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Fulltext Persistent müllerian duct syndrome--a case report

Yip CH, Chang KW

Singapore Med J, 1991 Oct;32(5):363-4.
PMID: 1788587

An eighteen month old phenotypically and genotypically normal male child was admitted with a left inguinal hernia and a right undescended testis. At operation, he was found to have a uterus, bilateral fallopian tubes, and a vagina in the left hernial sac. Bilateral orchidopexies and excision of the persistent Mullerian duct structures were carried out. This rare case of persistent Mullerian duct syndrome is due to a defect in Mullerian regression, which is in turn controlled by the Mullerian inhibiting substance (MIS). Orchidopexy with excision of the persistent Mullerian duct structures is usually not possible without damage to the vas deferens which is closely adherent to the wall of the uterus. The alternative of leaving the persistent Mullerian duct structures alone and performing a staged or primary orchidopexy has been suggested.

Matched MeSH terms: Mullerian Ducts/abnormalities*; Mullerian Ducts/surgery
Fulltext REPLY BY THE AUTHORS: Re: Persistent Mullerian Duct Syndrome: a rare entity with a rare presentation in need of multidisciplinary management

Aw LD, Zain MM, Esteves SC, Humaidan P

Int Braz J Urol, 2017 Sep-Oct;43(5):1005-1006.
PMID: 28792190 DOI: 10.1590/S1677-5538.IBJU.2017.0072.1
Matched MeSH terms: Mullerian Ducts*
Transverse testicular ectopia: correlation of embryology with laparoscopic findings

Thambidorai CR, Khaleed A

Pediatr Surg Int, 2008 Mar;24(3):371-4.
PMID: 17492292

Two patients with unilateral transverse testicular ectopia (TTE) without the persistence of Mullerian duct structures are described. Each presented with unilateral impalpable testis and a contralateral inguinal hernia. The diagnosis of unilateral TTE was made during laparoscopic evaluation for undescended testis. The first patient had unilateral TTE on the right side and the second on the left. In both patients, a long thin band resembling the round ligament of the uterus was seen extending from the region of the internal inguinal ring (IIR) on the side of the undescended testis to the opposite inguinal canal. In both patients, there was no patent processus vaginalis on the side of the undescended testis and on the contralateral side the internal rings were widely patent with large hernial sacs. In the second patient, the right vas was seen extending from the right IIR towards the right side of the pelvis. The right vas showed a short segment of discontinuity at the level of the right IIR while its proximal end extended into the left inguinal canal in close relation to the right spermatic vessels. The vasal anomaly was probably ischemic in origin, resulting from excessive mobility of the ectopic testis and its vas in TTE. Correlation of the current hypotheses on the embryology of TTE with the above mentioned laparoscopic findings is discussed.

Matched MeSH terms: Mullerian Ducts/abnormalities
Mullerian duct cyst (utricular cyst): treatment with the transvesical, transtrigonal approach

Goon HK, Tan KC, Sakijan AS

Aust N Z J Surg, 1987 Sep;57(9):683-6.
PMID: 3689258

The diagnosis of mullerian duct or utricular cyst should be considered in a child with urinary difficulties and a palpable midline, anterior rectal mass. Endoscopic cannulation of the cyst has been found to be the most useful diagnostic test. Infection should be treated with the appropriate antibiotics before definitive treatment. Surgical excision offers the best result. The transperitoneal and posterior parasacral approaches have been described but we favour the transvesical, transtrigonal approach which we find highly satisfactory. The risk of malignancy at a later age is an added indication for surgery.

Matched MeSH terms: Mullerian Ducts/surgery*
OHVIRA syndrome: clinical implications of a delayed diagnosis

Mohd Adzlan F, Mohd K, Ahmad N, Ramli R

BMJ Case Rep, 2024 May 22;17(5).
PMID: 38782440 DOI: 10.1136/bcr-2024-259861

Obstructed Hemi Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rarely encountered müllerian duct anomaly. Delayed diagnosis is common due to normal onset of puberty and menstruation. We report a case of a woman in her early 20s with a background history of multiple emergency department visits, ward admissions and surgeries for chronic abdominal pain. She was reviewed at 1 month postlaparotomy for recurrent pelvic abscess and was finally diagnosed to have an OHVIRA syndrome, 11 years after her first clinical presentation. Excision of the vaginal septum completely resolved her symptoms. We are reporting this case to highlight the clinical implications resulting from the delayed diagnosis, to look into factors contributing to the delay and to highlight the importance of having a high index of suspicion to diagnose this unique condition.

Matched MeSH terms: Mullerian Ducts/abnormalities; Mullerian Ducts/surgery
Successful pregnancy outcome following gamete intra-Fallopian transfer in a patient with Müllerian dysgenesis

Lee CS, Lie AT

Reprod Biomed Online, 2012 May;24(5):547-9.
PMID: 22410277 DOI: 10.1016/j.rbmo.2012.01.021

A 29-year-old lady with Müllerian dysgenesis was keen to have a baby. Clinically, she was medium built with well-developed secondary female sexual characteristics. There was a short and blind vagina. She had undergone surgery for an imperforated hymen. Her FSH and LH concentrations were normal. Laparoscopy revealed a patent right Fallopian tube, a rudimentary right uterus and extensive pelvic endometriosis. She subsequently underwent gamete intra-Fallopian transfer (GIFT). Oocyte retrieval was carried out laparoscopically and a total of nine oocytes were retrieved. Four of the oocytes were transferred together with motile spermatozoa into the right Fallopian tube and the remaining five oocytes were inseminated with spermatozoa for IVF. Three embryos resulted and were frozen. She subsequently developed moderate ovarian hyperstimulation syndrome. Serum β-human chorionic gonadotrophin concentration 14 days after GIFT was 1612 IU/l. Her antenatal care was relatively uneventful until 31 weeks of gestation when she was diagnosed to have intrauterine growth retardation and oligohydramnios. She then underwent an emergency Caesarean section at 32 weeks of pregnancy delivering a normal baby. This case study describes a successful pregnancy outcome following gamete intra-Fallopian transfer (GIFT) in a woman with malformation of the vagina (Müllerian dysgenesis). A 29-year-old lady with Müllerian dysgenesis diagnosed at 16 years of age was keen to become pregnant. Upon examination, a decision was made for a William's vulvovaginoplasty but as the patient was indecisive the surgery was deferred. Clinically, she is a medium-built lady with well-developed secondary female sexual characteristics. There was a short and blind vagina. Her serum FSH and LH concentrations were normal. Laparoscopy revealed a patent right Fallopian tube, a rudimentary right uterus and extensive pelvic endometriosis. She subsequently underwent GIFT. Nine oocytes were retrieved through laparoscopy. Four of the oocytes were transferred together with motile sperm into the right Fallopian tube and the remaining five oocytes were inseminated with sperm for IVF. Three embryos resulted and were frozen. Serum β human chorionic gonadotrophin concentration measured 14 days after GIFT was 1612 IU/l. An abdominal ultrasonography performed at 5 weeks showed one intrauterine gestational sac. Her antenatal care was uneventful until 31 weeks of gestation when she developed a deficiency of amniotic fluid in the amniotic sac. She then underwent an emergency Caesarean section at 32 weeks of pregnancy. She delivered a healthy, normal 1.24 kg baby boy. Her post-natal care was uneventful.

Matched MeSH terms: Mullerian Ducts/abnormalities*
The Missing Uterus, the Missed Diagnosis, and the Missing Care. Mayer-Rokitansky-Küster-Hauser Syndrome in the Lives of Women in Malaysia

Hatim H, Zainuddin AA, Anizah A, Kalok A, Daud TIM, Ismail A, et al.

J Pediatr Adolesc Gynecol, 2021 Apr;34(2):161-167.
PMID: 33189898 DOI: 10.1016/j.jpag.2020.11.009

STUDY OBJECTIVE: To explore the effect of the diagnosis of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome on affected Malaysian women.
DESIGN: Qualitative study with a quantitative component.
SETTING: Pediatric and adolescent gynecology unit at Universiti Kebangsaan Malaysia Medical Centre, Malaysia.
PARTICIPANTS: Twelve women with MRKH.
INTERVENTIONS: Face-to-face interview and short questionnaire.
MAIN OUTCOME MEASURES: Thematic analysis was used to understand participants' experiences.
RESULTS: There were 7 themes identified: (1) delayed diagnoses; (2) doctors' roles and attitudes; (3) gender identity; (4) family and society's response; (5) reaction toward infertility; (6) managing sexual intimacy; and (7) coping mechanisms. Several participants consulted their physicians regarding their primary amenorrhea at an opportunistic setting. When they were referred to the gynecologists, they were dismayed at the lack of information given. The term, "MRKH" plays an important role to ease information-seeking. Participants felt that the doctors were insensitive toward them. Mental illness is a significant complication of MRKH. All participants acknowledged that infertility was the hardest part of the condition. The importance of blood lineage affects their outlook on childbearing options. Some were afraid of sexual intimacy and worried that they would not be able to satisfy their partners. Participants gained support and bonded with their counterparts in the MRKH support group.
CONCLUSION: A multidisciplinary approach including medical, psychological, and social support is essential for the management of MRKH. Adequate information and sexual education plays the utmost importance in preventing social-related complications of MRKH.

Matched MeSH terms: Mullerian Ducts
Diagnostic pitfalls in the evaluation and management of amenorrhea in adolescents

Azurah AG, Zainuddin AA, Jayasinghe Y

J Reprod Med, 2013 Jul-Aug;58(7-8):324-36.
PMID: 23947083

Amenorrhea is a common menstrual problem seen in adolescents. Amenorrhea has been shown to have a negative impact on adolescents' quality of life. In this paper we discuss the various causes and investigations of amenorrhea in adolescents and address management dilemmas for specific conditions. Specific approaches in dealing with adolescents using the HEADSS (Home, Education, Activity, Drugs, Sexual activity, Suicidal) approach are discussed.

Matched MeSH terms: Mullerian Ducts/abnormalities