Ultrasound and MR Imaging and Intra-operative Findings of Obstructed Müllerian Duct Anomalies in Children and Adolescents

Beatriz L. P. Junqueira, Elka Miller*, Lisa Allen, Rachel Spitzer, Kerith Lucco, Andrea S. Doria*Presenting author

The Hospital for Sick Children, Toronto, Canada

Introduction• The development of the normal female

reproductive tract requires differentiation and development of the Müllerian ducts.

• These ducts develop cephalocaudally from embryonal mesoderm forming the fallopian tubes and fuse distally to create the uterus, cervix and upper vagina.

• The Müllerian duct anomalies (MDAs) can be divided into two major categories:

• Due to the close embryologic relationship of the paramesonephric and mesonephric ducts, MDAs may be associated with renal anomalies.

Patients and Methods• MR and/or Ultrasound imaging data from

teenage girls presenting with MDAs were collected from PACSweb (Department of Diagnostic Imaging, The Hospital for Sick Children), as well as corresponding pre-, intra- and/or post-surgical images.

• The surgical findings were considered as reference standard measures.

• The MRI and/or Ultrasound findings have been interpreted by two radiologists and were correlated with the corresponding surgical images, for evaluation of presence of true-positive, true-negative, false-positive and false-negative results.

• 20 girls (aged 0–17 years; median, 14 years; SD, 5.3) were included in the study.

• 1 patient didn’t undergo MRI, she had imperforate hymen.

• 4 patients haven’t undergone an Ultrasound scan.

Including Criteria• Diagnosis: Mullerian Anomalies.

• Age: 0–18 years old (at the surgery/imaging examination date).

• Surgery previously done.

• At least one imaging examination: MR or US.

• Surgery within 6 months of Imaging.

• Surgery performed between 2004 and 2007.

Results• The MRI suggested the correct diagnosis

(true positive rate [sensitivity] results) in 15 patients (79%) out of a total of 19 patients. The presence of the cervix could be evaluated in 13 patients (69%).

• The Ultrasound suggested the diagnosis in 11 cases (69%).

Conclusion• Ultrasound examination accurately demonstrates

an abnormal genital tract; although, it is not able to identify the MDA type.

• MRI was a sensitive imaging tool for assessment of MDAs in this small sample of patients (adequate sensitivity cutoff: >70%).

• Non-invasive diagnosis of gynecologic anomalies early on in the puberty, prior to initiation of sexual activity, may be of value for future reproductivity counselling and surgical management.

References1 Carrington BM, Hricak H, Nuruddin RN, Secaf E, Laros RK, Hill EC. Müllerian Duct Anomalies:

MR Imaging Evaluation. Radiology 1990; 176 (3):715-720;

2 Pelletiro JS, McCarthy SM, Doyle MB, Glickman MG, DeCherney AH. Diagnosis of Uterine Anomalies: Relative Accuracy of MR Imaging, Endovaginal Sonography, and Hysterosalpingography. Radiology 1992; 183 (3): 795-800;

3 Emans SJ, Laufer MR, Goldstein DP. Pediatric & Adolesent Gynecology. Lippincott Willian & Wilkins 2005; 362-386;

4 Balen AH, Creighton SM, Davies MC, MacDougall J, Stanhope R. Paediatric and Adolescent Gyneacology. Cambridge University Press 2004; 110-118, 267-274

Objective• Correlate MR and/or Ultrasound images

with pre-, intra- and/or post-operative images in children diagnosed with surgically confirmed complicated MDAs.

Both paramesonephric ducts fail to form

COMPLETE MÜLLERIAN APLASIA

UNILATERAL AGENESIS

DUPLICATION of the system

TRANSVERSE VAGINAL SEPTAE

LONGITUDINAL VAGINAL SEPTAE

Obstructive

ObstructiveNon-Obstructive

Non-obstructive

One paramesonephric ducts fail to form

Both paramesonephric ducts are present,

but fail to fuse

Failure of the urogenital sinus and the müllerian duct to meet or canalize

Paramesanephric ducts fail to fuse at their

lower border

1a. Obstructive 1b. Non-obstructive

2. Aplasia / Hypoplasia or agenesis – Mayer-Rokitansky-Küster-Hauser syndrome (MRKH or simply Rokitansky syndrome);

MRI (n = 19) US (n = 16)

True-positive

True-positive

15(79%)

11(69%)

Pathophysiology

Class I: “Müllerian” agenesis or hypoplasia Class II: Unicornuate uterus Class IV: Uterus bicornuate Class V: Septate uterus Class VI: Arcuate

Class VII: DES-related anomalies

A. Vaginal (uterus may be normal or exhibit a variety of malformations)

A. Communicating (endometrial cavity present)

A. Complete (division down to internal os)

A. Complete (septum to internal os)

A. T-shaped uterus

B. Cervical B. Noncommunicating (endometrial cavity present)

B. Partial B. Partial B. T-shaped with dilated hornsC. Fundal

C. Horn without endometrial cavityC. T-shaped

D. TubalD. No rudimentary hornE. Combined

Typically obstructed

dcba

Table 1. Classification of müllerian anomalies according to the American Society for Reproductive Medicine (ASRM) Classification System.4

a dcb

dcba

a

a b c d

dcba

a dcb

a b c

a dcb

a cb d e

dcba

dcba

a

a

e

dcb

Background• Both, MRI and Ultrasound, are non-invasive

procedures for assessment of Müllerian Duct Anomalies (MDAs).

• Previous studies in adults have shown that these imaging modalities are accurate tools for diagnosing MDAs. Therefore, they are preferable as investigative tests compared with diagnostic laparoscopy.

• To our knowledge, no previous investigation has been conducted in this field in children.

•Given differences in the anatomy of the growing uterus and ovaries in children as compared to adults, the diagnostic performance of MRI and Ultrasound in MDAs in the childhood should be further evaluated.

Figure 2: Seventeen-year-old girl with surgically diagnosed unicornuate uterus with non-communicating functional right uterine horn (class IIB – Table I). (a) MDA type. (b) Transverse US image demonstrates a non-communicating right uterine horn (long white arrow) distended with fluid and a normal appearing left uterine horn (short white arrow). (c) Sagittal T2-weighted FSE MR image. A normal left-sided uterine horn (long white arrow) with an endometrial stripe connected to a normal cervix (short straight white arrow) and a single vagina (curved white arrows) are seen. A large hematometra (arrowhead) is present within the non-communicating right horn. (d) Laparoscopy image shows the normal left-sided uterine horn (long white arrow), the left fallopian tube (curved white arrow) and the right obstructed uterus (short white arrow).

Figure 5: Fourteen-year-old girl with surgically diagnosed uterus didelphys, obstructed right hemi-vagina and ipsilateral renal agenesis (class III – Table I). (a) MDA type. (b) Axial T2-weighted fast FSE MR image. A large hematocolpos (white arrow) is noted to be centrally placed, corresponding to the right-sided obstructed hemi-vagina. (c) Coronal T1-weighted SE MR image shows a solitary left kidney. (d) Surgical picture shows a bulging right vagina.

Figure 1: Fifteen-year-old girl with surgically diagnosed Mayer-Rokitansky-Küster-Hauser syndrome presenting with functional uterine remnants (class IE – Table I). (a) MDA type. (b) Longitudinal US image demonstrates the left horn containing an endometrial stripe – 5 mm (white arrow). (c) Coronal T2-weighted FSE MR image. There is a left-sided functional uterus (short white arrow) with an abnormal connection to its cervix. On the right side, a rounded soft tissue mass (long white arrow) is seen with similar signal intensity to the myometrium, with no endometrial line, appearing to be the right remnant uterus. The vagina is atretic. (d) Laparoscopy image of the left uterus.

Figure 3: Fourteen-year-old girl with surgically diagnosed unicornuate uterus with a non-communicating right uterine horn (class IIB – Table I). (a) MDA type. (b) Coronal T2-weighted SSFSE FS MR image. There is an obstructed right-sided functional uterus horn (long white arrow) connected, but with no communication with the cavity of the normal left horn (short white arrow), which is communicated with a single cervix and a single vagina. (c) Coronal T2-weighted SSFSE FS MR image showing that both kidneys are present. (d) Laparoscopy image shows the right distended horn (long white arrow) and the left normal horn (short arrow).

Figure 4: A girl with didelphys uterus (class III – Table I). (a) MDA type. (b) Baseline axial FSE MR image, obtained when the patient was 7-years-old, shows a small uterus for the patient’s age (white arrow). (c) Transaxial US image, obtained 5 years later, demonstrates a slightly distended right uterine cavity (long white arrow) and a non-distended left uterine cavity (short white arrow). (d) Axial T2-weighted FRFSE MR image, obtained 6 years after the baseline MRI, shows mild fluid distension of both uterine cavities, more prominent on the right side (long white arrow) than on the left side (short white arrow). Nevertheless, no significant vaginal distension is noted suggesting the presence of an obstructing vaginal septum.

Figure 6: Fourteen-year-old girl with surgically diagnosed uterus didelphys, microperforated and obstructed right hemi-vagina and ipsilateral renal agenesis (class III – Table I). (a) MDA type. (b) Transverse US image demonstrates two cervices (white arrow). (c) Coronal T2-weighted FSE MR image reveals two separate uteri (long white arrows). Hematometra (short white arrow) is identified in the right vagina. (d) Surgical picture shows a bulging right vagina.

Figure 7: Bicornuate uterus. (a) MDA type. The following group of images contain two different patients. One-year-old girl with bicornuate uterus as an incidental finding (b,c). Ultrasound appearance of a complete bicornuate uterus - septum down to the internal os (Class IVA – Table I). (b) Transaxial US image demonstrates divergent endometrial cavities (white arrows) in a pre-pubertal bicornuate uterus. (c) Transaxial plane demonstrates both cervices (curved white arrows) and the septum to internal os (long white arrow). (d) 13-year-old girl presenting with dysmenorrhea. Transaxial US image shows a post-pubertal bicornuate uterus with two separate cavities, right-sided endometrial cavity (long white arrow) and a left-sided cavity (short white arrow).

Figure 13: Thirteen-year-old girl with uterus didelphys, complete longitudinal vaginal septum and obstructed right vagina (Class III – Table I; Class IIA – Table II). (a) MDA type. (b) Longitudinal US image demonstrates debris within the right hematocolpos (white arrow). (c) Coronal FSE PD MR image demonstrates the nonobstructed vagina (long white arrow). A right hematocolpos (short white arrow) is seen.

Figure 14: Fourteen-year-old girl with primary amenorrhea and surgically diagnosed imperforate hymen. (a) Drawing of this patient’s anomaly. (b) Lateral picture of the patient’s shows a protuberant lower abdomen. (c) Picture of the patient’s vulva shows a bulging bluish vaginal membrane. (d) Axial CT scan demonstrates a severe hematocolpos (white arrow). (e) Sagittal CT reformat shows once again the large hematocolpos (white arrow).

Figure 8: Twelve-year-old girl with partial bicornuate uterus – only one cervix is present (Class IVB – Table I). (a) MDA type. (b) Coronal FSE MR image. There is a large notch (long white arrow) seen between the two horns (curved arrows). (c) Longitudinal US image demonstrates one cervix.

Figure 12: Fourteen-year-old girl with surgically diagnosed low transverse vaginal septum (Class IA – Table II). (a) MDA type. (b) A picture of the low vaginal septum is shown as well as a pink thick membrane, extending into the introitus before the surgical drainage of the hematocolpos. (c) Longitudinal US image demonstrates once again a large hematocolpos (black arrow). (d) Sagittal T1-weighted MR image. A fluid-filled vagina (white arrow) is shown.

Figure 11: Fourteen-year-old girl with an arcuate uterus (Class VI – Table I). (a) MDA type. (b) Longitudinal US image demonstrates a flat fundal contour (long white arrow), a single endometrial cavity and a hypoplastic cervix (short white arrow). (c) Coronal T2-weighted MR image. The uteri fundus appears flattened on its outer surface (short white arrow). Note the hypoplastic cervix (long white arrow). (d) Axial T2-weighted MR image. A post-pubertal uterus is seen presenting with a single cavity (white arrow).

Figure 9: Fifteen-year-old girl with surgically diagnosed complete septate uterus (class VA – Table I). (a) MDA type. (b) Transverse US image demonstrates a solid mass with echotexture compatible with myometrium seen between two endometrial canals (white arrow). (c) Axial T2-weighted FSE MR image, demonstrates the fundus of uterus and two cervices. The septum is seen continuing through the cervix (white arrow). (d) Laparoscopy view of the external fundal contour.

Figure 10: Sixteen-year-old girl with surgically diagnosed complete septate uterus and left obstructed uterine horn with hematosalpinx (class VA – Table I). (a) MDA type. (b) Longitudinal US image demonstrates the left-sided hematosalpinx (long arrow). (c) Axial T2-weighted FSE MR image. A left-sided hematosalpinx (long white arrow) and left obstructed uterine cavity (short white arrow) are seen. (d) Hysteroscopic image shows the obstructed cavity once it was unobstructed at the second look hysteroscopy. (e) Transaxial US image reveals the uterus after the hysteroscopic resection of the obstructing septum.

Table 2. Classification of vaginal anomalies according to Digwani G, Falcone T. Congenital malformations of the female genital tract. Diagnosis and Management. Philadelphia: Lippincott Williams & Wilkins, 1999:146.

A. Obstructing A. Obstructing

Class II: Longitudinal

Class III:Stenosis/iatrogenic

Class I: Transverse

B. Nonobstructing B. Nonobstructing

Class III: Uterus Didelphys

dcb

b c