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Idiopathic Segmental Ureteral Ectasia in a Congenital Solitary Kidney
THE JOURNAL OF UROLOGY
Vol. 69, No. 2, February 1953 Printed in U.S.A.
IDIOPATHIC SEGMENTAL URETERAL ECTASIA IN A CONGENITAL SOLITARY KIDNEY JOSEPH A. LAZARUS
AND
MARIO W. RICCI
In 1896 Saintu reported the first example of congenital idiopathic ureteral dilatation in a 73,f month old fetus, w1th hydronephrosis, relaxed atonic ureteric orifices. There was no evidence of infravesical obstruction. Since then several reports appeared in the literature of urology pertaining to the condition in newborn infants and children. Caulk, in 1923, was the first to use the term "megaloureter" in describing the condition of a patient with an enormously dilated ureter and a normal renal pelvis, probably caused by a primary anatomic defect in the ureteral wall. A careful review of the literature failed to reveal many examples of ureteral ectasia where the defect was limited to a single segment of the ureteral lumen without evidence of hydronephrosis. Morris (Surgical Diseases of the Kidney and Ureter) contributes an illustration of a saccular dilatation of the ureter directly below, but not involving, the renal pelvis (Guy's Hospital Museum, No. 1704). Several reports have appeared of congenital acquired megalo-ureter, associated with a permanent dilatation of the ureteric orifice and without a concomitant stricture, impacted calculus or other kind of obstruction. Papin and Legueu (1912) reported four additional examples of congenital hydro-ureter similar to that of Saintu. They maintained that before a condition can be classified as congenital hydronephrosis, three criteria were necessary: 1) extreme dilatation and immobility of the ureteric orifice; 2) absence of interference with the continuity of the renal pelvis and the urinary bladder, and 3) complete filling of the ureter and renal pelvis with opaque medium following the same technique for the bladder. Eisenstaedt (1926) reported two similar cases. ETIOLOGY
An attempt to explain the etiology of this condition requires a discussion of the term "megalo-ureter" and whethe,r conditions thus classified really belong in this category. For example, if we are to follow the criteria set by Papin and Legueu, it must be definitely established that no mechanical obstruction is present. This, at times, may be extremely difficult unless painstakingly sought, as shown by Chwalla (1927). He found a possible cause for a dilated ureter in a vestigial membrane which he believed could function valvularly. Vermooten was inclined to agree with Chwalla's view as a result of a critical study of a number of ureteropyelograms. According to Campbell (1948) the term "megalo-ureter," formerly used, designated two distinct varieties, namely: 1) the kind described by Saintu and elaborated by Papin and Legueu, and 2) the form described by Caulk. Theoretically, at least, the causes of this abnormality, as defined by Campbell, fall into three categories: 1) Obstruction. Although several theoretic varieties of occult infravesical obAccepted for publication October 5, 1951. 222
IDIOPA'rnrc SEGMENTAL URETERAL ECT,~SIA
223
struction have been mentioned, e.g., urethral. valves or vestigieal membrane (111:ac:VIyn), these pm;sibilities can hardly he accepted in vie-rl' of the absence of any visible obstruetive lesions in any of the reported cases, and also because of examples of unilateral hydro-ureter reported by Hilliard. In regard to (:m1ceiyable occult obstruction i1wolving the ureter itself, Bottomley attributed congenital ureteral ectasia to valve formation in the lmYer end of the ureter. Campbell, hmrnver, following a careful surgical search in four patients, failed to find any valvular formation or any evidence of stricture, as shown by Eisendrnth, following the removal of the terminal segment of ureter from two patients. But Campbell believes that the cause of obstruction in the intramural part of the ureter i::; an acute angulation, probably cau:,;ed by a dmvnward pull of the dilated juxtavesical ureter, vVe believe that in :,;eg;meutal ureteral ectasia some degree of obstruction occurs owing to a twist or kink in the nreteral wall occasioned by sagging of the dilated segment of ureter. The area of obstruction may be situated at the uppermost 01 lowermost part of the dilated segment. In the first instance, the ureteral catheter is frequently found coiled in the dilated segment of ureter; in the latter, it may be impossible or difficnlt to achanc:e the tip of the catheter into the dilated portion of the ureter. 2) Neurogenic. The pos::;ibility of impaired innervation of the ureter wa:,; suggested Thompson in a discussion of a report by Couvelaire. This assumption ,yas probably based on a spinal cord lesion which, in turn, ,rnuld result in au atonic bladder. Then again, if t.he assumption was a valid one, it would lead to bilateral ureteral ectasia. Bui, :-;ince such premises have not been mentioned in reported case::;, the possibility :,;eems remote. 3) Developmental arrest. According to embryonic studies of Gerard, and also of Loraine, fetal ureters up to the fourth and fifth months are enormously dilated. The first appearance of muscle fibers in the nreteral wall occurs in the third month of intra-uterine life, arising in the juxtavesical part of the ureter and extending upward to the pelvis. Completion of the muscular layer occurs approximately in the mid-fourth month. Full mtrncular development of the ureteral wal1 corresponds closely to the time ffhen the ureter is still in its dilated form. Au arrest in development of the muscular layer may result in ectasia of a segment 01· even of the entire length of ureter, and may also involve tho renal pelYit'. Another possibility is 1,he absence of normal inhibition of growth, ffhich normally sets in after the fifth month of embryonic life, and results in the perpetuation of the dilated nreteral lmnen throughout adult life. In my view this theory had definite merit as an explanation of the anomaly. Campbell, on the other hand, found normal deYelopmeut of the muscular coal, of the meter in all specimens taken from his five patients, and thereby concluded that arrested development of the muscular layer is not the eause of this anomaly He maintained that obstruction appeared to be the main cause of congenital megalo-ureter and that the term "congenital hydro-ureter" be reserved for conditions occasioned by obstruction, and "megalo-ureter" for examples presenting no evidence of obstntction.
224
JOSEPH A. LAZARUS AND MARIO W. RICCI
The relationship between megalo-ureter and infection has been noted by many observers. When the infection begins in the renal parenchyma or pelvis, ureteral ectasia proceeds from above downward. The distal end of the ureter thus retains its spindle form for long periods of time. Eventually it may act as additional impediment to drainage and lead to further ectasia of the already dilated ureteral lumen. While this development may be true in a large percentage of patients, it is probable that infection may be the effect rather than the cause of the ectasia. Owing to impairment or absence of peristalsis in these megalo-ureters, pooling or stagnation of urine occurs above the intramural part of the ureter. This in turn may serve as an excellent culture medium for bacteria from the kidney. SYMPTOMS
r
Symptoms directly attributable to ureteral ectasia are probably of little or no significance. However, in the event of superimposed infection or failure of the dilated ureters to empty into the bladder, symptoms may occur which are severe enough to warrant medical advice. The main symptoms, although not diagnostic, may be a sense of fullness in the loin or groin, increased urinary frequency, dysuria, fever and pyuria, the last-named becoming conspicuous with the onset of infection. To confirm the diagnosis of the anomaly it is necessary to resort to cystoscopic and pyelographic procedures. A rigid, and especially a gaping, ureteral orifice is not invariably noted. Inability to advance the ureteral catheter into the renal pelvis, and coiling of the catheter in a segment of ureter, may suggest the diagnosis. Urographic studies always confirm the diagnosis. Where there is a dilated, gaping and rigid ureteric orifice, reflux of the pyelographic medium from the bladder into the renal pelvis discloses the condition. When the ureteral orifice is unaffected and where sufficient renal parenchyma is present, the excretion of dye shows the defect clearly. Retrograde pyelography remains the only procedure in examples of complete parenchymal destruction and the presence of an apparently normal ureteral orifice. TREATMENT
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The treatment of choice for patients with unilateral involvement (when the remaining kidney is unaffected) is complete nephro-ureterectomy. In dealing with one who has a solitary kidney, acquired or congenital, or in whom the remaining kidney is impaired, the facilities available to the urologist are limited. When symptoms are absent or minimal, treatment may be unnecessary. Irrigation of the renal pelvis with a mild antiseptic solution and properly selected antibiotics may be tried when infection is present. Attempts have been made to transplant the affected ureter to another part of the bladder with the view of facilitating drainage. Plastic operations upon the affected ureter have been attempted with varying degrees of success, especially when ectasia is limited to a part of the ureter.
IDIOPATHIC SEG:VIENTAL UHETERAL ECTASIA
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PROGNOSIS
The prognosis of unilateral megalo-ureter in a patient with a normal kidney on the opposite side is good following nephro-ureterectomy. In examples of bilateral involvement or solitary kidney, the prognosis is poor, or at best should be guarded because little or nothing can be accomplished except in palliation to improve drainage and minimize infection.
Fm. 1. .11, left slmdowgraphy showing catheter coiled up in dilated lower segment o[ ureter. B, excretory urogram. "-:ote extreme degree of dilatation of lower third of ureter, with apparently normal pelvis and upper two-thirds of ureter CASE REPORT
Miss B. H., aged a school teacher, first came under my care 12 years ago, complaining of attacks of left renal colic and hematuria of 3 years' duration. Following a complete examination of the urogenital tract, a diagnosis was made of left renal calculus in a congenital solitary kidney. The lower third of ureter ,vas extremely dilated. On September 3, Hl38 the calculus was removed through a small incision in the pelvic wall. Convalescence was uneventful. One year later patient returned for re-examination, stating that she experienced occasional left lumbar pain but no colic. Left shadowgraphy showed catheter coiled up in dilated lower segment of ureter (fig 1, A). No recurrence of calculi. Phenolsulphonphthalein injected intravenously returned in good concentration from the kidney in 4 minutes, and urine specimen showed occasional leukocytes.
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JOSEPH A. LAZARUS AND MARIO W. RICCI
For the next 5 years the patient felt little or no discomfort in the kidney, but December 14, 1944 she returned, stating that she had hematuria, left lumbar pain and increased diurnal and nocturnal urinary frequency. Cystoscopy disclosed mild cystitis; the left ureteral orifice appeared normal and showed normal peristaltic action. Ureteral catheter passed easily, but on x-ray was found coiled in lower ureter. Excretory urography showed extreme degree of dilatation of lower third of ureter, with normally appearing renal pelvis (fig. 1, B). The pelvis was completely filled 5 minutes after administration of dye, but failed to empty 75 minutes later. Urinalysis, negative. Specimens failed to show organisms on smear and culture. Blood chemistry showed no abnormality. The patient return.ed 5 years later (April 22, 1949) for re-examination. She stated that, except for easy fatiguability, she had no urinary symptoms. Urologic survey showed the same conditions as were noted 5 years previously. September 13, 1949 the patient returned, stating that for past 2 weeks she suffered pain in left lumbar region and left lower quadrant. Urinalysis now showed 2 plus albumin and several leukocytes. The urine was sterile bacteriologically. On the morning of October 18, 1950 the patient experienced severe attack of renal colic. Urine was definitely turbid (pus). Cystoscopy again showed a normal ureteral orifice. Several attempts were made to pass a catheter to renal pelvis but each time the catheter coiled in the dilated lower segment of ureter. Phenolsulfonphthalein injected intravenously returned in good concentration in 12 minutes. The urine showed a growth of E. coli and B. pyocyaneus from the bladder, and E.coli from the kidney. The patient was given course of terramycin (250 mg. every 4 hours), and on November 14, 1950 a ureteral catheter was successfully passed into the renal pelvis, which was then irrigated with silver nitrate solution (1: 500). Subsequent attempts to carry out pelvic lavage were unsuccessful owing to coiling of the catheter in dilated portion of ureter. Operation (November 16, 1950): Under spinal anesthesia supplemented with sodium pentothal, cystoscopy was carried out. Two No. 6 ureteral catheters were passed into renal pelvis, following which lower ureter was exposed extraperitoneally through 6-inch Gibson incision. The lower third of ureter appeared saccular and approximated size of loop of small intestine. This ectatic segment of ureter extended from juxtavesical part to point approximately joining the middle and lower thirds of ureter. The lowermost part of dilated segment was so redundant that it overlapped juxtavesical ureter and resembled large diverticulum. The ureteric wall was greatly thickened. The mucosa appeared hemorrhagic. Several aberrant blood vessels extended from the hypogastric artery across the ureter. Adhesions bound the lower part of the ureter to surrounding structures, which were sheet-like and others like narrow bands (fig. 2). The involved ureter was freed by dividing adhesions by blunt and sharp dissection and by ligating and dividing aberrant vessels. This severance was carried down to and into wall of bladder. A large elliptical segment of redundant ureteral wall was now excised. The defect was then closed over indwelling catheters with two layers of running 5-0 chromic catgut, passed so as not to
IDIOPATHIC SEGMENTAL URETERAL ECTASIA
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include ureteral mucosa. The wound was closed in layers over large rubber darn and soft tube. The left kidney was then exposed through 6-inch Albarran incision carried through old scar, and a (20F) 5 cc bag catheter passed into the renal
Fm. 2. Fm. 3. Fm. 2. Schematic drawing: extent of urntcrnl dilatation, aberrant blood vessels and fibrous bands. Fm. 3. A, position of splinting ureteml catheters and Foley catheter in renal pelvis. B, line of excision of rndund,mt portion of diltited ureter. C, closure of ureter over splinting catheters.
I Fw. 4. Segment of ureter removed at operation
through small incision in the posterior ·wall of renal pelvis. The incision in the pelvic wall was closed around a catheter with two layers of ,5-0 chromic sutures. The wound was closed in layers over a rubber dam and a tube placed in the renal fossa. A Foley catheter was introduced into bladder (fig. :3)
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JOSEPH A. LAZARUS AND MARIO W. RICCI
The urethral catheter removed on eighth postsurgical day and the ureteral catheter on the fifteenth day. Convalescence was completely uneventful and the patient discharged from hospital on the sixteenth day, with pyelotomy tube in situ, to be removed following period of ureteral dilatation to assure patency of reconstructed portion of ureter. For the first 10 days following surgery the patient was given 600,000 units of aqueous solution of penicillin and 1 gm. streptomycin daily, and then 500 mg. terramycin daily for 6 days. Pathologist's report (F. D. Speer, M.D., New York Medical College, Flower and Fifth Avenue Hospital): "Gross specimen consists of one large mass of tissue measuring 6 by 3 by 0.8 cm. It appears thick and brown, but cuts with ease (fig. 4). "Microscopic: The ureteral wall is thick and shows subepithelial and intramuscular fibrosis which accounts for most of the thickening. There is slight muscular hypertrophy and minimal lymphocytic infiltration. Diagnosis: Marked subepithelial fibrosis and slight muscular hypertrophy." DISCUSSION
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The history presented deals with a patient with a congenital solitary kidney who underwent a nephrolithotomy for renal calculus 12 years previously. At the time of the first examination, the lower third of ureter was found to be dilated. Although this condition progressed during the intervening years, the upper two-thirds of the ureter and renal pelvis failed to disclose evidence of dilatation. Symptoms were few and mild until a few months prior to surgery, when infection set in, causing loin pain, pyuria and increased urinary frequency. The ureteral orifice was apparently normal in appearance and function. There was no difficulty in passing a catheter through the ureteral orifice into the lower segment of ureter. However, the catheter almost invariably coiled in the dilated segment of ureter and could not be introduced into the renal pelvis. The cause of the obstruction at the upper end of the dilated segment, we concluded, was a kink produced by downward sagging of the heavy-walled, dilated ureter at the area of junction with the normal portion, thereby displacing the lumen of the unaffected segment. In view of the fact that there was 1) no evident obstruction in the intramural part of ureter, 2) an obvious impediment to the free passage of a ureteral catheter into the normal part of the ureter, and 3) absence of dilatation above the ectatic part of the ureter, two problems presented themselves: What was the cause of the marked dilatation of the affected part of ureter? And, why did the upper ureter and pelvis remain unaffected? It seems probable that the condition under discussion falls into the group of megalo-ureters described by Caulk, rather than that analyzed by Saintu, because of the absence of any of the criteria laid down by Papin and Legueu. In regard to the cause of the dilatation, the only cogent explanation appears to be that it is a congenital weakness involving all layers of the wall of the involved segment of ureter. Inasmuch as the pathologic report indicates the unusual thickness of the ureteral wall was attributable to fibrosis, and that there was only a moderate
lDIOP."'-THIC SEGMENT)cL URETEHAL ECTASB.
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degree of muscular hypertrophy, the cause cannot be ascribed to de,·elopmental arrest of muscular development or to faulty irmervation, because in either ease the muscular layer would shmv atrophy rather than hypertrophy. In our vie,Y the high degree of fibrosis ,ms a secondary manifestation caused, most likely, frequent attacks of ureteritis which, in turn, induced stagnation of urine and subsequent infection. Another hypothesis which comes to mind is that the presence of aberrant yessels may have been instrumental in preventing the affected segment of ureter from elongating, \\·ith the result that it retained its fetal state of enlargement. In explanation of the absence of ectasia of the upper ureter, it cau be postulated that a kink produced sagging of the dilated part of the ureter may have produced a valvular Yariety of obstruction ·which, while not preventing the free passage of urine from the renal pelvis into the dilated part of ureter, could have impeded regurgitation of urine back into the renal pelvis, thereby ob,·iating dilatation. The problem involved in treating patients with segmental megalo-ureter, particularly whe11 associated 1Yith a solitary kidney, is a difficult one, and its solution must of depend upon certain conditions. In the absence of symptoms, treatment is not warranted. 1Vhen they occur and are sufficiently severe to require serious consideration, they are usually attributable to colic:, infection, or a combination of both. Frequently patients falling into this category respond to conservative measures, such as the use of antibiotics and ureteral drainage. In patients with seyere symptoms and in those ,Yhere functional integrity of the renal parenchyma is in jeopardy, more radical measure" must be resorted to. '\Vhen only a short segment of ureter is involved, it may be feasible to excise it and reimplant the proximal end into another part of the urinary bladder. As a rule, hmYeYer, the ectasia involves too much of the ureter to ,,-arrant this procedure. It may then only be necessary to resort to other measures, such as 1) pyelostomy, 2) uretero-intestinal implantation, 3) ureteror:utaneous implantation, or 4) plastic procedures. Of the aforementioned measures, one which requires most serious consideration is ureteroplasty. ·while not completely devoid of hazard, it has the great advantages of establishing pelviovesical continuity with the smallest risk of subsequent infection, and avoiding the Yexation~physical and psychologic--uf carrying cumbersome appliances. In order to accomplish a proper plastic: operation, especially when dealing ,,,ith the lowermost part of the ureter, it i:-i desirable to mobilize it completely. In so doing, hmYever, it is almost impossible to preserve the extrinsic vascular supply of the mobilized ureter. While the intrinsic yascularity is usually adequate, it is possible, at times, that necrosis may occnr and the ureter become completely occluded as a result of fibrosis. For thi:,; reason it is desirable, when performing a tangential resection of the ec:tatic segment of ureter, to splint it with one or two large catheters which should be left in place and to supplement the procedure with a pyelostomy for two to three The pyelostomy tube should remain in place until subsequent cystoscopic examinations show complete patency of the reconstructed ureter. This may require
230
JOSEPH A. LAZARUS AND MARIO W. RICCI
the wearing of a pyelostomy tube for several weeks or months, and carrying out ureteral dilatation at least for one year to accomplish ureteral patency. SUMMARY AND CONCLUSIONS
Idiopathic megalo-ureter is an unusual anomaly and falls into two categories: 1) the ureter solely is ectatic and the renal pelvis normal, a condition first de-
scribed by Caulk; and 2) the ureter and renal pelvis are involved (most common variety). In the latter the ureteral orifice is patulous and there is absence of peristalsis, permitting free reflux from the bladder to the renal pelvis. Although some authorities have reported, after careful examination, the discovery of a valvular obstruction in the ureter or urethra, this has not been generally confirmed. Several theories have been advanced to explain the anomaly, but none is entirely satisfactory. The condition may be unilateral or bilateral. The lesion rarely involves a segment of one ureter. Symptoms may be entirely lacking. If present they are usually caused by superimposed infection, or to pain resulting from obstruction owing to impairment of drainage. Diagnosis is made by urography. Treatment depends upon: 1) presence or absence of debilitating conditions; 2) unilateral or bilateral lesion; 3) jeopardy to vital renal parenchyma. Treatment is not indicated in the absence of severe symptoms. Antibiotics and ureteral catheter drainage usually suffice to relieve symptoms consequent upon infection and impaired drainage. When surgical intervention becomes urgent, nephro-ureterectomy is the procedure of choice if the involvement is unilateral and the remaining kidney shows adequate function. For patients with bilateral involvement, or when dealing with a lesion in a solitary kidney, the choice of procedures is: 1) permanent pyelostomy; 2) uretero-intestinal anastomosis; 3) cutaneous ureterostomy; 4) excision of involved segment of ureter and re-implantation of the remaining ureter into another part of the bladder (a procedure rarely possible), and 5) ureteroplasty. The hazards, advantages and technical details involved in accomplishment of ureteroplasty are described. Segmental ectasia of the ureter in a patient with a congenital solitary kidney successfully treated by ureteroplasty is reported.
875 Park Ave., New York 21, N. Y. REFERENCES BOTTOMLEY, J. T.: Ann. Surg., 2: 597, 1910. CAMPBELL, E.W.: J. Urol., 60: 31, 1948. CAULK, J. R.: J. Urol., 9: 315, 1923. CHWALLA, R.: Urol. & Cutan. Rev., 31: 499, 1927. CouvELAIRE, A.: Bull. Soc. Anat., de Paris, 2: 287, 1900. EisENDRATH, D. N.: Ann. Surg., 65: 552, 1917. EisENSTAEDT, J. S.: Arch. Surg., 13: 1, 1926. GERARD, M.: These de Paris. HILLIARD, C.: Brit. Med. J., 2: 838, 1935. LORAINE, A.: These de Paris, 30: 1921. MAcMYN, D. J.: Brit. J. Urol., 1: 150, 1929. MORRIS, H.: Surgical Diseases of the Kidney and Ureter. Cassell & Co., Ltd., 1904, vol. 2, p. 299. PAPIN, E. AND LEGUEU, F.: Arch. Urol. de la Clin. de Necker, vol. 1, #4. SAINTU, 0.: J. med. de Paris, 8: 332, 1896. VERMOOTEN, V.: J. Urol., 23: 427, 1930; J. Urol., 41: 455, 1939.
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Vol. 69, No. 2, February 1953 Printed in U.S.A.
IDIOPATHIC SEGMENTAL URETERAL ECTASIA IN A CONGENITAL SOLITARY KIDNEY JOSEPH A. LAZARUS
AND
MARIO W. RICCI
In 1896 Saintu reported the first example of congenital idiopathic ureteral dilatation in a 73,f month old fetus, w1th hydronephrosis, relaxed atonic ureteric orifices. There was no evidence of infravesical obstruction. Since then several reports appeared in the literature of urology pertaining to the condition in newborn infants and children. Caulk, in 1923, was the first to use the term "megaloureter" in describing the condition of a patient with an enormously dilated ureter and a normal renal pelvis, probably caused by a primary anatomic defect in the ureteral wall. A careful review of the literature failed to reveal many examples of ureteral ectasia where the defect was limited to a single segment of the ureteral lumen without evidence of hydronephrosis. Morris (Surgical Diseases of the Kidney and Ureter) contributes an illustration of a saccular dilatation of the ureter directly below, but not involving, the renal pelvis (Guy's Hospital Museum, No. 1704). Several reports have appeared of congenital acquired megalo-ureter, associated with a permanent dilatation of the ureteric orifice and without a concomitant stricture, impacted calculus or other kind of obstruction. Papin and Legueu (1912) reported four additional examples of congenital hydro-ureter similar to that of Saintu. They maintained that before a condition can be classified as congenital hydronephrosis, three criteria were necessary: 1) extreme dilatation and immobility of the ureteric orifice; 2) absence of interference with the continuity of the renal pelvis and the urinary bladder, and 3) complete filling of the ureter and renal pelvis with opaque medium following the same technique for the bladder. Eisenstaedt (1926) reported two similar cases. ETIOLOGY
An attempt to explain the etiology of this condition requires a discussion of the term "megalo-ureter" and whethe,r conditions thus classified really belong in this category. For example, if we are to follow the criteria set by Papin and Legueu, it must be definitely established that no mechanical obstruction is present. This, at times, may be extremely difficult unless painstakingly sought, as shown by Chwalla (1927). He found a possible cause for a dilated ureter in a vestigial membrane which he believed could function valvularly. Vermooten was inclined to agree with Chwalla's view as a result of a critical study of a number of ureteropyelograms. According to Campbell (1948) the term "megalo-ureter," formerly used, designated two distinct varieties, namely: 1) the kind described by Saintu and elaborated by Papin and Legueu, and 2) the form described by Caulk. Theoretically, at least, the causes of this abnormality, as defined by Campbell, fall into three categories: 1) Obstruction. Although several theoretic varieties of occult infravesical obAccepted for publication October 5, 1951. 222
IDIOPA'rnrc SEGMENTAL URETERAL ECT,~SIA
223
struction have been mentioned, e.g., urethral. valves or vestigieal membrane (111:ac:VIyn), these pm;sibilities can hardly he accepted in vie-rl' of the absence of any visible obstruetive lesions in any of the reported cases, and also because of examples of unilateral hydro-ureter reported by Hilliard. In regard to (:m1ceiyable occult obstruction i1wolving the ureter itself, Bottomley attributed congenital ureteral ectasia to valve formation in the lmYer end of the ureter. Campbell, hmrnver, following a careful surgical search in four patients, failed to find any valvular formation or any evidence of stricture, as shown by Eisendrnth, following the removal of the terminal segment of ureter from two patients. But Campbell believes that the cause of obstruction in the intramural part of the ureter i::; an acute angulation, probably cau:,;ed by a dmvnward pull of the dilated juxtavesical ureter, vVe believe that in :,;eg;meutal ureteral ectasia some degree of obstruction occurs owing to a twist or kink in the nreteral wall occasioned by sagging of the dilated segment of ureter. The area of obstruction may be situated at the uppermost 01 lowermost part of the dilated segment. In the first instance, the ureteral catheter is frequently found coiled in the dilated segment of ureter; in the latter, it may be impossible or difficnlt to achanc:e the tip of the catheter into the dilated portion of the ureter. 2) Neurogenic. The pos::;ibility of impaired innervation of the ureter wa:,; suggested Thompson in a discussion of a report by Couvelaire. This assumption ,yas probably based on a spinal cord lesion which, in turn, ,rnuld result in au atonic bladder. Then again, if t.he assumption was a valid one, it would lead to bilateral ureteral ectasia. Bui, :-;ince such premises have not been mentioned in reported case::;, the possibility :,;eems remote. 3) Developmental arrest. According to embryonic studies of Gerard, and also of Loraine, fetal ureters up to the fourth and fifth months are enormously dilated. The first appearance of muscle fibers in the nreteral wall occurs in the third month of intra-uterine life, arising in the juxtavesical part of the ureter and extending upward to the pelvis. Completion of the muscular layer occurs approximately in the mid-fourth month. Full mtrncular development of the ureteral wal1 corresponds closely to the time ffhen the ureter is still in its dilated form. Au arrest in development of the muscular layer may result in ectasia of a segment 01· even of the entire length of ureter, and may also involve tho renal pelYit'. Another possibility is 1,he absence of normal inhibition of growth, ffhich normally sets in after the fifth month of embryonic life, and results in the perpetuation of the dilated nreteral lmnen throughout adult life. In my view this theory had definite merit as an explanation of the anomaly. Campbell, on the other hand, found normal deYelopmeut of the muscular coal, of the meter in all specimens taken from his five patients, and thereby concluded that arrested development of the muscular layer is not the eause of this anomaly He maintained that obstruction appeared to be the main cause of congenital megalo-ureter and that the term "congenital hydro-ureter" be reserved for conditions occasioned by obstruction, and "megalo-ureter" for examples presenting no evidence of obstntction.
224
JOSEPH A. LAZARUS AND MARIO W. RICCI
The relationship between megalo-ureter and infection has been noted by many observers. When the infection begins in the renal parenchyma or pelvis, ureteral ectasia proceeds from above downward. The distal end of the ureter thus retains its spindle form for long periods of time. Eventually it may act as additional impediment to drainage and lead to further ectasia of the already dilated ureteral lumen. While this development may be true in a large percentage of patients, it is probable that infection may be the effect rather than the cause of the ectasia. Owing to impairment or absence of peristalsis in these megalo-ureters, pooling or stagnation of urine occurs above the intramural part of the ureter. This in turn may serve as an excellent culture medium for bacteria from the kidney. SYMPTOMS
r
Symptoms directly attributable to ureteral ectasia are probably of little or no significance. However, in the event of superimposed infection or failure of the dilated ureters to empty into the bladder, symptoms may occur which are severe enough to warrant medical advice. The main symptoms, although not diagnostic, may be a sense of fullness in the loin or groin, increased urinary frequency, dysuria, fever and pyuria, the last-named becoming conspicuous with the onset of infection. To confirm the diagnosis of the anomaly it is necessary to resort to cystoscopic and pyelographic procedures. A rigid, and especially a gaping, ureteral orifice is not invariably noted. Inability to advance the ureteral catheter into the renal pelvis, and coiling of the catheter in a segment of ureter, may suggest the diagnosis. Urographic studies always confirm the diagnosis. Where there is a dilated, gaping and rigid ureteric orifice, reflux of the pyelographic medium from the bladder into the renal pelvis discloses the condition. When the ureteral orifice is unaffected and where sufficient renal parenchyma is present, the excretion of dye shows the defect clearly. Retrograde pyelography remains the only procedure in examples of complete parenchymal destruction and the presence of an apparently normal ureteral orifice. TREATMENT
r
The treatment of choice for patients with unilateral involvement (when the remaining kidney is unaffected) is complete nephro-ureterectomy. In dealing with one who has a solitary kidney, acquired or congenital, or in whom the remaining kidney is impaired, the facilities available to the urologist are limited. When symptoms are absent or minimal, treatment may be unnecessary. Irrigation of the renal pelvis with a mild antiseptic solution and properly selected antibiotics may be tried when infection is present. Attempts have been made to transplant the affected ureter to another part of the bladder with the view of facilitating drainage. Plastic operations upon the affected ureter have been attempted with varying degrees of success, especially when ectasia is limited to a part of the ureter.
IDIOPATHIC SEG:VIENTAL UHETERAL ECTASIA
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PROGNOSIS
The prognosis of unilateral megalo-ureter in a patient with a normal kidney on the opposite side is good following nephro-ureterectomy. In examples of bilateral involvement or solitary kidney, the prognosis is poor, or at best should be guarded because little or nothing can be accomplished except in palliation to improve drainage and minimize infection.
Fm. 1. .11, left slmdowgraphy showing catheter coiled up in dilated lower segment o[ ureter. B, excretory urogram. "-:ote extreme degree of dilatation of lower third of ureter, with apparently normal pelvis and upper two-thirds of ureter CASE REPORT
Miss B. H., aged a school teacher, first came under my care 12 years ago, complaining of attacks of left renal colic and hematuria of 3 years' duration. Following a complete examination of the urogenital tract, a diagnosis was made of left renal calculus in a congenital solitary kidney. The lower third of ureter ,vas extremely dilated. On September 3, Hl38 the calculus was removed through a small incision in the pelvic wall. Convalescence was uneventful. One year later patient returned for re-examination, stating that she experienced occasional left lumbar pain but no colic. Left shadowgraphy showed catheter coiled up in dilated lower segment of ureter (fig 1, A). No recurrence of calculi. Phenolsulphonphthalein injected intravenously returned in good concentration from the kidney in 4 minutes, and urine specimen showed occasional leukocytes.
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For the next 5 years the patient felt little or no discomfort in the kidney, but December 14, 1944 she returned, stating that she had hematuria, left lumbar pain and increased diurnal and nocturnal urinary frequency. Cystoscopy disclosed mild cystitis; the left ureteral orifice appeared normal and showed normal peristaltic action. Ureteral catheter passed easily, but on x-ray was found coiled in lower ureter. Excretory urography showed extreme degree of dilatation of lower third of ureter, with normally appearing renal pelvis (fig. 1, B). The pelvis was completely filled 5 minutes after administration of dye, but failed to empty 75 minutes later. Urinalysis, negative. Specimens failed to show organisms on smear and culture. Blood chemistry showed no abnormality. The patient return.ed 5 years later (April 22, 1949) for re-examination. She stated that, except for easy fatiguability, she had no urinary symptoms. Urologic survey showed the same conditions as were noted 5 years previously. September 13, 1949 the patient returned, stating that for past 2 weeks she suffered pain in left lumbar region and left lower quadrant. Urinalysis now showed 2 plus albumin and several leukocytes. The urine was sterile bacteriologically. On the morning of October 18, 1950 the patient experienced severe attack of renal colic. Urine was definitely turbid (pus). Cystoscopy again showed a normal ureteral orifice. Several attempts were made to pass a catheter to renal pelvis but each time the catheter coiled in the dilated lower segment of ureter. Phenolsulfonphthalein injected intravenously returned in good concentration in 12 minutes. The urine showed a growth of E. coli and B. pyocyaneus from the bladder, and E.coli from the kidney. The patient was given course of terramycin (250 mg. every 4 hours), and on November 14, 1950 a ureteral catheter was successfully passed into the renal pelvis, which was then irrigated with silver nitrate solution (1: 500). Subsequent attempts to carry out pelvic lavage were unsuccessful owing to coiling of the catheter in dilated portion of ureter. Operation (November 16, 1950): Under spinal anesthesia supplemented with sodium pentothal, cystoscopy was carried out. Two No. 6 ureteral catheters were passed into renal pelvis, following which lower ureter was exposed extraperitoneally through 6-inch Gibson incision. The lower third of ureter appeared saccular and approximated size of loop of small intestine. This ectatic segment of ureter extended from juxtavesical part to point approximately joining the middle and lower thirds of ureter. The lowermost part of dilated segment was so redundant that it overlapped juxtavesical ureter and resembled large diverticulum. The ureteric wall was greatly thickened. The mucosa appeared hemorrhagic. Several aberrant blood vessels extended from the hypogastric artery across the ureter. Adhesions bound the lower part of the ureter to surrounding structures, which were sheet-like and others like narrow bands (fig. 2). The involved ureter was freed by dividing adhesions by blunt and sharp dissection and by ligating and dividing aberrant vessels. This severance was carried down to and into wall of bladder. A large elliptical segment of redundant ureteral wall was now excised. The defect was then closed over indwelling catheters with two layers of running 5-0 chromic catgut, passed so as not to
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include ureteral mucosa. The wound was closed in layers over large rubber darn and soft tube. The left kidney was then exposed through 6-inch Albarran incision carried through old scar, and a (20F) 5 cc bag catheter passed into the renal
Fm. 2. Fm. 3. Fm. 2. Schematic drawing: extent of urntcrnl dilatation, aberrant blood vessels and fibrous bands. Fm. 3. A, position of splinting ureteml catheters and Foley catheter in renal pelvis. B, line of excision of rndund,mt portion of diltited ureter. C, closure of ureter over splinting catheters.
I Fw. 4. Segment of ureter removed at operation
through small incision in the posterior ·wall of renal pelvis. The incision in the pelvic wall was closed around a catheter with two layers of ,5-0 chromic sutures. The wound was closed in layers over a rubber dam and a tube placed in the renal fossa. A Foley catheter was introduced into bladder (fig. :3)
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The urethral catheter removed on eighth postsurgical day and the ureteral catheter on the fifteenth day. Convalescence was completely uneventful and the patient discharged from hospital on the sixteenth day, with pyelotomy tube in situ, to be removed following period of ureteral dilatation to assure patency of reconstructed portion of ureter. For the first 10 days following surgery the patient was given 600,000 units of aqueous solution of penicillin and 1 gm. streptomycin daily, and then 500 mg. terramycin daily for 6 days. Pathologist's report (F. D. Speer, M.D., New York Medical College, Flower and Fifth Avenue Hospital): "Gross specimen consists of one large mass of tissue measuring 6 by 3 by 0.8 cm. It appears thick and brown, but cuts with ease (fig. 4). "Microscopic: The ureteral wall is thick and shows subepithelial and intramuscular fibrosis which accounts for most of the thickening. There is slight muscular hypertrophy and minimal lymphocytic infiltration. Diagnosis: Marked subepithelial fibrosis and slight muscular hypertrophy." DISCUSSION
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The history presented deals with a patient with a congenital solitary kidney who underwent a nephrolithotomy for renal calculus 12 years previously. At the time of the first examination, the lower third of ureter was found to be dilated. Although this condition progressed during the intervening years, the upper two-thirds of the ureter and renal pelvis failed to disclose evidence of dilatation. Symptoms were few and mild until a few months prior to surgery, when infection set in, causing loin pain, pyuria and increased urinary frequency. The ureteral orifice was apparently normal in appearance and function. There was no difficulty in passing a catheter through the ureteral orifice into the lower segment of ureter. However, the catheter almost invariably coiled in the dilated segment of ureter and could not be introduced into the renal pelvis. The cause of the obstruction at the upper end of the dilated segment, we concluded, was a kink produced by downward sagging of the heavy-walled, dilated ureter at the area of junction with the normal portion, thereby displacing the lumen of the unaffected segment. In view of the fact that there was 1) no evident obstruction in the intramural part of ureter, 2) an obvious impediment to the free passage of a ureteral catheter into the normal part of the ureter, and 3) absence of dilatation above the ectatic part of the ureter, two problems presented themselves: What was the cause of the marked dilatation of the affected part of ureter? And, why did the upper ureter and pelvis remain unaffected? It seems probable that the condition under discussion falls into the group of megalo-ureters described by Caulk, rather than that analyzed by Saintu, because of the absence of any of the criteria laid down by Papin and Legueu. In regard to the cause of the dilatation, the only cogent explanation appears to be that it is a congenital weakness involving all layers of the wall of the involved segment of ureter. Inasmuch as the pathologic report indicates the unusual thickness of the ureteral wall was attributable to fibrosis, and that there was only a moderate
lDIOP."'-THIC SEGMENT)cL URETEHAL ECTASB.
229
degree of muscular hypertrophy, the cause cannot be ascribed to de,·elopmental arrest of muscular development or to faulty irmervation, because in either ease the muscular layer would shmv atrophy rather than hypertrophy. In our vie,Y the high degree of fibrosis ,ms a secondary manifestation caused, most likely, frequent attacks of ureteritis which, in turn, induced stagnation of urine and subsequent infection. Another hypothesis which comes to mind is that the presence of aberrant yessels may have been instrumental in preventing the affected segment of ureter from elongating, \\·ith the result that it retained its fetal state of enlargement. In explanation of the absence of ectasia of the upper ureter, it cau be postulated that a kink produced sagging of the dilated part of the ureter may have produced a valvular Yariety of obstruction ·which, while not preventing the free passage of urine from the renal pelvis into the dilated part of ureter, could have impeded regurgitation of urine back into the renal pelvis, thereby ob,·iating dilatation. The problem involved in treating patients with segmental megalo-ureter, particularly whe11 associated 1Yith a solitary kidney, is a difficult one, and its solution must of depend upon certain conditions. In the absence of symptoms, treatment is not warranted. 1Vhen they occur and are sufficiently severe to require serious consideration, they are usually attributable to colic:, infection, or a combination of both. Frequently patients falling into this category respond to conservative measures, such as the use of antibiotics and ureteral drainage. In patients with seyere symptoms and in those ,Yhere functional integrity of the renal parenchyma is in jeopardy, more radical measure" must be resorted to. '\Vhen only a short segment of ureter is involved, it may be feasible to excise it and reimplant the proximal end into another part of the urinary bladder. As a rule, hmYeYer, the ectasia involves too much of the ureter to ,,-arrant this procedure. It may then only be necessary to resort to other measures, such as 1) pyelostomy, 2) uretero-intestinal implantation, 3) ureteror:utaneous implantation, or 4) plastic procedures. Of the aforementioned measures, one which requires most serious consideration is ureteroplasty. ·while not completely devoid of hazard, it has the great advantages of establishing pelviovesical continuity with the smallest risk of subsequent infection, and avoiding the Yexation~physical and psychologic--uf carrying cumbersome appliances. In order to accomplish a proper plastic: operation, especially when dealing ,,,ith the lowermost part of the ureter, it i:-i desirable to mobilize it completely. In so doing, hmYever, it is almost impossible to preserve the extrinsic vascular supply of the mobilized ureter. While the intrinsic yascularity is usually adequate, it is possible, at times, that necrosis may occnr and the ureter become completely occluded as a result of fibrosis. For thi:,; reason it is desirable, when performing a tangential resection of the ec:tatic segment of ureter, to splint it with one or two large catheters which should be left in place and to supplement the procedure with a pyelostomy for two to three The pyelostomy tube should remain in place until subsequent cystoscopic examinations show complete patency of the reconstructed ureter. This may require
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the wearing of a pyelostomy tube for several weeks or months, and carrying out ureteral dilatation at least for one year to accomplish ureteral patency. SUMMARY AND CONCLUSIONS
Idiopathic megalo-ureter is an unusual anomaly and falls into two categories: 1) the ureter solely is ectatic and the renal pelvis normal, a condition first de-
scribed by Caulk; and 2) the ureter and renal pelvis are involved (most common variety). In the latter the ureteral orifice is patulous and there is absence of peristalsis, permitting free reflux from the bladder to the renal pelvis. Although some authorities have reported, after careful examination, the discovery of a valvular obstruction in the ureter or urethra, this has not been generally confirmed. Several theories have been advanced to explain the anomaly, but none is entirely satisfactory. The condition may be unilateral or bilateral. The lesion rarely involves a segment of one ureter. Symptoms may be entirely lacking. If present they are usually caused by superimposed infection, or to pain resulting from obstruction owing to impairment of drainage. Diagnosis is made by urography. Treatment depends upon: 1) presence or absence of debilitating conditions; 2) unilateral or bilateral lesion; 3) jeopardy to vital renal parenchyma. Treatment is not indicated in the absence of severe symptoms. Antibiotics and ureteral catheter drainage usually suffice to relieve symptoms consequent upon infection and impaired drainage. When surgical intervention becomes urgent, nephro-ureterectomy is the procedure of choice if the involvement is unilateral and the remaining kidney shows adequate function. For patients with bilateral involvement, or when dealing with a lesion in a solitary kidney, the choice of procedures is: 1) permanent pyelostomy; 2) uretero-intestinal anastomosis; 3) cutaneous ureterostomy; 4) excision of involved segment of ureter and re-implantation of the remaining ureter into another part of the bladder (a procedure rarely possible), and 5) ureteroplasty. The hazards, advantages and technical details involved in accomplishment of ureteroplasty are described. Segmental ectasia of the ureter in a patient with a congenital solitary kidney successfully treated by ureteroplasty is reported.
875 Park Ave., New York 21, N. Y. REFERENCES BOTTOMLEY, J. T.: Ann. Surg., 2: 597, 1910. CAMPBELL, E.W.: J. Urol., 60: 31, 1948. CAULK, J. R.: J. Urol., 9: 315, 1923. CHWALLA, R.: Urol. & Cutan. Rev., 31: 499, 1927. CouvELAIRE, A.: Bull. Soc. Anat., de Paris, 2: 287, 1900. EisENDRATH, D. N.: Ann. Surg., 65: 552, 1917. EisENSTAEDT, J. S.: Arch. Surg., 13: 1, 1926. GERARD, M.: These de Paris. HILLIARD, C.: Brit. Med. J., 2: 838, 1935. LORAINE, A.: These de Paris, 30: 1921. MAcMYN, D. J.: Brit. J. Urol., 1: 150, 1929. MORRIS, H.: Surgical Diseases of the Kidney and Ureter. Cassell & Co., Ltd., 1904, vol. 2, p. 299. PAPIN, E. AND LEGUEU, F.: Arch. Urol. de la Clin. de Necker, vol. 1, #4. SAINTU, 0.: J. med. de Paris, 8: 332, 1896. VERMOOTEN, V.: J. Urol., 23: 427, 1930; J. Urol., 41: 455, 1939.
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