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Management of an unusual case of atypical Mayer–Rokitansky–Kuster–Hauser syndrome, with unilateral gonadal agenesis, solitary ectopic pelvic kidney, and pelviureteric junction obstruction

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Abstract

Congenital absence of uterus and vagina, Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome, is mullerian agenesis and is the second most frequent cause of primary amenorrhea. Only atypical form of MRKH (type B) is associated with renal skeletal and ovarian abnormalities. We report the management of an unusual case of atypical MRKH, unilateral gonadal agenesis, and solitary ectopic pelvic kidney with pelviureteric junction obstruction (PUJO). After doing thorough Medline search, to the best of our knowledge, this is the first case reported with this combination.

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References

  1. Chervenak F, Stangel J, Nemec M, Amin H (1982) Mayer–Rokitansky–Kuster–Hauser syndrome. N Y State J Med 82:23–27

    PubMed  CAS  Google Scholar 

  2. Willemsen WN, Lemmens JA et al (1993) Mayer Rokitansky Kuster Hauser syndrome: distinction between two forms based on excretory urographic sonographic and laparoscopic findings. Am J Roentgenol 160:331–334

    Google Scholar 

  3. Heidenreich W (1988) Genitale and extragenitale Fehlbildungen beim Mayer–Rokitansky–Kuster syndrom. Dtsch Med Wochenschr 113:1092–1096

    Article  PubMed  CAS  Google Scholar 

  4. Carranza-Lira S, Forbin K, Martinez-Chequer JC (1999) Rokitansky syndrome and MURCS association-clinical features and basis for diagnosis. Int J Fertil Womens Med 44:250–255

    PubMed  CAS  Google Scholar 

  5. Gorrgojo JJ, Almodovar F, Lopez E, Donnay S (2002) Gonadal agenesis 46, XX associated with the atypical form of Rohitansky syndrome. Fertil Steril 77(1):185–187

    Article  Google Scholar 

  6. Murphy A, Krall A, Rock J (1987) Bilateral functioning uterine anlagen with the Mayer–Rokitansky–Kuster–Hauser syndrome. Int J Fertil 32:316–319

    PubMed  CAS  Google Scholar 

  7. Cramer DW, Goldstein DP, Fraer C, Reichardt JK (1996) Vaginal agenesis (Mayer–Rokitansky–Kuster–Hauser syndrome) associated with the N314D mutation of galactose-1-phosphate uridyl transferase (GALT). Mol Hum Reprod 2:145–148

    Article  PubMed  CAS  Google Scholar 

  8. Rock JA (1997) Surgery for anomalies of the mullerian ducts. In: Rock JA, Thompson JD (eds) TE Linde’s operative gynecology, 8th edn. Williams & Wilkins, Philadelphia, pp 687–729

    Google Scholar 

  9. Schmid-Tannwald I, Hauser G (1977) Deutung der “atypischen” Formen des Mayer–Rokitansky–Kuster Syndroms. Geburtshilfe Frauenheilkd 37:386–392

    PubMed  CAS  Google Scholar 

  10. Kaya H, Seizik M, Ozkaya O, Kose SA (2003) Mayer–Rokitansky–Kuster–Hauser syndrome associated with unilateral gonadal agenesis. A case report. J Reprod Med 48(11):902–904

    PubMed  Google Scholar 

  11. Davis DE, Wolf JS (2005) Laparoscopic pyelovesicostomy for ureteropelvic junction obstruction in pelvic kidney. J Endocrinol 19(4):469–470

    Google Scholar 

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Authors and Affiliations

  1. Department of Urology, All India Institute of Medical Sciences, New Delhi, India

    Anup Kumar, Saurabh Mishra & P. N. Dogra

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  1. Anup Kumar
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  2. Saurabh Mishra
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  3. P. N. Dogra
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Correspondence to P. N. Dogra.

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Kumar, A., Mishra, S. & Dogra, P.N. Management of an unusual case of atypical Mayer–Rokitansky–Kuster–Hauser syndrome, with unilateral gonadal agenesis, solitary ectopic pelvic kidney, and pelviureteric junction obstruction. Int Urogynecol J 18, 823–825 (2007). https://doi.org/10.1007/s00192-006-0238-z

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  • DOI: https://doi.org/10.1007/s00192-006-0238-z

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