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Congenital malformation of female reproductive organ
- 1. “Congenital malformation of femalereproductive organ” Prepared by: Anju Bista
- 2. Congenital Malformation • Congenitalabnormalities of the female reproductive tract are developmental issues that form in the embryo. • can be in the vagina, cervix, uterus and fallopian tubes that occur while the child is growing in the womb. • can arise out of abnormal or missing genes, but those who don’t have genetic abnormalities can also have this condition.
- 3. Congenital Malformation • Maygo unnoticed even during puberty or after menstruation has begun, getting diagnosed only when they are pregnant or trying to conceive. • Abnormalities of the reproductive tract may be accompanied by congenital disorders of the urinary tract, kidneys and spine.
- 5. Causes of malformation •Genetic defects • Heredity • Drugs used during pregnancy • Radiation • Chemical factors • Hormonal imbalance
- 6. Types of femalereproductive tract Congenital Abnormalities: 1. Development anomalies of the external genitalia:
- 7. 1. Development anomaliesof the external genitalia: a.Perineal or vestibular anus: detected at birth. • The usual anal opening site is evidenced by anal pit. • The anal opening is situated either close to the posterior end of the vestibule or in the vestibule. • Rarely, it is situated in the vagina (congenital recto vaginal fistula). • opening is usually sufficiently big and continence is present. no problem in future reproduction. • The delivery should be by cesarean section
- 8. b. Ectopic Ureter: The additional ureteric opening is usually in the vestibule close to the urethra or in the vagina. The main symptom is uncontrollable wetness. Partial nephrectomy and ureterectomy may be indicated or implantation of the ectopic ureter into the bladder may be done.
- 10. 2.Abnormalities of Vagina: •Narrow Introitus. • Hymen abnormality. • Septum. • Agenesis. • Associated abnormalities.
- 11. 1.Narrow Introitus: • Theexistence is revealed after marriage. • Dyspareunia may be the first complaint, or it may be detected during investigation of infertility. • Treatment is effective by manual stretching under general anesthesia or by surgical enlargement (Perineoplasty/Fenton’s operation).
- 12. 2.Hymen Abnormality: • Grosshymenal abnormality of significance is imperforate hymen. • Due to failure of disintegration of the central cells of the Müllerian eminence that projects into the urogenital sinus. • Always unnoticed until the girl attains the age of 14–16 years. • Uterus is functioning is normal - menstrual blood is pent up inside the vagina behind the hymen (cryptomenorrhea).
- 14. Hymen Abnormality(Contd…) • Dependingupon the amount of blood so accumulated, it first distends the vagina (hematocolpos). The uterus is next involved and the cavity is dilated (hematometra). • In the late and neglected cases, the tubes may also be distended after the fimbrial ends are closed by adhesions (Hematosalpinx). • Hematocolpos: accumulation of menstrual blood in the vagina • Hematometra: accumulation of blood in the uterus
- 15. Hymen abnormality Clinicalfeatures : • Age: 14-16 • Chief complaint: continuous periodic lower abdominal pain. • Primary amenorrhea . • Urinary symptoms like frequency ,dysuria or even retention of urine – due to elongation of the urethra .
- 16. On examination ofhymen abnormality • Abdominal examination Suprapubic swelling • Vulval inspection: Tense bulging membrane of bluish colouration • Rectal examination: Bulged vagina
- 17. • Treatment ofhymen abnormality Cruciate incision is made in the hymen. Quadrants of the hymen are partially excised not too close to the vaginal mucosa Spontaneous escape of dark tarry coloured blood is allowed. Antibiotic is given.
- 18. c. Vaginal septum: •Transverse vaginal septa are due to faulty fusion of canalization of the urogential sinus and the mullerian ducts. • About 45% occur in the upper vagina. 40% in mid vagina and 15% in the lower vagina. • Septum located in the lower vagina is often complete and the sign and symptoms are similar of that imperforated hymen.
- 19. c. Vaginal septum: •principles of surgical treatment are the same. Septum in the upper vagina is often perforated. • Incision of a complete (imperforate septum become easy when the upper vagina is distended - this reduces the risk of injury to adjacent organs.
- 20. • Longitudinal septumof the vagina may be present when the distal parts of the Müllerian ducts fail to fuse (fusion failure). • may be associated with double uterus and double cervix. • may be asymptomatic and needs no treatment. • may cause dyspareunia or may obstruct delivery. • In such circumstances, the septum is to be excised. Results of surgery are good in terms of achieving pregnancy.
- 22. d. PARTIALAGENESIS OFUPPER VAGINA A segment of vagina may be atretic in the upper- third. It is often associated with hypoplasia or even absence of cervix. Uterus may be normal and functioning or malformed. Primary amenorrhoea (cryptomenorrhea), hematometra, hematocolpos, cyclic lower abdominal pain and presence of lower abdominal mass (as felt per abdomen or per rectum) point to the diagnosis
- 23. PARTIALAGENESIS OF UPPERVAGINA • Conventional treatment is hysterectomy-ovaries should be conserved this gives the benefit of endogenous estrogen. • abdominovaginal approach is made to establish communication between the utero vaginal canal above and the newly created vagina below. • Prosthesis is used to prevent re stenosis. • result is, however, not always satisfactory though successful pregnancy and live birth have been reported.
- 24. e. COMPLETE AGENESISof vagina • Is almost always associated with absence of uterus. • There is, however, presence of healthy gonads and fallopian tubes. The patient is phenotypically female, with normal female karyo type pattern. • The entity is often associated with urinary tract (40%) and skeletal (12%) malformation. This is called Mayer-Rokitansky-Küster- Hauser syndrome. The patient usually seeks advice for primary amenorrhea and dyspareunia.
- 25. COMPLETE AGENESIS ofvagina Treatment - needs repeated psychological counseling for depressed concerning their sexual and reproductive life. • Nonsurgical: Repeated use of graduated vaginal dilators for a period of 6–12 months.Presence of a vaginal dimple (1 cm) is often seen. This method (Frank, 1938) is a simple and effective one. • Surgical:. various procedures of vaginal reconstruction (vaginoplasty) are done
- 26. Surgical procedure a. McIndoe-Reedprocedure (1938): A space is created digitally between the bladder and the rectum. Split thickness skin graft is used over a mould. This mould is kept in this neovaginal space. b. Williams Vulvovaginoplasty (1976): A vaginal pouch is created from skin laps of labia majora in the midline. • This is not done these days. c.Vaginoplasty with amnion graft:
- 27. 3. ASSOCIATED ABNORMALITIES: Associatedabnormalities with: a.Vesicovaginal fistula is formed when the Müllerian eminence ruptures into the vesicourethral part of the cloaca instead of the pelvic part of the urogenital sinus . b.Rectovaginal fistula when the Müllerian eminence opens in the dorsal segment of the endodermal cloaca. c.Persistent urogenital sinus with various irregularities of urethral and vaginal orifices in the sinus.
- 29. 4.Uterine Anomalies : •American Fertility Society (AFS) Classification of Müllerian Anomalies (1988) are : • Class I: Müllerian agenesis/Hypoplasia segmental, • Class II: Unicornuate uterus, • Class III: Didelphys uterus, • Class IV: Bicornuate uterus, • Class V: Septate uterus, • Class VI: Arcuate uterus, • Class VII:Diethylstilbestrol (DES)-related abnormality
- 30. CONTD… • Incidence ofMüllerian abnormalities: It varies between 3 and 4%. The incidence is found to be high in women suffering from recurrent miscarriage or preterm deliveries (5–20%).
- 31. Failure ofdevelopment of one or both Müllerian ducts • The absence of both ducts leads to absence of uterus, including oviducts. • There is absence of vagina as well. • Primary amenorrhea is the chief complaint. • The absence of one duct leads to a unicornuate uterus with a single oviduct.
- 32. CONTD… Failure ofre canalization of the Müllerian ducts • Agenesis of the upper vagina or of the cervix— This may lead to hematometra as the uterus is functioning
- 33. Failure of fusionof Müllerian ducts • In majority, the presence of deformity escapes attention. In some, the detection is made accidentally during investigation of infertility or repeated pregnancy wastage. • In others, the diagnosis is made during D + E operation, manual removal of placenta or during cesarean section
- 34. • Arcuate (18%):The cornual parts of the uterus remains separated.The uterine fundus looks concave with heart-shaped cavity outline. Types of fusion Anomalies :
- 35. • Uterus didelphys (8%):There is complete lack of fusion of the Müllerian ducts with a double uterus, double cervix and a double vagina .
- 36. Types of fusionAnomalies contd… • Uterus bicornis (26%): There is varying degrees of fusion of the muscle walls of the two ducts. Uterus bicornis bicollis: There are two uterine cavities with double cervix with or without vaginal septum. Uterus bicornis unicollis: There are two uterine cavities with one cervix. The horns may be equal or one horn may be rudimentary and have no communication with the developed horn .
- 38. Types of fusionAnomalies contd… • Septate uterus (35%): The two Müllerian ducts are fused together but there is persistence of septum in between the two either partially (sub septate) or completely . • Uterus unicornis (10%): Failure of development of one Müllerian duct.
- 39. Types of fusionAnomalies contd… • DES-related abnormality : It is due to DES exposure during intrauterine life. Varieties of malformations are included, e.g: Vagina: Adenosis, adenocarcinoma. Cervix: Cockscomb cervix, cervical collar. Uterus: Hypoplasia, T-shaped cavity, uterine synechiae. Fallopian tube: Cornual budding, abnormal fimbriae. Such cases are not seen now
- 40. Clinical features Condition maynot produce any clinical manifestation. • Gynecological Infertility and dyspareunia are often related in association with vaginal septum. Dysmenorrhea in bicornuate uterus or due to cryptomenorrhea (pent up menstrual blood in rudimentary horn). Menstrual disorders (menorrhagia, cryptomenorrhea) are seen. Menorrhagia is due to increased surface area in bicornuate uterus.
- 41. Obstetrical Manifestation Midtrimesterabortion which may be recurrent. Rudimentary horn pregnancy may occur due to transperitoneal migration of sperm or ovum from the opposite side. Cornual pregnancy (ectopic) inevitably ends in rupture around 16th week. Cervical incompetence. Increased incidence of malpresentation— transverse lie in arcuate or subseptate, breech inbicornuate, unicornuate or complete septate uterus.
- 42. Obstetrical Manifestation Preterm labor,IUGR, IUD. Prolonged labor—due to incoordinate uterine action. Obstructed labor—obstruction by the nongravid horn of the bicornuate uterus or rudimentary horn. Retained placenta and postpartum hemorrhage where the placenta is implanted over the uterine septum.
- 43. Diagnosis: • Internal examinationreveals septate vagina and two cervices. • Passage of a sound can diagnose two separate cavities. In fact in significant number of cases, the clinical diagnosis is made during uterine curettage,manual removal of placenta or cesarean section. • For exact diagnosis of the malformation, internal as well as external architecture of the uterus must be visualized.
- 44. Diagnosis: • several investigationsin different combinations are done, such as hysterography, hysteroscopy laparoscopy, ultrasonography (vaginal probe) and magnetic resonance imaging (MRI). • Ultrasonography and MRI are noninvasive procedures. • Urological tract is also evaluated at the same time. • The renal tract abnormality in association with Müllerian abnormality is about 40%. Skeletal system anomaly (12%) is also associated
- 45. Treatment • Many uterineAnamolies require no treatment. Reproductive outcome: • Better obstetric outcome in septate uterus (86%), bicornuate uterus (50%) has been mentioned. • Unicornuate uterus has very poor (40%) pregnancy outcome. No treatment is generally effective. • Uterus didelphys has best possibility of successful pregnancy (64%).Unification operation is generally not needed.
- 46. Treatment • Rudimentary hornshould be excised to reduce the risk of ectopic pregnancy (8%) • Unification operation (bicornuate/septate uterus) is, therefore, indicated in otherwise unexplained cases with uterine malformation. • Abdominal metroplasty could be done either by excising the septum (Strassman, Jones, and Jones) or by incising the septum (Tompkins). Success rate of abdominal metroplasty in terms of live birth is high (5–75%).
- 47. Treatment • Hysteroscopic metroplastyis more commonly done. Resection of the septum can be done either by a resectoscope or by laser . Advantages are: High success rate (80–89%), Short hospital stay, Reduced postoperative morbidity (infection or adhesions) Subsequent chance of vaginal delivery is high compared to abdominal metroplasty where cesarean section is mandatory.
- 48. 5. ABNORMALITIES OFTHE FALLOPIAN TUBES: • The tubes may be unduly elongated; may have accessory ostia or diverticula. • Rarely, the tube may be absent on one side. These conditions may lower the fertility or favor ectopic pregnancy.
- 49. 6. ANOMALIES OFTHE OVARIES: • There may be streak gonads or gonadal dysgenesis, which are usually associated with errors of sex chromosomal pattern. • No treatment is of any help. • Accessory ovary (division of the original ovary into two) may be rarely (1 in 93,000) present. • Rarely, supernumerary ovaries may be found (1 in 29,000) in the broad ligament or elsewhere. This can explain a rare event where menstruation continues even after removal of two ovaries
- 50. 7. WOLFFIAN REMNANT ABNORMALITIES: •The outer end of the Wolffian (Gartner) duct may be cystic, size of pea, often pedunculated (hydatid of Morgagni) and attached near the outer end of the tube. • The tubules of the Gartner’s duct may be cystic, the outer ones are Kobelt’s tubules, the middle set, the epoophoron and the proximal set, the paroophoron.
- 51. 7. WOLFFIAN REMNANT ABNORMALITIES: •Small cyst may arise from any of the tubules. A cystic swelling from the Gartner duct may appear in the anterolateral wall of the vagina, which may be confused with cystocele.
- 52. 8. PARO VARIANCYST: • It arises from the vestigial remnants of Wolffian tissue situated in the meso salpinx between the tubeand the ovary. • This can attain a big size. • The cyst is unilocular ; the wall is thin and contains clear translucent fluid.The ovarian fimbria with the ovary is stretched over the cyst. • The wall consists of connective tissue lined by single layer of low columnar epithelium.
- 53. 9.Other Abnormalities: 1. LABIAMINORA: Labial fusion: True due to developmental defect . Inflammatory 2. LABIA MAJORA: Hyperplastic or hypoplastic labia. Abnormal fusion in adrenogenital syndrome. 3. CLITORIS : Clitorial hypertrophy—often associated with various intersex problems .
- 54. 9.Other Abnormalities: 4. PERINEUM:Perineum differentiates from the area of contact between the urorectal septum (mesoderm) and the dorsal wall of cloaca (endoderm) at about 7th week. • This site of contact between the two is the perineal body. Malformations of the perineum are rare.
- 55. 4. PERINEUM: • Imperforateanus, anal stenosis or fistula are the result of abnormal development of the urorectal septum. This is due to the posterior deviation of the septum. Anal agenesis and fistula are rare. • The anal fistula may open into the posterior aspect of the vestibule of the vagina
- 56. Research study: • Congenitalanomalies of reproductive system among infertile women attending Center for Infertility and IVF in Maternity Teaching Hospital in Erbil city
- 57. Research study: Result: • Theprevalence of congenital reproductive system anomaly was 4.1% among infertile women. The sub- septate uterus was the most common type (23.24%) anomalies. The genetic factor was found to has a significant role in developing such a condition. Success rate after management significantly raised to 61.1% with sub – Septate uterus (91.35) , bicornuate /partial ( 85.7%) and Septate / complete (72.2%). One of the causative factors of female infertility is congenital anomaly. Most type have a good prognosis in achieving pregnancy after proper management.
- 58. Reference: • Dutta ,DC:text book of gyanacology. central book agency,culcatta.2009. • Ranabhat,R , Niraula, H.A text book of midwifery and reproduction health.Kathmandu.national health centre for health professions education;2017
- 59.