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JKAUSCIMED-V22-2-FINAL MRV-ORIGINAL 21X28.Indd The Role of Laparoscopy in Management of Congenital Anomalies of the Müllerian System: Literature Review and Case Series E.A. Aljahdali REVIEW ARTICLE The Role of Laparoscopy in Management of Congenital Anomalies of the Müllerian System: Literature Review and Case Series Ettedal A. Aljahdali, MBBCH, SBOG, CBG OBGYN AFSA Department of Obstetrics and Gynecology, Faculty of Medicine King Abdulaziz University, Jeddah, Saudi Arabia Correspondence Abstract Dr. Ettedal A. Aljahdali Structural defects of the female reproductive tract become apparent P.O. Box 80215, Jeddah 21589, Saudi Arabia e.M: [email protected] at varying chronologic times during intrauterine life. The diagnosis of all female reproductive tract abnormalities has benefi ted from Submission: 17 Dec. 2014 enhancements in imaging techniques and the surgical treatment Accepted: 9 Feb. 2015 thereof has improved due to advances in the techniques and instrumentation of both surgical and non-surgical procedures. In the Citation Aljahdali EA. The role of laparoscopy in last decade, laparoscopy has become increasingly popular and has management of congenital anomalies of the successfully replaced several open surgical procedures. It has been Müllerian system: Literature review and case applied to many aspects of gynecological surgery and has become series. JKAU Med Sci 2015; 22 (2): 39-46. DOI: the procedure of choice for gynecological surgery. A review of the 10.4197/Med. 22.2.6 recent literature continues to support the utilization of laparoscopy for the management of genital tract malformation. This review aims to appraise the recent literature to determine how laparoscopy may aid in the diagnosis of reproductive tract anomalies and their treatment, its success in replacing the open surgery in some cases, and the presentation of some cases that were successfully diagnosed and treated with laparoscopy. Keywords Laparoscopy; Müllerian system anomalies; Literature Review Introduction and instrumentation used for both surgical and non- Congenital abnormalities of the Müllerian ducts surgical procedures[2]. often escape diagnosis for a long period of time In the last decade, laparoscopy has become very due to a lack of symptoms. Despite this asymptomatic popular and has successfully replaced several open nature, these abnormalities may negatively impact a surgical procedures. It has been applied to many woman’s health, both obstetric and gynecological[1]. aspects of gynecological surgery, including accurate The management of all female reproductive tract diagnosis and treatment of anomalies. In addition, anomalies has seen dramatic improvement resulting laparoscopy off ers the critical benefi t of preventing or from enhancements in the imaging techniques used reducing adhesions, which makes it the procedure of for diagnosis, in addition to advances in the techniques choice for gynecological surgery[3,4]. Journal of King Abdulaziz University - Medical Sciences 2015 Volume 22, No. 2 www.jkaumedsci.sa 39 The Role of Laparoscopy in Management of Congenital Anomalies of the Müllerian System: Literature Review and Case Series E.A. Aljahdali Due to the, usually, asymptomatic nature, and second trimester of pregnancy. Primordial germ cells the resulting low rate of diagnosis, it is diffi cult to migrate to the genital ridge to begin the development accurately evaluate the incidence of congenital uterine of the gonads, the type of which is governed by anomalies. However, the current best estimates indicate the chromosomal sex. An absence of Sertoli cells approximately 4.3% of fertile women, 3.5% of infertile 6 to 7 weeks after conception indicates ovarian women, and 13% of women with recurrent pregnancy diff erentiation and after 13.5 weeks the ovarian loss suff er these abnormalities[5]. primordial follicles will be identifi able[8]. The Müllerian ducts can be identifi ed around the sixth week of One well-designed study evaluated the uteri of development. The fusion of the caudal part of the 679 women who had experienced normal reproductive Müllerian ducts forms the uterovaginal canal near the outcomes with laparoscopy or laparotomy before 12th week of gestation, which then inserts itself into the tubal ligation, and hysterosalpingogram fi ve months dorsal wall of the urogenital sinus at Müller’s tubercle. after sterilization[6]. The prevalence of abnormalities Cranial unfused parts form the fi mbria and fallopian was estimated to be 3.2% in total, with septate uteri tubes, while the caudal, fused parts form the uterus comprising 90%, bicornuate uteri 5%, and didelphic and upper two thirds of the vagina. The sinovaginal uteri 5%[5-7]. Other studies of congenital anomalies bulbs are two solid cords of tissues in the urogenital of the Müllerian system in general (including uterus, sinus at the distal aspect of the Müllerian tubercle. The vagina, cervix, tubes) estimate between 0.1% and 1.5% sinovaginal bulbs proliferate into the caudal end of the of women in the general population are aff ected, with uterovaginal canal to form a solid vaginal plate, which 90% of these anomalies mainly aff ecting the uterus. will be canalized after the degeneration of the central These anomalies are often only discovered cells to form the vaginal lumen; this process is normally incidentally, or during the investigation of infertility, complete by the 20th week. The vaginal lumen is or following repeated fetal loss. Laparoscopy has separated from the urogenital sinus by the hymenal become the primary choice for accurately diagnosing membrane which usually ruptures before birth due to and successfully treating uterovaginal abnormalities degeneration of the central epithelial cells. However, because it provides a superior visual fi eld, including of a thin fold of mucous membrane persists around the the pelvic structures, a good monitor for endometriosis vaginal introitus[9]. and hysteroscopic procedures, replace absent vagina, resects abnormal Müllerian structures with the benefi t of shorter recovery time, and reduced postoperative Classifi cation adhesion and pain[4]. A classifi cation system is a trial to arrange the disorders into groups based on symptomatology, treatment, or This review aims to appraise the recent literature prognosis. Unfortunately, Müllerian defects have yet to to determine how laparoscopy may aid in the diagnosis be classifi ed into a universally accepted and interpreted of reproductive tract anomalies and their treatment, system[9]. its success in replacing the open surgery in some cases, and the presentation of some cases that were The American Fertility Society in 1988 produced a successfully diagnosed and treated with laparoscopy. standard form for classifi cation of Müllerian defects. Type I: Müllerian agenesis or hypoplasia. The Literature Review Type II: Unicornuate uterus. The search was done for genital tract anomalies and laparoscopy between 1990-2014, including case Type III: Uterus didelphys. reports, reviews, and articles. The reference lists of the Type IV: Uterus bicornuate. identifi ed articles were searched for further relevant articles. A textbook was reviewed for basic science and Type V: Septate Uterus. understanding of anatomy. Type VI: Diethylstilbestrol related anomalies. This descriptive classifi cation concentrates mainly Emryology on defects of vertical fusion and dysgenesis of the Multiple overlapping steps are required for normal uterus. Consideration must also be given to related growth of the female genital tract; beginning three abnormalities in the vagina, cervix, fallopian tubes, and weeks after conception and continuing into the renal system[9]. 40 Journal of King Abdulaziz University - Medical Sciences 2015 Volume 22, No. 2 www.jkaumedsci.sa The Role of Laparoscopy in Management of Congenital Anomalies of the Müllerian System: Literature Review and Case Series E.A. Aljahdali Developmental Defects Etiology When considering the Müllerian system three common The underlying etiology of congenital Müllerian developmental abnormalities are encountered: defects is poorly understood. The karyotype of women with these anomalies is usually normal (46, XX in 92 • Agenesis percent). Most of the defects are likely to be related to • Lateral fusion defects polygenic and multifactorial causes. • Vertical fusion defects +Diethylstilbestrol induced defects, and hymenal Clinical Manifestations anomalies. Congenital anomalies of the Müllerian duct are often diagnosed incidentally during the evaluation Agenesis of gynecologic and obstetric problems, after hysterosalpingography during a work-up of infertility or The Mayer-Rokitansky-Küster-Hauser syndrome refers reproductive loss. The associated signs and symptoms to a congenital absence of the vagina in addition vary depending upon the defect involved: to variable uterine development, which results from Müllerian agenesis. Diff erent types of uterine • Adolescents may experience increased pain of the development may result in lateral hemi-uteri or uterine pelvis (either cyclic or noncyclic), dysmenorrhea, horns, a midline uterus sans cervix, or complete abnormal vaginal bleeding, or vaginal pain. absence of uterine structures. • Menstrual abnormalities: Hypomenorrhea may result from minimal endometrium; amenorrhea Lateral Fusion Defects may be a sign of Mayer-Rokitansky-Küster-Hauser The most common kind of Müllerian abnormality syndrome or a vertical fusion defect. are lateral fusion defects. The Müllerian anomalies • Longitudinal vaginal septum may occur alone, are symmetric or asymmetric, obstructed
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