Magnetic resonance imaging in the assessment of complex MuÈ llerian anomalies

Transcription

1 British Journal of Obstetrics and Gynaecology August 2001, Vol. 108, pp. 791±797 Magnetic resonance imaging in the assessment of complex MuÈ llerian anomalies Catherine L. Minto a, Nicholas Hollings b, Margaret Hall-Craggs b, Sarah Creighton a, * Objective To evaluate the accuracy of magnetic resonance imaging in assessment of adolescent patients with complex MuÈllerian anomalies and its contribution towards operative management. Design A retrospective review of magnetic resonance imaging and operative ndings. Setting A London teaching hospital that is a tertiary referral centre for complex reproductive tract disorders. Sample All adolescents referred for assessment of complex MuÈllerian anomalies, from 1996 to 1999, and undergoing both magnetic resonance imaging and surgical assessment. Method In the nine suitable patients magnetic resonance imaging and surgical ndings were compared and the role of magnetic resonance imaging in determining the route and type of surgery was evaluated. Main outcome measures Magnetic resonance imaging data on reproductive tract anatomy and surgical ndings detailing reproductive tract anatomy. Results There was good correlation of magnetic resonance imaging and operative ndings in all cases. The best correlation was with uterine structure. In four cases the magnetic resonance imaging ndings were essential for the appropriate choice of the surgical approach and type of procedure. Conclusions Magnetic resonance imaging is a valuable tool in the management of this particular complex group of patients. INTRODUCTION Complex congenital abnormalities of the MuÈllerian system can occur in isolation or in association with other developmental disorders involving the cloaca, urogenital sinus and anorectal areas. In the past these anomalies have been assessed using a combination of clinical examination, examination under anaesthetic, ultrasonography, hysterosalpingography and laparoscopy 1. Magnetic resonance imaging has been suggested as a valuable alternative to laparoscopy and hysterosalpingography for the assessment of simple MuÈllerian anomalies 2,3 but its accuracy has remained controversial 4,5. In the combined developmental disorders, survival has markedly improved in the past decade. Surgeons now face the challenge of suitable internal and external genital reconstruction to optimise sexual activity, menstruation and fertility 6. When surgery is indicated it remains dif cult in even the isolated complex MuÈllerian anomalies. Accurate knowledge of pelvic anatomy is a Department of Obstetrics and Gynaecology, University College London Hospitals, London, UK b Department of Radiology, University College London Hospitals, London, UK * Correspondence: Miss S. Creighton, Department of Obstetrics and Gynaecology, University College London Hospitals, Grafton Way, London WC1E, UK. q RCOG 2001 British Journal of Obstetrics and Gynaecology PII: S (00)00200-X essential to fully evaluate the patient and plan the correct management. In this study the correlation between magnetic resonance imaging and operative ndings in nine patients with complex MuÈllerian anomalies and the role of magnetic resonance imaging in preoperative assessment are examined. We report here the results of a collaborative study between the Departments of Radiology and Gynaecology in a London teaching hospital, a tertiary referral centre for complex reproductive tract disorders. METHODS Nine patients with MuÈllerian abnormalities requiring surgical intervention were studied. They presented consecutively over a three-year period (April 1996 to July 1999) to the Gynaecology Department. The age range was years (median 14 years). All had congenital developmental abnormalities involving the MuÈllerian duct and/or urogenital sinus. Six of the nine also had complex bladder or cloacal anomalies and had undergone extensive surgery in the neonatal and childhood periods. Table 1 provides clinical information. Patients 1 to 8 underwent laparotomy by the same experienced paediatric gynaecologist (S.M.C.). Patient 9 underwent laparoscopic surgery (by S.M.C.). Pre-operative investigation for all patients was by ultrasound scanning (transvaginal or transabdominal) and magnetic resonance imaging. None had hysterosalpingography. Each magnetic resonance imaging scan was

2 792 C. L. MINTO ET AL. Table 1. Clinical information. CAH ˆ congenital adrenal hyperplasia; EUA ˆ examination under anaesthesia; TOF ˆ trachea-oesophageal stula. Patient No. Age at referral (years) Diagnosis Surgical history with age at time of each operation Diagnostic surgery Reason for & date of referral Transverse vaginal septum 12 years: laparoscopy and hysteroscopic incision in vaginal septum (referring hospital) years: EUA Cloacal & MuÈllerian anomalies. 4 days: colostomy; 3 months: anorectal pullthrough; 13 years: EUA CAH & MuÈllerian anomaly. 3 months: clitoral reduction; 10 years: appendectomy Vaginal, anal and oesophageal agenesis, and MuÈllerian anomalies Vaginal agenesis, anorectal malformation, MuÈllerian anomalies, and tracheoesophageal stula. Birth: TOF ligation & colostomy; 2 years: colon graft for oesophageal atresia, anorectal pullthrough; 11 years: EUA; 12 years: Laparotomy x2; 13 years: EUA 14 years: skingraft vaginoplasty. Birth: TOF repair & anal cutback; 1 year: fundoplication, anal transposition & redo fundoplication; 6 years: colostomy prolapse repaired; 6 years: colostomy closed; 9 years: sigmoid colon resected, end colostomy; 11 years: Laparotomy Double bladder and vagina. 6 months: bladder & vaginal reconstruction, ileocystoplasty, then ileal mitrofanoff Classical cloacal anomaly Birth: colostomy; 1 year: anorectal pullthrough; 2 years: anoplasty; 9 years: ileocystoplasty and Mitrofanoff procedure; 11years: hartmanns procedure & ileostomy; 15 years: skingraft vaginoplasty years: vaginoplasty, abdominal and vaginal approach years: vaginoplasty (abdominal and vaginal approach) & Bilateral salpingectomy years: laparotomy, Strassman metroplasty & R salpingectomy. Age 14.5years: laparotomy; unable to connect uterus & vagina. Age 14: laparotomy, vaginoplasty (ileal) and right hemihysterectomy. Age 14: laparotomy; ovarian cystectomy, resection of vaginal septum, & salpingectomy. Age 16: laparotomy, proceeded to hysterectomy MuÈllerian anomaly 13.8 years: laparoscopy and hysteroscopy. Age 14.2 years: laparotomy & modi ed Tompkins metroplasty MuÈllerian anomaly 17 years: laparoscopy. Age 17.3 years: laparoscopy and removal of rudimentary uterine horn associated with unicornuate uterus. Haematocolpos, January 1997 Haematocolpos, December 1998 Pain & pelvic mass, December 1998 Vaginoplasty, April 1996 Haematometra, November 1997 Pelvic mass, January 1998 Haematometra, June 1996 Pain & pelvic mass, March 1996 Pain & pelvic mass, July 1999

3 MRI IN ASSESSMENT OF MUÈ LLERIAN ANOMALIES 793 interpreted by a radiologist (M.H.C.) with extensive experience in pelvic magnetic resonance imaging. This assessment was without knowledge of previous medical and surgical history and without the information gained from consequent surgical evaluation. Over the three-year study period there were changes in imaging equipment; patient 8 was scanned on a Siemens Magnetom SP 1.0 Tesla magnet with a standard body coil (Siemens Medical Systems, Erlangen, Germany), while all other patients (1-7 and 9) were scanned on a Siemens Magnetom Vision 1.5 Tesla magnet with a phased array body coil. Similarly, protocols also varied but with increasing experience were standardised to include T 2 -weighted turbo spin echo sequences in sagittal, coronal and axial planes through the true and false pelvis. In addition, T 1 -weighted axial sections were also obtained and a small eld of view employed (256 x 256 or less). Slice thickness ranged from ve to eight millimetres. Data review In each patient, after clinical examination and ultrasound, an initial management plan was determined. This initial plan included: need for surgery, type of surgical procedure and route of surgical approach (i.e. abdominal, vaginal, hysteroscopic and/or laparoscopic). The magnetic resonance imaging data and clinical information were then evaluated jointly by a gynaecologist (S.M.C.) and radiologist (M.H.C.), and the initial management plan was revised where appropriate to produce a nal surgical management plan. We assessed the impact of magnetic resonance imaging on the choice of operative procedure, need for surgery and surgical approach, by comparing the initial management plan with the nal management plan. The surgical and magnetic resonance imaging data were scored according to an agreed schedule (Table 2). The MuÈllerian anatomy was classi ed according to our adaptation of the American Fertility Society's Classi cation of MuÈllerian Anomalies (Fig. 1). This new pictorial representation of the anatomy encompasses uterine, cervical and vaginal information, with expanded sections to include the more complex anomalies. The magnetic resonance imaging and surgical ndings relating to uterus, fallopian tubes, ovaries, cervix and vagina, were compared. RESULTS The data obtained from surgery and MR imaging are presented for comparison in Table 3. A pictorial format has been used to display uterine type, cervical, tubal, ovarian and vaginal anatomy, supplemented with text to expand the magnetic resonance imaging ndings. Magnetic resonance imaging correctly showed gross uterine anatomy in all nine patients. There were two minor differences. In one patient magnetic resonance imaging did not show a small non-fused rudimentary uterine horn (patient 5) and in another patient (patient 9) a rudimentary horn was classi ed as non-fused while at surgery it was found to be fused. In all three patients with haematosalpinges the magnetic resonance imaging correctly demonstrated this problem. Magnetic resonance imaging was unable to show fallopian tubes in ve of the six patients with normal fallopian tubes at laparotomy. Magnetic resonance imaging demonstrated 12 of the 15 ovaries found at laparotomy. It was unable to nd the other three ovaries and described one ovary as normal that was found to be a tiny streak at operation. Concordance between cervical and vaginal data was not as good. Three patients had a normal vagina and these were correctly identi ed. Six patients had an abnormal vagina and magnetic resonance imaging demonstrated this in three of them, with the remaining three described as dif cult to visualise. One of eight surgically normal cervices was identi ed on magnetic resonance imaging as rudimentary without a patent canal. Additionally bilateral cervical agenesis (patient 4) was described on magnetic resonance imaging as normal cervices. Magnetic resonance imaging provided supplementary information to the laparotomy ndings in six of the nine patients. In four cases (patients 2,4,5 and 7) abnormal pelvic bones and muscles were identi ed. In one other case magnetic resonance imaging correctly identi ed an absent right kidney (patient 3). In one case magnetic resonance imaging showed an arcuate uterus (patient 1) which was not demonstrated at surgery as a hysteroscopy was not done, nor was the uterine cavity opened. In four out of the nine patients magnetic resonance Table 2. Schedule of detail. Uterus Tubes Ovaries Cervix Vagina Other Classi cation of uterine anomaly ±(see Fig.1); Dimensions (mm x mm x mm); Cavity (shape, size); Myometrium; Endometrium (thickness mm); Junctional zone; Septum (myometrium or brous tissue). Size and shape; Complications (hydro or haematosalpinx). Dimensions; Position; Presence of follicles or cysts; Other complications Type (single or double or partial agenesis); Dimensions; Endocervical canal present (Y/N/partial). Dimensions; Transverse septum (site, thickness, length of vagina above and below); Longitudinal septum (complete/partial, length, thickness); Complications (e.g. vaginal stones or brous remnants). Pelvic musculature; Bony anomalies; Urinary tract anomalies; Relationship to other organs (e.g. bladder or urinary diversion); Free uid in Pouch of Douglas.

4 794 C. L. MINTO ET AL. Fig. 1. MuÈllerian anomaly classi cations (American Fertility Society). (a) Type A- Bilateral aplasia; (b) Unilateral agenesis; (c) lateral fusion failure. imaging data altered the initial management plan. In patients 3 and 8, magnetic resonance imaging showed that these cases were not suitable for a hysteroscopic resection and that in both cases there was enough uterine tissue in both horns to allow a metroplasty as the best option. In patient 9, magnetic resonance imaging con rmed that the uterine horn was too small for metroplasty and required removal. This was done laparoscopically avoiding the need for laparotomy. In patient 1 magnetic resonance imaging con rmed a very thick vaginal septum. Consequently the surgical approach was changed from vaginal to a combined abdominoperineal approach. In the other ve patients the type and approach of surgery was not in uenced by the magnetic resonance imaging.

5 MRI IN ASSESSMENT OF MUÈ LLERIAN ANOMALIES 795 DISCUSSION The prevalence of MuÈllerian abnormalities is thought to be 0.5% in the female population 7. The causes are unclear but may be due to genetic errors or teratogenic events. The spectrum of anomalies is wide and around 75% of these women will remain asymptomatic 8. The remaining 25% will present in a variety of ways (Table Table 3. Data from surgery and MRI (FT ˆ Fallopian Tubes). Pt Surgery MRI MRI text Comments 1 FTs not seen; uterus is arcuate internally with normal external fundal contour. Normal FTs not seen by MRI; MRI extra; internal uterine contour is arcuate. 2 FTs not seen; vagina with no septum seen; Abnormal pelvic bones & muscle. MRI did not show FTs; Abnormal vagina missed by MRI; MRI extra: abnormal pelvic muscles & bones. 3 No R ovary seen; both FTs not seen; Absent R kidney. MRI did not show FTs or R ovary; MRI extra: absent R kidney. 4 Unusual uterine positions. Abnormal coccyx, sacrum & pelvic muscles; vagina poorly delineated; stulous tract from top of vagina to back of bladder. MRI did not show streak ovary; cervices wrongly described MRI extra; abnormal pelvic muscles & bones. 5 Right uterine horn not seen; no canal to cervix. L ovary not seen; L-sided complex cystic mass in tube;?haematosalpinx; abnormal pelvic muscles & absent coccygeus. 6 Unable to delineate vagina R ovary dif cult to visualise. FTs not seen; abnormal symphysis pubis; complex L ovarian cyst. MRI missed L ovary, cervix & uterine horn MRI extra: abnormal pelvic muscles & bones. Vagina not normal; MRI did not show vagina. FT not seen. 7 L-sided pelvic mass with fat/blood;? malformed uterus; neither ovary seen. abnormal bony pelvis and muscles. MRI extra; abnormal pelvic muscles & bones. 8 R. haematosalpinx; L. FT not seen; obstructed R uterine horn. Mild obstruction to L uterus, but normal cervix. Uterus slight discordance; L FT not seen. 9 R rudimentary uterine horn not fused to L unicornuate uterus. Full concordance, except fusion of horn to unicornuate uterus.

6 796 C. L. MINTO ET AL. Table 4. Presentation of MuÈllerian Anomalies. Abnormality Likely presentation Long term complications Vaginal agenesis, transverse vaginal septum, cervical agenesis, and non- communicating uterine horn. Haematocolpos & haematosalpinx with pain and abdominal mass. Infertility (tubal damage) and endometriosis Vaginal septum or hypoplasia Dyspareunia Psychosexual problems Bicornuate or septate uterus Obstetric complications Uterine septum Recurrent miscarriage 4). The much rarer developmental disorders of the bladder, cloaca and urogenital sinus can often be associated with MuÈllerian anomalies due to their close embryological development 9. In childhood the gynaecological aspects of these conditions are largely irrelevant and surgery naturally concentrates on the urinary and gastrointestinal aspects. However, as more of these girls are now surviving into adolescence due to improved neonatal care and surgical techniques, there is a corresponding increase in referral for evaluation of fertility, assessment of genitalia for sexual function, and monitoring or treatment for haematocolpos and haematometra. We assessed several classi cations of MuÈllerian abnormalities 10±12. None of these described all anomalies found in the nine patients in this study. The American Fertility Society had the clearest classi cation 13 but even so we needed to modify it to describe our patients accurately. Consequently we have adapted and expanded this classi cation to include: partial or complete vaginal aplasia (lower or upper), cervical hypoplasia, uterine hypoplasia with cavity, combined cervico-vaginal and cervicovaginal-uterine aplasia, non-fused rudimentary uterine horns, and an extended section of lateral fusion failure incorporating more detail of vaginal and uterine septae. To optimise the management of these girls, accurate knowledge of pelvic anatomy is essential. Knowledge of uterine and vaginal type allows prediction of risk of MuÈllerian duct obstruction. This allows effective monitoring and intervention to prevent the unnecessary complications of haematometrocolpos. Information on the cervix and uterus is essential for evaluating the potential for menstruation and pregnancy. There are very few reports of successful pregnancy in women with congenital absence of the cervix despite reconstructive surgery and hysterectomy is a more common outcome 14. Patients with a small unicornuate uterus are also unlikely to carry a pregnancy to term and have a high incidence of recurrent miscarriage 15. At present the prediction of fertility can only be speculative and longitudinal studies are required particularly in correlating uterine size and structure with pregnancy rate and outcome. Accurate imaging is required initially to make the correct diagnosis and to allow assessment of operability and surgical planning. It also allows the identi cation of any complications such as haematometra, as well as providing the diagnosis of other anomalies such as associated pelvic bone or muscular malformations. Before embarking on any surgery, it is essential to have an accurate estimation of the pelvic anatomy to allow correct pre-operative planning and full preparation of the patient for surgical outcome. Ideally, the surgeon should know whether there is a uterus with the potential for menstruation or pregnancy (i.e. uterine type, dimensions and presence of endometrium, and also whether the cervix is of normal patency and con guration). It is also important to know whether any complications have occurred (e.g. haematosalpinx, hydrosalpinx), and where the ureters and kidneys lie. Planning of vaginal surgery is enhanced with measurements of vaginal dimensions and septae. Where partial vaginal agenesis has occurred, measurements of the size of the aplastic segment and its distance from the perineum or cervix allow accurate choice of vaginoplasty technique. Differentiation between types of MuÈllerian anomaly is needed to decide whether surgery is appropriate and to plan the surgical approach 16. Septae within the vagina and uterine cavity can be effectively removed hysteroscopically. Conversely bicornuate uteri require an abdominal approach if metroplasty is being considered. Accessory horns need only be removed from unicornuate uteri if the obstructed horn contains endometrium. Partial vaginal agenesis may be effectively reconstructed with a perineal approach and didelphic uteri without vaginal septae require no surgery. Table 5. Traditional investigations in muèllerian anomalies. Investigation Advantages Disadvantages Hsg Tvs Laparoscopy Shows internal uterine contour; demonstrates tubal occlusion and cervical canal patency. Good measure of endometrial thickness; well tolerated by patients. Will demonstrate tubal patency. Clear information on external uterine contour. Painful for the patient. Involves radiation; no information on external uterine contour; will miss uncommunicating uterine horns and double cervices. Unable to perform in vaginal agenesis; poor views with previous surgery; operator dependant. No information on internal uterine contour; will miss cervical agenesis; may miss vaginal anomalies; involves general anaesthetic and post-operative pain.

7 MRI IN ASSESSMENT OF MUÈ LLERIAN ANOMALIES 797 The complications of unidenti ed obstructive MuÈllerian anomalies are haematocolpos and haematometra. Seven of our patients presented with these conditions. If untreated, tubal damage and endometriosis may eventually occur 17. Fertility, already reduced in these girls, will be further compromised and the incidence of ectopic pregnancy will increase 18. These complications may be avoided if children with known cloacal anomalies are scanned (either by ultrasound or preferably magnetic resonance imaging) before and during menarche. Imaging of simple MuÈllerian anomalies has been found to be inaccurate in a number of studies 6,19. Traditional investigations have been with ultrasound (preferably transvaginal) hysterosalpingogram, and diagnostic laparoscopy. Table 5 lists the advantages and disadvantages of each. Several studies have suggested that magnetic resonance imaging scanning has higher diagnostic sensitivity and speci city in this patient group 2,3,20. Our patients are a more dif cult diagnostic group due to their complex anomalies. They are all young and sexually inactive and therefore transvaginal ultrasound and hysterosalpingogram are inappropriate investigations. Transabdominal ultrasound may be dif cult in the presence of previous multiple abdominal operations and complex bladder adaptation. In six patients diagnostic laparoscopy was contraindicated due to previous abdominal surgery. Magnetic resonance imaging was the only modality that was feasible in all nine patients and provided the required information. Magnetic resonance imaging was best at de ning uterine anatomy and the complications associated with these complex conditions. It was less accurate with regards to the cervical and vaginal anatomy. Contrast enhanced magnetic resonance imaging may improve ovarian assessment but was not used in this study. We are currently evaluating the potential bene t of the use of contrast enhanced studies in improving the de nition of vaginal anatomy. CONCLUSION In this study we have con rmed the accuracy of magnetic resonance imaging as a sensitive and speci c diagnostic investigation for MuÈllerian anomalies in this complex group of adolescent patients. It is likely that ultrasound will remain the initial investigation for all patients with simple muèllerian anomalies. However, magnetic resonance imaging should now replace hysterosalpingogram and diagnostic laparoscopy as second line investigations. In the cases of complex muèllerian anomalies magnetic resonance imaging is the investigation of choice for both peripubertal anatomical de nition and management, and pre-operative surgical planning. 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