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ACOG Committee Opinion No. 728: Müllerian Agenesis: Diagnosis, Management, And Treatment.

Abstract Müllerian agenesis, also referred to as müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,500-5,000 females. Müllerian agenesis is caused by embryologic underdevelopment of the müllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with müllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. The most important steps in the effective management of müllerian agenesis are correct diagnosis of the underlying condition, evaluation for associated congenital anomalies, and psychosocial counseling in addition to treatment or intervention to address the functional effects of genital anomalies. The psychologic effect of the diagnosis of müllerian agenesis should not be underestimated. All patients with müllerian agenesis should be offered counseling and encouraged to connect with peer support groups. Future options for having children should be addressed with patients: options include adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with müllerian agenesis. Nonsurgical vaginal elongation by dilation should be the first-line approach. When well-counseled and emotionally prepared, almost all patients (90-96%) will be able to achieve anatomic and functional success by primary vaginal dilation. In cases in which surgical intervention is required, referrals to centers with expertise in this area should be considered because few surgeons have extensive experience in construction of the neovagina and surgery by a trained surgeon offers the best opportunity for a successful result.
PMID
Related Publications

Mayer-Rokitansky-Kuster-Hauser syndrome: complications, diagnosis and possible treatment options: a review.

ACOG Committee Opinion. Number 274, July 2002. Nonsurgical diagnosis and management of vaginal agenesis.

Using the Wharton-Sheares-George method to create a neovagina in patients with Mayer-Rokitansky-Küster-Hauser syndrome: a step-by-step video tutorial.

Committee opinion: no. 562: müllerian agenesis: diagnosis, management, and treatment.

ACOG Committee Opinion No. 728 Summary: Müllerian Agenesis: Diagnosis, Management, And Treatment.

Authors
Mayor MeshTerms

Practice Guidelines as Topic

Keywords
Journal Title obstetrics and gynecology
Publication Year Start

 



PMID- 29266078
OWN - NLM
STAT- MEDLINE
DCOM- 20180108
LR  - 20180108
IS  - 1873-233X (Electronic)
IS  - 0029-7844 (Linking)
VI  - 131
IP  - 1
DP  - 2018 Jan
TI  - ACOG Committee Opinion No. 728: Mullerian Agenesis: Diagnosis, Management, And
      Treatment.
PG  - e35-e42
LID - 10.1097/AOG.0000000000002458 [doi]
AB  - Mullerian agenesis, also referred to as mullerian aplasia,
      Mayer-Rokitansky-Kuster-Hauser syndrome, or vaginal agenesis, has an incidence of
      1 per 4,500-5,000 females. Mullerian agenesis is caused by embryologic
      underdevelopment of the mullerian duct, with resultant agenesis or atresia of the
      vagina, uterus, or both. Patients with mullerian agenesis usually are identified 
      when they are evaluated for primary amenorrhea with otherwise typical growth and 
      pubertal development. The most important steps in the effective management of
      mullerian agenesis are correct diagnosis of the underlying condition, evaluation 
      for associated congenital anomalies, and psychosocial counseling in addition to
      treatment or intervention to address the functional effects of genital anomalies.
      The psychologic effect of the diagnosis of mullerian agenesis should not be
      underestimated. All patients with mullerian agenesis should be offered counseling
      and encouraged to connect with peer support groups. Future options for having
      children should be addressed with patients: options include adoption and
      gestational surrogacy. Assisted reproductive techniques with use of a gestational
      carrier (surrogate) have been shown to be successful for women with mullerian
      agenesis. Nonsurgical vaginal elongation by dilation should be the first-line
      approach. When well-counseled and emotionally prepared, almost all patients
      (90-96%) will be able to achieve anatomic and functional success by primary
      vaginal dilation. In cases in which surgical intervention is required, referrals 
      to centers with expertise in this area should be considered because few surgeons 
      have extensive experience in construction of the neovagina and surgery by a
      trained surgeon offers the best opportunity for a successful result.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Obstet Gynecol
JT  - Obstetrics and gynecology
JID - 0401101
RN  - Mullerian aplasia
SB  - AIM
SB  - IM
MH  - 46, XX Disorders of Sex Development/*diagnosis/psychology/*surgery
MH  - Advisory Committees
MH  - Congenital Abnormalities/*diagnosis/psychology/*surgery
MH  - Female
MH  - Gynecologic Surgical Procedures/methods
MH  - Humans
MH  - Infant, Newborn
MH  - Mullerian Ducts/*abnormalities/surgery
MH  - *Practice Guidelines as Topic
MH  - Quality of Life
MH  - Reconstructive Surgical Procedures/*methods
MH  - Risk Assessment
MH  - Treatment Outcome
MH  - United States
MH  - Urogenital Abnormalities/diagnosis/surgery
EDAT- 2017/12/22 06:00
MHDA- 2018/01/09 06:00
CRDT- 2017/12/22 06:00
PHST- 2017/12/22 06:00 [entrez]
PHST- 2017/12/22 06:00 [pubmed]
PHST- 2018/01/09 06:00 [medline]
AID - 10.1097/AOG.0000000000002458 [doi]
AID - 00006250-201801000-00041 [pii]
PST - ppublish
SO  - Obstet Gynecol. 2018 Jan;131(1):e35-e42. doi: 10.1097/AOG.0000000000002458.