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Published in: BMC Pregnancy and Childbirth 1/2018

Open Access 01-12-2018 | Case report

Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report

Published in: BMC Pregnancy and Childbirth | Issue 1/2018

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Abstract

Background

Herlyn–Werner-Wunderlich syndrome (HWWS) is an uncommon congenital anomaly of the female urogenital tract, characterised by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We reported the difficult pregnancy course complicated by an extremely rare and unique case of this syndrome associated with ectrodactyly, a clinical combination never described in literature.

Case presentation

A 28- year-old nulliparous woman previously diagnosed for HWWS associated with ectrodactyly of the right foot and with a history of abdominal left hemi-hysterectomy, ipsilateral salpingectomy, vaginal reconstruction when she was an adolescent. She suffered from threats of abortion in the first trimester, recurrent urinary tract infections during all pregnancy. At 33 weeks + 5 days of gestational age, she was hospitalized for premature rupture of the membranes and uterine contractions and a caesarean section was performed because of breech presentation. Postpartum period was complicated by a pelvic abscess resolved with parental antibiotic therapies.

Conclusions

Our literature review shows an unusual aspect in our case: HWWS is not classically associated with skeletal anomalies. Moreover, the most frequent urogenital side affected is the right, not left side as in this woman. Preterm spontaneous rupture of membranes and fetal abnormal presentation represent frequent complications and probably post-caesarean infections are related to pregnancies in the context of this syndrome.
Literature
1.
go back to reference Herlyn U, Werner H. Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as atypical syndrome of abnormalities. Geburtshilfe Frauenheilkd. 1971;31:340–7.PubMed Herlyn U, Werner H. Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as atypical syndrome of abnormalities. Geburtshilfe Frauenheilkd. 1971;31:340–7.PubMed
2.
go back to reference Wunderlich M. Unusual form of genital malformation with aplasia of the right kidney. Zentralbl Gynakol. 1976;98(9):559–62.PubMed Wunderlich M. Unusual form of genital malformation with aplasia of the right kidney. Zentralbl Gynakol. 1976;98(9):559–62.PubMed
3.
go back to reference Burgis J. Obstructive Müllerian anomalies: case report, diagnosis, and management. AM J Obstet Gynaecol. 2001;185(2):338–44.CrossRef Burgis J. Obstructive Müllerian anomalies: case report, diagnosis, and management. AM J Obstet Gynaecol. 2001;185(2):338–44.CrossRef
4.
go back to reference Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol. 2007;37(7):657–65.CrossRef Orazi C, Lucchetti MC, Schingo PM, Marchetti P, Ferro F. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol. 2007;37(7):657–65.CrossRef
5.
go back to reference Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato L, Sansoni I, Grasso RF, Beomonte Zobel B. Herlyn –Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging. 2012;12:4.CrossRef Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato L, Sansoni I, Grasso RF, Beomonte Zobel B. Herlyn –Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging. 2012;12:4.CrossRef
6.
go back to reference Shavell VL, Montgomery SE, Johnson SC, Diamond MP, Berman JM. Complete septate uterus, obstructed hemivagina, and ipsilateral renal anomaly: pregnancy course complicated by a rare urogenital anomaly. Arch Gynaecol Obstet. 2009;280:449–52.CrossRef Shavell VL, Montgomery SE, Johnson SC, Diamond MP, Berman JM. Complete septate uterus, obstructed hemivagina, and ipsilateral renal anomaly: pregnancy course complicated by a rare urogenital anomaly. Arch Gynaecol Obstet. 2009;280:449–52.CrossRef
7.
go back to reference Afrashtehfar CD, Pigña-García A, Afrashtehfar KI. Müllerian anomalies. Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA). Cir Cir. 2014;82(4):460–71.PubMed Afrashtehfar CD, Pigña-García A, Afrashtehfar KI. Müllerian anomalies. Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA). Cir Cir. 2014;82(4):460–71.PubMed
8.
go back to reference Jindal G, Parmar VR, Gupta VK. Ectrodactyly/split hand feet malformation. Indian J Hum Genet. 2009;15(3):140–2.CrossRef Jindal G, Parmar VR, Gupta VK. Ectrodactyly/split hand feet malformation. Indian J Hum Genet. 2009;15(3):140–2.CrossRef
9.
go back to reference Hydir Z, Beale V, O’Connor R, Clayton-Smith J. Genitourinary malformations: an under-recognized feature of ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome. Clin Dysmorphol. 2017;26(2):78–82.CrossRef Hydir Z, Beale V, O’Connor R, Clayton-Smith J. Genitourinary malformations: an under-recognized feature of ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome. Clin Dysmorphol. 2017;26(2):78–82.CrossRef
10.
go back to reference Lin PC, Bhatnagar KP, Nettleton S, Nakajima ST. Female genital anomalies affecting reproduction. Fert Steril. 2002;78(5):899–915.CrossRef Lin PC, Bhatnagar KP, Nettleton S, Nakajima ST. Female genital anomalies affecting reproduction. Fert Steril. 2002;78(5):899–915.CrossRef
11.
go back to reference American Fertility Society. The American Fertility Society classification of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies. Müllerian duct anomalies and intrauterine adhesions. Fert Steril. 1988;49:944–55.CrossRef American Fertility Society. The American Fertility Society classification of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies. Müllerian duct anomalies and intrauterine adhesions. Fert Steril. 1988;49:944–55.CrossRef
12.
go back to reference Purslow CE. A case of unilateral haematocolpos, hematometra and haematosalpinx. J Obstet Gynaecol Br Emp. 1922;29:643.CrossRef Purslow CE. A case of unilateral haematocolpos, hematometra and haematosalpinx. J Obstet Gynaecol Br Emp. 1922;29:643.CrossRef
13.
go back to reference Karag’ozov I. Herlyn –Werner-Wunderlich syndrome. Akush Ginekol (Sofiia). 1983;22(1):70–6. Karag’ozov I. Herlyn –Werner-Wunderlich syndrome. Akush Ginekol (Sofiia). 1983;22(1):70–6.
14.
go back to reference Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fert Steril. 2007;87(4):918–22.CrossRef Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fert Steril. 2007;87(4):918–22.CrossRef
15.
go back to reference Živković K, Prka M, Živković N, Bucko A, Habek D. Unusual case of OHVIRA syndrome with a single uterus, unrecognized before labor and followed by an intrapartum rupture of obstructed hemivagina. Arch Gynaecol Obstet. 2014;290:855–8.CrossRef Živković K, Prka M, Živković N, Bucko A, Habek D. Unusual case of OHVIRA syndrome with a single uterus, unrecognized before labor and followed by an intrapartum rupture of obstructed hemivagina. Arch Gynaecol Obstet. 2014;290:855–8.CrossRef
16.
go back to reference Han B, Herndon CN, Rosen MP, Wang ZJ, Daldrup-Link H. Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Radiol Case Rep. 2015;5(1):327.CrossRef Han B, Herndon CN, Rosen MP, Wang ZJ, Daldrup-Link H. Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Radiol Case Rep. 2015;5(1):327.CrossRef
17.
go back to reference Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, Li TC, Tanos V, Brölmann H, Gianaroli L, Campo R. The ESHRE-ESGE consensus on the classification of female genital tract congenital anomalies. Gynecol Surg. 2013;10(3):199–212.CrossRef Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, Li TC, Tanos V, Brölmann H, Gianaroli L, Campo R. The ESHRE-ESGE consensus on the classification of female genital tract congenital anomalies. Gynecol Surg. 2013;10(3):199–212.CrossRef
18.
go back to reference Vercellini P, Daguati R, Somigliana E, Viganò P, Lanzani A, Fedele L. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys institutional case series and a systematic literature review. Fert Steril. 2007;87(4):719–24.CrossRef Vercellini P, Daguati R, Somigliana E, Viganò P, Lanzani A, Fedele L. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys institutional case series and a systematic literature review. Fert Steril. 2007;87(4):719–24.CrossRef
19.
go back to reference Muraoka A, Tsuda H, Kotani T, Kikkawa F. Severe hemoperitoneum during pregnancy with obstructed hemivagina and ipsilateral renal anomaly syndrome: a case report. J Reprod Med. 2016;61:290–4.PubMed Muraoka A, Tsuda H, Kotani T, Kikkawa F. Severe hemoperitoneum during pregnancy with obstructed hemivagina and ipsilateral renal anomaly syndrome: a case report. J Reprod Med. 2016;61:290–4.PubMed
20.
go back to reference Reis MI, Vicente AP, Cominho J, Gomes AS, Martins L, Nunes F. Pyometra and pregnancy with Herlyn –Werner-Wunderlich syndrome. Rev Bras Ginecol Obstet. 2016;38(12):623–8.CrossRef Reis MI, Vicente AP, Cominho J, Gomes AS, Martins L, Nunes F. Pyometra and pregnancy with Herlyn –Werner-Wunderlich syndrome. Rev Bras Ginecol Obstet. 2016;38(12):623–8.CrossRef
21.
go back to reference Wu T-H, Wu T-T, Ng Y-Y, et al. Herlyn-Werner-Wunderlich syndrome consisting of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis in a newborn. Pediatr Neonatol. 2012;53:68–71.CrossRef Wu T-H, Wu T-T, Ng Y-Y, et al. Herlyn-Werner-Wunderlich syndrome consisting of uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis in a newborn. Pediatr Neonatol. 2012;53:68–71.CrossRef
22.
go back to reference Bhoil R, Ahluwalia A, Chauhan N. Herlin Werner Wunderlich syndrome with hematocolpos: an unusual case report of full diagnostic approach and treatment. Int J Fert Steril. 2016;10(1):136–40. Bhoil R, Ahluwalia A, Chauhan N. Herlin Werner Wunderlich syndrome with hematocolpos: an unusual case report of full diagnostic approach and treatment. Int J Fert Steril. 2016;10(1):136–40.
23.
go back to reference Gungor Ugurlucan F, Bastu E, Gulsen G, Kurek Eken M, Akhan SE. OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature. Clin Imaging. 2014;38(3):357–9.CrossRef Gungor Ugurlucan F, Bastu E, Gulsen G, Kurek Eken M, Akhan SE. OHVIRA syndrome presenting with acute abdomen: a case report and review of the literature. Clin Imaging. 2014;38(3):357–9.CrossRef
24.
go back to reference Jeong J-H, Kim YJ, Chang C-H, Choi H-I. A case of Herlyn- Werner-Wunderlich syndrome with recurrent hematopyometra. J Women's Med. 2009;2(2):76–80. Jeong J-H, Kim YJ, Chang C-H, Choi H-I. A case of Herlyn- Werner-Wunderlich syndrome with recurrent hematopyometra. J Women's Med. 2009;2(2):76–80.
25.
go back to reference Stassart JP, Nagel TC, Prem KA, Phipps WR. Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis: the University of Minnesota experience. Fert Steril. 1992;57(4):756–61.CrossRef Stassart JP, Nagel TC, Prem KA, Phipps WR. Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis: the University of Minnesota experience. Fert Steril. 1992;57(4):756–61.CrossRef
26.
go back to reference Monks P. Uterus didelphys associated with unilateral cervical atresia and renal agenesis. Aust N Z J Obstet Gynaecol. 1979;19(4):245–6.CrossRef Monks P. Uterus didelphys associated with unilateral cervical atresia and renal agenesis. Aust N Z J Obstet Gynaecol. 1979;19(4):245–6.CrossRef
27.
go back to reference Candiani GB, Fedele L, Candiani M. Double uterus, blind hemivagina, and ipsilateral renal agenesis:36 cases and long-term follow-up. Obstet Gynaecol. 1997;90(1):26–32.CrossRef Candiani GB, Fedele L, Candiani M. Double uterus, blind hemivagina, and ipsilateral renal agenesis:36 cases and long-term follow-up. Obstet Gynaecol. 1997;90(1):26–32.CrossRef
28.
go back to reference Chen FP, Ng KK. Term pregnancy at the site of atresia following vaginal canalization in a case of uterus didelphys with hemivaginal atresia and ipsilateral renal agenesis. Taiwan J Obstet Gynaecol. 2006;45(4):366–8.CrossRef Chen FP, Ng KK. Term pregnancy at the site of atresia following vaginal canalization in a case of uterus didelphys with hemivaginal atresia and ipsilateral renal agenesis. Taiwan J Obstet Gynaecol. 2006;45(4):366–8.CrossRef
29.
go back to reference Cozzolino M, Corioni S, Magro Malosso ER, Sorbi F, Mecacci F. Two successful pregnancies in Herlyn –Werner-Wunderlich syndrome. J Obstet and Gynaecol. 2014;34(6):534–5.CrossRef Cozzolino M, Corioni S, Magro Malosso ER, Sorbi F, Mecacci F. Two successful pregnancies in Herlyn –Werner-Wunderlich syndrome. J Obstet and Gynaecol. 2014;34(6):534–5.CrossRef
30.
go back to reference Altchek A, Paciuc J. Successful pregnancy following surgery in the obstructed uterus in a uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: case report and literature review. J Pediatr Adolesc Gynaecol. 2009;22(5):e159–62.CrossRef Altchek A, Paciuc J. Successful pregnancy following surgery in the obstructed uterus in a uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: case report and literature review. J Pediatr Adolesc Gynaecol. 2009;22(5):e159–62.CrossRef
31.
go back to reference Haddad B, Barranger E, Paniel BJ. Blind hemivagina: long-term follow -up and reproductive performance in 42 cases. Hum Reprod. 1999;14(8):1962–4.CrossRef Haddad B, Barranger E, Paniel BJ. Blind hemivagina: long-term follow -up and reproductive performance in 42 cases. Hum Reprod. 1999;14(8):1962–4.CrossRef
32.
go back to reference Heinonen PK. Clinical implications of the didelphic uterus: long-term follow-up of 49 cases. Eur J Obstet Gynecol Reprod Biol. 2000;91(2):183–90.CrossRef Heinonen PK. Clinical implications of the didelphic uterus: long-term follow-up of 49 cases. Eur J Obstet Gynecol Reprod Biol. 2000;91(2):183–90.CrossRef
33.
go back to reference Adair L 2nd, Georgiades M, Osborne R, Ng T. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: common presentation of an unusual variation. J Radiol Case Rep. 2011;5(1):1–8.PubMedPubMedCentral Adair L 2nd, Georgiades M, Osborne R, Ng T. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: common presentation of an unusual variation. J Radiol Case Rep. 2011;5(1):1–8.PubMedPubMedCentral
34.
go back to reference Singh K, Thakur S, Soni A, Verma A. Herlyn-Werner-Wunderlich syndrome/OHVIRA syndrome; a rare urogenital anomaly with unusual presentation in two case with review of literature. Clinics Mother Child Health. 2016;13(1):1–4.CrossRef Singh K, Thakur S, Soni A, Verma A. Herlyn-Werner-Wunderlich syndrome/OHVIRA syndrome; a rare urogenital anomaly with unusual presentation in two case with review of literature. Clinics Mother Child Health. 2016;13(1):1–4.CrossRef
35.
go back to reference Li Z, Yu X, Shen J, Liang J. Scoliosis in Herlyn-Werner-Wunderlich syndrome: a case report and literature review. Medicine (Baltimore). 2014;93(28):e185.CrossRef Li Z, Yu X, Shen J, Liang J. Scoliosis in Herlyn-Werner-Wunderlich syndrome: a case report and literature review. Medicine (Baltimore). 2014;93(28):e185.CrossRef
36.
go back to reference Yavuz A, Bora A, Kurdoğlu M, Goya C, Kurdoğlu Z, Beyazal M, Akdemir Z. Herlyn-Werner-Wunderlich syndrome: merits of sonographic and magnetic resonance imaging for accurate diagnosis and patient management in 13 cases. J Pediatr Adolesc Gynecol. 2015;28(1):47–52.CrossRef Yavuz A, Bora A, Kurdoğlu M, Goya C, Kurdoğlu Z, Beyazal M, Akdemir Z. Herlyn-Werner-Wunderlich syndrome: merits of sonographic and magnetic resonance imaging for accurate diagnosis and patient management in 13 cases. J Pediatr Adolesc Gynecol. 2015;28(1):47–52.CrossRef
37.
go back to reference Zhun L, Chen N, Tong JL, Wang W, Zhang L, Lang JH. New classification of Herlyn –Werner-Wunderlich syndrome. Chin Med J (Engl). 2015;128(2):222–5.CrossRef Zhun L, Chen N, Tong JL, Wang W, Zhang L, Lang JH. New classification of Herlyn –Werner-Wunderlich syndrome. Chin Med J (Engl). 2015;128(2):222–5.CrossRef
38.
go back to reference Karat C, Madhivanan P, Krupp K, Poornima S, Jayanthi NV, Suguna JS, Mathai E. The clinical and microbiological correlates of premature rupture of membranes. Indian J Med Microbiol. 2006;24(4):283–5.CrossRef Karat C, Madhivanan P, Krupp K, Poornima S, Jayanthi NV, Suguna JS, Mathai E. The clinical and microbiological correlates of premature rupture of membranes. Indian J Med Microbiol. 2006;24(4):283–5.CrossRef
39.
go back to reference Tsai JL, Tsai SF. Case Report: A Rare Cause of Complicated Urinary Tract Infection in a Woman with Herlyn-Werner-Wunderlich Syndrome. Iran Red Crescent Med J. 2016;18(11):e40267.CrossRef Tsai JL, Tsai SF. Case Report: A Rare Cause of Complicated Urinary Tract Infection in a Woman with Herlyn-Werner-Wunderlich Syndrome. Iran Red Crescent Med J. 2016;18(11):e40267.CrossRef
40.
go back to reference Kawakita T, Landy HJ. Surgical site infection after cesarean delivery: epidemiology, prevention and treatment. Maternal Health Neonatol Perinatol. 2017;3:12.CrossRef Kawakita T, Landy HJ. Surgical site infection after cesarean delivery: epidemiology, prevention and treatment. Maternal Health Neonatol Perinatol. 2017;3:12.CrossRef
Metadata
Title
Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report
Publication date
01-12-2018
Published in
BMC Pregnancy and Childbirth / Issue 1/2018
Electronic ISSN: 1471-2393
DOI
https://doi.org/10.1186/s12884-018-2133-2

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