Herlyn-Werner-Wunderlich syndrome (ECR 2011 Case of the Day)

Clinical History

A 12-year-old girl experienced onset of symptoms shortly after menarche, including acute lower abdominal pain and progressive dysmenorrhea with normal menstrual cycles. A large left pelvic mass was palpated during the physical examination.

Imaging Findings

Abdominal US demonstrated absence of the left kidney. US of the pelvis showed a left-sided, lobulated structure with thin walls, containing low-level echoes, and increased through transmission (Fig. 1). An elongated structure of similar characteristics was seen adjacent to the first one (Fig. 2). On Doppler, neither structure was found to be vascularised (Fig. 3).
Abdominal T2-WI (Fig. 4) shows absence of the left kidney and a normal right kidney. A large lobulated structure in the left side and a small similar structure in the right side are depicted on the pelvic T2-WI (Fig. 5) Both are homogeneously hyperintense. Both structures are located behind the bladder (Fig. 6, 7). T2-WI shows the structures of the perineal region (Fig. 8). Coronal T2-WI (Fig. 9) depicts another hyperintense elongated tubular structure adjacent to the previous structure on the left. T1-WI FS (Fig. 10) shows the structures on the left side with hyperintense signal.

Discussion

The specific association of uterus didelphys or bicornuate bicollis uterus, obstructed hemivagina-hemicervix (haematocolpos-hemihaematometros) and ipsilateral renal agenesis is well recognised in Herlyn-Werner-Wunderlich syndrome [1, 2].

The internal genital organs and lower urinary tract are derived from two paired urogenital structures that develop in both sexes, the Wolffian (mesonephric) ducts and the Müllerian (paramesonephric) ducts [3]. The close interrelationship between the urinary and reproductive systems during embryogenesis may explain the coexistence of urinary and reproductive tract abnormalities. The Wolffian ducts, besides giving origin to the kidneys, are also inductor elements for adequate Müllerian duct fusion [1].
The uterus, fallopian tubes, cervix and upper two-thirds of the vagina develop from the paired Müllerian ducts while the lower one-third of the vagina develops separately from the urogenital sinus [2].

Due to maldevelopment or failure of fusion of the distal segments of the Müllerian ducts, various types of uterine anomalies can ensue, such as hypoplasia/agenesis, and unicornuate, didelphys, bicornuate, septate, and arcuate uterus [4]. The incidence is in between 0.5% and 5.0%. Most cases are diagnosed at menarche [3].

In this case, the imaging findings are suggestive of a uterine cystic mass with dilated left Fallopian tube in the context of uterine malformation (Fig. 1, 2 and 3).

Abdominal MRI detected left renal agenesis (Fig. 4). Pelvic MRI show uterus didelphys. The uterine duplication is seen in Fig. 5; the left uterus is larger in size and suggestive of a blind hemicervix. A large left uterus and a normal vagina, located behind the bladder, are seen in Fig. 6. The normal right uterus behind the bladder is depicted in Fig. 7. A single vagina of normal morphology can be seen in Fig. 8. Fig. 9 shows the left uterus and an elongated tubular structure corresponding to a dilated left Fallopian tube. The hyperintensity on T1 (Fig. 10) is suggestive of bloody content (hemihaematometros and haematosalpinx).
Laparoscopy and hysteroscopy confirmed the diagnosis. Hysteroscopic drainage of the hemihaematometros and haematosalpinx, and resection of the obstructive septum of the left cervix were performed.

The differential diagnosis of this entity includes Mayer-Rokitansky-Küster-Hauser syndrome, which is characterised by congenital aplasia of the uterus and the upper part of the vagina with renal abnormalities. Neither the clinical symptoms nor the radiologic images are suggestive of pelvic abscess.

Sonography and MR are both useful in the diagnosis of Müllerian malformations. The accuracy of MR has been reported at 100% [4].

Early recognition prompts surgical removal of the obstruction and prevent of complications [5].

Differential Diagnosis List

Final Diagnosis

Herlyn-Werner-Wunderlich syndrome: uterus didelphys, left blind hemicervix with hemihaematometros and haematosalpinx, and ipsilateral renal agenesis

URL:	https://eurorad.org/case/9850
DOI:	10.1594/EURORAD/CASE.9850
ISSN:	1563-4086