Eur Radiol (2004) 14:805–808 DOI 10.1007/s00330-003-2202-3
Carmen Trinidad Francisco Tardáguila Gabriel C. Fernández Concepción Martínez Elena Chávarri Isabel Rivas
Received: 26 May 2003 Revised: 9 October 2003 Accepted: 2 December 2003 Published online: 24 January 2004 © Springer-Verlag 2004
C. Trinidad (✉) · F. Tardáguila G. C. Fernández · C. Martínez E. Chávarri · I. Rivas Departments of Radiology and Gynecology, Povisa Medical Center, Salamanca St. 5, 36211 Vigo (Pontevedra), Spain e-mail:
[email protected] Tel.: +34-986-413-144 Fax: +34-986-421-439
U R O G E N I TA L
Ovarian maldescent
Abstract Undescended ovary is a rare disorder that can be associated with müllerian malformations. There is an unclear association with infertility and malignant disease. When an ovary is not in its normal location, it is sought in other locations above the pelvic brim. This is important in cases of undetermined cyclical abdominal pain, follicle aspiration and surgical castration. MRI has proven to be the best imaging method for finding an ovary in an anomalous position and for disregarding associated malformations. We report two patients with unilateral ovarian
Introduction
Case reports
Ovaries and testes have an analogous embryological development with similar migration disorders. However, these are less frequent in ovaries, as their migration pathway ends in the pelvic cavity. In some cases, US studies occasionally show that one ovary is not present. Nevertheless, this is not usually considered an indication for a thorough search for the ovary using other imaging methods, as is done when a testis is not found, because this entity and its possible consequences are not well known and it has received little attention. As far as we know, there are no reports in the radiological literature. We report two cases of ovarian maldescent proved by MRI and provide a review of the literature.
Case 1
maldescent, one of them with Rokitansky–Küster–Hauser syndrome. A review of the literature is included. Keywords Ovarian maldescent · Undescended ovary · Müllerian malformations · MRI · Ectopic ovary
A 20-year-old nulliparous woman presented with a history of menstrual irregularity and dysmenorrhoea. She neither used hormonal contraceptives nor did she have any history of abdominal or pelvic surgery or infection. Gynaecological examination was normal. Transvaginal sonography showed a normal uterus and a eutopic left ovary, without identification of the right ovary. In its place, a dubious mass was observed, showing an acoustic shadow that could be a dermoid tumour. MRI was suggested to complete the study and was performed with a 1.5-T unit using a torso phase-array coil. Axial, sagittal and coronal turbo spin-echo T2-weighted (TR 6,095, TE 150) and axial spin-echo T1-weighted (TR 550, TE 2), 5-mm thick and 0.5 GAP, images were obtained without i.v. contrast administration. The images confirmed the absence of the right ovary in a normal position and showed an ovoid mass with cysts, identical to a mature normal ovary, located in the right paracolic gutter. Two weeks later, another MRI examination was undertaken; it revealed changes in the follicle size. An abdominal sonography focused on this area showed a mass with morphology resembling a normal gonad. Hysterosalpingography showed a long fallopian tube and a normal relationship between the fallopian tube and the ovary, i.e.
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Fig. 1a–c A 20-year-old woman with irregular and painful menses. a Coronal T2-weighted MR image of the pelvis showing a normal left ovary and the absence of a right ovary in its normal location. b The same sequence at a different level reveals the right
ovary in the lumbar region, lateral to the ascending colon. c Hysterosalpingography showing a normal relationship between the fallopian tube and the ectopic ovary and the permeability of both tubes
Fig. 2a–c A 14-year-old girl with Rokitansky–Küster–Hauser syndrome type A. a Coronal T2-weighted MR image of the abdomen showing the right ovary in its normal location and the left one in the ipsilateral paracolic gutter. b The same sequence, 2 weeks later, showing the difference in follicle size in the undescended ovary. c Sagittal MR image showing the absence of the uterus and the presence of pelvic fluid
the fimbria near the upper pole and the isthmus part near the lower pole of the ovary. Abdominal US and MRI ruled out associated congenital anomalies (Fig. 1). Case 2 A 14-year-old nulliparous girl with primary amenorrhoea and albinism was referred for a gynaecological examination that revealed a rudimentary vagina. Abdominal sonography was performed revealing the absence of the left kidney and with no visualization of
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the uterus and left ovary. MRI was recommended to rule out other urinary and gynaecological malformations. Abdominal MRI with TSE T2-weighted and SE T1-weighted images confirmed left kidney agenesis and showed the absence of the uterus, cervix and upper third of the vagina. The left ovary was not in the ovarian fosse, but was found in the left paracolic gutter instead. Hysterosalpingography was not performed due to the agenesis of the uterus (Fig. 2).
Discussion Male and female gonads are initially located on the medial surface of the urogenital ridge, on each side of the spine, inside the wolffian body. During the third month of fetal life, the gonads descend; ovaries take their place in the ovarian fosse, and testes in the scrotum, reaching their final position at month nine [1]. Ovarian descent is guided by the gubernaculum, which connects the lower pole of the gonad and attaches to the uterus, forming the utero-ovarian and round ligaments of the uterus. The suspensory ligament is attached to the upper pole of the ovary, which elongates forming the infundibulopelvic ligament. Like testes, ovaries can fail to descend and may be found in an ectopic position, ranging from the lumbar region to the pelvis. Ovarian maldescent results in the upper pole being located too high, above the pelvic brim, associated with a short infundibulopelvic ligament and mesovarium and an elongated utero-ovarian ligament [2]. However, ovarian maldescent is less known and less frequent than cryptorchidism, because in most cases, cryptorchidism is due to a migration problem from the inguinal duct to the scrotum, whereas ovarian descent disorders only occur between the paraspinal region and the pelvis. Two different entities should be considered for the differential diagnosis: ectopic ovary, which is a supernumerary ovary usually associated with inflammation or pelvic surgery, and adhesions, which may be formed during normal ovarian displacement in pregnancy, impeding the return to a normal position after delivery [3]. Ovarian maldescent has a very low incidence, between 0.3% and 0.5% [2, 4], and it may be unilateral or bilateral. Even though ovaries have a different embryological origin from the uterus and the fallopian tubes, arising from the müllerian duct, the incidence of ovarian maldescent is significantly higher in women with congenital uterine anomalies (22%) [4]. Dabirashrafi found this disorder only in patients with congenital absence of the uterus or unicornuate uterus, but not in association with other congenital anomalies [4]. More recently, Ombelet [5] reported an undescended ovary in five out of eight patients with unicornuate uterus (62.5%). One of our patients had Rokitansky–Küster–Hauser syndrome type A, consisting in the absence of the uterus and cervix and associated with renal agenesis. In the other case, the uterus was normal.
As in case 1, all the reported cases presented an elongated fallopian tube whose fimbriated end had a normal relationship with the undescended ovary [2, 4–7]. This fact suggests that a migration disorder in the müllerian duct is associated with the undescended ovary and that congenital uterine anomalies are not only due to an abnormal fusion of the müllerian ducts, but we can hypothesize that an abnormal migration could affect their normal fusion [4]. The relationship between ovarian maldescent and the associated tube maldescent and infertility is not clear. Verkauf and Bernhisel [2] found 5 cases out of 2,025 patients investigated for infertility. Three of those patients achieved pregnancy after correcting other factors, and only two remained infertile, one having bilateral ovarian maldescent and the other associated unicornuate uterus. Their results were congruent with other reports in the literature [5, 6]. Our patients were very young and had had no previous pregnancies, but we can infer normal ovary functioning as shown by variations in follicle size observed with MRI during different ovarian cycles. Thus, we can assume that women with a normal uterus can be fertile. Consequently, we do not know whether ectopic pregnancy is more frequent in these cases, because there is only one case reported in the literature [8]. Another important aspect is that, as in cryptorchidism, these gonads may have a higher risk of developing tumours than normal ovaries. It should be pointed out that there are about 12 reported cases of primary retroperitoneal cystadenomas, all in women [9]. This leads us to consider the possibility that some of these tumours occur in undiagnosed ectopic ovaries, whether supernumerary or maldescended. As far as we know, there are no specific reports in the literature about this possibility, and thus further evaluation is required. In a recent report, Ombelet described the value of MRI after clomiphene citrate ovarian stimulation for the diagnosis of undescended ovaries [5]. We agree with the authors that it is the best imaging technique thanks to its excellent ovary characterization capabilities on T2weighted sequences; it helps to diagnose, to determine the ovary location and to disregard other urinary and gynaecological anomalies. In summary, undescended ovary due to migration disorders is a rare entity that should be known by radiologists in order to diagnose it when the ovary cannot be found in its normal location. It is also important for patients to be aware of this so that atypical menstrual pain is not mistaken for other entities (appendicitis, pyelonephritis, diverticulitis, renal colic, etc.) and so as to avoid diagnostic errors with potentially serious consequences. Further studies should be performed in order to prove a possible high risk of tumours in these patients. MRI is the best technique for its diagnosis and follow-up, whereas sonography is less sensitive.
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8. Scoud MAF, Khayyat H, Mufarrij SK (1987) Ectopic pregnancy in an undescended fallopian tube: an unusual presentation. Obstet Gynecol 69:455–457 9. Subramony C, Habibpour S, Hashimoto LA (2001) Retroperitoneal mucinous cystadenoma. Arch Pathol Lab Med 125:691
