INTERNATIONAL AYURVEDIC MEDICAL JOURNAL

International Ayurvedic Medical Journal, (ISSN: 2320 5091) (March, 2017) 5 (3)

DEVELOPMENTAL ABNORMALITIES OF MULLERIAN DUCT- A REVIEW Kowsalya.R.G1, Padmasaritha.K2, Ramesh M3

1PG Scholar, 2Assistant professor, 3Professor; Dept of PTSR, Sri Kalabyreshwara Swamy Ayurvedic College and Hospital And Research Centre, Vijayanagar, Bangalore, Karnataka, India

Email: [email protected]

ABSTRACT The beeja (sukra and artava rupa) has chromosomes with genes representing the future organs to be developed. Any abnormality in the beeja, beejabhaga, beejabhagaavayava leads to various conge- nital abnormalities in foetus. Mullerian duct anomalies are one of the congenital abnormalities of the female reproductive tract resulting from failure in the development of the Mullerian ducts and their as- sociated structures. Their cause has yet to be fully clarified, and it is currently believed to be multi fac- torial. Symptoms appear during adolescence or early adulthood, and affect the reproductive capacity of these women. When clinically suspected, investigations leading to diagnosis include imaging methods such as , ultrasonography and MRI. Mullerian duct anomalies consist of a wide range of defects that may vary from patient to patient. The aim is to understand the congential malfor- mation of mullerian duct through Ayurveda.

Keywords: Beeja, Beejabhaga, Mullerian duct anomalies.

INTRODUCTION

The beeja and its component are the subtle form bhagas lead to defective formation of the garb- of the future organs and parts of the body and hasaya and artava in fetus. Different degrees of the particular parts consequently develop into mullerian duct anomalies can be considered as the specific organs and parts. Acharyas states defective formation of garbhasaya and artava. that if specific part of beejabhaga avayava is Mullerian ducts are Paired ducts derived vitiated, it leads to the vitiation of that specific from intermediate mesoderm in females known organs being generated from the part. Vata do- as . Named after Johannes sha also vitiated and hampers the development peter mullero -described them in 1830. of reproductive organs. Some of the particular The mullerian ducts are paired embryologic beejabhaga is responsible for formation of structures that undergo fusion and resorption in garbhasaya and artava, vitiation of these beeja utero to give rise to the , fallopian tubes, Kowsalya.R.G Et Al: Developmental Abnormalities Of Mullerian Duct- A Review and upper 3/5 of the . Interruption via dorsal mesentry populate the posterior body of the normal development of the mullerian wall. Induce the coelomic epithelium to form ducts can result in formation of mullerian duct primitive sex cords. Sex cords proliferate and anomalies (MDA). MDA are a broad and com- produce genital ridges. The sex cords surround plex spectrum of abnormalities that are often the germ cells and will become ovarian follicle associated with many gynaecological and obste- in female sertoli cells in males. At the end of 6 tric complications. The prevalence of these weeks of fetal life, primitive ducts will be anomalies ranges from 0.001 to 10% in the gen- formed which includes both mesonephric duct eral population and from 8-10% in women with and paramesonephric ducts. After this point an adverse reproductive history1. The embryo- male and female phenotype diverges. The para- logical development of the female reproductive mesonephric ducts are situated lateral to meso- system is closely related to the development of nephric duct. In the absence of hormonal (Tes- the urinary system, and anomalies in both sys- tosterone and MIF) input these ducts will get tems may occur in up to 25% of these patients. close to each other. The two ducts are initially Other associated malformations may affect the separated by a septum but later fuse to form the gastrointestinal tract (12%) or musculoskeletal uterine canal. The united lower vertical parts system (10-12%) 2,3. form the uterovaginal canal. The unfused cranial EMBRYOLOGY part of each paramesonephric duct forms uterine At 5th week of intra uterine life tubes. The distal open end forms the abdominal primordial germs cells migrate from yolk sac ostium4. Picture 1: Embryology of Female Reproductive Organs

UNDESTANDING OF MULLERIAN DUCT To understand the origins of uterine and anomalies the process is typically related to

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 Failure of mullerian duct to fuse at a particu- Complete formation and differentiation of mul- lar point lerian duct into female reproductive system de-  Failure of the tubes to fully descend to the pends on completion of 3 phases of develop- urogenital sinus ment.

Picture 2: Differentiation of Mullerian Duct Into Female Reproductive System

A. Formation septal resorption fails to occur it will lead to B. Fusion Septate uterus. C. Septal resorption Asymmetric obstructed disorders of ute- Any defects in these three processes can lead to rus and vagina, as these tend to be associated MDA. with ipsilateral . Though there is FORMATION no direct reference for mullerian duct anomalies  One or both mullerian duct may not develop in ayurveda, it can be considered under the fully – uterine agenesis or hypoplasia, un- broad heading of beeja dusti and beeja bhaga icornuate uterus. dusti mainly. FUSION-  Lateral fusion – process during which lower BEEJA DUSTI segment of paired mullerian duct fuse. Im- Aggravated vata in mother affects arta- proper fusion can lead to uterine didelphys, va rupa beeja during garbhadana. Due to ab- , . normalities of beeja, atma, karma, asaya, kala  Vertical fusion – fusion of ascending sino- and ahara vihara, the vitiated doshas produce vaginal bulb with descending mullerian duct abnormalities of fetus, affecting its appearance, – Improper fusion can lead to Transverse varna and indriyas5.Abnormalities in beeja . leads to yonivyapad like sandi yonivyapad, su- SEPTAL RESORPTION- chimuki yonivyapad. After fusion central septum persist later resorps to form single uterocervical cavity – if

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SANDI YONIVYAPAD Whatever part of the beeja is defective; the body Acharya charaka says, due to abnormali- part developing from that portion of beeja will ties of beeja (artava) the uterus of the female is be abnormal. Beeja-bhaga is responsible for affected with vata. A woman with female phe- formation of various organs. Vitiation of beeja- notype having beeja doshas (chromosomal ab- bhaga responsible for the formation of repro- normalities) and suffering with deficient sec- ductive organ (uterus, , cervix and ondary sexual characters and un established re- vagina) leads to defective formation that par- productive functions like lack of menarche and ticular organ which exhibits congenital deformi- menstruation should be considered under sandi ties of female genital organs leading to repro- yonivayapad.6,7 ductive failure9. Sandi yonivyapad can be considered as Turner’s syndrome (XO monosomy) VANDHYA The beeja-bhaga responsible for SUCHIMUKI YONIVYAPAD formation of garbhashaya is totally vitiated it Acharya charaka says, that when a pregnant leads to vandhya. Vandhya can be consider as woman consumes vata aggravating ahara viha- different degrees of mullerian agenesis i.e. ab- ra, this aggravated vayu due to its dryness vi- sence or incomplete development of mullerian tiates yoni of female fetus, thus the orifice of ducts leading to complete absence of uterus, yoni becomes very narrow. It can be considered septate uterus, , bicornuate ute- as different grades of mullerian agenesis and rus, , arcuate uterus10,11. dysgenesis.8 MULLERIAN DUCT ANOMALIES BEEJA BHAGA DUSHTI UTERINE ABNORMALITIES Acharya Charaka has explained garbhanga vi- CLASSIFICATION OF MULLERIAN ANO- kruti depends upon the condition of beeja. MALIES-

According to AFS12 CLASS I – Agenesis/hypoplasia CLASS III – Didelphys  Vaginal CLASS IV – Bicornuate  Cervical  Complete  Fundal  Partial  Tubal CLASS V – Septate CLASS II – Unicornuate  Complete  Communicating  Partial  Non communicating CLASS VI – Arcuate  No cavity CLASS VII – DES related anomalies  No horn

INCIDENCE OF MDA13  Unicornuate - 10% Total mullerian duct anomalies – 3-4%  Uterine didelphys - 8%  Agenesis - 4%  Bicornuate uterus - 26%

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 Septate uterus - 35% dus, the endometrial cavity, which demonstrates  Arcuate uterus - 18% a small fundal cleft or impression (>1.5 cm). Mild thickening of the midline fundal myome- Class I (HYPOPLASIA/AGENESIS) includes trium resulting in fundal cavity indentation but entities such as uterine/ or hy- normal outer fundal contour. poplasia. The most common form is the Mayer- Class VII (- Rokitansky-Kuster-Hauser syndrome, which is RELATED ANOMALY) has occurred in sever- combined agenesis of the uterus, cervix, and al million women who were treated with die- upper portion of the vagina. thylstilbestrol (DES), an estrogen analogue pre- Class II (UNI-CORNUATE UTERUS) is the scribed to prevent from 1945-1971. result of complete, or almost complete, arrest of Affected female fetuses have a variety of ab- development of one Mullerian duct. If the arrest normal findings that include uterine hypoplasia is incomplete, as in 90% of patients, a rudimen- and a T-shaped uterine cavity. tary horn with or without functioning endome- CLINICAL IMPLICATIONS trium is present. are frequently asymp- Class III (DIDELPHYS UTERUS) results from tomatic and are often missed in routine gy- complete non-fusion of both Mullerian ducts. necological examinations. Nevertheless, a The individual horns are fully developed and history of pelvic pain following the me- almost normal in size. Two cervices are inevita- narche, , primary bly present. and changes to menstrual flows may be Class IV (BI-CORNUATE UTERUS) results present. from partial non-fusion of the Mullerian ducts. Among the ductal differentiation malforma- The central myometrium may extend to the lev- tions, vaginal agenesis presents with primary el of the internal cervical is (bi-cornuate unicol- amenorrhea and . In cases of lis) orexternal cervical is (bi-cornuate bicollis). uteri with a functional endometrium, hema- The latter is distinguished from didelphys uterus tometra and are frequent find- because it demonstrates some degree of fusion ings. between the 2 horns, while in classic didelphys In unicornuate uterus if a rudimentary, non- uterus, the 2 horns and cervices are separated communicating uterine horn is present to- completely. gether with a functional endometrium, he- Class V (SEPTATE UTERUS) results from matometra and sometimes hematosalpinges failure of resorption of the septum between the 2 may be found. . The septum can be partial or is generally an asymptomat- complete, in which case it extends to the inter- ic condition and is often only diagnosed nal cervical is. The uterine fundus is typically when couples with a history of repeated convex but may be flat or slightly concave (<1 miscarriage or infertility are undergoing in- cm fundal cleft). vestigation. Likewise, lateral fusion defects, Class VI (ARCUATE UTERUS) has a single which are responsible for uterus didelphys uterine cavity with a convex or flat uterine fun- and bicornuate uterus.

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Anomalies resulting from failure in vertical or with unsuccessful fusion, such as cervical agenesis, transverse outcomes vaginal septum and imperforated hymen, are These anomalies are not easily discovered on associated with primary amenorrhea, hema- routine gynaecological exams, diagnose acci- tocolpos and . dentally during imaging evaluation for another Mullerian malformations include decreased condition or during surgery. intraluminal volume, inadequate vasculari- zation of regions such as the septum, pres- INVESTIGATIONS ence of a medial wall or an unfused uterine horn and greater uterine contractility and ir- Hysterosalpingography (HSG) is the method ritability, thereby leading to used to evaluate the cervical canal, uterine cavi- and premature deliveries. ty and Fallopian tubes. Its efficacy in diagnosing anomalies is debatable and varies according to CLINICAL DIAGNOSIS the specific type of malformation Uterine anomalies are unnoticed until puberty in Three-Dimensional Ultrasonography (3D- a majority of cases. USG)has shown high specificity and sensitivity At puberty- Few cases are typically discov- in evaluations on all uterine anomalies, includ- ered with subsequent menstrual abnormali- ing Mullerian malformation ties like primary amenorrhea, primary dys- MRI- it provides high resolution images of the menorrhea. uterine body, fundus and internal structures. At childbearing age- patients present with

Table 1: Mullerian Duct Anomalies With Imaging And Findings Classification Anatomical Representation Imaging Findings Imaging AGENESIS/ HY-  Absence of the cervix / uterus POPLASIA with a blind-ending vagina.  In uterine agenesis, no uterine tissue is present.  In uterine hypoplasia, the en- dometrial cavity is small with intercornual distance < 2cm

UNICORNUATE  Banana shaped uterine UTERUS cavity.  Full development of sin- gle uterine horn  A normal appearing cer- vix

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UTERUS DIDEL-  2 uterine horn with cervix PHYS and vagina

BICORNUATE  Two uterine cavity with UTERUS single cervix

SEPTATE UTE-  Uterine cavity with sep- RUS tum

ARCUATE UTE-  A single uterine cavity RUS with a broad saddle shaped indentation at the uterine fundus

DES- RELATED  T- shaped uterus ANOMALIES

VARTA DISCUSSION The beeja-bhaga responsible for forma- In Ayurveda description of beeja, bee- tion of both garbhashaya and artava is vitiated jabhaga, beejabhagaavayava which probably it leads to varta, who is having only external resemble pronucles of gametes chromosomes feminine characters in abundance but not fe- and gene. Vitiation of dosha affects beeja, bee- male14. jabhaga, beejabhagaavayava. Beeja dusti and Varta can be considering as intersexuality with beejabhaga dusti are main cause for garbha vi- a female phenotype as in testicular feminisation kriti and yoni vyapad. Garbha vikriti is one of syndrome, triple X chromosome. the ashubha bhava of pregnancy. Developmen- tal anomalies of the mullerian duct system represent some of the most fascinating disorders that obstetricians and gynecologists encounter. They range from uterine and vaginal agenesis to

IAMJ: MARCH, 2017 953 Kowsalya.R.G Et Al: Developmental Abnormalities Of Mullerian Duct- A Review duplication of the uterus and vagina to minor sthana-30/34 editted by Vaidya yadavji tri- uterine cavity abnormalities. These abnormali- kamji acharya published by chaukhamba ties in minor form can be asymptomatic but surbharati prakashan,Varanasi 2014. when gross anomalies are present, may have a 7) Prof.K.B. Srikantha murthy, Astanga san- grave impact on health of a woman. Establish- graha, Uttara sthana- 38/45 published by ing an accurate diagnosis is essential for plan- chaukhambha orientalia,Varanasi 2005. ning treatment and management strategies. Ni- 8) Prof. R.H.Singh, Caraka samhita, chiktisa dana parivarjana, rajaswala paricharya, garb- sthana-30/31 editted by Vaidya yadavji tri- hadhana vidhi, garbini paricharya and normal- kamji acharya published by chaukhamba cy of doshas are having the prime importance in surbharati prakashan,Varanasi 2014. prevention of garbha vikriti. 9) Prof. R.H.Singh, Caraka samhita, sarira sthana-4/30 editted by Vaidya yadavji tri- CONCLUSION kamji acharya published by chaukhamba Beeja, beejabhaga, beejabhagaavayava surbharati prakashan,Varanasi 2014. dusti, directly correlated Mullerian duct anoma- 10) Prof. R.H.Singh, Caraka samhita, sarira lies. Mullerian duct developmental abnormali- sthana-4/30 editted by Vaidya yadavji tri- ties can affect the normal physiology of a wom- kamji acharya published by chaukhamba an ranging from amenorrhoea to abor- surbharati prakashan,Varanasi 2014. tions.Hence a depth knowledge of these abnor- 11) Prof.K.B. Srikantha murthy, Astanga san- malities is very much essential which will fur- graha, sarira sthana- 2/30 published by ther pave a way to diagnosis and treatment. chaukhambha orientalia,Varanasi 2005. 12) D.C.Dutta’s- Textbook Of Gynecology 6th REFERENCES edition pg no- 42-43, pp- 686 1) Pui MH. Imaging diagnosis of congenital 13) D.C.Dutta’s- Textbook Of Gynecology 6th . Comput Med Imaging edition pg no- 44, pp- 686 Graph. 2004;28(7):425-33. 14) Prof. R.H.Singh, Caraka samhita, sarira 2) Propst AM, Hill JA 3rd. Anatomic factors sthana-4/30 editted by Vaidya yadavji tri- associated with recurrent pregnancy loss. kamji acharya published by chaukhamba Semin Reprod Med. 2000;18(4):341-50. surbharati prakashan,Varanasi 2014. 3) Golan A, Langer R, Bukovsky I, Caspi E. Congenital anomalies of the müllerian sys- Source of Support: Nil tem. Fertil Steril. 1989:51(5):747-55. Conflict Of Interest: None Declared 4) D.C.Dutta’s- Textbook Of Gynecology 6th

edition pg no- 38, pp- 686. How to cite this URL: Kowsalya.R.G Et Al: 5) Prof. R.H.Singh, Caraka samhita, sarira Developmental Abnormalities Of Mullerian Duct- A sthana-4/30 editted by Vaidya yadavji tri- Review. International Ayurvedic Medical Journal {online} 2017 {cited March, 2017} Available from: kamji acharya published by chaukhamba http://www.iamj.in/posts/images/upload/947_954.pdf surbharati prakashan,Varanasi 2014. 6) Prof. R.H.Singh, Caraka samhita, chiktisa

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