Association of the rectovestibular fistula with MRKH Syndrome and the paradigm shift in the management in view of the future uterine transplant




Rectovestibular fistula, Vaginal atresia, Cervicovaginal atresia, MRKH Syndrome, Vaginoplasty, Bowel vaginoplasty, Ecchietti vaginoplasty, Uterine transplantation, VCUA classification, ESHRE/ESGE classification, AFC classification, Krickenbeck classification


Uterine transplantation in Mayer-Rokitansky-Küster-Hauser (MRKH) patients with absolute uterine function infertility have added a new dimension and paradigm shift in the management of females born with rectovestibular fistula coexisting with vaginal agenesis. The author reviewed the relevant literature of this rare association, the popular and practical classifications of genital malformations that the gynecologists use, the different vaginal reconstruction techniques, and try to know what shall serve best in this small cohort of these patients lest they wish to go for uterine transplantation in future.


Download data is not yet available.


Metrics Loading ...

Author Biography

Yogesh Kumar Sarin, Lady Hardinge Medical College, New Delhi

Dr. Yogesh Kumar Sarin,



Dr. Yogesh Kumar Sarin did his graduation and post-graduation in Surgery in Christian Medical College, Ludhiana. He was trained in Paediatric Surgery in Jaipur. He later acquired MBA in health care administration from the prestigious faculty of Management Studies, University of Delhi. He worked as Director Professor & Head of Department of Paediatric Surgery in Maulana Azad Medical College (MAMC) for 22 years (1998 to 2020) and Medical Superintendent and officiating Dean, MAMC during 2015-16. He is presently posted as Director Professor & Head of Department of Paediatric Surgery in Lady Hardinge Medical College, New Delhi sinced last 3 months. His special areas of interests are Paediatric Urology, Paediatric Surgical Oncology, and Neonatal Surgery. Besides being an accomplished academician (with total teaching experience of nearly 33 years) and astute administrator, he is a skilled paediatric surgeon with many firsts to his credit. At the age of 33 years, he had successfully separated ischiopagus tetrapus conjoined twins.

Among the many Fellowships that has been conferred upon him, the notable include WHO Fellowship (2001-02) and Commonwealth Fellowship (2003) that helped him to get trained in traumatology in Westmead Hospital, Sydney and in Paediatric Laparoscopic Surgery in St. John's Hospital, Leeds and Westminster Hospital, London respectively. He did Pediatric Surgical Oncology Short Fellowship in St. Jude’s Research Cancer Institute, Memphis (USA) in 2008. He has been conferred Fellowships by many prestigious Academies and Professional bodies such as Association of Indian Surgeons (FAIS; 1996), International Medical Science Academy, International College of Surgeons (Indian Section) (FIMSA; 1998), International College of Surgeons, Indian Section (FICS-Pediatric Surgery; 1998), National Academy of Medical Sciences (India) (FAMS- Pediatric Surgery; 2007), National Neonatology Forum (FNNF; 2017), and Indian Academy of Pediatrics (FIAP; 2019). He was conferred honorary membership of South Africa Children’s Cancer Study Group (SACCSG) in 2009. He was conferred Delhi State Award for Doctors for year 2012-13.

He has been visiting Professor to many universities/ teaching hospitals in India and abroad including Nelson R Mandela School of Medicine, KwaZulu-Natal University, and Red Cross War Memorial Children’s Hospital, Cape town, South Africa in 2009, Boston Children’s Hospital, Boston, USA in 2010, Children Hospital and the Institute of Child Health, Lahore, Pakistan in 2013 and 2015, and Dhaka Shishu (Children’s) Hospital, Dhaka, Bangladesh in 2017 and 2015. He had been an observer in Department of Urology, the Hospital for Sick Children (Sickkids), Toronto in 2014.

He has participated in/ supervised 47 research projects/ thesis and had been part of one multi-site research project with extramural funding.  

He is credited to 280 publications in indexed national and international scientific journals with more than 1000 citations with h-index of 14 and i10-index of 35 (many of them in national and international textbooks). He has contributed many chapters in books and is presently editing a multi-author book on Pediatric Surgery. 

He is also associated with publication of many scientific journals. Besides being reviewer for many medical journals, he is presently Emaritus editor-in-chief of international ‘Journal of Neonatal Surgery’ and editorial board member of Indian Journal of Pediatrics (IJP), Global Journal of Medical Sciences; Pediatric Health, Medicine and Therapeutics; World Journal of Clinical Pediatrics; Open Pediatrics Medicine journal; Frontiers in Surgery, Wudpecker Journal of Educational Research, Advances in Surgical Sciences, Journal of Indian Medical Association (JIMA), Journal of Pediatrics and Neonatal Care and Pediatric Hematology Oncology. He has served on editorial board of three other journals, namely, Indian Journal of Surgery, Indian Pediatrics and Journal of Indian association of Pediatric Surgeons.

He has been a faculty in many scientific conferences and continuing medical education programs in India and abroad and presented more than 250 free papers/ CME lectures. He has been awarded many medals, and honors including IAPS-Ethicon Teacher Travelling Fellowship (2000), IPSO best poster award in Auckland, New Zealand (2011), IJP Travelling Professor Fellowship (2012), Avantika Dhanwantri Sewa Samman’ by NGO Avantika Saikripa Sewa Sansthan (2007), Bhagirathi Award by NGO Bhagirathi Samajik Saanskritik Manch (2011), Shri Santosh Sharma Award 2015 by NGO Santosh Sharma Trust (2016), Rejoice AYUSH Promotion Award 2019 for Yoga by Rejoice Health Foundation (2019) and Bharat ke Anmol Ratan-2019 (Lifetime Achievement Award by Tejasvi Astitva Foundation

He has organized many local and National Conferences/ CME programs. He has been very active in proceedings of Medical Council of India (MCI), National Board of Examinations, Indian Council of Medical Research (ICMR) and Council of Scientific & Industrial Research (CSIR). He has been a member of the 3-member Board of Specialty in Pediatric Surgery by Medical Council of India (MCI) to suggest changes in curriculum in 2010 and was member of postgraduate (super-specialty) committee of MCI in 2013. He has served member of Advisory Committee of CSIR for Shanti Swarup Bhatnagar (SSB) Prize for Science & Technology in Medical Sciences in the years 2013 and 2016. He has been member of ICMR Project Review Committee for the Task Force Project on Descriptive Epidemiology of Childhood injuries since 2013 till date.

He has been an active member of more than 20 national and international scientific bodies and associations. He has played a very long inning on IAPS in different capacities since 1997. He was Secretary-cum treasurer of IAPS in 2005-07. As Secretary IAPS, he was responsible for creation of many sections/ chapters of IAPS, consolidation of different IAPS funds and drafting of revised constitution. He was singularly responsible for the creation of Delhi Chapter of IAPS in 1999. He served as executive member and later Secretary cum treasurer of Delhi Chapter of IAPS. He has served as the Secretary cum treasurer of Paediatric Urology Chapter of IAPS. He had been Chairperson of Basic Research Section of IAPS. He had been also executive member of Pediatric Endoscopic society of India (PESI-IAPS).

He had been executive member of Delhi Chapter of IAP for the years 2011, 2016 and 2018. He has served as Treasurer and Joint Secretary, Indian Child Abuse, Neglect, and Child Labor (ICANCL) Group of IAP in the year 2015-16 and its Chairperson for Delhi-NCR during 2018-19. He is presently Convener of Spina Bifida Foundation- India Delhi/ NCR region and executive member of Delhi Chapter of IAP.

He has been an active member of NNF since 1998 and in the year 2000, he was nominated as IAPS nominee to have better coordination with IAPS and NNF.

He has been organising 3-4-day long Pediatric Surgery Annual Update since 2008 (12 years in a row) that is attended by as many as 50-120 MCh/ DNB students for which no registration fee has been ever charged; subsidized accommodation is provided. About 40 faculty members from India and abroad assemble every year in MAMC New Delhi to teach the students. The stawlarts who have graced these updates include notable faculty from India and abroad. 

Last not but the least, he has been associated with charitable voluntary work and NGOs such as Indian Alliance for Child Rights and Udaan that works for rehabilitation of spastic children. He has organised many free operative camps in far flung areas such as Port Blair, Ujjain. He has been also an avid Yoga practitioner and has special interest in horticulture and landscaping. He recently did a Course on Health Promotion through Ayurveda and Yoga conducted by Indian Red Cross Society, New Delhi (fity hours program) in collaboration with Central Council of Research in Ayurveda and Sidha and Morarji Desai Institute of Yoga.



Holschneider A, Hutson J, Peña A, Beket E, Chatterjee S, Coran A, et al. Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg. 2005;40:1521-6.

Sarin YK, Sinha A. Two orifices in the perineum of a girl with imperforate anus: possibility of uterovaginal agenesis associated with rectovestibular fistula. J Pediatr Surg. 2002; 37:1217-9.

Cook RCM. Anorectal malformations. In: Neonatal Surgery. 3rd edition. Edited by Lister J, Irving IM. London, England, Butterworth 1990, p. 547-70.

Galifer RB, Chavrier Y, Viala JS. Principles of the surgical treatment of cloacal malformation. Apropos of 3 cases. Chir Pediatr. 1985; 26:95-103.

Peña A. Anorectal malformations. Semin Pediatr Surg. 1995; 4:35-47.

Peña A. Imperforate anus and cloacal malformations. In: Pediatric Surgery. 3rd edition. Edited by Ashcraft KW. Saunders, Philadelphia, 2000. p. 473-92.

Choudhury SR, Khan NA, Debnath PR, Yadav PS, Shah S, Chadha R. Anorectal agenesis with rectovaginal fistula: a rare/regional variant. J Indian Assoc Pediatr Surg. 2017; 22:79-82.

Torres R, Levitt MA, Tovilla JM, Rodriguez G, Peña A. Anorectal malformations and Down's syndrome. J Pediatr Surg. 1998; 33:194-7.

Bischoff A, Frischer J, Dickie BH, Peña A. Anorectal malformation without fistula: a defect with unique characteristics. Pediatr Surg Int. 2014; 30:763-6.

Sarin YK, Pathak D, Sengar M. Bowel vaginoplasty in children. J Indian Assoc Pediatr Surg. 2006; 11:92-96.

Sinha A, Sarin YK. Mayer-Rokitansky Syndrome with imperforate anus - a unique association. J Indian Assoc Pediatr Surg. 2001; 6:30-3.

Gross RE. Malformation of the anus and rectum. In: The surgery of infancy and childhood. Edited by Gross RE. Philadelphia, PA: Saunders Company; 1953. p. 348–68.

Wakhlu A, Kureel SN, Tandon RK, Wakhlu AK. Long-term results of anterior sagittal anorectoplasty for the treatment of vestibular fistula. J Pediatr Surg. 2009; 44:1913-9.

De la Torre L, Cogley K, Calisto JL, Santos K, Ruiz A, Zornoza M. Vaginal agenesis and rectovestibular fistula. Experience utilizing distal ileum for the vaginal replacement in these patients, preserving the natural fecal reservoir. J Pediatr Surg. 2016; 51:1871-6.

Levitt MA, Stein DM, Peña A. Rectovestibular fistula with absent vagina: A unique anorectal malformation. J Pediatr Surg. 1998; 33:986-99.

Skerritt C, Vilanova Sánchez A, Lane VA, Wood RJ, Hewitt GD, Breech LL, et al. Menstrual, sexual, and obstetrical outcomes after vaginal replacement for vaginal atresia associated with anorectal malformation. Eur J Pediatr Surg. 2017; 27:495-502.

Ahmed H, Almomani M, Strine AC, Reddy PP, Sheldon C, Frischer J, et al. Clinical urologic and urodynamic outcomes in patients with anorectal malformation and absent vagina after vaginal replacement [published online ahead of print, 2020 Feb 4]. J Pediatr Surg. 2020;S0022-3468(20)30093-2.

Levitt MA, Bischoff A, Breech L, Peña A. Rectovestibular fistula--rarely recognized associated gynecologic anomalies. J Pediatr Surg. 2009; 44:1261-7.

Cohn BD, Murphy DR. Imperforate anus with agenesis of the vagina. Ann Surg. 1956; 143:430-2.

Ein SH, Stephens CA. Vaginal construction in children with absent vagina and imperforate anus. J Pediatr Surg. 1971; 6:435-9.

Fujiwara Y, Oizumi T, Sasahara M, Kato E, Kakizaki G. A case of congenital imperforate anus and absent vagina with a functioning uterus. Tohoku J Exp Med. 1974; 113:283-9.

King SL, Ladda RL, Shochat SJ. Monozygotic twins concordant for tracheoesophageal fistula and discordant for the VATER association. Acta Paediatr Scand. 1977; 66:783-5.

Tolete-Velcek F, Hansbrough F, Kugaczewski J, Coren CV, Klotz DH, Price AF, et al. Utero vaginal malformations: a trap for the unsuspecting surgeon. J Pediatr Surg. 1989; 24:736-40.

Digray NC, Mengi Y, Goswamy HL, Thappa DR. Rectovaginoplasty for vaginal atresia with anorectal malformation. J Urol. 1999; 162:514-5.

Okoye BO, Parikh DH, Buick RG, Lander AD. Pyloric atresia: five new cases, a new association, and a review of the literature with guidelines. J Pediatr Surg. 2000; 35:1242-5.

Adejuyigbe O, Sowande OA, Olayinka OS, Fasubaa OB. Rectovestibular fistula with absent distal vagina in an adolescent Nigerian girl. J Pediatr Surg. 2002; 37:1479-80.

Günşar C, Genç A, Sencan A, Dağlar Z, Alparslan O, Mir E. MURCS association and rectovestibular fistula: case report of a patient treated with one-stage posterior sagittal anorectoplasty and sigmoid loop vaginoplasty. J Pediatr Surg. 2003; 38:262-4.

Tei E, Yamataka A, Segawa O, Kobayashi H, Lane GJ, Tobayama S, et al. Laparoscopically assisted anorectovaginoplasty for selected types of female anorectal malformations. J Pediatr Surg. 2003; 38:1770-4.

Deshpande AV, Sanghani HH, Sanghavi BV, Borwankar SS. Delayed presentation of vaginal agenesis with anorectal malformation—a unique problem. Ind J Ped Surg. 2003; 8:242-4.

Patankar JZ, Mali VP, Yashpal R, Neo GTH, Prabhakaran K. Anorectal malformation with congenital absence of vagina: a case report and review of the literature. Pediatr Surg Int. 2004; 20:295-7.

Patankar SP, Kalrao V, Patankar SS. Mayer-Rokitansky syndrome and anorectal malformation. Indian J Pediatr. 2004; 71:1133-5.

Banu T, Hannan MJ, Aziz MA, Hoque M, Laila K. Rectovestibular fistula with vaginal malformations. Pediatr Surg Int. 2006; 22:263-6.

Wester T, Läckgren G, Christofferson R, Rintala RJ. The congenital pouch colon can be used for vaginal reconstruction by longitudinal splitting. J Pediatr Surg. 2006; 41:e25–e28.

Komura M, Kanamori Y, Sugiyama M, Tomonaga T, Suzuki K, Hashizume K, et al. A female infant who had both complete VACTERL association and MURCS association: report of a case. Surg Today. 2007; 37:878-80.

Matignas ALA, Delos Reyes RH. Rectovestibular fistula as neovagina in congenital cervico-vaginal agenesis associated with imperforate anus. J Obstet Gynaecol Res. 2008; 34:428-35.

Chatterjee SK. Rare/regional variants. In: Anorectal malformations in children. Edited by Holschneider AM, Hutson JM. Berlin Heidelberg New York: Springer; 2008.p. 251-62.

Wang S, Lang JH, Zhu L. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with rectovestibular fistula and imperforate anus. Eur J Obstet Gynecol Reprod Biol. 2010; 153:77-80.

Mane SB, Shastri P, Dhende NP, Obaidah A, Acharya H, Reddy S, et al. Our 10-year experience of variable Müllerian anomalies and its management. Pediatr Surg Int. 2010; 26:795-800.

Wester T, Tovar JA, Rintala RJ. Vaginal agenesis or distal vaginal atresia associated with anorectal malformations. J Pediatr Surg. 2012; 47:571-6.

Yamataka A, Goto S, Kato Y, Koga H, Lane GJ, Okazaki T. Fecal and urinary continence after scope-assisted anorectovaginoplasty for female anorectal malformation. Pediatr Surg Int. 2012; 28:907-12.

Jessel RH, Laufer MR. Management of lower vaginal agenesis in a patient with unicornuate uterus. J Pediatr Adolesc Gynecol. 2013; 26:e21–e23.

de Blaauw I, Midrio P, Breech L, Bischoff A, Dickie B, Versteegh HP, et al. Treatment of adults with unrecognized or inadequately repaired anorectal malformations: 17 cases of rectovestibular and rectoperineal fistulas. J Pediatr Adolesc Gynecol. 2013; 26:156-60.

Kisku S, Barla RK, Sen S, Karl S, Mathai J, Varghese L. Rectovestibular fistula with vaginal atresia: our experience and a proposed course of management. Pediatr Surg Int. 2014; 30:6333-9.

Kapczuk K, Friebe Z, Iwaniec K, Kędzia W. Creation of a neovagina in a patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and previously corrected rectovestibular fistula concomitant with imperforate anus. J Pediatr Adolesc Gynecol. 2015; 28:e17–9.

Pandya KA, Koga H, Okawada M, Coran AG, Yamataka A, Teitelbaum DH. Vaginal anomalies and atresia associated with imperforate anus: diagnosis and surgical management. J Pediatr Surg. 2015; 50:431-7.

Teo XL, Narasimhan KL, Chua JH. Müllerian agenesis in the presence of anorectal malformations in female newborns: a diagnostic challenge. Singapore Med J. 2015; 56:e82–e84.

Gupta R, Gupta A, Gupta A, Singh A. Mayer- Rokitansky-Küster-Hauser Syndrome with Imperforate Anus: A Rare Association and an Innovative Surgical Management. IJSS Case Rep Reviews. 2016; 2:1-3.

Bjørsum-Meyer T, Herlin M, Qvist N, Petersen MB. Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence: two case reports and a review of the literature. J Med Case Rep. 2016; 10:374.

Tiwari C, Shah H, Bothra J, Sandlas G. Congenital rectovaginal fistula with anorectal agenesis: A rare anorectal malformation. Int J Pediatr Adolesc Med. 2017; 4:138-40.

Kölle A, Taran FA, Rall K, Schöller D, Wallwiener D, Brucker SY. Neovagina creation methods and their potential impact on subsequent uterus transplantation: a review. BJOG. 2019; 126:1328-35.

Mayer CA. [About doubling of the uterus and its types, along with remarks about rabbits and wolf throats] J Chir Augen. 1829; 13:525-64. [Article in German]

Rokitansky KF. [About the so-called doubling of the uterus]. Med Jahrb Österr Staat. 1838; 26:39-77. [Article in German]

Kuester H. [Rudimentary solid bipartite uterus with solid vagina]. Z Geburtshilfe Gynäkol. 1910; 67;692-718. [Article in Latin]

Hauser GA, Schreiner WE. [Das Mayer-Rokitansky-Küster-Syndrome]. Schweiz Med Wochenschr. 1961; 91:381-4. [Article in German]

Schmid-Tannwald I, Hauser GA. [Atypical forms of the Mayer-Rokitansky-Küster syndrome]. Geburtshilfe Frauenheilkd. 1977; 37:386-92. [Article in German]

Duncan PA, Shapiro LR, Stangel JJ, Klein RM, Addonizio JC. The MURCS association: Müllerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. J Pediatr. 1979; 95:399-402.

Liszewska-Kapłon M, Strózik M, Kotarski Ł, Bagłaj M, Hirnle L. Mayer-Rokitansky-Küster-Hauser syndrome as an interdisciplinary problem. Adv Clin Exp Med. 2020; 29:505-11.

Oppelt P, Renner SP, Kellermann A, Brucker S, Hauser GA, Ludwig KS, et al. Clinical aspects of Mayer-Rokitansky-Küster-Hauser syndrome: recommendations for clinical diagnosis and staging. Hum Reprod. 2006; 21:792-7.

Deng S, He Y, Chen N, Zhu L. Spectrum of type I and type II syndromes and associated malformations in Chinese patients with Mayer-Rokitansky-Küster-Hauser syndrome: A retrospective analysis of 274 cases. J Pediatr Adolesc Gynecol. 2019; 32:284-7.

Oppelt P, Renner SP, Brucker S, Strissel PL, Strick R, Oppelt PG, et al. The VCUAM (Vagina Cervix Uterus Adnex-associated Malformation) classification: a new classification for genital malformations. Fertil Steril. 2005; 84:1493-7.

Kisku S, Verghese L, Kekre A, Sen S, Karl S, Mathai J, et al. Bowel vaginoplasty in children and young women: an institutional experience with 55 patients. Int Urogynecol J. 2015; 26:1441-8.

Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, et al. The ESHRE/ ESGE consensus on the classification of female genital tract congenital anomalies. Hum Reprod. 2013; 28:2032-44.

Buttram VC Jr, Gomel V, Siegler A, DeCherney A, Gibbons W, March C. American Fertility Society. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion secondary due to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions. Fertil Steril. 1988; 49:944-55.

Acién P, Acién M, Sánchez-Ferrer M. Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod. 2004; 19:2377-84.

Callens N, De Cuypere G, De Sutter P, Monstrey S, Weyers S, Hoebeke P, et al. An update on surgical and non-surgical treatments for vaginal hypoplasia. Hum Reprod Update. 2014; 20:775-801.

Committee on Adolescent Health Care. ACOG Committee opinion No. 728: Müllerian agenesis: Diagnosis, management, and treatment. Obstet Gynecol. 2018; 131:e35–e42.

Cheikhelard A, Bidet M, Baptiste A, Viaud M, Fagot C, Khen-Dunlop N, et al. Surgery is not superior to dilation for the management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome: A multicenter comparative observational study in 131 patients. Am J Obstet Gynecol. 2018; 219:281.e1-281.e9.

Adeyemi-Fowode OA, Dietrich JE. Assessing the experience of vaginal dilator use and potential barriers to ongoing use among a focus group of women with Mayer-Rokitansky-Küster-Hauser syndrome. J Pediatr Adolesc Gynecol. 2017; 30:491-4.

Liszewska-Kapłon M, Strózik M, Kotarski Ł, Bagłaj M, Hirnle L. Mayer-Rokitansky-Küster-Hauser syndrome as an interdisciplinary problem. Adv Clin Exp Med. 2020; 29:505-11.

Frank RT. The formation of an artificial vagina without operation. Am J Obstet Gynecol. 1938; 35:1053-5.

Ingram JM. The bicycle seat stool in the treatment of vaginal agenesis and stenosis: A preliminary report. Am J Obstet Gynecol. 1981; 140:867-73.

Williams JK, Lake M, Ingram JM. The bicycle seat stool in the treatment of vaginal agenesis and stenosis. J Obstet Gynecol Neonatal Nurs. 1985; 14:147-50.

McQuillan SK, Grover SR. Dilation and surgical management in vaginal agenesis: A systematic review. Int Urogynecol J. 2014; 25:299-311.

Edmonds DK, Rose GL, Lipton MG, Quek J. Mayer-Rokitansky-Küster-Hauser syndrome: A review of 245 consecutive cases managed by a multidisciplinary approach with vaginal dilators. Fertil Steril. 2012; 97:686-90.

Ketheeswaran A, Morrisey J, Abbott J, Bennett M, Dudley J, Deans R. Intensive vaginal dilation using adjuvant treatments in women with Mayer-Rokitansky-Küster-Hauser syndrome: Retrospective cohort study. Aust N Z J Obstet Gynaecol. 2018; 58:108-13.

Baldwin JF. The formation of an artificial vagina by intestinal transplantation. Ann Surg. 1904; 40:398-403.

Lima M, Ruggeri G, Randi B, Domini M, Gargano T, Pergola EL, et al. Vaginal replacement in the pediatric age group: a 34-year experience of intestinal vaginoplasty in children and young girls. J Pediatr Surg. 2010; 45:2087-91.

Hensle TW, Reiley EA. Vaginal replacement in children and young adults. J Urol. 1998; 159:1035-38.

Georgas K, Belgrano V, Andréasson M, Elander A, Selvaggi G. Bowel vaginoplasty: A systematic review. J Plast Surg Hand Surg. 2018; 52:265-73.

Özkan Ö, Özkan Ö, Çinpolat A, Dogan NU, Bektas U, Doley K, et al. Vaginal reconstruction with the modified rectosigmoid colon: Surgical technique, long-term results and sexual outcomes. J Plast Surg Hand Surg. 2018; 52:210-6.

van der Sluis WB, Bouman MB, Meijerink WJHJ, Elfering L, Mullender MG, de Boer NKH, et al. Diversion neovaginitis after sigmoid vaginoplasty: Endoscopic and clinical characteristics. Fertil Steril. 2016; 105:834.e1.-839.e1.

Djordjevic ML, Stanojevic DS, Bizic MR. Rectosigmoid vaginoplasty: clinical experience and outcomes in 86 cases. J Sex Med. 2011; 8:3487-94.

Zhang M, Li S, Huang X, Du H, Wang C, Zhang L, et al. Transumbilical single-incision laparoscopic vaginoplasty hybrid transperineal approach using a sigmoid colon segment: Initial twenty-five cases. Int Urol Nephrol. 2016; 48:1401-6.

Boztosun A, Olgan S. Robotic sigmoid vaginoplasty in an adolescent girl with Mayer-Rokitansky-Küster-Hauser syndrome. Female Pelvic Med Reconstr Surg. 2016; 22:e32-e35.

Gauthier T, Lavoue V, Piver P, Aubard Y, Ayoubi JM, Garbin O, et al. Which neovagina reconstruction procedure for women with Mayer-Rokitansky-Küster-Hauser syndrome in the uterus transplantation era? Editorial from the French Uterus Transplantation Committee (CETUF) of CNGOF. J Gynecol Obstet Hum Reprod. 2018; 47:175-6.

Vecchietti G. [Neovagina in the syndrome of in Rokitansky-Küster-Hauser]. Attualita Obstet Ginecol. 1965; 11:131-47. [Article in Italian]

Vecchietti G. [The neo-vagina in Rokitansky syndrome-Küster-Hauser]. Rev Med Suisse Romande. 1979; 99:593. [Article in French]

Fedele L, Bianchi S, Frontino G, Fontana E, Restelli E, Bruni V. The laparoscopic Vecchietti’s modified technique in Rokitansky syndrome: anatomic, functional, and sexual long-term results. Am J Obstet Gynecol. 2008; 198:377.e1-377.e6.

Borruto F, Camoglio FS, Zampieri N, Fedele L. The laparoscopic Vecchietti technique for vaginal agenesis. Int J Gynaecol Obstet. 2007; 98:15-9.

Brucker SY, Gegusch M, Zubke W, Rall K, Gauwerky JF, Wallwiener D. Neovagina creation in vaginal agenesis: Development of a new laparoscopic Vecchietti-based procedure and optimized instruments in a prospective comparative interventional study in 101 patients. Fertil Steril. 2008 ;90:1940-52.

Rall K, Schickner MC, Barresi G, Schonfisch B, Wallwiener M, Wallwiener CW, et al. Laparoscopically assisted neovaginoplasty in vaginal agenesis: A long-term outcome study in 240 patients. J Pediatr Adolesc Gynecol. 2014; 27:379-85.

McIndoe AH, Bannister JB. An operation for the cure of congenital absence of the vagina. J Obstet Gynecol Brit Emp. 1938; 45:490-4.

McIndoe A. The treatment of congenital absence and obliterative conditions of the vagina. Br J Plast Surg. 1950; 2:254-67.

Cali RW, Pratt JH. Congenital absence of the vagina. Long-term results of vaginal reconstruction in 175 cases. Am J Obstet Gynecol. 1968; 100:752-63.

Han SE, Go JY, Choi DS, Seo GH, Lim SY. Experience with specially designed pored polyacetal mold dressing method used in McIndoe-style vaginoplasty. J Pediatr Urol. 2017; 13:621.e1-e621.e6.

McQuillan SK, Grover SR. Dilation and surgical management in vaginal agenesis: A systematic review. Int Urogynecol J. 2014; 25:299-311.

Hopkins MP, Morley GW. Squamous cell carcinoma of the neovagina. Obstet Gynecol. 1987; 69:525-7.

Baltzer J, Zander J. Primary squamous cell carcinoma of the neovagina. Gynecol Oncol 1989; 35:99-103.

Idrees MT, Deligdisch L, Altchek A. Squamous papilloma with hyperpigmentation in the skin graft of the neovagina in Rokitansky syndrome: Literature review of benign and malignant lesions of the neovagina. J Pediatr Adolesc Gynecol. 2009; 22:e148–e155.

Williams EA. Congenital absence of the vagina; a simple operation for its relief. J Obstet Gynecol Commonw. 1964; 71:511–4.

Creatsas G, Deligeoroglou E, Makrakis E, Kontoravdis A, Papadimitriou L. Creation of a neovagina following Williams vaginoplasty and the Creatsas modification in 111 patients with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. 2001; 76:1036–40.

Sheares BH. Congenital atresia of the vagina—a technique for tunneling the space between the bladder and rectum and construction of a new vagina by a modified Wharton-technique. J Obstet Gynecol Br Emp. 1960; 67:24-31.

Kuessel L, Wenzl R, Marschalek ML, Slavka G, Doerfler D, Husslein H. Using the Wharton-Sheares-George method to create a neovagina in patients with Mayer-Rokitansky-Küster-Hauser syndrome: a step-by-step video tutorial. Fertil Steril. 2016; 106:e20-e21.

Kriplani A, Karthik SD, Kriplani I, Kachhawa G. Laparoscopic peritoneal vaginoplasty for Mayer–Rokitansky–Küster–Hauser syndrome: an experience at a tertiary care center. J Gynecol Surg. 2018; 34:63-7.

Bianchi S, Frontino G, Ciappina N, Restelli E, Fedele L. Creation of a neovagina in Rokitansky syndrome: comparison between two laparoscopic techniques. Fertil Steril. 2011; 95:1098-100.e1-3.

Fedele L, Frontino G, Restelli E, Ciappina N, Motta F, Bianchi S. Creation of a neovagina by Davydov’s laparoscopic modified technique in patients with Rokitansky syndrome. Am J Obstet Gynecol. 2010; 202:33.e1-e6.

Ding JX, Chen LM, Zhang XY, Zhang Y, Hua KQ. Sexual and functional outcomes of vaginoplasty using acellular porcine small intestinal submucosa graft or laparoscopic peritoneal vaginoplasty: a comparative study. Hum Reprod. 2015; 30:581-9.

Zhang X, Ding Y, Hua K, Liu S, Jia N. Combined Laparoscopic and Vaginal Cervicovaginal Reconstruction Using Acellular Porcine Small Intestinal Submucosa Graft in a Patient with Mayer-Rokitansky-Küster-Hauser Syndrome (U5aC4V4). J Minim Invasive Gynecol. 2019; 26:396-7.

Ozkan O, Akar ME, Ozkan O, Erdogan O, Hadimioglu N, Yilmaz M, et al. Preliminary results of the first human uterus transplantation from a multiorgan donor. Fertil Steril. 2013; 99:470–6.

Brännström M, Dahm-Kähler P. Uterus transplantation and fertility preservation. Best Pract Res Clin Obstet Gynaecol. 2019; 55:109-16.

Flyckt R, Falcone T, Eghtesad B, Fung J, Tzakis A. Uterus transplantation: medical considerations. Curr Transpl Rep. 2016; 3:380-4.

Fageeh WRH, Jabbad H, Marzouki A. Transplantation of the human uterus. Int J Gynaecol Obstet. 2002; 76:245–51.

Erman A, Ozkan O, Aydinuraz B, Dirican K, Cincik M, Mendilcioglu I, et al. Clinical pregnancy after uterus transplantation. Fertil Steril. 2013; 100:1358-63.

Brännström M, Diaz-Garcia C, Hanafy A, Olausson M, Tzakis A. Uterus transplantation: animal research and human possibilities. Fertil Steril. 2012; 97:1269-76.

Brännström M, Johannesson L, Dahm-Kahler P, Enskog A, Molne J, Kvarnstrom N, et al. First clinical uterus transplantation trial: a six month report. Fertil Steril. 2014; 101:1228-36.

Johannesson L, Jarvholm S. Uterus transplantation: current progress and future prospects. Int J Womens Health. 2016; 8:43-51.

Kisu I, Liu Y, Chen G, Song MJ, Chang CY, Koon TH, et al. Current progress in uterus transplantation research in Asia. J Clin Med. 2019; 8:245.

Daolio J, Palomba S, Paganelli S, Falbo A, Aguzzoli L Uterine transplantation and IVF for congenital or acquired uterine factor infertility: A systematic review of safety and efficacy outcomes in the first 52 recipients. PLoS ONE. 2020; 15:e0232323.

Gautheir T, Piver P, Pichon N, Bibes R, Guillaudeau A, Piccardo A, et al. Uterus retrieval process from brain dead donors. Fertil Steril. 2014; 102:476-82.

Johannesson L, Kvarnström N, Mölne J, Dahm-Kähler P, Enskog A, Diaz-Garcia C, et al. Uterus transplantation trial: 1-year outcome. Fertil Steril. 2015; 103:199-204.

Flyckt R, Davis A, Farrell R, Zimberg S, Tzakis A, Falcone T. Uterine Transplantation: Surgical Innovation in the Treatment of Uterine Factor Infertility. J Obstet Gynaecol Can. 2018; 40:86-93.

Puntambekar S, Telang M, Kulkarni P, Jadhav S, Sathe R, Warty N, et al. Laparoscopic-assisted uterus retrieval from live organ donors for uterine transplant. J Minim Invasive Gynecol. 2018; 25:571-2.

Georgopapadakos N, Manoli A, Passia G, Skandalakis PN, Filippou D. Uterus transplantation as a therapy method in Mayer-Rokitansky-Küster-Hauser Syndrome. Cureus. 2019; 11:e6333.

Ejzenberg D, Andraus W, Baratelli Carelli Mendes LR, Ducatti L, Song A, Tanigawa R, et al. Livebirth after uterus transplantation from a deceased donor in a recipient with uterine infertility. Lancet. 2019; 392:2697-704.

Pluta D, Lemm M, Franik G, Kowalcyzk K, Blucakz L, Tekieli-Balon A, et al. Mayer-Rokitansky-Küster-Hauser syndrome - case studies, methods of treatment and the future prospects of human uterus transplantation. Eur Rev Med Pharmacol Sci. 2020; 24:549-63.

Brännström M, Johannesson L, Bokström H, Kvarnström N, Mölne J, Dahm-Kähle P, et al. Livebirth after uterus transplantation. Lancet. 2015; 385:607-16.



How to Cite

Sarin YK. Association of the rectovestibular fistula with MRKH Syndrome and the paradigm shift in the management in view of the future uterine transplant. J Neonatal Surg [Internet]. 2020Oct.30 [cited 2024Jul.22];9:5. Available from:

Most read articles by the same author(s)

<< < 1 2 3 4 > >>