Müllerian Aplasia with Severe Hematometra: A Case Report of Diagnosis and Management in a Low Resource Setting
Müllerian aplasia or Mayer–Rokitansky–Küster–Hauser syndrome is described as congenital absence of the proximal vagina with or without absence of the cervix and uterus, most often recognized after the onset of primary amenorrhea. An 18-year-old woman presented to a free medical clinic in Arcahaie, H...
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| Veröffentlicht in: | Journal of pediatric & adolescent gynecology 2019-04, Vol.32 (2), p.189-192 |
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| container_title | Journal of pediatric & adolescent gynecology |
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| creator | Howard, Lucille A. Mancuso, Abigail C. Ryan, Ginny L. |
| description | Müllerian aplasia or Mayer–Rokitansky–Küster–Hauser syndrome is described as congenital absence of the proximal vagina with or without absence of the cervix and uterus, most often recognized after the onset of primary amenorrhea.
An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Transabdominal and transperineal ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix was present.
Diagnosis of vaginal and cervical agenesis is complicated in low-resource settings and treatment must be modified when subspecialty care and consistent follow-up are not available. |
| doi_str_mv | 10.1016/j.jpag.2018.11.006 |
| format | Article |
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An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Transabdominal and transperineal ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix was present.
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An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Transabdominal and transperineal ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix was present.
Diagnosis of vaginal and cervical agenesis is complicated in low-resource settings and treatment must be modified when subspecialty care and consistent follow-up are not available.</description><subject>46, XX Disorders of Sex Development - complications</subject><subject>46, XX Disorders of Sex Development - diagnosis</subject><subject>46, XX Disorders of Sex Development - surgery</subject><subject>Adolescent</subject><subject>Congenital Abnormalities - diagnosis</subject><subject>Congenital Abnormalities - surgery</subject><subject>Female</subject><subject>Haiti</subject><subject>Hematometra - etiology</subject><subject>Hematometra - surgery</subject><subject>Humans</subject><subject>Laparotomy - methods</subject><subject>Low resource management</subject><subject>Mullerian aplasia</subject><subject>Mullerian Ducts - abnormalities</subject><subject>Mullerian Ducts - surgery</subject><subject>Primary amenorrhea</subject><subject>Ultrasonography</subject><subject>Uterovaginal anomalies</subject><issn>1083-3188</issn><issn>1873-4332</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kMtu1DAUQC0EoqXwAyyQl2wS_I6D2IyGR5GmqtTC2vI414NHiR1sTyv-rTt-jIymsGR17-LcI92D0GtKWkqoerdv97PdtYxQ3VLaEqKeoHOqO94IztnTZSeaN5xqfYZelLInhHRS6efojBPRUS75OUpXvx_GEXKwEa_m0ZZg8X2oP_At3EEGfAmTrWmCmu17vMJrWwDfwJxyxcnjj8HuYiqhYBsHfGWj3cEEseIQscWbdL-wJR2yg8VXa4i7l-iZt2OBV4_zAn3__Onb-rLZXH_5ul5tGselqo1UVlrB9KC2UnrpQVMGStJB9T30ljqieiGk6pUfeq2EYEw754FvvdBCeX6B3p68c04_D1CqmUJxMI42QjoUwyjvRKc7RhaUnVCXUykZvJlzmGz-ZSgxx9Bmb46hzTG0odQsoZejN4_-w3aC4d_J37IL8OEEwPLlXYBsigsQHQwhg6tmSOF__j_MKo8F</recordid><startdate>201904</startdate><enddate>201904</enddate><creator>Howard, Lucille A.</creator><creator>Mancuso, Abigail C.</creator><creator>Ryan, Ginny L.</creator><general>Elsevier Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>201904</creationdate><title>Müllerian Aplasia with Severe Hematometra: A Case Report of Diagnosis and Management in a Low Resource Setting</title><author>Howard, Lucille A. ; Mancuso, Abigail C. ; Ryan, Ginny L.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c356t-56a5a428d6b55f5fe812e651d699e9a1c069445696fd98644228ccfe3bf4846f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>46, XX Disorders of Sex Development - complications</topic><topic>46, XX Disorders of Sex Development - diagnosis</topic><topic>46, XX Disorders of Sex Development - surgery</topic><topic>Adolescent</topic><topic>Congenital Abnormalities - diagnosis</topic><topic>Congenital Abnormalities - surgery</topic><topic>Female</topic><topic>Haiti</topic><topic>Hematometra - etiology</topic><topic>Hematometra - surgery</topic><topic>Humans</topic><topic>Laparotomy - methods</topic><topic>Low resource management</topic><topic>Mullerian aplasia</topic><topic>Mullerian Ducts - abnormalities</topic><topic>Mullerian Ducts - surgery</topic><topic>Primary amenorrhea</topic><topic>Ultrasonography</topic><topic>Uterovaginal anomalies</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Howard, Lucille A.</creatorcontrib><creatorcontrib>Mancuso, Abigail C.</creatorcontrib><creatorcontrib>Ryan, Ginny L.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of pediatric & adolescent gynecology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Howard, Lucille A.</au><au>Mancuso, Abigail C.</au><au>Ryan, Ginny L.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Müllerian Aplasia with Severe Hematometra: A Case Report of Diagnosis and Management in a Low Resource Setting</atitle><jtitle>Journal of pediatric & adolescent gynecology</jtitle><addtitle>J Pediatr Adolesc Gynecol</addtitle><date>2019-04</date><risdate>2019</risdate><volume>32</volume><issue>2</issue><spage>189</spage><epage>192</epage><pages>189-192</pages><issn>1083-3188</issn><eissn>1873-4332</eissn><abstract>Müllerian aplasia or Mayer–Rokitansky–Küster–Hauser syndrome is described as congenital absence of the proximal vagina with or without absence of the cervix and uterus, most often recognized after the onset of primary amenorrhea.
An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Transabdominal and transperineal ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix was present.
Diagnosis of vaginal and cervical agenesis is complicated in low-resource settings and treatment must be modified when subspecialty care and consistent follow-up are not available.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>30471353</pmid><doi>10.1016/j.jpag.2018.11.006</doi><tpages>4</tpages></addata></record> |
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| ispartof | Journal of pediatric & adolescent gynecology, 2019-04, Vol.32 (2), p.189-192 |
| issn | 1083-3188 1873-4332 |
| language | eng |
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| source | MEDLINE; Elsevier ScienceDirect Journals Complete |
| subjects | 46, XX Disorders of Sex Development - complications 46, XX Disorders of Sex Development - diagnosis 46, XX Disorders of Sex Development - surgery Adolescent Congenital Abnormalities - diagnosis Congenital Abnormalities - surgery Female Haiti Hematometra - etiology Hematometra - surgery Humans Laparotomy - methods Low resource management Mullerian aplasia Mullerian Ducts - abnormalities Mullerian Ducts - surgery Primary amenorrhea Ultrasonography Uterovaginal anomalies |
| title | Müllerian Aplasia with Severe Hematometra: A Case Report of Diagnosis and Management in a Low Resource Setting |
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