Herlyn Werner Wunderlich syndrome (HWWS): an unusual presentation of acute abdominal pain

Herlyn Werner Wunderlich syndrome (HWW) is a very rare congenital malformation of the urogenital tract secondary to Müllerian developmental abnormalities MDA.

HWW syndrome results from agenesis or hypoplasia, defective vertical or lateral fusion, or resorption failure of paramesonephric ducts.

Anatomically it is characterised by a triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis (Fig. 1). This syndrome was described the first time on 1922 [1].

Fig. 1.

The drawing illustrates anatomical variations (renal agenesis, didelphys uterus and obstructed hemivagina) and consequences of the HWW: hematometra, hematosalpinx and hematocolpos on the same side of the renal agenesis; on the opposite side, the zonal anatomy is preserved

Didelphys uterus is generated by the absence of fusion of the Müllerian cavity, lacking of communication between the two uterine cavities, each hemiuterus show regular zonal anatomy, with its vagina, cervix, Fallopian tube and adnexa [2, 3].

Being obstructed the vaginal ostium, after menarche, at every menstrual cycle; blood material accumulates gradually into the vagina (hematocolpos), in the uterus (hematometra) and along the course of the ipsilateral tube (hematosalpinx). Some of the blood is absorbed between the periods, and therefore, patients with HWW syndrome can go unrecognised for several years after the onset of menstruation.

One other reason for delayed diagnosis is the presence of regular menses.

This anomaly is generally observed in post-menarche adolescents and young women with irregular menses, dysmenorrhoea, abdominal pain and pelvic mass. The diagnosis may be difficult due to the infrequency of this syndrome, and a high index of suspicion is required.

Reproductive performance of these patients is affected by the delayed diagnosis: retrograde blood flow destroys tubal function and leads to endometriosis [4]. Another important factor affecting fertility is the occurrence of infectious complications.

Radiologic imaging is fundamental tools in the diagnosis of HWWS. Ultrasound is the first step in the evaluation of undifferentiated pelvic pain; even though US is necessary to pone the suspect of Müllerian anomalies, ultrasound is not able to clearly identify the anatomical abnormality in this patient. MRI further characterises structural abnormalities with high accuracy, and results necessary to plan for surgical management.

We report a case of a 10-year-old female who presented to our Emergency Department with severe abdominal pain that occurred, less intense, since the patient achieved menarche, 6 months before; the patient had also irregular cycle with severe dysmenorrhoea, and she reported that the pain begun immediately prior to menstruation and worsened the second and third day of the menstrual period. Abdominal examination revealed a palpable mass.

A transabdominal ultrasound examination was perfomed using a convex (3.5–5.0 MHz) and a linear probe (5.0–12.0 MHz).

US findings showed two separate uterine cavities and cervices, suggestive of uterus didelphys (Fig. 2). The right uterine cavity and cervix were normal (Fig. 3). The left uterine cavity and cervical canal were dilated and filled with fluid that was hypoechoic with few hyperechoic areas (Fig. 4). In addition, a tubular structure was noted in left adnexa location extending laterally from left uterine corn with fluid within, suggestive for left hematosalpinx (Fig. 5). Right and left ovaries were normal (Fig. 6). In addition, renal agenesis of the left kidney (Fig. 7) with compensatory hypertrophy of the right kidney was identified.

Fig. 2.

US shows dilated left hemivagina, Fallopian tube and hemiuterus. On the right side is recognisable the right normal hemiuterus (white arrow). Axial turbo spin-echo T1-weighted fat-saturated MR image US shows analogous findings

Fig. 3.

US sagittal scan shows right uterine cavity with normal cervix and vagina (white arrow); same findings are showed in the sagittal T2W MRI scan

Fig. 4.

US shows centrally the vagina (V) and laterally on the left the Fallopian tube (T) and the hemiuterus (U) filled by inhomogeneous hypoechoic fluid. Axial turbo spin-echo T1-weighted fat-saturated MR image confirms the findings: vagina, left tube and hemiuterus are distended by inhomogeneous hyperintense fluid, signal due to products of haemoglobin degradation

Fig. 5.

US shows in the left hemipelvis, over the bladder a tubular structure dilated and filled with inhomogeneous hypoechoic fluid; behind it is recognisable the left hemivagina filled by inhomogeneous material (arrow). Sagittal T2-weighted MR image confirms the findings: vagina and the left tube are distended by inhomogeneous hypointense fluid; coronal turbo spin-echo T1-weighted fat-saturated MR images show the haematosalpinx

Fig. 6.

US shows dilated left hemivagina and Fallopian tube. The left normal adnexa (white arrow) is located in front. Analogous findings are showed in axial T2W MRI scan

Fig. 7.

US coronal image trough the left flank reveals absent kidney

MRI was performed to confirm the hypothesis of Müllerian duct anomalies and to precise anatomical delineation of the uterus, tubes, cervix and vagina. Status of these reproductive organs and extent of endometriosis, pelvic inflammation and adhesions can be well evaluated with MRI.

MRI showed two separate uterine cavities, cervices and vaginas. Normal zonal anatomy was revealed on the right side.

Left uterine cavity, cervical canal and salpinx were dilated and filled with fluid hyperintense on both T1w and T2w images with few T2 hypointense areas suggesting blood products.

The presence of hematocolpos, hematometra and hematosalpinx supported the hypothesis of being an obstructing left vaginal septum.

At examination under general anaesthesia, an obstructed left hemivagina was identified with a longitudinal vaginal septum. A vaginal septectomy was performed, and a large amount of haematic fluid was drained.