mullerian duct anamolies

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1 : Mullerian Duct Anomalies
2 : MULLERIAN DUCTS Paired ducts derived from intermediate mesoderm. Known as paramesonephric duct. Named after Johannes peter mullero-described them in 1830.
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4 : EMBRYOLOGICAL DEVELOPMENT Appear between 5-6 wks. Derived from intermediate mesoderm lateral to wolffian duct as invegination of dorsal coelomic epithelium. Depends on absence of male determining factor which is present in Y-chromosome.
5 : Cont. Female development called Basic developmental path of the human embryo –requiring not estrogen but the absence of testosterone. Ambisexual period persist upto 8 wks,thereafter one type of duct system persist & other disappears.
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7 : Cont. Mullerian ducts form as buds of coelomic epithelium . Grows downward & lateral to corresponding wolffian ducts. Turn inwards & crosses anterior to it joining its fellow from opposite side.
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9 : Cont. Consists of- Upper vertical part lateral to wolffian duct ? fallopian tube. Middle horizontal part crossing wolffian duct ? remaining part of fallopian tube. Lower vertical part fusing to opposite part ? uterus, cervix, upper 1/3rd of vagina.
10 : Cont. VAGINA Develops in 3rd month of embryonic life. From lower end of uterovaginal canal (mullarian duct) & urogenital sinus. Uterovaginal canal fuses with sinovaginal bulb (develops from posterior aspect of urogenital sinus)forming vaginal plate. Later canalizes to form vaginal canal.
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12 : Cont. Upper 1/3rd develops from mullerian duct – mesodermal. Lower 2/3rd develops from vaginal plate - endodermal.
14 : Complete formation & differentiation of mullerian duct into female reproductive system depends on completion of 3 phases of development as follows- ? Organogenesis ? Fusion ? Septal resorption
15 : ORGANOGENESIS One or both mullerian duct may not develop fully-Uterine agenesis or hypoplasia Unicornuate uterus. FUSION Lateral fusion- process during which lower segment of paired mullerian duct fuse – Uterine didelphys or Bicornuate uterus,Arcuate uterus.
16 : Cont. ? Vertical fusion- Fusion of ascending sinovaginal bulb with descending mullerian duct – Transverse vaginal septum, Imperforate hymen. SEPTAL RESORPTION – after fusion central septum persist later resorps to form single uterocervical cavity – Septate uterus
17 : INCIDENCE There are irregularities in incidence & prevalence rate because of – Non standardization of classification system. Non-uniform diagnostic modalities. Different study population.
18 : Cont. Normal / Fertile women-1.5-4.5% Infertile patients – 3-6% Women with recurrent miscarriage - 5-10% DISTRIBUTION Septate uterus Bicornuate Arcuate uterus Unicornuate uterus Didelphys uterus
19 : CLASSIFICATION  AMERICAN FERTILTY SOCIETY CLASSIFICATION (1988) I. Segmental or complete agenesis or hypoplasia Agenesis and hypoplasia may involve the vagina, cervix, fundus, tubes, or any combination of these structures. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is the most common example in this category. II. Unicornuate uterus With rudimentary horn – ? With endometrial cavity- -Communicating -Non communicating ? Without endometrial cavity Without rudimentary horn
20 : Cont. III. Didelphys uterus Complete or partial duplication of the vagina, cervix, and uterus characterizes this anomaly. IV. Bicornuate uterus Complete bicornuate uterus -uterine septum that extends from the fundus to the cervical os. Partial bicornuate uterus - septum, which is located at the fundus. In both variants, the vagina and cervix each have a single cavity.
21 : Cont. V. Septate uterus A complete or partial midline septum is present within a single uterus. VI. Arcuate uterus A small septate indentation is present at the fundus. VII. DES-related abnormalities T-shaped uterine cavity with or without dilated horns is evident.
22 : Mullerian Agenesis/Hypoplasia Recently termed-mullerian aplasia. C/by absence or hypoplasia of uterus proximal vagina & in some cases fallopian tubes. TWO VARIANT ? Partial-rare ? Complete- more common Mayer-Rokitansky-Kuster-Hauser syndrome
23 : Mayer-Rokitansky-Kuster-Hauser Syndrome Congenital absence of uterus & vagina, small rudimentary uterus may be present. Normal ovarian function including ovulation. Genotype - 46xx. Phenotype – female. Associated with other congenital anomalies- (skeletal, renal).
24 : Cont. Usually diagnosed at puberty with c/o primary amenorrhea. Age appropriate secondary sexual character. Normal development of breast, body, proportionate hair distribution, external genitalia. Vaginal vault can be either absent completely or short vaginal port can be present. Hormonal profile – Normal.
25 : Cont. USG Absence of uterus & fallopian tube with normal ovaries. MRI Uterus & vagina absent. Rudimentary uterus can be seen. Coexisting renal abnormality identified.
26 : UNICORNUATE UTERUS One mullerian duct develops normally while opposite fails to develop or develop incompletely. With rudimentary horn- ? With endometrial cavity- communicating non communicating ? Without endometrial cavity- Without rudimentary horn
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28 : Cont. Associated with urological anomalies-44%. Poorest fetal survival among all mullerian anomalies b/c of – ? Insufficient muscular mass. ? Decreased uterine volume. ? Decreased ability to expand. Non communicating accessory horn having endometrial cavity-most common & most clinically significant. Can cause-hematometra , endometriosis
29 : Cont. Ectopic pregnancy in horn f/by rupture can happen-should be removed before pregnancy.
30 : HSG Useful for diagnosis, Can not detect non communicating horn. MRI Reliable. Only one fallopian tube identified. Uterine volume reduced
31 : Cont. Accessory horn appears solid but not Opacified when endometrium is absent.
32 : USG Useful for identifying rudimentary horn. Can identify communication with main uterine cavity.
33 : Cont Laproscopy Rarely indicated. Additional : IVP, Renal USG Indication of surgery-Presence of accessory horn with endometrium. Laparoscopic hemi hysterectomy of rudimentary horn treatment of choice.
34 : UTERINE DIDELPHYS Failure of medial fusion of two mullerian ducts. C/by 2 hemiuterus, 2 endocervical canal, 2 cervix, vagina can be single or double. Each hemiuterus having one fallopian tube. Renal agenesis most commonly associated with uterine didelphys.
35 : Cont. Simultaneous pregnancy can occur in each uterus –are always dizygotic. Usually asymptomatic (non obstructive) – diagnosed during pelvic ex.- two cervix seen. Associated with best possibility of successful pregnancy after arcuate uteus. May have history of recurrent second trimester abortion.
36 : Unilateral vaginal obstruction can cause hematocolpos, hemetometra, endometriosis-can be overlooked as there is cyclical menstruation from opp. Side.
37 : Cont. HSG Two uterine cavity with two cervix with two vagina. Intercornual distance-> 4 cm. MRI Two widely separeted uterus. Two cervix, two vagina. Associated renal anomaly identified.
38 : Cont. Indication of vaginal septum resection-obstructed unilateral hemivagina to preserve fertility. In non obstructive vagina – surgical correction is limited.(severe dyspareunia). Metroplasty – Rarely indicated. Results are disappointing.
39 : BICORNUATE UTERUS Incomplete fusion at the level of uterine fundus. Lower uterus & cervix are fused completely. Two separate but commmunicating endometrial cavity. Important to differentiate bicornuate uterus from septate uterus –different reproductive outcome & treatment strategies.
40 : Cont. Subclassification : depending on septum length. COMPLETE : Septum upto cervix. BICORNUATE UNICOLLIS : Septum extends to internal os. BICORNUATE BICOLLIS : Septum extends upto external os. PARTIAL : Septum confined to fundal region.
41 : Complete Partial
42 : Cont. Usually don’t have reproductive associated problem. Depends on length of septum. USG Should be done in luteal phase- endometrial echo complex is better identified. Not useful for distinguising bicornuate uterus form septate uterus.
43 : Cont. HSG Not reliable. Two chambered uterine cavity. Septal thickness is important. MRI Two uterine body with single cervix. Myometrial tissue separating two cavity has Intensity identical to that of myometrium.
44 : Cont. LAPROSCOPY Can distinguish. Surgery seldom required. Preserved for pts. with recurrent spontaneous abortion, preterm birth. Straussmann procedure –treatment of choice.
45 : SEPTATE UTERUS Failure of resorption of medial septum after complete fusion of mullerian duct. Most common. Subclassification-depending on septal length. COMPLETE : Septum upto os. PARTIAL : Septum does’t extand upto os.
46 : Cont. Fertility is not compromised yet has poorest reproductive outcome. Decreased intrauterine space for fetal growth. Implantation of placenta on poorly vascularized septum.
47 : Decision to prefer surgical correction of septum should be based on poor reproductive outcome rather than on presence of septum alone.
48 : Cont. INDICATION FOR SURGERY Recurrent spontaneous abortion. Single 2nd trimester abortion. Preterm delivery. HSG Two chambered uterine cavity. Length & thickness of septa should be assessed.
49 : Cont. LAPROSCOPY Best for distinguishing septate from bicornuate uterus. Normal fundal contour. MRI Normal fundal contour with outward convexity. Low signal intensity for septum.
50 : Cont. Surgical procedure of choice : Hysteroscopy metroplasty with concurrent laparoscopy.
51 : ARCUATE UTERUS Near complete resorption of uterovaginal septum. C/by small intrauterine indentation <1 cm in fundal region. Considered as mild form of bicornuate uterus. Clinically benign.
52 : HSG Single uterine cavity with saddle shaped fundal indentation. MRI Convex or flate contour. Cavity with broad & smooth indentation similar to myometrium.
53 : TRANSVERSEVAGINAL SEPTUM Formed when tissue between vaginal plate & caudal aspect of fused mullerian ducts fail to reabsorb. Develop at all levels. Superior vagina-46% Mid vagina-40% Inferior vagina-14%
54 : Cont. NEONATES & INFANTS Rarely diagnosed in neonates & infants – hydromucocolpos. Unlike imperforate hymen bulging vagina is not seen. USG is initial study, MRI is more useful. Management : surgical excision of septum.
55 : Cont. POST MENARCHAE Symptoms depends on whether septum is complete or incomplete. Presents with primary amenorrhea. Cyclical pelvic pain. O/E Palpable central lower abdominal or pelvic mass secondary to hematometra, hematocolpos, hematosalpinx.
56 : Cont. Local ex.- no bulging membrane. Incomplete TVS allows menstrual flow to escape periodically but hematometra, hematocolpos can later develop. SURGICAL MANAGEMENT Excision of membrane followed by vaginoplasty. Depends on location & thickness of septum.
57 : Cont. High TVS Surgical correction is more difficult. Transverse incision made in vaginal vault. Dissection done between bladder & rectum upto cervix. Lateral margin of septum excised. Hematocolpos drained. An form channel for drainage placed in vagina- allows epithelialization & maintains patency.
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59 : Cont. LOW, MID, THIN TVS Excised with multiple radial incision. Upper & lower segments joined with multiple delayed absorbable sutures.
60 : Surgica] cortection of transverse vaginal septum. A: The upper end of a short vagina. The small sinus tract opening, through which the patient menstruated, is shown. The line of incision is drawn through the mucous membrane between the vaginal dimple and the sinus. B: Areolar tissue is dissected through to the pocket of mucosa that covered the cervix. The rnucosa is incised. C: An anastomosis is made between the lower vagina and the upper vagina. 0: Completed vagina. It is slightly shorter than normal but of normal caliber.
63 : HYSTEROSALPINGOGRAM- Primary imaging modality. Normal uterus- typical trigone, configuration. Diagnostic criteria. Intercornual distance Distance between the distal ends of horns. Septate uterus- <2 cm. Didelphys uterus- >4 cm. Normal uterus (indeterminate in an abnormal cavity)- 2-4 cm
64 : Cont. Intercornual angle Angle formed by the most medial aspect of two uterine hemicavities. Septate uterus- <60º Bicornuate uterus- >105º T- shaped uterus A hypoplastic, irregular, T shaped uterine cavity – inutero DES exposure. Only anomaly in which HSG plays a significant role- DES exposure.
65 : Cont. Allows misdiagnosis between- Partial saptate & bicornuate uterus. Complete saptate & Uterus didelphys.
66 : ULTRASONOGRAPHY Most commonly 2D is used to evaluate. ( 75-100% sensitivity, upto 90% specificity). Diagnose associated urological anomaly. I Hypoplasia/Agenesis Absence of uterus & cervix- Agenesis. Hypoplastic uterus <2 cm intercornual distance-Hypoplasia.
67 : Cont. Unicornuate uterus- Difficult to differantiate from normal. Banana shaped uterus. Laterally positioned. Rudimentary horn- soft tissue mass with echogenicity similar to myometrium. Obstructed horn with functioning endometrium-complex hemorrhagic cyst.
68 : Cont. Uterus didelphys- Two separate uterus with two cervix, separate vagina difficult to see. Endometrial & myometrial zonal width are preserved. Bicornuate uterus- Two uterine cavity with normal endometrium. Reliable means of distinguisihing bicornuate from septate uterus.
69 : Cont. Concave fundus with fundal cleft >1 cm. Increased intercornual distance>4 cm. Intervening septum echogenicity similar to myometrium. Septate uterus Convex fundal contour. Intercornual distance <2 cm. Intervening septum composed of muscle or fibrous tissue.
70 : Cont. Arcuate uterus May be ditected. Fundal cleft <1 cm. Clinically not significant. DES related Uterine hypoplasia. Diagnosed confidently by HSG.
71 : 3 D ULTRASONOGRAPHY Permits accurate diagnosis. Sensitivity & specficity-98.4%,100% resp. It is best performed during the secretory phase of the menstrual cycle so the endometrial cavity is easier to outline. The coronal plane is the most valuable in the detection of uterine anomalies, shows the entire endometrial canal & its relation to myometrium and the uterine serosa. Multiple planes can be constructed regardless of uterine position.
72 : Cont. Accurately analyses uterine structure, contour of fundus, muscular thickness, septum length.
73 : MAGNETIC RESONANCE IMAGING Gold standard for diagnosing uterine anomaly. Evaluate concomitant urinary tract anomaly. Hypoplasia/agenesis Absence of uterus, cervix & upper vagina. Hypoplastic uterus <2 cm intercornual distance-Hypoplasia. Zonal differentiation is poor.
74 : Cont. Unicoruate uterus- Banana or cigar shaped uterine cavity. Laterally deviated. Preserved zonal anatomy. Rudimentary horn- soft tissue mass with intensity similar to myometrium. Obstructed horn with functioning endometrium-distended with blood or blood products.
75 : Cont. Uterus didelphys Two separated uterus & cervix. Preserved zonal anatomy. Septum of low signal intensity seen within the upper vagina. Bicornuate uterus Two uterine cavity with single cervix. Preserved zonal anatomy.
76 : Cont. Concave fundus with fundal cleft >1 cm. Increased intercornual distance>4 cm. Intervening septum intensity similar to myometrium. Septate uterus Normal shaped uterus. Two separate uterine cavity. Fundal segment of septum with intensity similar to myometrium. Fibrous segment with low intensity.
77 : Cont. Arcuate uterus Normal shaped uterus with single uterine cavity. Slightly concave or flate external contour. DES related Small uterus with T-shaped cavity.
79 : VAGINAL AGENESIS Aim:- to create a neovagina. Non surgical Initial therapy. Frank (1938) described non surgical method to create neovagina using sequential application of wider & longer dilator. Ingram technique- Series of graduated dilator dilate vaginal space. Patient instructed to sit on racing type bicycle seat for at least 2 hrs/day at interval of 15-30 min.
80 : Cont. SURGICAL Non surgical treatment fails-considered. Without use of abdominal contents- Without dissecting cavity- Williams vulvovaginoplasty(1964) Vecchietti procedure(1965) Dissecting cavity & lining with graft- Mc Indoe operation Dermis graft Amnion graft Flaps-Musculocutaneous, fasciocutaneous With use of abdominal contents- Peritoneum Intestinal contents.
81 : Mc Indoe operation- Most common. Most satisfactory results - Procedure of choice. Three important principles- Dissection of adequate space between bladder & rectum. Inlay a split thickness skin graft. Prolonged dilatation during the contractile phase of healing.
82 : Cont. Technique Skin graft taken. 0.018 inch thick, 8-9 cm wide, 16-20 cm in length. Vaginal form of adequate size is prepared by applying skin graft over it. Neo vaginal space is created. Form placed in neo vagina, edge of graft sutured with skin edge.
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85 : Cont. Post operative management Bed rest in upright and flate position for one week. Antibiotics. Low residue diet. Replacing with new form- Removed after 7-10 days.vaginal cavity irrigated with warm saline. Inspection of cavity to determine the take of graft. New form applied. Applied continuously for 6 wks except at the time of urination & defication. Next 12 months-new form applied only during night.
86 : Cont. Complication Post operative infection. Intra - postop haemorrhage. Post operative fistula formation. Failure of graft takeup. Later granulation formation. Malignant transplantation.
87 : Williams Vulvo vaginoplasty Full thickness skin flap of labia majora to create a vaginal pouch. Advantages- Technique is simple. Less local complication. Early recovery. Operation of choice for unsatisfactory Mc Indoe operation. After extensive pelvic surgery or radiation therapy.
88 : The Williams vulvovaginoplasty. A through C: No. 3-0 polyglycolic acid sutures can be used throughout to close both inner aud outer skin margins and the tissue between. D: The entrance to the ponch should not cover the external urethral tneatus.
89 : VECCHIETTI OPERATION An olive-shaped device placed at vaginal opening and with laparoscopic guidance connected to a traction device on lower abdomen. The traction device is tightened every day, gradually pulling the olive-shaped device inward to create a vagina over about a week. removal needs further manual dilation.
90 : UNICORNUATE UTERUS Indication of operation- Presence of endometrium in the accessory horn. Treatment of choice- Laproscopic hemihyserectomy of rudimentary horn.
91 : UTERUS DIDELPHYS Uterus didelphys with obstructed unilateral vagina- Full excision and marsupalization of vaginal septum. Non obstructed- Surgery rarely indicated. Recommended procedure is Strassmann metroplasty.
92 : BICORNUATE UTERUS Seldom requires surgical reconstruction. Treatment of choice – Strassmann procedure. Older procedures – Jones metroplasty Tompkins metroplasty
93 : Cont. STRASSMANN METROPLASTY Incision given in medial side of each hemicorpus, deep enough to enter the endometrial cavity. The incision extends from the superior aspect of each horn, near the interstitial region of the fallopian tubes, to the inferior aspect of the uterus. The goal is to achieve a single endocervical canal. If 2 cervices are present, their unification is not recommended.
94 : Cont. Apposition of the myometrium After resecting the wedge, the myometrial edges naturally evert. Apposition of the opposing myometrium is achieved using interrupted vertical figure-8 sutures along the posterior and anterior uterine walls. The final layer is closed using continuous subserosal sutures, without exposing any suture material to the peritoneal cavity. Transvaginal dilatation of the cervix is performed, assuring proper endometrial cavity drainage.
95 : FIGURE 25.21. The Strassmann metropinsty with modification. A: If a rectovesical ligament is found, it should be removed. B: Ao incision is made on the medial side of each hemicorpus and carried deep enough to enter th uterine cavity. The edges of the myometrium will evert to face the opposite side. C and D: The myometriurn is approximated by use of interrupted vertical figure-of-eight 3—0 polyglycolic acid sutures. O... should avoid placing sutures too lose to the interstitial portion of the fallopian tubes. E: A contiouaus.S—0 polyglycolic acid subserosal suture is used as a final layer. Tourniquets are removed, aad defects in the broad ligament are closed.
96 : Jones Metroplasty Abdominal approach. Wedge shaped incision given at the top of fundus within 1 cm of insertion of tubes. Uterine septum excised as wedge. Uterus closed in three layers with interrupted stitches.
97 : TOMPKINS METROPLASTY Abdominal approach. Single median incision given divides uterine corpus in half. Each lateral half incised to within 1 cm of tubes. Myometrium is reapproximated. Leaves the uterotubal junction in a more normal and lateral position.
98 : SEPTATE UTERUS Indication of surgery- Recurrent spontaneous abortion History preterm labour. Procedure of choice- Transcervical hysteroscopic lysis of uterine septum with concurrent laproscopy.
99 : Cont. The laparoscope is placed. Hysteroscopy commences,and the hysteroscope is inserted to the level of the external os. Operative hysteroscopic metroplasty can be performed by using microscissors, electrosurgery, or a laser. Dissection of the septum is complete when The hysteroscope can be moved freely from 1 tubal ostium to the other . the tubal ostia are visualized simultaneously, and bleeding occurs from small vessels at the fundal myometrium.
100 : Cont. Post op management- Placement of intrauterine device for a month - controversial. Conjugated estrogen and progesteron added to facilitate epithelialization. After one month follow with HSG & Hysteroscopy.
101 : CONCLUSION Müllerian anomalies are a morphologically diverse group of developmental disorders that involves the internal female reproductive tract. Establishing an accurate diagnosis is essential for planning treatment and management strategies. The surgical approach for correction of müllerian duct anomalies is specific to the type of malformation and may vary in a specific group. For most surgical procedures, the critical test of the procedure's value is the patient's postoperative ability to have healthy sexual relations and achieve successful reproductive outcomes.
102 : Thank you


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