Intestinal Obstruction Secondary to Malformation in a Child with Mayer-Rokitansky-Küster-Hauser Syndrome
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by underdevelopment of the uterus and vagina. A 4-year-old girl was declared dead on arrival to the hospital with a history of chronic constipation since birth. Postmortem examination showed Müllerian remnants atta...
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| Published in | Journal of pediatric & adolescent gynecology Vol. 35; no. 2; pp. 192 - 195 |
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| Main Authors | , , , , |
| Format | Journal Article |
| Language | English |
| Published |
United States
Elsevier Inc
01.04.2022
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| Abstract | Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by underdevelopment of the uterus and vagina.
A 4-year-old girl was declared dead on arrival to the hospital with a history of chronic constipation since birth. Postmortem examination showed Müllerian remnants attached to abnormal fibrous tissue. The fibrous tissue extended from the descending colon to the rectum and connected to a urinary bladder duplication cyst. The fibrous tissue formed into a constricted band around the rectum. The cause of death was Streptococcus pyogenes sepsis with intestinal obstruction secondary to the rectal fibrotic band and urinary bladder duplication cyst.
MRKH syndrome is a rare congenital disorder, and the malformations rarely cause death. We describe the features of abnormal fibrous tissue and urinary bladder duplication cyst in a patient with MRKH syndrome. |
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| AbstractList | Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by underdevelopment of the uterus and vagina.
A 4-year-old girl was declared dead on arrival to the hospital with a history of chronic constipation since birth. Postmortem examination showed Müllerian remnants attached to abnormal fibrous tissue. The fibrous tissue extended from the descending colon to the rectum and connected to a urinary bladder duplication cyst. The fibrous tissue formed into a constricted band around the rectum. The cause of death was Streptococcus pyogenes sepsis with intestinal obstruction secondary to the rectal fibrotic band and urinary bladder duplication cyst.
MRKH syndrome is a rare congenital disorder, and the malformations rarely cause death. We describe the features of abnormal fibrous tissue and urinary bladder duplication cyst in a patient with MRKH syndrome. |
| Author | Othman, Mohammad Shafie Tan, Lii Jye Lai, Shau-Kong Hussin, Huzlinda Tan, Geok Chin |
| Author_xml | – sequence: 1 givenname: Lii Jye surname: Tan fullname: Tan, Lii Jye organization: Department of Forensic Medicine, Hospital Raja Permaisuri Bainun, Ipoh, Perak Darul Ridzuan, Malaysia – sequence: 2 givenname: Geok Chin surname: Tan fullname: Tan, Geok Chin organization: Department of Pathology, Universiti Kebangsaan Malaysia Medical Center, Kuala Lumpur, Malaysia – sequence: 3 givenname: Huzlinda surname: Hussin fullname: Hussin, Huzlinda organization: Department of Pathology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia – sequence: 4 givenname: Mohammad Shafie surname: Othman fullname: Othman, Mohammad Shafie organization: Department of Forensic Medicine, Hospital Raja Permaisuri Bainun, Ipoh, Perak Darul Ridzuan, Malaysia – sequence: 5 givenname: Shau-Kong surname: Lai fullname: Lai, Shau-Kong email: pathsklai@gmail.com organization: Department of Pathology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/34687904$$D View this record in MEDLINE/PubMed |
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| Cites_doi | 10.1016/j.urology.2020.11.001 10.1016/j.ejogrb.2010.07.003 10.1016/j.fertnstert.2008.08.132 10.1186/s13023-020-01491-9 10.3348/kjr.2013.14.2.233 10.1016/j.fertnstert.2012.12.002 10.1016/j.jpedsurg.2018.05.009 10.1097/00004728-200011000-00001 10.3390/pathogens10020248 10.1007/s00330-017-4919-4 10.1007/s00192-013-2214-8 |
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| Keywords | Intestinal obstruction Mayer-Rokitansky-Küster-Hauser syndrome Urinary bladder duplication MRKH Müllerian aplasia |
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| References | Wang, Lang, Zhu (bib0006) 2010; 153 Wang, Lu, Zhu (bib0007) 2017; 27 Li, Qayyum, Coakley (bib0002) 2000; 24 Avire, Whiley, Ross (bib0011) 2021; 10 Lamarca, Navarro, Ballesteros (bib0009) 2009; 91 Pradhan, Vilanova-Sanchez, McCracken (bib0005) 2018; 53 Yoo, Cho, Kim (bib0008) 2013; 14 Huebner, Rall, Brucker (bib0010) 2014; 25 Delcont, Guglielmetti, Rajbhandari (bib0004) 2021; 149 Herlin, Petersen, Brännström (bib0001) 2020; 15 Rall, Barresi, Wallwiener (bib0003) 2013; 99 Herlin (10.1016/j.jpag.2021.10.002_bib0001) 2020; 15 Delcont (10.1016/j.jpag.2021.10.002_bib0004) 2021; 149 Pradhan (10.1016/j.jpag.2021.10.002_bib0005) 2018; 53 Avire (10.1016/j.jpag.2021.10.002_bib0011) 2021; 10 Lamarca (10.1016/j.jpag.2021.10.002_bib0009) 2009; 91 Wang (10.1016/j.jpag.2021.10.002_bib0006) 2010; 153 Rall (10.1016/j.jpag.2021.10.002_bib0003) 2013; 99 Yoo (10.1016/j.jpag.2021.10.002_bib0008) 2013; 14 Li (10.1016/j.jpag.2021.10.002_bib0002) 2000; 24 Huebner (10.1016/j.jpag.2021.10.002_bib0010) 2014; 25 Wang (10.1016/j.jpag.2021.10.002_bib0007) 2017; 27 |
| References_xml | – volume: 91 start-page: 931 year: 2009 ident: bib0009 article-title: Leiomyomas in both uterine remnants in a woman with the Mayer-Rokitansky-Küster-Hauser syndrome publication-title: Fertil Steril contributor: fullname: Ballesteros – volume: 53 start-page: 2164 year: 2018 ident: bib0005 article-title: The Mullerian Black Box: predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment publication-title: J Pediatr Surg contributor: fullname: McCracken – volume: 14 start-page: 233 year: 2013 ident: bib0008 article-title: Magnetic resonance evaluation of Müllerian remnants in Mayer-Rokitansky-Küster-Hauser syndrome publication-title: Korean J Radiol contributor: fullname: Kim – volume: 24 start-page: 829 year: 2000 ident: bib0002 article-title: Association of renal agenesis and Mullerian duct anomalies publication-title: J Comput Assist Tomogr contributor: fullname: Coakley – volume: 27 start-page: 5215 year: 2017 ident: bib0007 article-title: Evaluation of Mayer-Rokitansky-Küster-Hauser syndrome with magnetic resonance imaging: three patterns of uterine remnants and related anatomical features and clinical settings publication-title: Eur Radiol contributor: fullname: Zhu – volume: 10 start-page: 248 year: 2021 ident: bib0011 article-title: A review of publication-title: Pathogens contributor: fullname: Ross – volume: 15 start-page: 1 year: 2020 ident: bib0001 article-title: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update publication-title: Orphanet J Rare Dis contributor: fullname: Brännström – volume: 99 start-page: 1392 year: 2013 ident: bib0003 article-title: Uterine rudiments in patients with Mayer-Rokitansky-Küster-Hauser syndrome consist of typical uterine tissue types with predominantly basalis-like endometrium publication-title: Fertil Steril contributor: fullname: Wallwiener – volume: 153 start-page: 77 year: 2010 ident: bib0006 article-title: Mayer–Rokitansky–Küester–Hauser (MRKH) syndrome with rectovestibular fistula and imperforate anus publication-title: Eur J Obstet Gynecol Reprod Biol contributor: fullname: Zhu – volume: 25 start-page: 323 year: 2014 ident: bib0010 article-title: The rectovaginal septum: visible on magnetic resonance images of women with Mayer-Rokitansky-Küster-Hauser syndrome (Müllerian agenesis) publication-title: Int Urogynecol J contributor: fullname: Brucker – volume: 149 start-page: 199 year: 2021 ident: bib0004 article-title: Bladder duplication - 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| Snippet | Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by underdevelopment of the uterus and vagina.
A 4-year-old girl was... |
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| SubjectTerms | Intestinal obstruction Mayer-Rokitansky-Küster-Hauser syndrome Müllerian aplasia Urinary bladder duplication |
| Title | Intestinal Obstruction Secondary to Malformation in a Child with Mayer-Rokitansky-Küster-Hauser Syndrome |
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