 1906.
Miss . aged forty. History of slowly growing abdominal tumor, beginning
in the region of the bladder and growing upward, with gradual onset of pressure symptoms, especially difficult respiration, pain, and impaired digestion. The abdomen was enormously distended, but not tender, nor did it bulge much in the flanks. It was rather firm, and was flat on percussion from the pubes to the ensifonn cartilage. Its appearance is well shown in Fig. 239.
Fig. 239. — The Abdominal Contour in a Case of Vest Large D
After T. L. Macdonald.)
"Operation (October 6. 1907). — Through the usual incision the cyst-wall was perforated and the fluid drawn off. Two-thirds came away clear: the remainder was turbid, and. lastly, thick, cheesy masses were wiped out. Investigation of the inside of the sac disclosed several thick, nodular masses which were strikingly carcinomatous in character. So far. the peritoneal cavity had not been opened, the sac being situated in front of it. The task of separating the cyst-wall from the peritoneum and viscera was begun by first stripping and cutting it from the epigastric region and from beneath the ribs, and here the peritoneal cavity was opened. It was hoped that from this point downward the dissection would be less difficult, but it was more so. The anterior surface of the peritoneum seemed to be fused with the sac, and the posterior with the viscera generally: and the character of the adhesions was the most dense ever encountered by the writer. These were followed deeply into the pelvis, in all directions, and freed: and finally the firm, fibrous
- Macdonald, T. L.: Ann. Surg:.. July-December, 1907, xlvi. 230.
LARGE URACHAL CYSTS.
attachment to the bladder was severed and the sac removed. The appendix, six inches in length, bright red, and surrounded by adhesions, was also removed. The abdomen now presented a most unusual sight. With the exception of the anterior surface of the stomach, not a vestige of normal peritoneum was visible. All the abdominal contents, including tubes, ovaries, uterus, and bladder, could be seen outlined through the thin, raw film of peritoneum to which they were firmly attached. The abdominal cavity was filled with normal salt solution and closed with three layers of buried absorbable sutures without drainage.
"Fig. 240 shows some of the nodular masses. There are others on the opposite side. These were on the inner surface of the sac, which was photographed in this way. The cyst was turned inside out, and through the incision, which had served for the evacuation of the contents, a large, thin, collapsed rubber punching bag was thrust, then inflated, thus distending the sac for photographic purposes.
"The report of our hospital pathologist, Dr. Birdsall, shows the cyst-wall to be fibrous, and the nodular masses, which, during operation we feared were carcinomatous, were papillomata. Of course, in a cyst of this size, which had been growing presumably for forty years, and subjected to the ever-increasing pressure of the accumulating fluid, we could not expect to find the normal histologic features of the urachus. Naturally, all except the fibrous structures would disappear by pressure absorption ; even bone has been known to do the same.
"Postoperative Course. — The patient's condition was critical for the two following days, active stimulation and intravenous saline infusion being demanded. The wound healed by primary union. The bowels were loose. The temperature ranged from 101° to 102° F. Daily palpation of the abdomen revealed fluctuation, and the percussion-note
was flat, showing that the salt solution was not being absorbed. On the seventh day a chill occurred, followed by a rise in temperature to 104° F. Assuming that the unabsorbed solution had become infected through the raw surface of the intestines, the lower end of the now healed wound was cocainized and cut through, allowing the escape of quarts of the salt solution, which had become purulent, and which presented the colon bacillus characteristics. This was followed by prompt improvement. Drainage and irrigation were continued for a week, after which the wound closed and convalescence and return to health were satisfactory.
"Comments. — The density of the adhesions cannot be appreciated unless encountered. It is true, incision, evacuation, and drainage would probably have been successful after a long period of waiting for the cavity to undergo obliteration. The assumption, however, that portions of the sac had become carcinomatous made extirpation seem imperative.
"Extirpation is evidently not commonly resorted to. Among the 86 cases re
Fig. 240. — A Urachal Cyst Turned Inside Oct and Showing Papillary Masses, Particularly in the Lower Part of the Picture. (After T. L. Macdonald.)
560 THE UMBILICUS AXD ITS DISEASES.
viewed by Dr. Weisef, only eight were extirpated. Xone of these was said to be large, and with one or more the history and result were lacking."
Dilated Urachus Treated by Incision and Drainage.* -The patient. W. J. P.. was a man aged fifty-four who consulted Dr. Pratt on June 8. 1889. complaining of pain and distention in the abdomen and increasing general weakness. He had been quite well until the previous November, when he complained of pain in the lower part of the abdomen. He remained in bed for three weeks and in the house for four months. He could not account for the onset of the trouble in any way. There had been no blow, no lifting of heavy weights, nor straining of any kind. His occupation was that of a store-keeper and clerk near Xew York, where he had lived for many years. He had had a gonorrheal infection when twenty-one. but had never had symptoms of syphilis. He had led a very intemperate life until seven or eight years previously. Since then he had been a moderate drinker. On examination the abdomen was found to be much distended in the lower half and in front : the distended area was dull on percussion and reached as high as three fmgerbreadths above the umbilicus. The pain extended as far as the pubes. laterally, on either side, as far as vertical lines drawn through the anterior superior iliac spines. He had no trouble with micturition or defecation. The urine appeared to be normal. The prostate was not enlarged, but there was a fulness of the left side of the pelvis.
On June 15. 1889. the tumor was aspirated and about one dram of a gummy, semitransparent fluid, which blocked the tube was withdrawn. It contained only a trace of albumin, but a large quantity of mucin, as shown by the precipitate it gave with acetic acid. Microscopically it showed many leukocytes.
On July 9th Mr. Bond made a four-inch median incision midway between the umbilicus and the pubes. After division of the linea alba a very thick membrane was reached, resembling a peritoneum much thickened by tubercular peritonitis. It proved, however, to be the outer wall of the cyst. It was divided, and a very large quantity of a ropy, gummy, semisolid material came away, of which over a gallon was measured. This had the appearance and consistence of semi-decolorized fibrin, was partly squeezed and partly drawn out in stringy layers. A considerable quantity was left in the cavity, as any attempt to sponge it off the inner surface of the cyst-wall left a red. raw surface which bled freely. On exploration of the cavity with the hand and arm it was found to extend upward to and beneath the liver and downward into the pelvis. The intestines could be made out behind and at the sides of the cyst, though shut off and separated from it. The peritoneal cavity was not opened. A Keith drainage-tube was placed in the wound, and reached to the floor of the pelvis. The rest of the incision was closed.
Chemical examination showed that there was only a trace of albumin, that the fluid was practically mucus and fibrin, with a large predominance of the former. Microscopic examination showed mucus-corpuscles and blood.
The cyst-walls shrank, and the patient gradually improved. In December 1889, on his departure for America, he seemed to be in good health, could walk nine miles at a stretch, and his appetite was excellent. There still remained, however, an irregular shaped cavity with thickened walls capable of holding half a pint of fluid. Mucoid material was secreted daily. The discharge, however, was not fetid and did not seem to in any way depress his health. In a letter dated February
- Pratt. R.. and Bond. C. J.: The Lancet. 1890, i, 898.
LARGE URACHAL CYSTS. 561
27, 1890, the patient said that the wound was still kept open by a glass tube, and that there was a discharge of clear, watery fluid, with very little of the jelly-like material. The man was in excellent health and was working thirteen hours a day.
A True Urachal Cyst. — Von Recklinghausen* demonstrated a cyst, about the size of a walnut, which had been removed from a man thirty years of age. The cyst varied from 1 to 3 cm. in diameter, and contained tenacious, colorless mucus. It was situated directly at the top of the bladder, with which it was intimately connected. It lay in the median line in the subperitoneal adipose tissue, and was completely cut off from the bladder. It was polycystic. There was a main cavity with many bays running off from it, and in addition to this there was a small cystic mass which was attached to the bladder, and which contained a labyrinth of microscopic spaces looking like gland loops, or, at any rate, like dilated crypts. The dense connective-tissue walls were nearly everywhere covered over with bundles of smooth muscle-fibers. The epithelium was several (or usually two) layers in thickness, and was definitely squamous in type. Here and there in the crypts were abundant numbers of goblet-cells. On account of the presence of goblet-cells it was necessary to consider the possibility of an enterocystoma; in other words, a derivative from the omphalomesenteric duct. But von Recklinghausen said that this could be excluded, because the tumor was entirely extraperitoneal and because it was in no way connected with the peritoneum.
Cyst of the Urachus. — Reedf cites a case (his Fig. 321) in which the sac had extended from near the ensif orm cartilage to the pubes and forced the viscera from their normal positions. The cyst was enucleated without any opening into the peritoneal cavity. He gives a schematic picture of the condition. Microscopic details are lacking.
Probably a Urachal Cyst.t — This case was also reported by Freer. A divinity student had from infancy been remarkable for his large abdomen, which had made him an object of ridicule to his companions. Thinking adipose tissue to be the cause, he had tried to reduce it by fasting, but without avail. It caused him no trouble until his twenty-fourth year, when a marked increase in size took place. This seriously impeded his respiration and led to an examination, which revealed fluctuation in and around the umbilical region. The dyspnea having increased to such a degree that relief became imperative, a puncture was made and a considerable. quantity of reddish-yellow fluid escaped. The procedure was followed by vomiting and intense abdominal pain. The puncture afforded him some relief, and with the exception of occasional fainting spells, his health remained good for a period of two years, after which his abdomen again commenced to increase in size, the dyspnea returned, and his general appearance became cachectic. He again entered the hospital and six liters of bloody fluid were withdrawn. The operation was repeated three times during the ensuing nine months — the remainder of his life. The amounts of fluid were 18^, 17, and 6 liters respectively. At his death he weighed about 192 pounds. At autopsy the contents of the cyst were found to amount to 50 liters, which weighed about 100 pounds. The cyst fluid contained
- Von Recklinghausen: Eine richtige TJrachuscyste. Deutsche med. Wochenschr., 1902,
xxviii, Vereinsbeilage, 266.
t Reed, Charles A. L.: A Text-Book of Gynecology, 1901, 805.
i Rippmann, G. : Eine serose Cyste in der Bauchhohle, mit einem Inhalt von 50 Liter Fliissigkeit. Deutsche Klinik, 1870, xxii, 267. 37
562 THE UMBILICUS AND ITS DISEASES.
cholesterin crystals, flat epithelium, and fat-droplets. A minute examination of the cyst-wall showed it to consist of three layers, the external being a serous coat. This rested on a layer composed of elastic and fibrous tissue, and the interior was lined with pavement epithelium. The bladder contained a little yellowish urine. It was contracted, and its lining mucous membrane was pale. The urachus was found closed at the bladder end. In its course toward the umbilicus below the commencement of the large cyst, a small cyst was situated near the umbilicus. The fibrous tissue passed into the subperitoneal coat of the larger cyst, which occupied almost the whole abdominal cavity, but the cyst was absolutely independent of the abdominal cavity and the abdominal organs were normal.
Probably a Urachal Cyst. — Schaad's* patient was a married woman thirty-two years of age. Nothing was known about the condition of the umbilicus at birth. She had had two normal labors. At the last labor a tumor had been noted below the umbilicus. The patient was supposed to have had a severe inflammation of the bowels seven years before. Several fingerbreadths below the umbilicus could be felt an elastic tumor the size of a child's head. It could be sharply outlined and pushed in all directions.
A cyst the size of a five-franc piece was found situated about two fingerbreadths below the umbilicus, and attached to the abdominal wall in the mid-line. It was separated from the peritoneum and drawn out of the abdomen. The omentum was tied off; the cyst was found adherent to the appendix. The left ovary was hard and atrophic; the right ovary was normal. The patient recovered.
The cyst was oval in form, and measured 7.5 x 6 x 4.5 cm. The walls varied from 2 to 4 mm. in thickness. The outer surface was fairly smooth, except where it was adherent. The inner surface resembled mucosa and was light yellow in color, with dark spots. On the right side of the cyst was a secondary cyst opening into the larger one. The opening was the size of a pin-head. The inner surface of this second cyst was smooth and yellow; its walls were 1 mm. thick. The large cyst contained about 200 c.c. of a chocolate-colored, cloudy, tenacious fluid, showing much cholesterin, detritus, fat-droplets, etc. The contents of the small cyst were similar in character, but thicker. The wall of the large cyst consisted of connective tissue and large quantities of smooth muscle arranged in bundles. These ran in all directions. The inner surface was lined with high cylindric epithelium; there were also glands opening upon the surface. In places the epithelium and glands were absent. The small cyst was lined with granulation tissue, in which were encountered giant-cells, some containing as many as 20 or 30 nuclei, arranged at the margin or irregularly scattered or in the center. [These are suggestive of foreign-body giant-cells.] Schaad felt sure that he was dealing with an omphalomesenteric duct, a portion of which had remained open, with a resulting retention cyst. [From the cases followed in the literature the case strongly suggests a urachal cyst. The question, however, is an open one. — T. S. C]
A C y s t i c Urachus. — Scholzf reports the case of a sixteen-year-old girl who complained of difficulty in micturition and a painful tumor in the abdomen. The abdomen was prominent, the largest measurement being between the umbilicus and symphysis. The tumor was very painful. On both sides there was tympany.
- Schaad, T.: Ueber die Exstirpation einer Cyste des Dotterganges. Correspondenzbl.
f. Schweizer Aerate, 1886, xvi, 345.
fScholz: Wien. med. Wbchenschr., 187S, xxviii, 1327.
LARGE URACHAL CYSTS. 563
After a time an opening, about the size of a hair, developed at the umbilicus, and fluid escaped from it. The opening was dilated and about 300 c.c. of colorless, transparent, thick, tenacious fluid escaped, and finally a thick yellow pus. The wound closed in the course of two months.
A Large Urachal Cyst.* — Case 1. — "This case was sent to me by Dr. Lamb, of Albrighton. She had complained of abdominal pain and tenderness, and in October, 1880, she began to suffer from somewhat serious symptoms, more particularly frequent vomiting and disinclination to take solid food. Some swelling in the lower part of the abdomen was noticed about the same time, this being then regarded as ascitic. The symptoms slowly increased in severity until February 11, 1881, when a consultation was held between Drs. Lamb, Heslop, and Saundby. As a result of this consultation she was tapped, and 10 pints of fluid were removed, although this was by no means the amount of fluid in the cavity, because large masses of flocculi obstructed the tube of the trocar and prevented the complete emptying of the cyst. Some of this fluid was submitted to me for an opinion, and from the fact that it was brown and thick and gave an abundant flaky yellow deposit, which consisted chiefly of pus, I unhesitatingly gave the opinion that it was not ascitic, but a fluid that must have been contained in some cyst cavit3 T , probably a cyst of the parovarium. I saw her on February 13th, when we found that the abdomen was quite as much distended as before the tapping. I therefore proposed an exploratory incision for the removal of the tumor, if it were possible to remove it, although the extremely exhausted condition of the patient gave no very great prospect of success. It was perfectly clear, however, that if let alone nothing but death could be the result, and therefore an operation was accepted by her attendants and relatives.
" I opened the abdomen at the usual site, and after cutting through all the layers except the peritoneum I came upon the cyst-wall. I opened the cyst and removed about 30 pints of fluid, exactly the same as that which had been removed at the tapping; and mixed up with it I found large masses of the fibrinous deposit, which accounted for the failure of the tapping to remove the whole of the fluid. I then proceeded to remove the enormous cyst, which was uniformly attached to the parietal wall on its outer aspect, and to the outer surface of the thickened peritoneum on its posterior aspect. The cyst did not dip into the pelvis at all, and the anterior parietal peritoneum did not reach the wall lower than the ensiform cartilage. The intestines and the pelvic organs could be felt through the anterior peritoneal fold, non-adherent, and, as far as could be determined, perfectly healthy. The cyst lay, therefore, entirely between the transversalis fascia on the outer side and the parietal peritoneum on the inner, the peritoneal cavity having been nowhere opened during the severe and protracted operation. The cyst was removed in its entirety, and its inner surface consisted of broken-down mucoid epithelium, infiltrated everywhere with pus, lying upon the basement membrane, wmich consisted almost entirely of muscular fibers.
"The conclusion concerning the nature of this cyst, at which I have arrived, is that it was developed from the urachus, a part of which had been occluded at both ends, but during the developmental changes of embryonic and infantile existence had not become obliterated. I entirely fail to see any other possible origin for it, and, if my explanation be correct, it is very marvelous that this structure should have re
- Tait, Lawson: Twelve Cases of Extraperitoneal Cysts. Brit. Gyn. Jour., 1886-87, ii, 32S.
564 THE UMBILICUS AND ITS DISEASES.
mained quiescent for fifty-six years and then should suddenly undergo an inflammatory change which developed it into this enormous cyst. The patient went on very well for three days, and then rapidly sank from exhaustion. No postmortem examination was allowed, and therefore I can shed no further light upon it; and, as far as I know, the observation is unique, although it is perfectly well known, as I myself have repeatedly had occasion to observe, that small cysts of the urachus are opened in abdominal section. I do not know that any such cyst has previously been met with sufficiently large to be of pathologic importance. It was noted and published at the time that the basement membrane of this cyst consisted almost entirely of muscular fiber, an observation which is absolutely concurrent with the examination of the cyst in Case X, made by Mr. Bland-Sutton."
Probably a Large Urachal Cyst.* — Case XI. — -"This case was sent to me by Dr. T. S. Bourne, of Kenilworth, as a case of acute inflammatory disease of the abdomen, of which he said: "I find it impossible to make an exact diagnosis." When I saw her I found her with a high pulse and temperature, and abdomen distended with a large quantity of free fluid. My opinion, expressed at the time, was that it was a case of tubercular peritonitis. I made the usual section, and found it another of these cases of congenital cysts belonging to the category of the cases already described in numbers IV, V, VI, VII, VIII, IX, and X. I removed a small piece of the cyst-wall for examination, and the reports of the microscopic examination by Dr. Arthur Johnstone and Mr. J. Bland-Sutton of Cases X and XI are annexed. I used the circular drainage method, and the patient has completely recovered. The following is Mr. Bland-Sutton's report:
"Sections of the cyst-wall exhibited under the microscope a mixture of fibrous and non-striated muscle tissue arranged in fasciculi, closely corresponding to the disposition of the bundles of tissue which make up the walls of the urinary bladderScattered throughout the whole thickness of the sections were small calcareous nodules. It was difficult to make out any definite epithelial investment to the sections, but on scraping the smooth surface of the specimen with a cover-glass, the field of the microscope became crowded with flattened, rounded, and pyriform cells, similar to those found lining the interior of the urinary bladder, only very much smaller.
"As the urachus is lined with epithelium agreeing in shape, and continuous with that found in the interior of the bladder, the evidence that these cysts are allantoic seems to me to be complete (J. Blancl-Sutton)."
[Tait cites a considerable amount of literature and discusses other cases at length. It is very difficult to tell in the majority of these cases whether he was right in his assumption or not. His entire paper, however, is a very interesting one.— T. S. C]
A Urachal Cyst. — Wolff | reports two cases which came under his observation in the clinic in Marburg in 1872, and which, according to his view, were urachal cysts. I shall here report only Wolff's Case I.
Mrs. K., aged thirty-one, was always healthy in childhood. Two years before her admission she noticed a tumor in the left side of the lower abdomen. This gradually increased. In March, 1872, there was a pregnancy which terminated nor
- Tait, Lawson: Loc. cit., Case xi.
f Wolff, C. C: Beitrag zur Lehre von den Urachuscysten. Inaug. Diss., Marburg, 1873.
LARGE URACHAL CYSTS. 565
mally, but was followed by an acute fever, with severe pain in the left part of the abdomen. The abdomen suddenly reached enormous proportions in a few days. The patient was treated by her physician for peritonitis. Convalescence was slow, but the patient again became quite strong. On palpation of the abdomen, a tense, elastic, fluctuant, rounded tumor could be felt. This filled the entire left side of the lower abdomen, and extended over to the right a handbreadth beyond the linea alba. Upward it reached beyond the umbilicus. The tumor could not be pushed from side to side. It had a smooth surface, and apparently consisted of one mass. A median incision was made, but the peritoneum did not become visible. After careful dissection the cyst was opened and yellowish, serum-like fluid escaped. The patient was laid on her side and the contents of the cyst gradually flowed out. After 5 liters of fluid had been removed in this way, the tumor was gradually loosened. The peritoneum was thickened, evidently as a result of inflammation. In the inner part of the cyst were large, lumpy coagula of fibrin. The connection of the cyst with the peritoneum was in part firm and in part very loose. The tumor was shelled out without difficulty. It was possible to do the operation almo.st entirely extraperitoneally; only at one point was the peritoneum opened for a distance of 1 cm. This was closed with silk. The patient made a good recovery.
The cyst was egg-shaped. Its largest circumference was 63 cm. When flattened out it was 31 cm. in breadth. The cyst-walls varied from 1 to 3 or 4 mm. in thickness. The outer surface was rough, with numerous string-like processes which indicated where the adhesions to the peritoneum had been cut. It had a poor blood-supply. The cyst-wall had a tough consistence. The interior of the cyst was smooth, like a serous wall. It had over its surface fibrinous deposits. According to Lieberkuhn, who made the histologic examination, the cyst-wall consisted of fine connective tissue with fibers running in various directions; here and there were non-striated muscle-fibers. A definite epithelium was not detected on the inner surface. The fluid consisted of large granular masses of detritus and pus-cells.
LITERATURE CONSULTED ON LARGE NON-INFECTED URACHAL CYSTS. Atlee, W. L.: Ovarian Tumors, Lippincott, Philadelphia, 1873, 50. Baldwin: Large Cyst of the Urachus. Surg., Gyn., and Obst., 1912, xiv, 636. Bantock: See Tait's article.
Bryant, T.: Discussion on Doran's paper, Brit. Med. Jour., 1898, i, 1390. Carroll, J. W.: Cystic Urachus. Buffalo Med. Jour., 1895-96, xxxv, 869. Cotte et Delore: Gros kyste de l'ouraque. Lyon med., 1905, cv, 373. Doran, A. H. G.: Urachal Cyst Simulating Appendicular Abscess; Arrested Development of
Genital Tract; with Notes on Recently Reported Cases of Urachal Cysts. The Lancet,
1909, i, 1304. Dossekker: Klin. Beitr. z. Lehre von den Urachuscysten. Beitrage z. klin. Chir., 1893, x, 102. Douglas, R.: Cysts of the Urachus. Trans. Amer. Assoc, of Obstet. and Gynecologists, 1897,
x, 177. Ferguson, E. D.: Cysts of the Urachus. Phila. Med. Jour., 1899, hi, 830. Ill, E. J.: Tumors of the Urachus. Trans. Amer. Assoc, of Obstet. and Gynecologists, 1892, v,
238.— Amer. Jour. Obstr., 1897, xxxvi, 568. Le Count, E. R. : Cyst of Urachus. Trans. Chicago Path. Soc, Dec, 1895, to April, 1897, ii, 215. Macdonald, T. L.: An Enormous Cyst of the Urachus. Annals of Surg., July-December, 1907,
xlvi, 230. Pratt and Bond: Dilated Urachus Treated by Incision and Drainage. The Lancet, 1890, i,
Von Recklinghausen: Eine richtige Urachuscyste. Deutsche med. Wochenschr., 1902, xxviii,
Vereinsbeilage, 266. Reed, C. A. L. : Cyst of the Urachus. A Text-Book of Gynecology, 1901, 805. Rippmann, G.: Eine serose Cyste in der Bauchhohle, mit einem Inhalt von 50 Liter Fliissigkeit.
Deutsche Klinik, 1870, xxii, 267. Schaad, T.: Ueber die Exstirpation einer Cyste des Dotterganges. Correspondenzbl. f. Schweizer
Aerzte, 1S86, xvi, 345. Scholz: Cystis urachi. Bericht des k. k. Allg. Krankenhauses, Wien, 1877 (quoted by Wutz) . Tait, L.: Twelve Cases of Extraperitoneal Cysts. Brit. Gyn. Jour., 1886-87, ii, 328. Weiser, W. R.: Cysts of the Urachus. Annals of Surg., 1908, xliv, 529. Wolff, C. G: Beitrag zur Lehre von den Urachuscysten. Inaug. Diss., Marburg, 1873. Wutz, J. B.: Ueber Urachus und Urachuscysten. Virchows Arch., 1883, xcii, 387.
Cullen TS. Embryology, anatomy, and diseases of the umbilicus together with diseases of the urachus. (1916) W. B. Saunders Company, Philadelphia And London.
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