The Egyptian Journal of Radiology and Nuclear Medicine 47 (2016) 1815–1818

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Case Report

A rare case of Herlyn-Werner-Wunderlich syndrome-withpregnancy

http://dx.doi.org/10.1016/j.ejrnm.2016.09.0130378-603X/� 2016 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier.This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Peer review under responsibility of The Egyptian Society of Radiology andNuclear Medicine.⇑ Corresponding author at: H No. - 27, Latif Garden, Assandh Road,

Panipat, Haryana 132103, India.E-mail address: vikeydeshwal@gmail.com (V. Deswal).

Vikas Deswal (DNB Trainee) ⇑, Nauratmal Kumawat (DNB Trainee), Sunita Purohit (HOD),Rajendra Jat (DNB Trainee)Deptt. of Radiodiagnosis, SDM Hospital, Jaipur, Rajasthan, India

a r t i c l e i n f o a b s t r a c t

Article history:Received 9 August 2016Accepted 25 September 2016Available online 30 September 2016

Keywords:Uterus didelphysHemivaginal septumIpsilateral renal agenesisHematocolposHematometra

Herlyn-Werner-Wunderlich syndrome also known as uterus didelphys with obstructinghemivaginal septum and ipsilateral renal agenesis (OHVIRA) is a very rare syndrome withonly a few case reported. A female patient referred to our hospital with retention of urinerevealed uterus didelphys with 8 weeks gestation sac in the left moiety and hematometraand hematocolpous in the right moiety with absent right kidney. Diagnosing this syndromeat an early stage is important to prevent the complications in these patients.� 2016 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting byElsevier. This is an open access article under the CC BY-NC-ND license (http://creativecom-

mons.org/licenses/by-nc-nd/4.0/).

1. Introduction

Uterus didelphys with obstructing hemivaginal septumand ipsilateral renal agenesis, also known as Herlyn-Werner-Wunderlich syndrome is itself a very raresyndrome and pregnancy in such patients is even a rarefinding. Mullerian duct anomalies (MDA) are congenitalanatomic abnormalities of the female genital tract whicharises from non-development or non-fusion of the mulle-rian ducts or failed resorption of the uterine septum. Theoverall prevalence of MDAs is 0.4% [1] and among themobstructing hemivaginal septum and ipsilateral renalagenesis (OHVIRA) constitutes 0.16–10% [2].

2. Case report

19 years old nulliparous female married since 2 yearswith normal menstrual cycle with her last menstrual per-iod (LMP) 25/4/2016, was relatively alright before 10 days,and consulted local neurosurgeon for retention of urine.She was advised ultrasound for whole abdomen and mag-netic resonance imaging (MRI) abdomen for evaluation ofurinary retention. Her MRI report outside suggestedhydroureteronephrosis in right pelvic ectopic kidney, buther clinical findings were not correlating with the MRIfindings and hence she was referred to our institution.She was diagnosed with intrauterine pregnancy of 8 weeksin ultrasonography (USG) and MRI reported outside.

At our hospital she was examined in gynecology outpa-tient department (OPD). Her per abdomen examinationrevealed a mass of around 6 � 4 cm was felt arising frompelvis on right side, which was mobile and cystic, but tenseand mildly tender on palpation. On per speculum examina-tion large boggy cystic swelling 5 � 6 cm was seen fromright side of vagina which was tense but compressibleand swelling obliterated the vagina. No cervical opening

Fig. 1. 3D transvaginal scan showing content filled dilated uterine cavityand cervical canal on the right side. In the left moiety is seen a gestationalsac with fetal pole.

Fig. 2. 3D transvaginal scan showing hematometra with hematocolpousin the right moiety and a gestational sac with a fetal pole in the leftmoiety. There is seen a thin linear tract (arrows) which is compressed inbetween the dilated right cervix and the left moiety.

Fig. 3. (a and b) Transvaginal sonography showing a single intrauterine gestation5 days. Fetal cardiac activity present.

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was seen. On bimanual examination, vaginal movement isdirectly transmitted to the abdominal mass. She wasreferred to our radiology department to reconfirm theimaging findings reported outside.

3. Imaging findings

2-dimensional (2D) and 3-dimensional (3D) transab-dominal and transvaginal sonography revealed 2 large welldefined thick walled communicating cystic lesions withthick moving echoes within them in the right adnexalregion postero-lateral to urinary bladder. In the left adnexaa single gestational sac with fetal pole and yolk sac withcrown rump length (CRL) corresponding to a gestationage of 8 weeks were seen (Figs. 1 and 2). Fetal cardiacactivity was present (Fig. 3). On upper abdomen ultra-sonography (USG), right kidney was absent. Ultrasoundimpression was a bicornuate or didelphys uterus showinghematometra and hematocolpous in right moiety and sin-gle live intrauterine pregnancy of 8 weeks gestation in leftmoiety. Possibility of lower vaginal obstruction withanomalous uterus was raised from the USG findings.

Review reporting of MRI was done. Our MRI findingsalso suggested absent right kidney with hematometra,hematocolpous in right moiety and gravid left moiety. Anelongated and compressed left sided cervix was seen(Figs. 4 and 5).

So according to clinical and imaging findings our provi-sional diagnosis was uterine didelphys with hematometra,hematocolpous in right moiety and live intrauterine preg-nancy of 8 weeks in left moiety with absent right kidney.

These findings were later confirmed on diagnosticlaparoscopy done by the obstetrician on the next day. Itwas done to confirm the cause for repeated episodes of uri-nary retention. The collection was drained per vaginally atthe same time, to reduce the risk of urinary retention andabortion. Our only aim was to continue the precious preg-

al sac with fetal pole of CRL corresponding to a gestational age of 8 weeks

Fig. 4. MRI pelvis – T2WI Cor section showing hematometra, hematocolpous in the right moiety and a small hyperintense (arrow) linear tract on the leftside adjacent to the dilated right side cervix; between the right cervix and the left side gravid uterus as the compressed left cervical canal.

Fig. 5. MRI abdomen – T2WI Cor section showing empty right renal fossa(absent right kidney) and a normal left kidney in the left renal fossa.

Fig. 6. Viable fetus in the left moiety in the follow-up scan after a month.

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nancy till a period of viability with minimum number ofcomplications.

Patient again came for follow-up sonography in the OPDaround 1 month later. Follow-up sonography showed us 2cervical canals with 2 different uterine cavities and singlelive healthy fetus in the left moiety (Figs. 6 and 7).

4. Discussion

Uterus didelphys is a anomaly in which there are twocompletely separate uterine cavities and cervix with nocommunication among them [3]. In 75% of such cases com-plete or partial longitudinal vaginal septum is associated[4]. Some patients with uterus didelphys present with a

unilateral hemivaginal septum, which may result inobstruction with consequent hematometra and hemato-colpous [5]. The majority of cases reported in the literatureare associated with renal agenesis on the same side as theobstructing transverse hemivaginal septum [4]. Thissyndrome has been referred to as obstructed hemi-vagina–ipsilateral renal agenesis anomaly. When bothparamesonephric ducts fail to fuse at the 9th week ofgestation, a duplicated system results [6]. The presenceor absence of a vaginal septum is defined by the degreeof fusion failure.

Uterus didelphys with an obstructed hemivaginabecomes symptomatic after menarche, generating cyclicpelvic pain (dysmenorrhea) that coincides with regularmenses from the unobstructed side. This obstruction cancause hematometra, hematocolpous and retrograde men-strual flow, which explains the increased prevalence ofendometriosis, retention of urine, subfertility and pelvic

Fig. 7. Follow-up scan after 1 month. (a) Shows 2 different cervical canals. (b) 3D scan shows 2 different cervical canals arising from vagina. Left moietyshows G-Sac. Right moiety is seen completely and shows minimal fluid in the endometrial canal with a very small fibroid in posterior uterine wall.

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adhesions in affected patients [7]. Obstructed anomalieswith a vaginal septum manifest as a bulging mass withinthe vagina. Uterus didelphys is associated with a sponta-neous abortion rate of 23% and an unfavorable obstetricoutcome [8].

5. Conclusion

As it is very rare for these patients to become pregnantand even after getting pregnancy, to carry it to a full viableperiod is very difficult. So it becomes important to identifythis condition at an early stage to help these patientsimprove their obstetrics outcome.

Financial statement

There is no source of funding.

Conflict of interest

There is no conflict of interest.

Presentation at a meeting

Nil.

Acknowledgment

All Staff and Residents of SDM Hospital, Jaipur,Rajasthan.

References

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[3] Carrington BM, Hricak H, Nuruddin RN, Secaf E, Laros Jr RK, Hill EC.Mullerian duct anomalies: MR imaging evaluation. Radiology1990;176(3):715–20.

[4] Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging andclinical issues. Radiology 2004;233(1):19–34.

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