Summary: A fluid-filled VAGINA that is obstructed.

Top Publications

  1. Shono T, Taguchi T, Suita S, Nakanami N, Nakano H. Prenatal ultrasonographic and magnetic resonance imaging findings of congenital cloacal anomalies associated with meconium peritonitis. J Pediatr Surg. 2007;42:681-4 pubmed
    ..Prenatal ultrasonograms (US) of cloacal malformations sometimes show ascites, hydrocolpos, and hydronephrosis...
  2. Bischoff A, Levitt M, Breech L, Louden E, Pena A. Hydrocolpos in cloacal malformations. J Pediatr Surg. 2010;45:1241-5 pubmed publisher
    b>Hydrocolpos is a condition rarely mentioned in the literature...
  3. Cherian M, Al Sanna a N, Ayyat F. Hydrometrocolpos and acute renal failure: a rare neonatal presentation of Bardet-Biedl syndrome. J Pediatr Urol. 2008;4:313-6 pubmed publisher
    ..The patient developed end-stage renal failure towards the end of her first decade, possibly due to underlying renal pathology associated with Bardet-Biedl syndrome. ..
  4. Drut R. Squamous cell peritonitis associated with hydrometrocolpos in a multimalformed newborn. Fetal Pediatr Pathol. 2005;24:161-8 pubmed
    ..The possible relationship of the SUA with the constellation of infradiaphragmatic malformations is discussed. ..
  5. Hulleman J, Nguyen A, Ramprasad V, Murugan S, Gupta R, Mahindrakar A, et al. A novel H395R mutation in MKKS/BBS6 causes retinitis pigmentosa and polydactyly without other findings of Bardet-Biedl or McKusick-Kaufman syndrome. Mol Vis. 2016;22:73-81 pubmed
    ..Our observations reaffirm the notion that mutations in MKKS/BBS6 cause phenotypic heterogeneity and do not always result in classic MKKS or BBS findings. ..
  6. Dosedla E, Kacerovsky M, Calda P. Prenatal diagnosis of hydrometrocolpos in a Down syndrome fetus. J Clin Ultrasound. 2011;39:169-71 pubmed publisher
    ..In this case, sonographic evaluation in the 37th week revealed a large retrovesical, sharply marginated, heterogeneous, unilocular cystic structure within the fetal abdomen extending to the left side of the umbilicus. ..
  7. Hayashi S, Sago H, Kashima K, Kitano Y, Kuroda T, Honna T, et al. Prenatal diagnosis of fetal hydrometrocolpos secondary to a cloacal anomaly by magnetic resonance imaging. Ultrasound Obstet Gynecol. 2005;26:577-9 pubmed
    ..MRI is a useful complementary tool for assessing fetal urogenital anomalies when ultrasonography is inconclusive. ..
  8. Kos S, Roth K, Korinth D, Zeilinger G, Eich G. Hydrometrocolpos, postaxial polydactyly, and hypothalamic hamartoma in a patient with confirmed Pallister-Hall syndrome: a clinical overlap with McKusick-Kaufman syndrome. Pediatr Radiol. 2008;38:902-6 pubmed publisher
    ..This is the second genetically confirmed case revealing the previously described association of PHS with hydrometrocolpos due to vaginal atresia as a clinical overlap with MKKS. ..
  9. Sumi A, Sato Y, Kakui K, Tatsumi K, Fujiwara H, Konishi I. Prenatal diagnosis of anterior sacral meningocele. Ultrasound Obstet Gynecol. 2011;37:493-6 pubmed publisher

More Information


  1. Wu T, Wu T, Ng Y, Ng S, Su P, Chen J, 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 pubmed publisher
    ..The patient presented with a protruding mass over the vaginal introitus that was associated with an obstructed hemivagina and uterine didelphys. ..
  2. Güdücü N, Gönenç G, Isci H, Yigiter A, Dunder I. Herlyn-Werner-Wunderlich syndrome--timely diagnosis is important to preserve fertility. J Pediatr Adolesc Gynecol. 2012;25:e111-2 pubmed publisher
    ..When unilateral renal agenesis and uterus didelphys coexist the first thing that we should remember is to confirm or refute the presence of a blind vagina. ..
  3. de Jesus L, Dekermacher S, Manhães C, Faria L, Barros M. Acquired labial sinechiae and hydrocolpos secondary to Stevens-Johnson syndrome. Urology. 2012;80:919-21 pubmed publisher
    ..After a diagnosis of Stevens-Johnson syndrome in girls, it is prudent to schedule a prepubertal genital examination to diagnose genital disease preemptively and avoid obstructed menstruation and future sexual problems. ..
  4. Valentini A, Giuliani M, Gui B, Laino M, Zecchi V, Rodolfino E, et al. Persistent Urogenital Sinus: Diagnostic Imaging for Clinical Management. What Does the Radiologist Need to Know?. Am J Perinatol. 2016;33:425-32 pubmed publisher
    ..tract system (33%) such as intersex, rectovaginal communication, bladder agenesis, absence of vagina, and hydrocolpos. The correct radiological assessment of PUGS is especially useful for clinicians since the exact anatomical ..
  5. Cianciosi A, Mancini F, Busacchi P, Carletti A, de Aloysio D, Battaglia C. Increased amniotic fluid volume associated with cloacal and renal anomalies. J Ultrasound Med. 2006;25:1085-90 pubmed
  6. Akimoto C, Sakashita E, Kasashima K, Kuroiwa K, Tominaga K, Hamamoto T, et al. Translational repression of the McKusick-Kaufman syndrome transcript by unique upstream open reading frames encoding mitochondrial proteins with alternative polyadenylation sites. Biochim Biophys Acta. 2013;1830:2728-38 pubmed
    ..Two uORFs are translated in vivo and imported onto the mitochondrial membrane. Our findings provide unique insights into production of uORF-derived peptides and functions of uORFs. ..
  7. Adaletli I, Ozer H, Kurugoglu S, Emir H, Madazli R. Congenital imperforate hymen with hydrocolpos diagnosed using prenatal MRI. AJR Am J Roentgenol. 2007;189:W23-5 pubmed
  8. Han B, Park S, Lee Y, Lee K, Lee Y. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) suspected on the presence of hydrocolpos on prenatal sonography. J Clin Ultrasound. 2013;41:380-2 pubmed publisher
    ..Postnatal evaluation confirmed that the cystic lesion was a hydrocolpos associated with double uterus and blind hemivagina (HWW syndrome)...
  9. Zorn K, Spiess P, Salle J, Jednak R. Mucocolpos associated with bladder exstrophy: a case report. Can J Urol. 2005;12:2614-5 pubmed
    ..Surgical drainage of the collection was required. Mucocolpos should be considered in the differential diagnosis of a pelvic mass in a patient with bladder exstrophy. ..
  10. Ameh E, Mshelbwala P, Ameh N. Congenital vaginal obstruction in neonates and infants: recognition and management. J Pediatr Adolesc Gynecol. 2011;24:74-8 pubmed publisher
    ..Congenital vaginal obstruction is an uncommon presentation in this setting. Missing the diagnosis can lead to disastrous consequences. A high index of suspicion is required for prompt recognition and treatment. ..
  11. Breech L. Gynecologic concerns in patients with anorectal malformations. Semin Pediatr Surg. 2010;19:139-45 pubmed publisher
    ..Appropriate counseling for patients and families about potential reproductive concerns that may develop many years after the definitive surgical repair allows preparation and planning to preserve future fertility. ..
  12. Walsh B, Shih R. An unusual case of urinary retention in a competitive gymnast. J Emerg Med. 2006;31:279-81 pubmed
    b>Hydrocolpos is the accumulation of blood behind an imperforate hymen. Patients often present in early adolescence with cyclic abdominal pain...
  13. Gul A, Yildirim G, Gedikbasi A, Gungorduk K, Ceylan Y. Prenatal ultrasonographic features of persistent urogenital sinus with hydrometrocolpos and ascites. Arch Gynecol Obstet. 2008;278:493-6 pubmed publisher
    ..Hydrometrocolpos is a rare congenital disorder very rarely reported on prenatal sonography. In this study we report a persistent urogenital sinus with hydrometrocolpos and ascites at 24 weeks of gestation...
  14. Agnes A, Novelli D, Doglietto G, Papa V. A case report of a giant rectal adenoma causing secretory diarrhea and acute renal failure: McKittrick-Wheelock syndrome. BMC Surg. 2016;16:39 pubmed publisher
    ..We describe a case of this condition in detail and review the related literature, underlining the typical diagnostic features and exploring the possible therapeutic options. ..
  15. Algin O, Erdogan C, Kilic N. Ultrasound-guided percutaneous drainage of neonatal pyometrocolpos under local anesthesia. Cardiovasc Intervent Radiol. 2011;34 Suppl 2:S271-6 pubmed publisher
    ..Ultrasound-guided percutaneous drainage under local anesthesia is a simple, minimally invasive, safe, and effective procedure that facilitates later successful corrective surgery and avoids the need for more complex drainage procedures. ..
  16. Scott C, Marsden A, Rebagliati M, Zhang Q, Chamling X, Searby C, et al. Nuclear/cytoplasmic transport defects in BBS6 underlie congenital heart disease through perturbation of a chromatin remodeling protein. PLoS Genet. 2017;13:e1006936 pubmed publisher
    ..Our work identifies a new function for BBS6 in nuclear-cytoplasmic transport, and provides insight into the disease mechanism underlying the congenital heart defects in McKusick-Kaufman syndrome patients. ..
  17. Kraus S. Radiologic diagnosis of a newborn with cloaca. Semin Pediatr Surg. 2016;25:76-81 pubmed publisher
    ..The following discussion will outline the most important radiologic tests and demonstrate examples of images from newborn females with cloaca. ..
  18. Ayaz U, Dilli A, Api A. Ultrasonographic diagnosis of congenital hydrometrocolpos in prenatal and newborn period: a case report. Med Ultrason. 2011;13:234-6 pubmed
    ..At physical examination imperforate hymen was detected and a hymenotomy was done. After the hymenotomy, complete regression of the hydrometrocolpos and of the left renal pelvicaliectasis was demonstrated sonographically. ..
  19. Zewdneh D, Shewarega Z. A case of McKusick-Kaufman Syndrome. Ethiop Med J. 2011;49:279-82 pubmed
    ..Further pelvic exam under anaesthesia revealed hydrometrocolpos with vaginal agenesis. This, to the best knowledge of the authors, is the first ever reported case of the syndrome in Ethiopia. ..
  20. Ciftci I, Tastekin A, Annagur A, Koplay M. Early abdomino-perineal pull-through vaginoplasty. Afr J Paediatr Surg. 2013;10:188-91 pubmed publisher
    ..Pull-through vaginoplasty was performed with an abdomino-perineal approach. We report the unique treatment approach in this case. ..
  21. Lee S, Carlan S, Locksmith G, Perez J. Ascites, hydrocolpos, hydrometrocolpos with normal amniotic fluid in a fetus with partial urorectal septum malformation sequence. J Clin Ultrasound. 2013;41 Suppl 1:28-31 pubmed publisher
    ..b>Hydrocolpos, hydrometrocolpos, and ascites were seen in utero and confirmed on postnatal surgery...
  22. Toma H, Tan P, McKusick V, Katsanis N, Adams N. Bardet-Biedl Syndrome in an African-American patient: should the diagnostic criteria be expanded to include hydrometrocolpos?. Ophthalmic Genet. 2007;28:95-9 pubmed
  23. Nagy R, Márkus C, Jáger L, Toth C, Locsei Z. [A rare cause of hyponatraemia: McKittrick-Wheelock syndrome. Case report]. Orv Hetil. 2017;158:711-714 pubmed publisher
    ..The authors keep their case to be worthy to present, owing to the syndrome's rarity and difficulties of differential diagnosis. Orv Hetil. 2017; 158(18): 711-714. ..
  24. Subramanian S, Sharma R, Gamanagatti S, Agarwala S, Gupta P, Kumar S. Antenatal MR diagnosis of urinary hydrometrocolpos due to urogenital sinus. Pediatr Radiol. 2006;36:1086-9 pubmed
    ..Urinary hydrometrocolpos occurs in patients with urogenital sinus or cloacal anomaly. A rare case of antenatal MR diagnosis of urinary hydrometrocolpos due to urogenital sinus is presented. ..
  25. Tilahun B, Woldegebriel F, Wolde Z, Tadele H. Hydrometrocolpos Presenting as a Huge Abdominal Swelling and Obstructive Uropathy in a 4 Day Old Newborn: A Diagnostic Challenge. Ethiop J Health Sci. 2016;26:89-91 pubmed
    ..Hydrometrocolpos should be considered as a differential diagnosis in neonates who present with huge abdominal swelling. ..
  26. Nazir Z, Rizvi R, Qureshi R, Khan Z, Khan Z. Congenital vaginal obstructions: varied presentation and outcome. Pediatr Surg Int. 2006;22:749-53 pubmed
    ..A comprehensive management is imperative to preserve the reproductive potentials, as significant proportion of patients may experience sexual difficulties, menstrual irregularity, and infertility. ..
  27. Bischoff A, Calvo Garcia M, Baregamian N, Levitt M, Lim F, Hall J, et al. Prenatal counseling for cloaca and cloacal exstrophy-challenges faced by pediatric surgeons. Pediatr Surg Int. 2012;28:781-8 pubmed publisher
    ..at expected rectal location (7), vertebral anomaly (7), non-visualize bladder (5), distended bladder (5), hydrocolpos (4), dilated or echogenic bowel (3), umbilical cord cyst (3), separated pubic bones (2), and the "elephant ..
  28. Deveault C, Billingsley G, Duncan J, Bin J, Theal R, Vincent A, et al. BBS genotype-phenotype assessment of a multiethnic patient cohort calls for a revision of the disease definition. Hum Mutat. 2011;32:610-9 pubmed publisher
    ..Comprehensive phenotyping of these patients demonstrate that the spectrum of clinical features is greater than expected and overlapped with the features of other ciliopathies; specifically Alström and McKusick-Kauffman syndromes. ..
  29. Hamada T, Hirose R, Kosaka T, Taniguchi K, Noguchi M, Kihara T, et al. Giant cystic meconium peritonitis associated with a cloacal anomaly: case report. J Pediatr Surg. 2008;43:E21-3 pubmed publisher
    ..The baby girl showed duplicated hydrocolpos and a single orifice of the cloaca with a long common channel inducing a urinary outflow obstruction...
  30. Elsayes K, Narra V, Dillman J, Velcheti V, Hameed O, Tongdee R, et al. Vaginal masses: magnetic resonance imaging features with pathologic correlation. Acta Radiol. 2007;48:921-33 pubmed
    ..In this article, we will review pertinent vaginal anatomy, vaginal and pelvic MRI technique, and the MRI features of a variety of vaginal lesions with pathological correlation. ..
  31. Kawar B, Sakran W, Chervinsky L, Siplovich L. Unusual presentation of McKusick-Kaufman syndrome in a female Bedouin Arab baby. Eur J Pediatr Surg. 2005;15:446-8 pubmed
    ..We present a case of an Arab Bedouin girl who presented with features resembling this syndrome. ..
  32. Hübner A, Cavanagh Kyros J, Rincon M, Flavell R, Davis R. Functional cooperation of the proapoptotic Bcl2 family proteins Bmf and Bim in vivo. Mol Cell Biol. 2010;30:98-105 pubmed publisher
    ..Thus, developmental ablation of interdigital webbing on mouse paws and normal lymphocyte homeostasis require the cooperative activity of Bim and Bmf. ..
  33. El Messidi A, Fleming N. Congenital imperforate hymen and its life-threatening consequences in the neonatal period. J Pediatr Adolesc Gynecol. 2006;19:99-103 pubmed
    ..We report a 5-day-old infant with a large hydrometrocolpos causing severe renal compromise and abdominal ascites, successfully surgically treated in the neonatal period. Associated polydactyly suggested McKusick-Kaufman syndrome...
  34. Schumacher R, Brzezinska R. Hydrometrocolpos: the Fjord sign. Ultraschall Med. 2007;28:518-20 pubmed
    We want to stress a diagnostic sign in the diagnosis of pelvic cysts in newborns. In these cases the detection of an attached uterus is proof of a hydrocolpos or in cases with a dilated uterine cavity of a hydrometrocolpos.
  35. Buyukbayrak E, Ozyapi A, Karsidag Y, Pirimoglu Z, Unal O, Turan C. Imperforate hymen: a new benign reason for highly elevated serum CA 19.9 and CA 125 levels. Arch Gynecol Obstet. 2008;277:475-7 pubmed
    ..In this case report we describe a relation between imperforate hymen and elevated serum CA 19-9 and CA 125 levels. ..
  36. Taori K, Krishnan V, Sharbidre K, Andhare A, Kulkarni B, Bopche S, et al. Prenatal sonographic diagnosis of fetal persistent urogenital sinus with congenital hydrocolpos. Ultrasound Obstet Gynecol. 2010;36:641-3 pubmed publisher
    We report a case of persistent urogenital sinus with hydrocolpos and associated renal anomalies diagnosed prenatally at 30 weeks' gestation...
  37. Picone O, Laperelle J, Sonigo P, Levaillant J, Frydman R, Senat M. Fetal magnetic resonance imaging in the antenatal diagnosis and management of hydrocolpos. Ultrasound Obstet Gynecol. 2007;30:105-9 pubmed
    b>Hydrocolpos should be considered systematically when an abdominopelvic cystic mass is diagnosed in a female fetus...
  38. Sarathi V, Naregal A, Lila A, Bandgar T, Shah N. Mucocolpos in a toddler: central precocious puberty with vaginal atresia. Endocr Pract. 2012;18:e144-6 pubmed publisher
    ..To report the first case of imperforate hymen and vaginal atresia in a patient with mucocolpos during toddlerhood who was found to have central precocious puberty...
  39. Tseng J, Ho J, Chen W, Chou M. Prenatal diagnosis of isolated fetal hydrocolpos secondary to congenital imperforate hymen. J Chin Med Assoc. 2008;71:325-8 pubmed publisher
    ..Therefore, the diagnosis of congenital imperforate hymen with hydrocolpos was established...
  40. Sidatt M, Ould Sidi Mohamed Wedih A, Ould Boubaccar A, Ould Ely Litime A, Feil A, Ould Moussa A. [Hydrocolpos and hydrometrocolpos in newborns]. Arch Pediatr. 2013;20:176-80 pubmed publisher
    b>Hydrocolpos and the hydrometrocolpos are anomalies resulting from vaginal and uterine retention and accumulation of cervicovaginal secretions caused by congenital malformation...
  41. Dhombres F, Jouannic J, Brodaty G, Bessiere B, Daffos F, Benifla J. Contribution of prenatal imaging to the anatomical assessment of fetal hydrocolpos. Ultrasound Obstet Gynecol. 2007;30:101-4 pubmed
    b>Hydrocolpos may be associated with a lower urinary tract obstruction in a spectrum of urorectal malformations ranging from persistent urogenital sinus to cloacal dysgenesis...
  42. Gilboa Y, Lipitz S, Zalel Y, Achiron R. Prenatal diagnosis of ascites with hydrometrocolpos and uterus didelphys in a monoamniotic twin pregnancy. J Ultrasound Med. 2007;26:373-6 pubmed
  43. Jackson L, Murphy F, Hiorns M, Duffy P. The role of dynamic magnetic resonance urography in complex neonatal hydrometrocolpos. Pediatr Surg Int. 2008;24:625-7 pubmed publisher
    ..We report the case of a neonate in which the use of dynamic MRI allowed accurate preoperative diagnosis of urinary hydrometrocolpos secondary to ectopic drainage of a small left pelvic kidney, associated with a bicornuate uterus. ..
  44. Sharifiaghdas F, Abdi H, Pakmanesh H, Eslami N. Imperforate hymen and urinary retention in a newborn girl. J Pediatr Adolesc Gynecol. 2009;22:49-51 pubmed
    ..Mucocolpos with an abdominal mass in neonatal age is extremely rare. We report a case of a 20-day-old newborn girl with acute urinary retention due to isolated imperforate hymen and mucocolpos...
  45. Soman A, Thayyil S, Ogilvy Stuart A, Set P, Brain J, Samuel M. Fetal ascites secondary to urinary hydrocolpos. J Perinat Med. 2006;34:347-8 pubmed
  46. Malik F, Tahir A, Bashir H. McKusik Kaufman syndrome. J Ayub Med Coll Abbottabad. 2012;24:154-5 pubmed
    ..At this time, there is no molecular testing available to distinguish these two syndromes. ..
  47. Schaefer E, Durand M, Stoetzel C, Doray B, Viville B, Hellé S, et al. Molecular diagnosis reveals genetic heterogeneity for the overlapping MKKS and BBS phenotypes. Eur J Med Genet. 2011;54:157-60 pubmed publisher
  48. Sonmez G, Aydinoz S, Mutlu H, Ozturk E, Sildiroglu O, Akyol I. Medical image. Hydrocolpos in a child. N Z Med J. 2008;121:115-6 pubmed
  49. Yildirim G, Gungorduk K, Aslan H, Sudolmus S, Ark C, Saygin S. Prenatal diagnosis of imperforate hymen with hydrometrocolpos. Arch Gynecol Obstet. 2008;278:483-5 pubmed publisher
    ..Rarely, obstetric sonography may prenatally detect imperforate hymen due to presentation of hydrocolpos or hydrometrocolpos in the fetus if fetal cervical and vaginal secretions accumulate in response to circulating ..
  50. Hung Y, Tsai C, Ou C, Cheng B, Yu P, Hsu T. Late prenatal diagnosis of hydrometrocolpos secondary to a cloacal anomaly by abdominal ultrasonography with complementary magnetic resonance imaging. Taiwan J Obstet Gynecol. 2008;47:79-83 pubmed publisher
    ..MRI was performed and demonstrated hydrocolpos/hydrometrocolpos originating from a uterine didelphis with left dysgenesis and a possible vesicovaginal fistula...
  51. Witters I, Meylaerts L, Peeters H, Coumans A, Wirjosoekarto S, Fryns J. Fetal hydrometrocolpos, uterus didelphys with low vaginal and anal atresia: difficulties in differentiation from a complex cloacal malformation: a case report. Genet Couns. 2012;23:513-7 pubmed
    ..Care should be taken in decision making and counselling patients with these complex prenatal malformations. ..
  52. Shaked O, Tepper R, Klein Z, Beyth Y. Hydrometrocolpos--diagnostic and therapeutic dilemmas. J Pediatr Adolesc Gynecol. 2008;21:317-21 pubmed publisher
  53. Karteris E, Foster H, Karamouti M, Goumenou A. Congenital imperforate hymen with hydrocolpos and hydronephrosis associated with severe hydramnios and increase of maternal ovarian steroidogenic enzymes. J Pediatr Adolesc Gynecol. 2010;23:136-41 pubmed publisher