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Ovarian syndrome: Is it a myth?
REVIEW ARTICLE
OVARIAN PETER
SYNDROME:
DURE-SMITH,
M.B.,
IS IT A MYTH? F.R.C.R.
From the Department of Radiology, Division of Uroradiology, University of California, Irvine Medical Center, Orange, California
In 1964Clark’ published details of 130 cases of the right ovarian vein syndrome in the second edition of Emmett’s Clinical Urogruphy. This appears to have given the seal of respectability to an entity which had previously been offered somewhat tentatively as an occasional case report. Yet a review of the literature on this syndrome, which is not extensive, is disquietingly unconvincing. The diagnosis appears to devolve on two assumptions: (1) that the demonstration of upper ureteric dilatation, the essential feature of this syndrome, is secondary to obstruction; and (2) that demonstration of a dilated ovarian vein or veins, at or near the site where the ureteric dilatation ends, is the cause of the obstruction. Neither of these assumptions appears likely to be true. Although today a good deal of skepticism is still evident when this syndrome is discussed, many accept it as an established entity and a number of women are subjected to surgical cure. For this reason alone it appears justifiable to examine the evidence for and against the existence of this syndrome and whether or not it is possible with today’s expertise in urodynamics to settle the issue.
Embryologic
and anatomic
considerations’,2*‘5-19
In its passage from the pelvic region to its definitive lumbar position the metanephros passes through a ring of embryonic venous channels (Fig. 1). Persistence ofthe right posterior cardinal vein as the adult vena cava results in
Ovarian Vein Syndrome’-l4 Definition
Clark defined the right ovarian vein syndrome as an aberrant ovarian vein causing right ureteral obstruction occurring most frequently during pregnancy and persisting following pregnancy. The symptoms were related primarily to the development of acute pyelonephritis occurring in the obstructed ureter with pain, chills, fever, pyuria, and tenderness over the right costovertebral angle. Without infection, symptoms consisted of backache and pain in the right lower quadrant. Frequently symptoms occur during the days preceding the onset of menstruation.
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FIGURE 1. In its passage from pelvic region to its definitive lumbar position metanephros passes through ring of embryonic venous channels.
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FIGURE 2. Normal anatomy of ovarian vessels. (Reproduced the Ciba Collection-Reproductive System, Netter.)
the well-recorded entity of retrocaval ureter. The ovarian veins normally develop from the subcardinal veins. Persistence of anastomoses from any of the three systems will determine the number and relationship of the vein or veins to the definitive ureter. A typical arrangement of the ovarian vessels is depicted in Figure 2. The veins start as a plexus around the ovary, similar to the pampiniform plexus around the testis in the male, and communicate by one or more large veins with the uterine venous plexus.
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by permission,
One, two, or more veins ascend to reach the pelvic brim. Here the ovarian vessels (artery and vein) usually lie lateral or anterolateral to the ureter and do not cross the ureter until about L3 or L4 level (Fig. 3, arrow). It is not unusual to see an oblique indentation of the ureter at this level during excretory urography (Fig. 3), particularly in multiparous women. It is important to note that ureteric dilatation in the ovarian vein syndrome does not end where the ovarian vein normally crosses the ureter but always extends beyond it to the pelvic brim.
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The single ovarian vein crossing the ureter exactly at the pelvic brim, as usually described in the ovarian vein syndrome, is therefore the exception rather than the rule. It is presumably why Clark described this as an “aberrant” vein. The left ovarian vein drains into the left renal vein, and the right ovarian vein usually drains into the inferior vena cava, occasionally into the right renal vein. This difference in the termination of the two ovarian veins may have a functional significance which will be discussed later. Conditions abdominal
producing ureter
ureteric
obstruction
in
Except at the pelvic brim, the ureter is freely mobile within the loose retroperitoneal connective tissue and is capable of considerable displacement without obstruction. Indeed, if obstruction occurs it may be assumed that the ureter has become fixed by a periureteral process, its wall invaded or its lumen compromised. This principle is well exemplified by aneurysms of the aortofemoral arteries. Aneurysms will normally push aside the ureter; and if the displaced ureter becomes obstructed, there is always an additional factor, such as periureteral fibrosis, which is usually ascribed to local leakage from the aneurysm. For these and other reasons to be discussed simple enlargement of the ovarian vein, however marked, would not be expected, and indeed has never been conclusively shown, to cause obstruction of the abdominal ureter; it may and frequently does cause indentation of the midabdominal ureter, usually at L3 to L4 level (Fig. 3). In some cases purporting to show the ovarian vein syndrome and obstruction of the ureter ending at this level, the dilated ureter is not shown beyond the L3/4 level. In all cases in which the ureter is shown in its entirety, ureteric dilatation always extends beyond this level to the pelvic brim. Postpartum thrombophlebitis of the ovarian vein is a life-threatening condition in which the abdominal ureter is frequently obstructed by extension of the inflammatory process to the periureteral tissues. It should not be considered as a manifestation of the ovarian vein syndrome, for reasons which will be discussed. Anatomy
of ureters
i
FIGURE 3. Typical indentation of ureter (arrow) by ovarian vein frequently seen during excretory urography. Indentation is wider in women who have been pregnant.
anatomic arrangements of the structures at this level. Here the ureters make a near right-angle bend over the iliac vessels and lateral displacement is limited by the shallow concavity formed by the underlying sacral ala (Fig. 2). A possible additional factor fixing the ureters at this level has been described by Dykhuizen.’ At operation he noted that there appeared to be a condensation of connective tissue which formed a sheath, binding together the ovarian vessels and ureter, which was adherent posteriorly to the iliac vessels. He postulated that this sheath was a retroperitoneal continuation of the suspensory ligament of the ovary which, in anatomy textbooks, is described as extending up along the ovarian vein. The existence or, if it exists, the possible role of this sheath must be questioned. It has not been demonstrated so far by other workers, nor is it apparent radiologically, as will be discussed. Thus fixed, and with bony support behind, the ureters at the pelvic brim are readily compressed anteriorly. The well-recognized greater susceptibility of the right ureter to compression compared with the left will be discussed under pregnancy dilatation.
of pelvic brim
The ureter at the pelvic brim differs from the ureter at any other point, being relatively fixed. This appears to be due largely or entirely to the
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Ureteric
“spindles”
With the body supine the ureter takes an uphill course to the pelvic brim where it changes
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occur with the body supine unless the vein and ureter were tightly bound together, as indeed is said to be the case according to Dykhuizen.’ Even so, for a thin-walled vein to obstruct a thick-walled muscular ureter, the intravenous pressure would have to exceed the intraureteric pressure generated during the passage of a bolus of urine. Pressure measurements suggest this is unlikely to occur under normal circumstances. A rapid rise in intravenous pressures has been demonstrated during the Valsalva maneuver, as during labor and defecation, but this is transitory. During pregnancy there is a marked increase in blood flow through the ovarian vein and a sustained increase in intravenous pressure which is, however, slight (not statistically significant) and does not reach a level which would not be overcome by normal or slightly increased ureteric peristalsis. Obstruction of ureters gravid uterus
FIGURE 4. Anteroposterior and cross-table lateral views of ureters in postmortem study on woman dying during third month of pregnancy showing differential enlargement of right ureter compared with left, dilatation ending where ureters bend over iliac arteries at pelvic brim. Catheters outline position of iliac artery, and ureters are opacified by antegrade injection of contrast medium. (Reproduced by permission, Dure-Smith. 20)
direction to become precipitously downhill (Fig. 4). This near right-angled bend is apparently sufficient to close off the lumen of the ureter lightly except during the passage of a bolus of urine. Radiographically this is seen by contrast material tending to “pool” in the abdominal ureter, whereas the pelvic ureter tends to empty rapidly. This difference in behavior of the two sections of the normal ureter may explain the radiographic description of abdominal and pelvic ureteric “spindles,” despite the absence of any true intrinsic anatomic basis for such spindles. Conditions necessary for ovarian vein to produce obstruction of ureters at pelvic brim
This light closing off of the lumen of the ureter at the pelvic brim between peristaltic activity might, at least theoretically, be accentuated simply by the weight of the blood-filled ovarian vein lying on it at this level. This could only
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at pelvic brim by
On the other hand, it has been established that the weight of the gravid uterus can and does obstruct the ureter at the pelvic brim. Despite this, a dilated ovarian vein has been held to be the more important factor, and the vein has been excised during pregnancy to relieve obstruction; it would surely be more rational and effective to excise the uterus. However, most cases of the ovarian vein syndrome have been described as following pregnancy when pressure in the ovarian vein is likely to be lower than during pregnancy; the ovarian veins have been observed to collapse immediately after delivery with no recordable pressure. Objections to hypothesis sheath at pelvic brim
of connective
tissue
Even if the pressure aspect of this hypothesis could be substantiated, there are other objections to accepting the existence of a connective tissue sheath binding the ureter and the ovarian vein at the pelvic brim. From the radiologic point of view such a sheath might be expected to produce a “conical” type of constriction, analogous to the “conical” constriction usually seen in any retroperitoneal process be it neoplastic, fibrotic, or inflammatory (Fig. 5). In fact, the ureteric obstruction in cases depicting the ovarian vein syndrome, when clearly demonstrated (which is rather infrequent), is sharply demarcated precisely at the level of the true bony pelvic brim and showing the iliac artery sign (an
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oblique filling defect in the line of the iliac artery produced by the ureter crossing the artery) (Fig. 5). In other words, the ureteric dilatation in cases depicting the ovarian vein syndrome appears to be identical to that which occurs during all pregnancies with or without a dilated ovarian vein being present. Pregnancy Etiology
and the Ovarian
of pregnancy
Vein Syndrome
dilatation20-33
The etiology of pregnancy dilatation of the urinary tract has incited controversy for some thirteen decades. Even in the enlightened atmosphere of medicine today, diametrically opposite schools of thought are still being expressed, one postulating that the hormonal climate during pregnancy is the most important factor and the other that dilatation is simply due to mechanical pressure by the gravid uterus. However, there now appears to be general agreement that the primary cause for ureteric dilatation must be mechanical pressure by the gravid uterus pressing on the ureter as it crosses the iliac artery at the pelvic brim. The dominance on the right side, which is a characteristic feature of pregnancy dilatation, is related principally to the asymmetry of the anatomic structures at the pelvic brim, particularly the iliac arteries (Fig. 2) making the right ureter more prominent and more susceptible to compression than the left (Fig. 4). Persistent
postpregnancy
dilatation
Minimal persistence of these changes after pregnancy is the rule, but more marked changes are found not uncommonly. The cause for the more marked residual dilatation remains uncertain. There is some evidence that infection alone may result in ureteric dilatation; and when this occurs during pregnancy, it may retard or prevent resolution of the pregnancy dilatation. Ovarian vein as cause of persistent postpregnancy dilatation Some of the objections for postulating a dilated ovarian vein as the cause for persistent postpartum ureteric dilatation have been mentioned. The ovarian vein may be associated with unequivocal ureteric obstruction in patients who survive postpartum thrombophlebitis. This occurrence is probably extremely rare and the obstruction due to periureteritis. It will be discussed under postpartum thrombophlebitis.
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FIGURE5. Case depicting ouarian vein syndrome. Young multiparous woman with long-standing histmy of right flank pain and urinary tract infection. There was no surgical exploration. Right retrograde pyelogram is said to show obstruction at Sl level suggestive of right ovarian vein syndrome. Arteriogram is said to show point of ureteral obstruction where ureter crosses iliac vessels. Appearance is indistinguishable from nonobstructive persistent postpregnancy dilatation. {Compare with Figure 4.) (Reproduced by permission from Emmett.‘)
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Dominance of pregnancy dilatation and ovarian vein syndrome
in right side
Like pregnancy and postpregnancy dilatation, the ovarian vein syndrome is depicted as showing a strong dominance on the right side. Unlike pregnancy dilatation no satisfactory explanation has been offered for this dominance in the ovarian vein syndrome. In fact the reverse, dominance on the left, might be expected since the left ovarian vein during and after pregnancy is usually larger than the right. This dominance on the right in the ovarian vein syndrome would be readily accounted for if the ureteric dilatation were in fact due to pregnancy dilatation and its persistence after pregnancy, as seems likely. Ureteric
dilatation
and hormones34-3s
Although the role of hormones in pregnancy dilatation has been relegated by most to that of a possible secondary effect, there are still those
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favorite experimental animals, mice and rats), were used by Roberts13 and his co-workers and failed to demonstrate any effect of progestin given either by parenteral administration or periureteral implantation. These workers concluded that if there were a hormonal effect on the ureter this would most likely be progestin and none is demonstrable. Experimental
dilatation
of ovarian
vein
Southwell and Bourne14 produced experimental dilatation of the ovarian vein in dogs and monkeys by vein ligation leaving the ovarian veins as the major venous channels from the lower extremities. They were unable to produce ureteric obstruction. Further Roberts13 has shown that excision of the ovarian vein had no effect on the normal course of hydronephrosis of pregnancy in rhesus monkeys. Physiologic considerations during pregnancy37-42
FIGURE 6. Left renal arteriogram, venous phase, showing spontaneous filling of left ovarian vein crossing upper ureter (arrow).
who propose that hormones by themselves may produce ureteric dilatation. Thus Marshall, Lyon, and MinkleP described 2 cases of ureteric dilatation which they ascribed to the use of oral contraceptives, since the dilatation disappeared when the contraceptives were discontinued. These hormonal ureteric dilatations when demonstrated also end at the pelvic brim, yet there is no evidence to suggest the ureter above the pelvic brim differs anatomically or physiologically from the ureter below the pelvic brim. Some authors have suggested that administration of progesterone compounds to patients with the ovarian vein syndrome accentuates the premenstrual flank and lower abdominal pain and is a useful diagnostic test. The literature on the possible effect of hormones on the ureter is extensive and contradictory. Some of the most recent work must be considered to be the most convincing yet produced. Rhesus monkeys, which show ureteric dilatation during pregnancy identical to that of humans (unlike all quadrapeds, including those
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of ovarian
vein
The external diameter and pressure within the ovarian vein was studied by Hodgkinson3g,40 when the abdomen was opened for various surgical procedures during pregnancy. He found an almost trebling in the diameter of the ovarian vein during pregnancy which he calculated could result in more than a sixty-fold increase in blood flow. Despite these marked changes in size and calculated blood flow, this was asssociated with a small (but not statistically significant) rise in the average ovarian vein pressure. During pregnancy the average pressure was 15.43 cm. of water compared with 11.43 cm. in nonpregnancy. The ovarian veins lie in the loose retroperitoneal connective tissue. They are thought normally to be capable of responding to any increase in blood flow by dilatation rather than a rise in pressure. This contrasts with veins in the extremities which have a firm nonexpansile supportive fibrous tissue framework, which is essential to promote blood flow by the pump action of surrounding muscles. The ability to expand in response to an increased blood flow is also characteristic of the entire pelvic venous system. It has at least two important recognizable roles: first, to absorb the marked increase in blood flow during pregnancy without a significant rise in intravenous pressure which would hinder drainage; and second, to absorb sudden changes in intravenous pressure which would occur for example and most dramatically during labor. If no such compensatory
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mechanism were available, a sudden rise in intravenous pressure might result in rupture and hemorrhage from the thin-walled venous plexuses. In support of this concept Barcroft, Herkel, and Hi1142a showed that obstruction of a major vein during pregnancy was associated with uterine hemorrhage. Possible
Functional Significance of Difference Termination of Ovarian Veins
in
The left ovarian vein drains at right angles into the left renal vein. Drainage of the left renal vein may be impeded by having to cross anterior to the aorta to reach the inferior vena cava (Fig. 2). In the supine position this may result in a higher pressure in the proximal part of the left renal vein into which the ovarian vein drains. This may be a factor in the ready filling of the left ovarian vein during angiography (particularly selective renal angiography) (Fig. 6) compared with the right ovarian vein, which usually drains at an acute angle into the inferior vena cava and may only be opacified by selective catheterization. Retrograde filling of the left ovarian (or testicular) vein during angiography may be only to the first competent valve. If the valves are absent or incompetent, the entire vein may be filled and, if films are delayed for a sufficient length of time after injection, the contrast may pass via the uterine venous plexus and fill the right ovarian vein in an antegrade manner (Fig. 7). Melnich and Bramwit* suggested that this may be the mechanism producing a bilateral ovarian vein syndrome, but this is not supported by the radiographic evidence offered by these authors. In a postmortem study Ahlberg and colleagues 16,17~43found that valves were absent in the gonadal vein about twice as often on the left as on the right side and that women have bilateral incompetent valves more often than men. Incompetent valves were found most often in multiparous women which they related to overdistention of the veins during pregnancy. Both veins after pregnancy remain larger but with the left larger than the right. Radiographic
Demonstration
of Ovarian
Vein43-45
As already mentioned, the left ovarian vein is frequently demonstrated fortuitously during various angiographic procedures. Both ovarian veins may be selectively catheterized but retrograde filling may be achieved only up to the first competent valve.
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FIGURE 7. Selective injection of left renal vein showing reversed jlow filling valveless left ovarian vein to pelvic plexus and antegrade filling of right ovarian vein. (Reproduced by permission from Melnick and Bramwit. ‘)
a technique Wegryn and Harron 45 described to demonstrate the ovarian veins which is based on the free anastomosis between the uterine and ovarian veins (Fig. 2). The uterine veins are filled by injection of contrast material into the fundus of the uterus and results in opacification of both ovarian veins (Fig. 8). Essentially this technique consists of cannulating the vagina and dilating the cervix to introduce a flexible needle into the myometrium. After 150 units of 20 ml. of contrast material are hyaluronidase,
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rapidly injected. Films are taken once per second for five to ten seconds. This technique should show antegrade filling of both ovarian veins. Postpartum
FIGURE 8. Tip of flexible needle has been introduced in myometrium via cannula in vagina. After 20 ml. of contrast 150 units of hyaluronidase, medium has been rapidly injected. Film taken approximately ten seconds after injection showing filling of pelvic venous anastomoses and both ovarian veins. (Reproduced by permission from Wegryn and Harron. “)
Ovarian
Vein Thrombophlebitis46-65
Postpartum thrombosis of the ovarian vein appears to be a rather infrequent phenomenon but many cases may go unrecognized and some fatal postpartum pulmonary emboli may have originated from the ovarian vein. It is important to separate this entity from the ovarian vein syndrome. Postpartum ovarian vein thrombophlebitis is a well-defined entity with characteristic clinical features and is potentially lethal; surgery may be lifesaving. Definite ureteric obstruction commonly occurs. The ovarian vein syndrome on the other hand is a doubtful entity with poorly defined clinical features. The symptoms are apparently those of an upper urinary tract infection. The results of surgical correction have been equivocal. Definite ureteric obstruction by simple enlargement of an ovarian vein has not been conclusively established.
The salient features of postpartum ovarian vein thrombophlebitis are as follows: it usually presents in the immediate postpartum period (one to four days); it is more common in multiparous women, involves the right side much more commonly than the left but may be bilateral; causes pain in the lower abdominal quadrant, but often only on the right side, and the pain may radiate to the flank or the costovertebra1 angle. There is a moderate associated fever and leukocytosis with a definite shift to the left. A poorly defined adnexal mass may sometimes be palpable.
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FIGURE 9. Characteristic presentation and findings of postpartum ovarian vein thrombophlebitis. This twenty-three-year-old gravida 4, para 3, was admitted with right lower quadrant pain starting two days after delivery of normal female child. Physical findings revealed right lower quadrant tenderness and positive rebound tenderness. Preoperative diagnosis was acute appendicitis. At operation right ovarian vein was dilated (2.5 cm. maximum diameter) and thrombosed throughout its length. Right ovarian vein and normal appendix were excised. No preoperative excretory urogram was obtained. Postoperative excretory urogram shows partial ureteric obstruction at L3 level.
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Etiology Coagulation defects are not uncommon in association with pregnancy and in the immediate postpartum period. These have been related to elevation of factors 7 and 8 in pregnancy, decreased blood flow in the legs, elevated plasma proconvertin, elevated plasma prothrombin, increased fibrinogen, and an increased platelet adhesion; there is also postulated to be a hypercoagulable state which exists in late pregnancy and in the immediate postpartum period. Diagnosis The principal differential diagnosis includes thrombosis of an adnexal mass, broad ligament hematoma with abscess formation, appendicitis, complete or partial volvulus, perinephric abscess, and subdiaphragmatic abscess. Preoperative excretory urography has shown either a normal urinary system or partial ureteric obstruction usually at L3-4 level (Fig. 9). It is worth emphasizing that ureteric obstruction has always occurred well above the pelvic brim. Phlebography might be of some diagnostic value but has not been undertaken so far.
not been established that simple dilatation of the ovarian veins has ever produced a significant ureteric obstruction at any level nor, from theoretical considerations, would it be expected to do so. The ureteric dilatation which is said to be characteristic of the ovarian vein syndrome is identical to the dilatation which occurs during pregnancy and which may persist as unobstructed dilatation after pregnancy. It should therefore be a relatively simple matter to establish, using the newly developing techniques of percutaneous pyelostomy, whether or not the dilatation in cases labelled as the ovarian vein syndrome are obstructed or simply represent persistent nonobstructed postpregnancy dilatation, as seems likely. Postpartum ovarian vein thrombophlebitis is an uncommon, potentially fatal, entity with well-defined clinical features frequently associated with obstruction of the upper right ureter, well above the pelvic brim. It should not be confused with the ovarian vein syndrome. 101 City Drive South Orange, California 92668 References
Treatment Theoretically, treatment by anticoagulation therapy alone might suffice but so far has been used only as an adjunct to surgery. At operation there is always edema of the adnexae with upward peritoneal extension. The ovarian vein is palpably thrombosed and may extend from its origin as far as the inferior vena cava or renal vein. Operative treatment has consisted of ligation of both ovarian veins and usually extirpation of the veins. Ligation of the inferior vena cava has also been undertaken and is considered to be mandatory in suppurative pelvic thrombophlebitis or when pulmonary embolization has occurred. There is a limited experience of pregnancy subsequent to ligation of the inferior vena cava and ovarian vessels, but there appear to be no adverse effects related to the ligation and the pregnancy pursues a normal course. Conclusion During pregnancy marked dilatation of the ovarian veins occurs in response to a marked increase in blood flow but without a significant rise in intravenous pressure. Some dilatation of the ovarian veins persists after pregnancy. It has
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14. Southwell TH, and Bourne CW: Experimental ovarian vein dilatation, J. Urol. 195: 346 (1971). 15. Arey LB: Developmental Anatomy: A Textbook and Laboratory Manual of Embryology, 6th ed., Philadelphia, W. B. Saunders Company, 1954, p. 292. 16. Ahlberg NE, Bartley 0, and Chidekel N: Circumference of left gonadal vein; anatomical and statistical study, Acta Radial. (Diag.) 3: 593 (1965). 17. IDEM: Right and left gonadal veins; anatomical and statistical study, ibid. 4: 593 (1966). 18. Ferris EJ, et al: Venography of inferior vena cava and its branches, Baltimore, Williams and Wilkins Company, 1969, pp. 296-216. 19. Ohlson L: Ovarian vessels and ureter as indicators of the expansion of the uterus in pregnancy, Acta Radiol. (Diag.) 14: 467 (1973). 20. Dure-Smith P: Pregnancy dilatation of the urinary tract. The iliac sign and its significance, Radiology 96: 545 (1970). 21. IDEM: Ureteric dilatation: its anatomical basis and relation to sex and pregnancy, in O’Grady and Brumfitt, Eds: Urinary Tract Infection, Oxford University Press, 1968, pp. 172-175. 22. Fainstat T: “Physiological hydroureter” of pregnancy in a four-legged animal, Am. J. Obstet. Gynecol. 87: 486 (1963). 23. IDEM: Ureteral dilatation in pregnancy: a review, Obstet. Gynecol. Sure. 18: 845 (1963). 24. Harrow BR, Sloane JA, and Salhanick L: Etiology of the hydronephrosis of pregnancy, Surg. Gynecol. Obstet. 119: 1042 (1964). 25. Hofbauer J: Structure and function of the ureter during pregnancy, J. Ural. 20: 413 (1928). 26. Hundlev TM. Siegel IA. Hachtee IW. and Dumler IC: Some physiolog&l obse&ations on the urinary tract during pregnancy, Surg. Gynecol. Obstet. 66: 360 (1938). 27. Hundley JM Jr, et al: Physiological changes occurring in the urinary tract during pregnancy, Am. J. Obstet. Gynecol. 30: 625 (1935). 28. Lapides JA: A report regarding the physiology of the intact human ureter, J. Urol. 59: 591 (1948). 29. Lindheimer MD, and Katz AL: Current concepts, the kidney in pregnancy, N. Engl. J. Med. 283: 1095 (1970). 30. Roberts JA, and Wolf RH: Hydronephrosis of pregnancy: a naturally occurring disorder in non-human primates closely resembling that in man, Folia Primat. 15: 143 (1971). 31. Rubi RA, and Sala NL: Ureteral function in pregnant women. III: Effect of different wsitions and of fetal deliverv uuon ’ ureteral tonus, Am. J. Obstet. -Gynecol. 101: 230 (1968). 32. Traut HF, and McLane CM: Physiological changes in the ureter associated with pregnancy, Surg. Gynecol. Obstet. 62: 65 (1936). 33. Van Wagenen G, and Jenkins RH: An experimental examination of factors causing ureteral dilatation of pregnancy, J. Urol. 42: 1010 (1939). 34. Clayton JD, and Roberts JA: The effect of progesterone on ureteral physiology in a primate model, ibid. 107: 945 (1972). 35. Dure-Smith P: Urinary tract dilatation and oral contraceptives, Br. Med. J. 1: 230 (1971). 36. Marshall S, Lyon RP, and Minkler D: Ureteral dilatation following use of oral contraceptives. J.A.M.A. 198: 782 (1966). 37. Burwell CS: The placenta as a modified arteriovenous fistula, considered in relation to the circulatory adjustments to pregnancy, Am. J. Med. Sci. 195: 1 (1938). 38. Cohen ME, and Thomson KJ: Studies on circulation in pregnancy, J. Clin. Invest. 15: 607 (1936). 39. Hodgkinson CP: Ovarian vein pressure studies, Am. J. Obstet. Gynecol. 61: 321 (1951). 40. IDEM: Physiology of ovarian veins during pregnancy, Obstet. Gynecol. 1: 26 (1953).
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41. Hodgkinson CP, and Christensen RC: Hemorrhage from ruptured utero-ovarian veins during pregnancy, Am. J. Obstet. Gynecol. 59: 1112 (1959). 42. Spitzer W: The rate of blood flow in normal and abnormal pregnancy, Arch. Gynecol. 154: 449 (1933). 42a. Barcroft J. Herkel W, and Hill S: The rate of blood Row and the gaseous metabolism of the uterus during pregnancy, J. Phvsiol. 77: I94 (1933). 43. Ahlberg NE, Bartley 0, and Chidekel N: Retrograde contrast filling of left gonadal vein; roentgenologic and anatomical _ study, A& Radiol. (Diag.) 3: 385 (1965): 44. Touolanski-Sierra R: Pelvic uhleboeranhv. Am. .,1. Obstet. -*,I Gynecol.~76: 44 (1958). 45. Wegryn SP, and Harron RA: Pelvic phlebography, Obstet. Gynecol. 15: 73 (1966). 46. Alexander B, et al: Blood coagulation in pregnancy: proconvertin and prothrombm, and the hypercoagulable state, N. Engl. J. Med. 254: 358 (1956). 47. Austin OG: Massive thrombophlebitis of the ovarian vein, Am. J. Obstet. Gynecol. 72: 428 (1958). 48. B&s R, and MacFarlane RG: Human Blood Coagluation and Its Disorders. 3rd ed.. Surinefield. Illinois. Charles C Thomas, 1961, chap. 4, pp. 50-85. y 49. Collins CG: Suppurative pelvic thrombophlebitis, Surgery 30: 298 (1951). 50. Collins JH, and Batson HWK: Pregnancy subsequent to ligation of ovarian vessels and the inferior vena cava, Am. J. Obstet. Gynecol. 67: 1202 (1954). 51. Dieckmann WJ, and Wegner CR: Studies of blood in nurmal pregnancy. IV. Percentages and grams per kilogram of serum protein and fibrin and variations in total amount of each, Arch Intern. Med. 53: 353 (1934). 52. IDEM: Measurement of the rate of venous blood flow in the legs of women at term and in the puerperium, using radioactive sodium, West. J. Obstet. Gynecol. 56: 36 (1949). 53. Klee F: Velocity of blood in pregnancy, Lischr. f Geburtsh. W Gynak. 88: 308 (1924). 54. Lotze EC, Kaufman RH, and Kaplan AL: Postpartum ovarian vein thrombophlebitis, Obstet. Gynecol. Surv. 21: 853 (1966). 55. Lynch KJ, Screenivas V, and Pelliccia 0: Ovarian vein thrombophlebitis, N. Engl. J. Med. 275: 1112 (1966). 56. Mackler MA, andRoyster HP: Right ovarian vein thrombouhlebitis and ovarian arteritis. 1. Ural. 100: 683 (1968). 57. Manchester B, and Loube SD: Velocity of blood flow in normal pregnant women, Am. Heart J. 32: 215 (1946). 58. Meyer IO: Thrombosis of an ovarian vein varicocele in the postpartum period, Am. J. Obstet. Gynecol. 78: 111 (1959). 59. Moore TC, and Stout FE: Postpartum adnexal phlegmasia cerulea dolens with adnexal infarction, Obstet. Gynecol. 21: 584 (1963). 60. Revelli E, Durando C, and Barbanti A: A il valore clinic0 della flebografia pelvica per via endouterina in campo ginecologico, Minerva Ginecol. 10: 611 (1958). 61. Revelli E, and Faccini M: Possibilita e tecnica della flebografia pelvica per via endouterina, ibid. 8: 695 (1956). 62. Rosenblum R, Derrick FC lr. and Willis AC: Postmutum thrombosis of the right ovarian din, Obstet. Gynecol. 28: 121 (1966). 63. Sanders JG, Mali& LR, and Gready TG: Ovarian vein thrombosis: a postpartum surgical emergency, Obstet. Gynecol. 24: 903 (1964). 64. Wright HP: Changes in adhesiveness of blood platelets following parturition and surgical operations, J. Pathol. Bacterial. 54: 461 (1942). 65. Wright HP, Osborn SB, and Edmonds DG: Changes in rate of flow of venous blood in leg during pregnancy, measured with radioactive sodium, Surg. Gynecol. Obstet. 90: 481 (1959).
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OVARIAN PETER
SYNDROME:
DURE-SMITH,
M.B.,
IS IT A MYTH? F.R.C.R.
From the Department of Radiology, Division of Uroradiology, University of California, Irvine Medical Center, Orange, California
In 1964Clark’ published details of 130 cases of the right ovarian vein syndrome in the second edition of Emmett’s Clinical Urogruphy. This appears to have given the seal of respectability to an entity which had previously been offered somewhat tentatively as an occasional case report. Yet a review of the literature on this syndrome, which is not extensive, is disquietingly unconvincing. The diagnosis appears to devolve on two assumptions: (1) that the demonstration of upper ureteric dilatation, the essential feature of this syndrome, is secondary to obstruction; and (2) that demonstration of a dilated ovarian vein or veins, at or near the site where the ureteric dilatation ends, is the cause of the obstruction. Neither of these assumptions appears likely to be true. Although today a good deal of skepticism is still evident when this syndrome is discussed, many accept it as an established entity and a number of women are subjected to surgical cure. For this reason alone it appears justifiable to examine the evidence for and against the existence of this syndrome and whether or not it is possible with today’s expertise in urodynamics to settle the issue.
Embryologic
and anatomic
considerations’,2*‘5-19
In its passage from the pelvic region to its definitive lumbar position the metanephros passes through a ring of embryonic venous channels (Fig. 1). Persistence ofthe right posterior cardinal vein as the adult vena cava results in
Ovarian Vein Syndrome’-l4 Definition
Clark defined the right ovarian vein syndrome as an aberrant ovarian vein causing right ureteral obstruction occurring most frequently during pregnancy and persisting following pregnancy. The symptoms were related primarily to the development of acute pyelonephritis occurring in the obstructed ureter with pain, chills, fever, pyuria, and tenderness over the right costovertebral angle. Without infection, symptoms consisted of backache and pain in the right lower quadrant. Frequently symptoms occur during the days preceding the onset of menstruation.
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FIGURE 1. In its passage from pelvic region to its definitive lumbar position metanephros passes through ring of embryonic venous channels.
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FIGURE 2. Normal anatomy of ovarian vessels. (Reproduced the Ciba Collection-Reproductive System, Netter.)
the well-recorded entity of retrocaval ureter. The ovarian veins normally develop from the subcardinal veins. Persistence of anastomoses from any of the three systems will determine the number and relationship of the vein or veins to the definitive ureter. A typical arrangement of the ovarian vessels is depicted in Figure 2. The veins start as a plexus around the ovary, similar to the pampiniform plexus around the testis in the male, and communicate by one or more large veins with the uterine venous plexus.
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by permission,
One, two, or more veins ascend to reach the pelvic brim. Here the ovarian vessels (artery and vein) usually lie lateral or anterolateral to the ureter and do not cross the ureter until about L3 or L4 level (Fig. 3, arrow). It is not unusual to see an oblique indentation of the ureter at this level during excretory urography (Fig. 3), particularly in multiparous women. It is important to note that ureteric dilatation in the ovarian vein syndrome does not end where the ovarian vein normally crosses the ureter but always extends beyond it to the pelvic brim.
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The single ovarian vein crossing the ureter exactly at the pelvic brim, as usually described in the ovarian vein syndrome, is therefore the exception rather than the rule. It is presumably why Clark described this as an “aberrant” vein. The left ovarian vein drains into the left renal vein, and the right ovarian vein usually drains into the inferior vena cava, occasionally into the right renal vein. This difference in the termination of the two ovarian veins may have a functional significance which will be discussed later. Conditions abdominal
producing ureter
ureteric
obstruction
in
Except at the pelvic brim, the ureter is freely mobile within the loose retroperitoneal connective tissue and is capable of considerable displacement without obstruction. Indeed, if obstruction occurs it may be assumed that the ureter has become fixed by a periureteral process, its wall invaded or its lumen compromised. This principle is well exemplified by aneurysms of the aortofemoral arteries. Aneurysms will normally push aside the ureter; and if the displaced ureter becomes obstructed, there is always an additional factor, such as periureteral fibrosis, which is usually ascribed to local leakage from the aneurysm. For these and other reasons to be discussed simple enlargement of the ovarian vein, however marked, would not be expected, and indeed has never been conclusively shown, to cause obstruction of the abdominal ureter; it may and frequently does cause indentation of the midabdominal ureter, usually at L3 to L4 level (Fig. 3). In some cases purporting to show the ovarian vein syndrome and obstruction of the ureter ending at this level, the dilated ureter is not shown beyond the L3/4 level. In all cases in which the ureter is shown in its entirety, ureteric dilatation always extends beyond this level to the pelvic brim. Postpartum thrombophlebitis of the ovarian vein is a life-threatening condition in which the abdominal ureter is frequently obstructed by extension of the inflammatory process to the periureteral tissues. It should not be considered as a manifestation of the ovarian vein syndrome, for reasons which will be discussed. Anatomy
of ureters
i
FIGURE 3. Typical indentation of ureter (arrow) by ovarian vein frequently seen during excretory urography. Indentation is wider in women who have been pregnant.
anatomic arrangements of the structures at this level. Here the ureters make a near right-angle bend over the iliac vessels and lateral displacement is limited by the shallow concavity formed by the underlying sacral ala (Fig. 2). A possible additional factor fixing the ureters at this level has been described by Dykhuizen.’ At operation he noted that there appeared to be a condensation of connective tissue which formed a sheath, binding together the ovarian vessels and ureter, which was adherent posteriorly to the iliac vessels. He postulated that this sheath was a retroperitoneal continuation of the suspensory ligament of the ovary which, in anatomy textbooks, is described as extending up along the ovarian vein. The existence or, if it exists, the possible role of this sheath must be questioned. It has not been demonstrated so far by other workers, nor is it apparent radiologically, as will be discussed. Thus fixed, and with bony support behind, the ureters at the pelvic brim are readily compressed anteriorly. The well-recognized greater susceptibility of the right ureter to compression compared with the left will be discussed under pregnancy dilatation.
of pelvic brim
The ureter at the pelvic brim differs from the ureter at any other point, being relatively fixed. This appears to be due largely or entirely to the
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Ureteric
“spindles”
With the body supine the ureter takes an uphill course to the pelvic brim where it changes
357
occur with the body supine unless the vein and ureter were tightly bound together, as indeed is said to be the case according to Dykhuizen.’ Even so, for a thin-walled vein to obstruct a thick-walled muscular ureter, the intravenous pressure would have to exceed the intraureteric pressure generated during the passage of a bolus of urine. Pressure measurements suggest this is unlikely to occur under normal circumstances. A rapid rise in intravenous pressures has been demonstrated during the Valsalva maneuver, as during labor and defecation, but this is transitory. During pregnancy there is a marked increase in blood flow through the ovarian vein and a sustained increase in intravenous pressure which is, however, slight (not statistically significant) and does not reach a level which would not be overcome by normal or slightly increased ureteric peristalsis. Obstruction of ureters gravid uterus
FIGURE 4. Anteroposterior and cross-table lateral views of ureters in postmortem study on woman dying during third month of pregnancy showing differential enlargement of right ureter compared with left, dilatation ending where ureters bend over iliac arteries at pelvic brim. Catheters outline position of iliac artery, and ureters are opacified by antegrade injection of contrast medium. (Reproduced by permission, Dure-Smith. 20)
direction to become precipitously downhill (Fig. 4). This near right-angled bend is apparently sufficient to close off the lumen of the ureter lightly except during the passage of a bolus of urine. Radiographically this is seen by contrast material tending to “pool” in the abdominal ureter, whereas the pelvic ureter tends to empty rapidly. This difference in behavior of the two sections of the normal ureter may explain the radiographic description of abdominal and pelvic ureteric “spindles,” despite the absence of any true intrinsic anatomic basis for such spindles. Conditions necessary for ovarian vein to produce obstruction of ureters at pelvic brim
This light closing off of the lumen of the ureter at the pelvic brim between peristaltic activity might, at least theoretically, be accentuated simply by the weight of the blood-filled ovarian vein lying on it at this level. This could only
358
at pelvic brim by
On the other hand, it has been established that the weight of the gravid uterus can and does obstruct the ureter at the pelvic brim. Despite this, a dilated ovarian vein has been held to be the more important factor, and the vein has been excised during pregnancy to relieve obstruction; it would surely be more rational and effective to excise the uterus. However, most cases of the ovarian vein syndrome have been described as following pregnancy when pressure in the ovarian vein is likely to be lower than during pregnancy; the ovarian veins have been observed to collapse immediately after delivery with no recordable pressure. Objections to hypothesis sheath at pelvic brim
of connective
tissue
Even if the pressure aspect of this hypothesis could be substantiated, there are other objections to accepting the existence of a connective tissue sheath binding the ureter and the ovarian vein at the pelvic brim. From the radiologic point of view such a sheath might be expected to produce a “conical” type of constriction, analogous to the “conical” constriction usually seen in any retroperitoneal process be it neoplastic, fibrotic, or inflammatory (Fig. 5). In fact, the ureteric obstruction in cases depicting the ovarian vein syndrome, when clearly demonstrated (which is rather infrequent), is sharply demarcated precisely at the level of the true bony pelvic brim and showing the iliac artery sign (an
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oblique filling defect in the line of the iliac artery produced by the ureter crossing the artery) (Fig. 5). In other words, the ureteric dilatation in cases depicting the ovarian vein syndrome appears to be identical to that which occurs during all pregnancies with or without a dilated ovarian vein being present. Pregnancy Etiology
and the Ovarian
of pregnancy
Vein Syndrome
dilatation20-33
The etiology of pregnancy dilatation of the urinary tract has incited controversy for some thirteen decades. Even in the enlightened atmosphere of medicine today, diametrically opposite schools of thought are still being expressed, one postulating that the hormonal climate during pregnancy is the most important factor and the other that dilatation is simply due to mechanical pressure by the gravid uterus. However, there now appears to be general agreement that the primary cause for ureteric dilatation must be mechanical pressure by the gravid uterus pressing on the ureter as it crosses the iliac artery at the pelvic brim. The dominance on the right side, which is a characteristic feature of pregnancy dilatation, is related principally to the asymmetry of the anatomic structures at the pelvic brim, particularly the iliac arteries (Fig. 2) making the right ureter more prominent and more susceptible to compression than the left (Fig. 4). Persistent
postpregnancy
dilatation
Minimal persistence of these changes after pregnancy is the rule, but more marked changes are found not uncommonly. The cause for the more marked residual dilatation remains uncertain. There is some evidence that infection alone may result in ureteric dilatation; and when this occurs during pregnancy, it may retard or prevent resolution of the pregnancy dilatation. Ovarian vein as cause of persistent postpregnancy dilatation Some of the objections for postulating a dilated ovarian vein as the cause for persistent postpartum ureteric dilatation have been mentioned. The ovarian vein may be associated with unequivocal ureteric obstruction in patients who survive postpartum thrombophlebitis. This occurrence is probably extremely rare and the obstruction due to periureteritis. It will be discussed under postpartum thrombophlebitis.
UROLOGY
FIGURE5. Case depicting ouarian vein syndrome. Young multiparous woman with long-standing histmy of right flank pain and urinary tract infection. There was no surgical exploration. Right retrograde pyelogram is said to show obstruction at Sl level suggestive of right ovarian vein syndrome. Arteriogram is said to show point of ureteral obstruction where ureter crosses iliac vessels. Appearance is indistinguishable from nonobstructive persistent postpregnancy dilatation. {Compare with Figure 4.) (Reproduced by permission from Emmett.‘)
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Dominance of pregnancy dilatation and ovarian vein syndrome
in right side
Like pregnancy and postpregnancy dilatation, the ovarian vein syndrome is depicted as showing a strong dominance on the right side. Unlike pregnancy dilatation no satisfactory explanation has been offered for this dominance in the ovarian vein syndrome. In fact the reverse, dominance on the left, might be expected since the left ovarian vein during and after pregnancy is usually larger than the right. This dominance on the right in the ovarian vein syndrome would be readily accounted for if the ureteric dilatation were in fact due to pregnancy dilatation and its persistence after pregnancy, as seems likely. Ureteric
dilatation
and hormones34-3s
Although the role of hormones in pregnancy dilatation has been relegated by most to that of a possible secondary effect, there are still those
359
favorite experimental animals, mice and rats), were used by Roberts13 and his co-workers and failed to demonstrate any effect of progestin given either by parenteral administration or periureteral implantation. These workers concluded that if there were a hormonal effect on the ureter this would most likely be progestin and none is demonstrable. Experimental
dilatation
of ovarian
vein
Southwell and Bourne14 produced experimental dilatation of the ovarian vein in dogs and monkeys by vein ligation leaving the ovarian veins as the major venous channels from the lower extremities. They were unable to produce ureteric obstruction. Further Roberts13 has shown that excision of the ovarian vein had no effect on the normal course of hydronephrosis of pregnancy in rhesus monkeys. Physiologic considerations during pregnancy37-42
FIGURE 6. Left renal arteriogram, venous phase, showing spontaneous filling of left ovarian vein crossing upper ureter (arrow).
who propose that hormones by themselves may produce ureteric dilatation. Thus Marshall, Lyon, and MinkleP described 2 cases of ureteric dilatation which they ascribed to the use of oral contraceptives, since the dilatation disappeared when the contraceptives were discontinued. These hormonal ureteric dilatations when demonstrated also end at the pelvic brim, yet there is no evidence to suggest the ureter above the pelvic brim differs anatomically or physiologically from the ureter below the pelvic brim. Some authors have suggested that administration of progesterone compounds to patients with the ovarian vein syndrome accentuates the premenstrual flank and lower abdominal pain and is a useful diagnostic test. The literature on the possible effect of hormones on the ureter is extensive and contradictory. Some of the most recent work must be considered to be the most convincing yet produced. Rhesus monkeys, which show ureteric dilatation during pregnancy identical to that of humans (unlike all quadrapeds, including those
360
of ovarian
vein
The external diameter and pressure within the ovarian vein was studied by Hodgkinson3g,40 when the abdomen was opened for various surgical procedures during pregnancy. He found an almost trebling in the diameter of the ovarian vein during pregnancy which he calculated could result in more than a sixty-fold increase in blood flow. Despite these marked changes in size and calculated blood flow, this was asssociated with a small (but not statistically significant) rise in the average ovarian vein pressure. During pregnancy the average pressure was 15.43 cm. of water compared with 11.43 cm. in nonpregnancy. The ovarian veins lie in the loose retroperitoneal connective tissue. They are thought normally to be capable of responding to any increase in blood flow by dilatation rather than a rise in pressure. This contrasts with veins in the extremities which have a firm nonexpansile supportive fibrous tissue framework, which is essential to promote blood flow by the pump action of surrounding muscles. The ability to expand in response to an increased blood flow is also characteristic of the entire pelvic venous system. It has at least two important recognizable roles: first, to absorb the marked increase in blood flow during pregnancy without a significant rise in intravenous pressure which would hinder drainage; and second, to absorb sudden changes in intravenous pressure which would occur for example and most dramatically during labor. If no such compensatory
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mechanism were available, a sudden rise in intravenous pressure might result in rupture and hemorrhage from the thin-walled venous plexuses. In support of this concept Barcroft, Herkel, and Hi1142a showed that obstruction of a major vein during pregnancy was associated with uterine hemorrhage. Possible
Functional Significance of Difference Termination of Ovarian Veins
in
The left ovarian vein drains at right angles into the left renal vein. Drainage of the left renal vein may be impeded by having to cross anterior to the aorta to reach the inferior vena cava (Fig. 2). In the supine position this may result in a higher pressure in the proximal part of the left renal vein into which the ovarian vein drains. This may be a factor in the ready filling of the left ovarian vein during angiography (particularly selective renal angiography) (Fig. 6) compared with the right ovarian vein, which usually drains at an acute angle into the inferior vena cava and may only be opacified by selective catheterization. Retrograde filling of the left ovarian (or testicular) vein during angiography may be only to the first competent valve. If the valves are absent or incompetent, the entire vein may be filled and, if films are delayed for a sufficient length of time after injection, the contrast may pass via the uterine venous plexus and fill the right ovarian vein in an antegrade manner (Fig. 7). Melnich and Bramwit* suggested that this may be the mechanism producing a bilateral ovarian vein syndrome, but this is not supported by the radiographic evidence offered by these authors. In a postmortem study Ahlberg and colleagues 16,17~43found that valves were absent in the gonadal vein about twice as often on the left as on the right side and that women have bilateral incompetent valves more often than men. Incompetent valves were found most often in multiparous women which they related to overdistention of the veins during pregnancy. Both veins after pregnancy remain larger but with the left larger than the right. Radiographic
Demonstration
of Ovarian
Vein43-45
As already mentioned, the left ovarian vein is frequently demonstrated fortuitously during various angiographic procedures. Both ovarian veins may be selectively catheterized but retrograde filling may be achieved only up to the first competent valve.
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FIGURE 7. Selective injection of left renal vein showing reversed jlow filling valveless left ovarian vein to pelvic plexus and antegrade filling of right ovarian vein. (Reproduced by permission from Melnick and Bramwit. ‘)
a technique Wegryn and Harron 45 described to demonstrate the ovarian veins which is based on the free anastomosis between the uterine and ovarian veins (Fig. 2). The uterine veins are filled by injection of contrast material into the fundus of the uterus and results in opacification of both ovarian veins (Fig. 8). Essentially this technique consists of cannulating the vagina and dilating the cervix to introduce a flexible needle into the myometrium. After 150 units of 20 ml. of contrast material are hyaluronidase,
361
rapidly injected. Films are taken once per second for five to ten seconds. This technique should show antegrade filling of both ovarian veins. Postpartum
FIGURE 8. Tip of flexible needle has been introduced in myometrium via cannula in vagina. After 20 ml. of contrast 150 units of hyaluronidase, medium has been rapidly injected. Film taken approximately ten seconds after injection showing filling of pelvic venous anastomoses and both ovarian veins. (Reproduced by permission from Wegryn and Harron. “)
Ovarian
Vein Thrombophlebitis46-65
Postpartum thrombosis of the ovarian vein appears to be a rather infrequent phenomenon but many cases may go unrecognized and some fatal postpartum pulmonary emboli may have originated from the ovarian vein. It is important to separate this entity from the ovarian vein syndrome. Postpartum ovarian vein thrombophlebitis is a well-defined entity with characteristic clinical features and is potentially lethal; surgery may be lifesaving. Definite ureteric obstruction commonly occurs. The ovarian vein syndrome on the other hand is a doubtful entity with poorly defined clinical features. The symptoms are apparently those of an upper urinary tract infection. The results of surgical correction have been equivocal. Definite ureteric obstruction by simple enlargement of an ovarian vein has not been conclusively established.
The salient features of postpartum ovarian vein thrombophlebitis are as follows: it usually presents in the immediate postpartum period (one to four days); it is more common in multiparous women, involves the right side much more commonly than the left but may be bilateral; causes pain in the lower abdominal quadrant, but often only on the right side, and the pain may radiate to the flank or the costovertebra1 angle. There is a moderate associated fever and leukocytosis with a definite shift to the left. A poorly defined adnexal mass may sometimes be palpable.
I
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FIGURE 9. Characteristic presentation and findings of postpartum ovarian vein thrombophlebitis. This twenty-three-year-old gravida 4, para 3, was admitted with right lower quadrant pain starting two days after delivery of normal female child. Physical findings revealed right lower quadrant tenderness and positive rebound tenderness. Preoperative diagnosis was acute appendicitis. At operation right ovarian vein was dilated (2.5 cm. maximum diameter) and thrombosed throughout its length. Right ovarian vein and normal appendix were excised. No preoperative excretory urogram was obtained. Postoperative excretory urogram shows partial ureteric obstruction at L3 level.
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Etiology Coagulation defects are not uncommon in association with pregnancy and in the immediate postpartum period. These have been related to elevation of factors 7 and 8 in pregnancy, decreased blood flow in the legs, elevated plasma proconvertin, elevated plasma prothrombin, increased fibrinogen, and an increased platelet adhesion; there is also postulated to be a hypercoagulable state which exists in late pregnancy and in the immediate postpartum period. Diagnosis The principal differential diagnosis includes thrombosis of an adnexal mass, broad ligament hematoma with abscess formation, appendicitis, complete or partial volvulus, perinephric abscess, and subdiaphragmatic abscess. Preoperative excretory urography has shown either a normal urinary system or partial ureteric obstruction usually at L3-4 level (Fig. 9). It is worth emphasizing that ureteric obstruction has always occurred well above the pelvic brim. Phlebography might be of some diagnostic value but has not been undertaken so far.
not been established that simple dilatation of the ovarian veins has ever produced a significant ureteric obstruction at any level nor, from theoretical considerations, would it be expected to do so. The ureteric dilatation which is said to be characteristic of the ovarian vein syndrome is identical to the dilatation which occurs during pregnancy and which may persist as unobstructed dilatation after pregnancy. It should therefore be a relatively simple matter to establish, using the newly developing techniques of percutaneous pyelostomy, whether or not the dilatation in cases labelled as the ovarian vein syndrome are obstructed or simply represent persistent nonobstructed postpregnancy dilatation, as seems likely. Postpartum ovarian vein thrombophlebitis is an uncommon, potentially fatal, entity with well-defined clinical features frequently associated with obstruction of the upper right ureter, well above the pelvic brim. It should not be confused with the ovarian vein syndrome. 101 City Drive South Orange, California 92668 References
Treatment Theoretically, treatment by anticoagulation therapy alone might suffice but so far has been used only as an adjunct to surgery. At operation there is always edema of the adnexae with upward peritoneal extension. The ovarian vein is palpably thrombosed and may extend from its origin as far as the inferior vena cava or renal vein. Operative treatment has consisted of ligation of both ovarian veins and usually extirpation of the veins. Ligation of the inferior vena cava has also been undertaken and is considered to be mandatory in suppurative pelvic thrombophlebitis or when pulmonary embolization has occurred. There is a limited experience of pregnancy subsequent to ligation of the inferior vena cava and ovarian vessels, but there appear to be no adverse effects related to the ligation and the pregnancy pursues a normal course. Conclusion During pregnancy marked dilatation of the ovarian veins occurs in response to a marked increase in blood flow but without a significant rise in intravenous pressure. Some dilatation of the ovarian veins persists after pregnancy. It has
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1. Clark JC: The right ovarian vein syndrome, in Emmett JL, Ed: Clinical Urography: An Atlas and Textbook of Roentgenologic Diagnosis, 2nd ed., Philadelphia, W. B. Saunders Company, 1964, pp. 1227-1236. 2. IDEM: The right ovarian vein syndrome, in Emmett JL, and Witten DM, Eds: Clinical Urography, 3rd ed., Philadelphia, W. B. Saunders Company, 1971, pp. 1997-2016. 3. Derrick FC Jr. Rosenblum R, and Frensilli FJ: Right ovarian vein syndrome. Six-year critique, Urology 1: 383 (1973). 4. Derrick FC Jr, Rosenblum RR, and Lynch KM Jr: Pathological association of the right ureter and right ovarian vein, J. Ural. 97: 633 (1967). 5. Derrick FC Jr, Turner WR, and House EE: Incidence of right ovarian vein syndrome in pregnant females, Obstet. Gynecol. 35: 37 (1970). 6. Dykhuizen RF: The ovarian vein syndrome. U.S. Navy Technical Report: HA-PMB-505, 16 mm. color, sound, 16 min., 1965. Available from Motion Picture Library, American College of Surgeons, ACS-513. 7. Dykhuizen RF, and Roberts JA: The ovarian vein syndrome, Surg. Gynecol. Obstet. 130: 443 (1970). 8. Melnick GS, and Bramwit DN: Bilateral ovarian vein syndrome, Am. J. Roentgenol. Radium Ther. Nucl. Med. 113: 599 (1971). 9. Otnes B, Enge I, and Matheisen W: The ovarian vein syndrome - a cause of ureteral obstruction, Stand. J. Ural. Nephrol. 6: 206 (1972). 10. Pereira RM, Ferreira AA, and Lane E: Diagnosis of the right ovarian vein syndrome, Am. J. Obstet. Gynecol. 103: 888 (1969). 11. Polse S, and Bobo E: Bilateral ureteral obstruction secondary to enlarged ovarian veins, J. Ural. 192: 395 (1969). 12. Reynolds SRM: Right ovarian vein syndrome, Obstet. Gynecol. 37: 398 (1971). 13. Roberts JA: The ovarian vein and hydronephrosis of pregnancy. Experimental studies in the rhesus monkey (Macaca mulatta), Invest. Urol. 8: 610 (1971).
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14. Southwell TH, and Bourne CW: Experimental ovarian vein dilatation, J. Urol. 195: 346 (1971). 15. Arey LB: Developmental Anatomy: A Textbook and Laboratory Manual of Embryology, 6th ed., Philadelphia, W. B. Saunders Company, 1954, p. 292. 16. Ahlberg NE, Bartley 0, and Chidekel N: Circumference of left gonadal vein; anatomical and statistical study, Acta Radial. (Diag.) 3: 593 (1965). 17. IDEM: Right and left gonadal veins; anatomical and statistical study, ibid. 4: 593 (1966). 18. Ferris EJ, et al: Venography of inferior vena cava and its branches, Baltimore, Williams and Wilkins Company, 1969, pp. 296-216. 19. Ohlson L: Ovarian vessels and ureter as indicators of the expansion of the uterus in pregnancy, Acta Radiol. (Diag.) 14: 467 (1973). 20. Dure-Smith P: Pregnancy dilatation of the urinary tract. The iliac sign and its significance, Radiology 96: 545 (1970). 21. IDEM: Ureteric dilatation: its anatomical basis and relation to sex and pregnancy, in O’Grady and Brumfitt, Eds: Urinary Tract Infection, Oxford University Press, 1968, pp. 172-175. 22. Fainstat T: “Physiological hydroureter” of pregnancy in a four-legged animal, Am. J. Obstet. Gynecol. 87: 486 (1963). 23. IDEM: Ureteral dilatation in pregnancy: a review, Obstet. Gynecol. Sure. 18: 845 (1963). 24. Harrow BR, Sloane JA, and Salhanick L: Etiology of the hydronephrosis of pregnancy, Surg. Gynecol. Obstet. 119: 1042 (1964). 25. Hofbauer J: Structure and function of the ureter during pregnancy, J. Ural. 20: 413 (1928). 26. Hundlev TM. Siegel IA. Hachtee IW. and Dumler IC: Some physiolog&l obse&ations on the urinary tract during pregnancy, Surg. Gynecol. Obstet. 66: 360 (1938). 27. Hundley JM Jr, et al: Physiological changes occurring in the urinary tract during pregnancy, Am. J. Obstet. Gynecol. 30: 625 (1935). 28. Lapides JA: A report regarding the physiology of the intact human ureter, J. Urol. 59: 591 (1948). 29. Lindheimer MD, and Katz AL: Current concepts, the kidney in pregnancy, N. Engl. J. Med. 283: 1095 (1970). 30. Roberts JA, and Wolf RH: Hydronephrosis of pregnancy: a naturally occurring disorder in non-human primates closely resembling that in man, Folia Primat. 15: 143 (1971). 31. Rubi RA, and Sala NL: Ureteral function in pregnant women. III: Effect of different wsitions and of fetal deliverv uuon ’ ureteral tonus, Am. J. Obstet. -Gynecol. 101: 230 (1968). 32. Traut HF, and McLane CM: Physiological changes in the ureter associated with pregnancy, Surg. Gynecol. Obstet. 62: 65 (1936). 33. Van Wagenen G, and Jenkins RH: An experimental examination of factors causing ureteral dilatation of pregnancy, J. Urol. 42: 1010 (1939). 34. Clayton JD, and Roberts JA: The effect of progesterone on ureteral physiology in a primate model, ibid. 107: 945 (1972). 35. Dure-Smith P: Urinary tract dilatation and oral contraceptives, Br. Med. J. 1: 230 (1971). 36. Marshall S, Lyon RP, and Minkler D: Ureteral dilatation following use of oral contraceptives. J.A.M.A. 198: 782 (1966). 37. Burwell CS: The placenta as a modified arteriovenous fistula, considered in relation to the circulatory adjustments to pregnancy, Am. J. Med. Sci. 195: 1 (1938). 38. Cohen ME, and Thomson KJ: Studies on circulation in pregnancy, J. Clin. Invest. 15: 607 (1936). 39. Hodgkinson CP: Ovarian vein pressure studies, Am. J. Obstet. Gynecol. 61: 321 (1951). 40. IDEM: Physiology of ovarian veins during pregnancy, Obstet. Gynecol. 1: 26 (1953).
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41. Hodgkinson CP, and Christensen RC: Hemorrhage from ruptured utero-ovarian veins during pregnancy, Am. J. Obstet. Gynecol. 59: 1112 (1959). 42. Spitzer W: The rate of blood flow in normal and abnormal pregnancy, Arch. Gynecol. 154: 449 (1933). 42a. Barcroft J. Herkel W, and Hill S: The rate of blood Row and the gaseous metabolism of the uterus during pregnancy, J. Phvsiol. 77: I94 (1933). 43. Ahlberg NE, Bartley 0, and Chidekel N: Retrograde contrast filling of left gonadal vein; roentgenologic and anatomical _ study, A& Radiol. (Diag.) 3: 385 (1965): 44. Touolanski-Sierra R: Pelvic uhleboeranhv. Am. .,1. Obstet. -*,I Gynecol.~76: 44 (1958). 45. Wegryn SP, and Harron RA: Pelvic phlebography, Obstet. Gynecol. 15: 73 (1966). 46. Alexander B, et al: Blood coagulation in pregnancy: proconvertin and prothrombm, and the hypercoagulable state, N. Engl. J. Med. 254: 358 (1956). 47. Austin OG: Massive thrombophlebitis of the ovarian vein, Am. J. Obstet. Gynecol. 72: 428 (1958). 48. B&s R, and MacFarlane RG: Human Blood Coagluation and Its Disorders. 3rd ed.. Surinefield. Illinois. Charles C Thomas, 1961, chap. 4, pp. 50-85. y 49. Collins CG: Suppurative pelvic thrombophlebitis, Surgery 30: 298 (1951). 50. Collins JH, and Batson HWK: Pregnancy subsequent to ligation of ovarian vessels and the inferior vena cava, Am. J. Obstet. Gynecol. 67: 1202 (1954). 51. Dieckmann WJ, and Wegner CR: Studies of blood in nurmal pregnancy. IV. Percentages and grams per kilogram of serum protein and fibrin and variations in total amount of each, Arch Intern. Med. 53: 353 (1934). 52. IDEM: Measurement of the rate of venous blood flow in the legs of women at term and in the puerperium, using radioactive sodium, West. J. Obstet. Gynecol. 56: 36 (1949). 53. Klee F: Velocity of blood in pregnancy, Lischr. f Geburtsh. W Gynak. 88: 308 (1924). 54. Lotze EC, Kaufman RH, and Kaplan AL: Postpartum ovarian vein thrombophlebitis, Obstet. Gynecol. Surv. 21: 853 (1966). 55. Lynch KJ, Screenivas V, and Pelliccia 0: Ovarian vein thrombophlebitis, N. Engl. J. Med. 275: 1112 (1966). 56. Mackler MA, andRoyster HP: Right ovarian vein thrombouhlebitis and ovarian arteritis. 1. Ural. 100: 683 (1968). 57. Manchester B, and Loube SD: Velocity of blood flow in normal pregnant women, Am. Heart J. 32: 215 (1946). 58. Meyer IO: Thrombosis of an ovarian vein varicocele in the postpartum period, Am. J. Obstet. Gynecol. 78: 111 (1959). 59. Moore TC, and Stout FE: Postpartum adnexal phlegmasia cerulea dolens with adnexal infarction, Obstet. Gynecol. 21: 584 (1963). 60. Revelli E, Durando C, and Barbanti A: A il valore clinic0 della flebografia pelvica per via endouterina in campo ginecologico, Minerva Ginecol. 10: 611 (1958). 61. Revelli E, and Faccini M: Possibilita e tecnica della flebografia pelvica per via endouterina, ibid. 8: 695 (1956). 62. Rosenblum R, Derrick FC lr. and Willis AC: Postmutum thrombosis of the right ovarian din, Obstet. Gynecol. 28: 121 (1966). 63. Sanders JG, Mali& LR, and Gready TG: Ovarian vein thrombosis: a postpartum surgical emergency, Obstet. Gynecol. 24: 903 (1964). 64. Wright HP: Changes in adhesiveness of blood platelets following parturition and surgical operations, J. Pathol. Bacterial. 54: 461 (1942). 65. Wright HP, Osborn SB, and Edmonds DG: Changes in rate of flow of venous blood in leg during pregnancy, measured with radioactive sodium, Surg. Gynecol. Obstet. 90: 481 (1959).
UROLOGY
/ APRIL 1979
/ VOLUME
XIII,
NUMBER4