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Urogenial sinus and vagial atresias

Urogenial sinus and vagial atresias

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Urogenial sinus and vagial atresias

  1. 1. Urogenital sinus and vaginal atresias By Dr Sasidhar Moderator: Dr Rajesh
  2. 2. Introduction • Abnormalities of the female Urogenital tract can present and be identified at birth through adult hood. • Most of them are congenital. • Many of them are now identified even in the fetal life owing to the advancement in the diagnostic procedures. • A large number of these patients may also present at puberty with primary amenorrhea, pelvic/abdominal pain, and difficulty using a tampon or having sex.
  3. 3. embryology • Functionally the urogenital system can be divided into the urinary system and the genital system. • Embryologically and anatomically, however, they are intimately interwoven. • Two embryonic structures play an important role in the development of the urogenital system, intermediate mesoderm and cloaca. • Both urinary system and the genital system develop from a common mesodermal ridge (intermediate mesoderm) along the posterior wall of the abdominal cavity, and initially enter into a common cavity, the cloaca.
  4. 4. Intra embryonic mesoderm: •Paraxial mesoderm •Lateral plate mesodem •intermediate mesoderm lying between these two.
  5. 5. • Intermediate mesoderm now forms a bulging on the posterior abdominal wall lateral to the attachment of the dorsal mesentery– nephrogenic cord. • It extends from the cervical to sacral region of the embryo.
  6. 6. Gonad develops from the coelomic epithelium lining the medial side of the nephrogenic cord. Excretory tubules (mesonephros) are associated with the development of the kidney. The nephric duct is formed in relation to the developing excretory tubules. At later stages it becomes the mesonephric duct.( 3rd week) Paramesonephric duct is formed lateral to the nephric duct (6th week)
  7. 7. Cloaca: • The part of the hind gut caudal to the attachment of the allantoic diverticulum is called the cloaca. • This cloaca soon subdivides into a broad ventral part (primitive urogenital sinus) and a narrow dorsal part (primitive rectum). • These two parts are separated from each other by the formation of the urorectal septum.
  8. 8. • The urorectal septum grows towards the cloacal membrane and eventually fuses with it. • The cloacal membrane is now divided into a ventral urogenital membrane and dorsal anal membrane. • The mesoderm around the anal membrane becomes heaped up with the result that the anal membrane comes to lie at the bottom of a pit called the anal pit or proctodeum. The anal pit contributes to the formation of the anal canal.
  9. 9. • In further development the primitive urogenital sinus is subdivided into a cranial part called the vesico-urethral part and caudal part called the definitive urogenital sinus. • The openings of the mesonephric ducts lie at the junction of these two. • Still later the definitive UGS shows a subdivision into cranial pelvic part and caudal phallic part.
  10. 10. Development of the uterus PARAMESONEPHRIC DUCTS: • These ducts are present in the intermediate mesoderm formed by the invagination of the coelomic epithellium.(early 6th week) • The paramesonephric ducts develop into the main genital ducts of the female.
  11. 11. • Initially, three parts can be recognized in each duct: – (a) a cranial vertical portion that opens into the abdominal cavity, – (b) a horizontal part that crosses the mesonephric duct, and – (c) a caudal vertical part that fuses with its partner from the opposite side. • With descent of the ovary, the first two parts develop into the uterine tube and the caudal parts fuse to form the uterine canal. • When the second part of the paramesonephric ducts moves mediocaudally, the urogenital ridges gradually come to lie in a transverse plane. ( end of 6th week).
  12. 12. • After the ducts fuse in the midline, a broad transverse pelvic fold is established - the broad ligament of the uterus. • The uterine tube lies in its upper border, and the ovary lies on its posterior surface. The original points of invagination of the ducts into the coelomic epithelium remain as the abdominal openings of the tubes. Fimbria are formed in this situation. • The uterus and broad ligaments divide the pelvic cavity into the uterorectal pouch and the uterovesical pouch. The fused paramesonephric ducts give rise to the corpus and cervix of the uterus. • They are surrounded by a layer of mesenchyme that forms the muscular coat of the uterus, the myometrium, and its peritoneal covering, the perimetrium.
  13. 13. • Anomalies of the uterus: – Duplication- complete (uterus didelphys) or partial. – The lumen may be completely or partially subdivided by septum – Uterine agenesis – Unicornuate – Rhudimentary uterus – Atresia of the lumen either body of the cervix • Anomalies of the uterine tubes: – Absent on one or both sides – Partial or complete duplication – Atresia.
  14. 14. Development of the vagina • Shortly after the solid tip of the paramesonephric ducts reaches the urogenital sinus, two solid evaginations grow out from the pelvic part of the sinus, the sinovaginal bulbs, proliferate and form a solid vaginal plate. (9th week) • Proliferation continues at the cranial end of the plate, increasing the distance between the uterus and the urogenital sinus. (15th to 26th week) • By the 5th month, the vaginal outgrowth is entirely canalized. The winglike expansions of the vagina around the end of the uterus, the vaginal fornices, are of paramesonephric origin. • Thus, the vagina has a dual origin, with the upper portion derived from the uterine canal and the lower portion derived from the urogenital sinus
  15. 15. Anomalies of the vagina • Duplication (usually associated with duplication of the uterus). • Lumen may be subdivided into longitudinally or transversely by a septum. • Absent (may or may not be associated with the absence of uterus). • Imperforate hymen • Vesicovaginal or rectoveginal fistula (usually associated with ARM).
  16. 16. Urogenital sinus abnormalities • A urogenital sinus abnormality is present when a persistent communication occurs between the genital and urinary tract in the female. • Typically, the urethra and vagina are joined and exit to the perineum as a common channel. • If there is associated rectal involvement and imperforate anus with a single perineal opening for all three structures, a persistent cloaca or cloacal anomaly exists. • Patients with urogenital sinus anomalies commonly have associated abnormalities of the clitoris, labia, and external genitalia. • The most common etiology of a persistent urogenital sinus is congenital adrenal hyperplasia (CAH)
  17. 17. classification • relate to the level of the confluence of the urethra and vagina. • Hendren divided urogenital anomalies into those with a confluence above or below the female external sphincter. • In most females, the sphincter mechanism may be indistinct and the level of the pelvic floor a more reliable landmark. • It is important to recognize that the level of the confluence should be considered relative to the bladder neck and not the external meatus.
  18. 18. • Virilization of the distal urethra or urogenital sinus may create a confluence that is distant from the meatus yet still relatively low. The critical determinant, therefore, is the distance from the bladder neck to the confluence as it affects reconstructive surgery. • The nature of the urogenital sinus distal to the confluence should also be considered. Hendren described the urogenital sinus distal to the confluence as urethra- like or vagina-like based on circumference. • The difference between the two has potential ramifications on surgical reconstruction in terms of the amount of local tissue available for reconstruction of the urethra or vagina.
  19. 19. evaluation • Immediate attention to and stabilization of any fluid or electrolyte imbalance is paramount. • Initial screening studies in such patients include assessment of serum electrolytes, biochemical assays screening for CAH, and evaluation of karyotype using cultured peripheral leukocytes. • Multidisciplinary assessment is initiated. • Gender assignment based on karyotype and potential for genitourinary function. child should not be named until the appropriate gender assignment is made..
  20. 20. History/physical examination • maternal and family history – – Maternal use of alcohol or drugs and exposure to androgenic substances – family history of previous neonatal death, infertility, genital ambiguity, abnormal pubertal development or inguinal hernias associated with retained müllerian structures
  21. 21. • Physical examination – general well being, signs of dehydration and lethargy, – Patients with 11 β-hydroxylase deficiency may be hypertensive – abdominal examination should note any lower, midline abdominal masses – back should be assessed for any findings associated with an underlying neurologic anomaly – Gonads are palpated to determine location, number, size, and associated structures such as an epididymis and vas deferens – Rectal examination – Careful inspection of the female introitus is required to identify an isolated urogenital sinus if no clitoral or labial abnormalities are present
  22. 22. Radiographic evaluation • Ultrasound- – The newborn period is the optimal time for evaluation because maternal hormones make the ovaries and uterus more prominent.(sensitivity for locating is at best 60%). – Hydrocolpos, bladder distention, hydronephrosis, dysplasia, and agenesis of kidneys. – patients with CAH usually have a cerebriform appearance of their adrenal glands that may be helpful in diagnosis • MRI • contrast genitography
  23. 23. • Endoscopic evaluation- If the anatomy is not well understood after radiographic evaluation, particularly in the presence of a high confluence or after failure to identify any vaginal structure on genitography, cystoscopy may be performed alone in order to better understand or delineate the anatomy. • Further studies – – hormonal stimulation, – level of 17- OH-progesterone, level of other steroids (testosterone, dihydrotestosterone, delta-4-androstenedione, DHEA, 17-OH-progesterone), ACTH, cortisol, and renin will allow a precise picture of the gonadal and adrenal steroidogenesis, – Gonadal biopsy.
  24. 24. • The most valuable stimulation tests for evaluating steroid genesis are: – hCG test for the study of testicular functionality. – ACTH test for the study of adrenal steroid genesis. – Normal or elevated levels of testosterone and dihydrotestosterone may be suggestive of receptor resistance to androgens. • Molecular biology techniques are more sensitive and specific tests for assessment of the tissue sensibility to androgens, but are not always available.
  25. 25. Surgical techniques Timing • Controversies remain • It is generally accepted that clitoroplasty for CAH patients is best performed in the first few months of life. • Relatively early intervention may carry the benefit of thicker, more vascular skin and easier dissection or manipulation of the clitoris and paravaginal tissue related to maternal estrogen stimulation. • In contrast to timing of clitoral reduction, the optimal age for vaginal reconstruction is more controversial.
  26. 26. • Patients with a low-confluence urogenital sinus can be operated once their metabolic management is well controlled; in most cases an elective reconstruction at 3 to 6 months of age is done. • Patients with a midlevel or high confluence can be electively repaired at 9 to 12 months of age.
  27. 27. VAGINOPLASTY- EARLY INTERVENSION PROPONENTS • improved tissue transfer and reduced scarring secondary to estrogen stimulation • minimization of parental anxiety over the child’s condition, and • earlier self-acceptance of the child’s gender identity and genital anatomy OPPONENTS • May carry an increased risk for an element of vaginal stenosis
  28. 28. Patient preparation • full bowel preparation (unless the confluence is extremely) low. • series of oral antibiotic doses are often administered as well as perioperative broad-spectrum intravenous antibiotics • dorsal lithotomy position for endoscopy. • For higher lesions, a Foley catheter is placed into the bladder, and a second catheter, either a Fogarty or Foley, is placed into the vagina • Surgical preparation- full, lower body prep from the nipple to toes, front and back
  29. 29. Feminizing genitoplasty • Has three components – Clitoroplasty – Labioplasty – Vaginoplasty
  30. 30. clitoroplasty • clitoral amputation - impairment in orgasm and psychosexual development were later noted. • The goal of current techniques is to preserve sensation (neurovascular integrity) for future orgasms, provide an acceptable cosmesis, and avoid painful erections • Kogan described a subtunical excision of the erectile tissue, which has been used extensively and led to newer nerve-sparing techniques. • Baskin and colleagues (1999) found that distribution of the sensory nerves of the clitoris is similar to the sensory nerves of the penis
  31. 31. These nerves are found on the top or dorsal aspect of the clitoris and course under the pubis; circumferential branches from the dorsal neurovascular bundle encircle the clitoral shaft toward the ventrum. In most cases we try to avoid reduction of the glans clitoris, aiming to preserve sensation, but in patients with a large clitoris a wedge of glans tissue may be cautiously excised from its ventral aspect.
  32. 32. steps
  33. 33. LABIOPLASTY Most girls with CAH have labioscrotal swellings that are more anterior than normal labia majora. Y- shaped incisions are outlined with an extension posterior to the swellings The scrotal flaps are cautiously defatted and moved posteriorly, beside the introitus, as bilateral Y-V advancements. The medial aspects of these skin flaps are then sutured to the lateral edges of the preputial skin flaps mobilized during clitoroplasty (now labia minora)
  34. 34. vaginoplasty • There are six types of vaginoplasty: – cut-back, – flap, – pull-through, – TUM – PUM, and – vaginal replacement
  35. 35. Cutback vaginoplasty • simple procedure performed in the patient with labial fusion. • Technique involves incision of the fused skin posteriorly to the perineum to expose the vaginal orifice. • The incised lateral edges are oversewn. • it is only a consideration in patients with a common urogenital sinus on the phallus with confluence of a normal vaginal opening at the level of the perineum.
  36. 36. Flap vaginoplasty • Flap vaginoplasty is only indicated for cases with a very- lowconfluence UGS. • Fortunoff and colleagues - an inverted perineal skin U-flap that could be advanced into the opened vagina. • In a low vaginoplasty there is no need to do a complete mobilization of the UGS. • there is no need to insert a finger in the rectum, but a roll of petroleum jelly (Vaseline) gauze can be inserted in the rectum to avoid rectal injury
  37. 37. inverted perineal skin U-flap A thick, long, flap is mobilized and then the posterior wall of the vagina is dissected with care not to enter the rectum The apex of the flap is inserted into the apex of the vagina wall and secured in place
  38. 38. PULL-THROUGH VAGINOPLASTY FOR MID AND HIGH-LEVEL VAGINAL CONFLUENCE • Before the advent of the urogenital sinus mobilization procedures, the majority of girls were reconstructed using a pullthrough vaginoplasty. • described by Hendren and Crawford • All patients undergo a total lower body preparation from nipples to toes, the legs are wrapped. • Operation begins with a panendoscopy, during which the confluence is localized.
  39. 39. Place a finger in the rectum and dissect against the rectal wall beneath the external urethral sphincter. Have your assistant gently pull on the balloon so that you can identify the junction of the vagina with the sinus. Incise the vagina almost completely circumferentially about the base of the balloon (dashed line). Withdraw the Fogarty catheter. Insert a straight metal sound into the bladder through the urethra and complete the division of the vaginal rim, leaving a little vaginal tissue on the urethra to allow it to be closed without stricture.
  40. 40. close the urethra with interrupted 6-0 chromic catgut sutures. Replace the sound with a balloon catheter. Dissect the vagina from the proximal urethra as high as the back of the trigone. Suture the posterior flap into the back wall of the vagina with 5-0 chromic catgut sutures. Incise the perineum anterior to the vagina as an inverted U to form a second flap
  41. 41. Mobilize this skin flap anteriorly, and suture it to the vaginal rim. Insert a small suction drain or Penrose drain to exit through a stab wound. Complete the closure by joining the lateral margins of the anterior and the posterior skin flaps to the perineal skin. Place a loose vaginal pack. The proximal urogenital sinus at the site of prior confluence is closed, and the urogenital sinus used as a functional urethra.
  42. 42. Total urogenital mobilization • Total urogenital sinus mobilization (TUM) was described in 1997 by Alberto Pena. • Circumferential, partial mobilization of the UGS allows the midlevel vaginal confluence to be brought down to the perineum without tension, avoiding the need for separation of the vagina from the urethra as in the classical pull-through vaginoplasty. • Fogarty balloon, placed in the vagina during the panendoscopy, allows the identification of the confluence. • Urogenital sinus mobilization has the advantage of better visualization of the merging point.
  43. 43. • In this technique, because the confluence is brought closer to the perineum, the mobilization of skin flaps is minimized • The posterior dissection is similar to that done for a pull-through or flap procedure. • The anterior dissection in cases requiring a total urogenital sinus mobilization is done, staying close to the urogenital sinus under the pubis, and dividing the ligament from the pubis to the urogenital sinus.
  44. 44. Partial urogenital mobilization • Rink and colleagues (2006) proposed the use of a PUM. • In response to concerns for possible complications of urinary incontinence, resulting from the circumferential dissection of the UGS beyond the pubourethral ligament. • In this technique the anterior dissection stops at the pubourethral ligament, aiming to avoid compromising the innervation to the bladder outlet
  45. 45. • In both total and partial urogenital sinus mobilization, as previously described for low- confluence vaginoplasty, the distal segment of the vagina can be quite narrow; hence its posterior wall must be incised up to a normal- caliber vagina to avoid a vaginal stricture.
  46. 46. Place a temporary 5-0 Prolene holding stitch through the clitoris
  47. 47. Separate the urogenital sinus from the virilized clitoris to the level of the pubis. Divide the ligaments, attaching the urogenital sinus to the pubis and thus freeing the urogenital sinus. Identify the vaginal confluence by palpation of the Fogarty catheter. open the vagina posteriorly over the Fogarty catheter. Continue until a normal caliber is encountered, as the distal third of the vagina is typically narrow and prone to stenosis. Complete the anterior dissection of the urogenital sinus from the corpora of the clitoris underneath the pubic bone. This frees the anterior urogenital sinus. Complete the posterior dissection of the vagina, allowing the entire urogenital sinus to become mobile and, in most cases, easily able to reach the perineum.
  48. 48. SPLITTING THE UROGENITAL SINUS • proposed by Rink • to enhance the feminizing genitoplasty. • The common urogenital sinus can be used in the following manners: – (1) In very-lowconfluence cases we do not mobilize the urogenital sinus and incise it longitudinally on its ventral aspect up to the confluence point; the lateral aspects of the opened sinus are sutured to medial aspects of the prepuce wings, thus resulting in a more normal anatomic configuration; – (2) ventrally to fashion a mucosa-lined vestibule, – (3) dorsally to create a flap to enhance the anterior half of the vaginoplasty, and – (4) laterally to create a flap that will be rotated to extend the vagina, thus completing the vaginoplasty
  49. 49. Vaginal agenesis • pure mullerian agenesis/ can also be androgen insensitivity • Mullerian agenesis (also known as Mayer- Rokitansky-Kuster-Hauser [MRKH] syndrome) probably results from a failure of the mullerian ducts to reach the urogenital sinus. • occurring between the 4th and 12th weeks of gestation
  50. 50. • 1 in 4000 to 1 in 5000 live female births • By definition, patients are 46,XX females with normal secondary sex characteristics that commonly present with amenorrhea. • Associated with – Renal anomalies (agenesis, fusion anomalies) – 30% – Skeletal anomalies – MURCS association- mullerian duct aplasia, renal aplasia, and cervicothoracic somite association – TAR syndrome (thrombocytopenia-absent radius)
  51. 51. • TheMcKusick-Kaufman syndrome (MKS) – Hydrometrocolpos due to vaginal atresia or aplasia, – postaxial polydactyly, and – congenital heart disease • Bardet-Biedl syndrome (BBS) – genital abnormalities and polydactyly – in addition to retinitis pigmentosa, learning disabilities, central – obesity, and cardiac abnormalities – Mutations in the MKKS, mutations of BBS6 gene on chromosome 20p12.
  52. 52. diagnosis • Bimodal distribution – neonatal and pubertal • Diagnosis of vaginal agenesis is most frequently made at the time of evaluation of primary amenorrhea (15% of girls presenting with primary amenorrhea will have MRKH syndrome) • It is important to differentiate MRKH and androgen insensitivity syndrome (AIS), both of which present with primary amenorrhea and absence of a functional vagina
  53. 53. AIS • there is absent or scant pubic hair, • “male range” serum testosterone • In the prepubertal time period, before elevation of testosterone, a chromosomal analysis is necessary • The presence of a Y chromosome increases the risk of dysgerminoma in the gonadal tissue, and thus removal of gonadal tissue is indicated. • a lack of mullerian structures (uterus, cervix, and upper vagina), due to the presence of mullerian inhibiting substance (MIS). In contrast, women with MRKH have absence of mullerian structure with normal functioning ovaries and hormones.
  54. 54. • prenatal ultrasound, Prenatal magnetic resonance imaging (MRI) • in the neonatal period- abdominal mass • In the post pubertal period- primary amenorrhoea, cyclical abdominal pain, abdominal mass.
  55. 55. Treatment • Treatment of vaginal agenesis depends on the anatomy of the individual patient • The goal of therapy is to provide adequate sexual function and deal with the psychologic impact that the patient has no uterus or vagina. • The timing of creation of a functional vagina is important. In cases of MRKH and AIS it is best to wait for intervention until the patient is postpubertal and the young woman is interested in creating a functional vagina.
  56. 56. • The goal for creation of a functional vagina is pleasurable sexual intercourse with acceptable cosmesis of the external genitalia with minimal short- and long-term morbidity. • Ideally, the neovagina – should be located appropriately (posterosuperiorly), – be of adequate dimensions, – be lined by elastile tissue (either mucosa or skin), – be neither constantly moist nor malodorous, – be hairless, and – be sensate at least at the introitus
  57. 57. • Non operative management and operative management. • Non operative management – – using vaginal dilators for 20 minutes twice daily. – The dilation technique is best managed by a team of nurse and physician – lack of patient compliance affecting the results – The angle of insertion of the dilator is a key to success
  58. 58. Patients are asked to return monthly for examination to assure that the vagina is being created in the appropriate location and at the appropriate angle. The next size dilator is dispensed when appropriate.
  59. 59. • Advantages – Technique is under the control of the young woman, – it does not require anesthesia or a surgical procedure. – cost effective • Critics have concerns regarding – adequate length of the vagina, – poor lubrication, and – Dyspareunia during intercourse
  60. 60. Operative procedures Open procedures: – insertion of a skin-covered vaginal mold (Abbe- McIndoe procedure), – artificial skin grafts, – various fasciocutaneous and myocutaneous flaps (gluteal-thigh flap, gracilis, rectus abdominis, Malaga flap, Singapore flap, vulvovaginoplasty of Williams, vulvovaginal flap (lotus petal labia majora flap), horseshoe flap using labia minora, – labial flaps using tissue expanders – full-thickness skin grafts with vacuum-assisted wound closure, and the use of amnion and peritoneum
  61. 61. • laparoscopic procedures: – The Vecchietti procedure consists of intra- abdominal traction on the perineal membrane causing invagination of the shallow vaginal dimple. – Wharton procedure simply places a condom- covered mold in a created space. – Sheares’ modification of the Wharton technique - adjustable vaginal stent was placed in the space.
  62. 62. McIndoe procedure • The most popular tissue for vaginal replacement during the past 3 decades has been the split-thickness skin graft as described by McIndoe • uses several split-thickness skin grafts, which are usually harvested from the buttocks
  63. 63. The labia are sutured over the mold to hold it in position. After 7 days of bedrest the patient is taken back to the operating room for cutting of the labial sutures, removal of the mold, and sewing of the edge of the perineal skin to the skin grafts Due to the use of dermis, lubricants are required significant incidence of inadequate vaginal length, vaginal stenosis, and dyspareunia due to contraction of the split-thickness skin graft are drawbacks of this procedure
  64. 64. • Davydov procedure: – The peritoneum has been used to line the neovaginal space. – but this requires laparotomy or laparoscopy. • Williams vulvovaginoplasty – Use of vulvar tissues to create a vagina grossly change the appearance of the external genitalia – an abnormal angle for intercourse is problematic, resulting in dyspareunia and making this operation undesirable
  65. 65. Other materials used • In order to avoid the need for a skin graft donor site, the use of an acellular human dermal allograft has been reported. • Amnion has been used to avoid the need for autologous graft sites. • Use of tissue expanders to create more tissue for a labia minora graft may prove useful. • Autologous buccal mucosa has been used successfully for vaginoplasty
  66. 66. Bowel vaginoplasty • Rectum, sigmoid, small intestine • Rectum is not used because of the need for colostomy. • Sigoid colon is the preferred part currently Bowel vaginoplasty • uses a section of bowel approximately 10 cm that is mobilized and retains its vascular pedicle to reach the perineum without compromise to the graft or the pedicle. • Most surgeons advocate anchoring the proximal bowel segment in order to decrease the risk of prolapse of the bowel vagina
  67. 67. Complications – Syed and colleagues described the occurrence of diversion colitis in 3 of 18 children – Hiroi and colleagues in Japan described the only case of malignancy occurring in a sigmoid vaginoplasty – Many women do complain of the natural mucus production – Prolapse -avoided by retroperitoneal fixation of the sigmoid segment
  68. 68. Congenital vaginal obstruction • can result from – imperforate hymen, – agenesis of the lower vagina, or – a transverse vaginal septum
  69. 69. IMPERFORATE HYMEN • Perforation of the hymen usually occurs during the fifth month of gestation, Failure of perforation to occur results in an imperforate hymen. • Present with hydro/ muco/ pyo/ hematocolpos or metrocolpos. • Congenital vaginal obstruction is most commonly caused by simple obstructive anomalies and can include agenesis of the lower vagina, segmental agenesis, or transverse vaginal septum.
  70. 70. • Diagnosis: prenatal/ newborn period/ pubertal period. – Ultrasound/ MRI – Present with midline mass, frequently associated with urinary tract obstruction. – Primary amenorrhoea at puberty with cyclical abdominal pain. • Treatment- elliptical incision not cruciate
  71. 71. Agenesis of the Lower Vagina • presentation is similar to an imperforate hymen, and the diagnosis is determined by physical examination and imaging studies • Diagnosis- The external appearance of ALV can easily be confused with an imperforate hymen. The key means to a correct diagnosis include the observation that there are normal fronds of hymen and there is not just a membrane of tissue. • P/R - appreciate a space between the obstructed vagina and the location for the introitus.
  72. 72. If there is some amount of areolar tissue until the obstructed vagina is reached, then this is ALV and not an imperforate hymen Once the obstruction is relieved and the old blood is evacuated, the native upper vagina will tend to retract back up toward the uterus. This is the reason the native vaginal tissue must be sewn to the newly created introitus
  73. 73. Transverse Vaginal Septum • can occur at varying levels of the vagina. • referred to as low, middle, and high transverse vaginal septum. • approximately 46% of vaginal septa occur in the upper vagina, 40% in the middle vagina, and 14% in the lower vagina. • The presentation will vary depending on whether there is a perforation. • Pelvic ultrasonography is usually diagnostic of hematocolpos.
  74. 74. best treated by draining the vagina by cutting into the septum, resecting the septum, and performing a simultaneous transperineal vaginal pull-through procedure attaching the upper and lower vagina using absorbable sutures
  75. 75. Anomalies of fusion • Vaginal duplication • OHVIRA • Longitudenal vaginal septum
  76. 76. OHVIRA • obstructed hemivagina with ipsilateral renal anomaly (OHVIRA) or Herlyn-Werner-Wunderlich syndrome • Urinary tract anomalies may confound the situation with an ectopic ureter inserting into a duplicated imperforate hemivagina, resulting in a mass. • The mass usually pushes the bladder forward and the normal vagina in a posterior direction. • simple incision of the obstructing septum with resection of a “window of tissue” often provides adequate drainage
  77. 77. Longitudinal Vaginal Septum • For nonobstructed longitudinal vaginal septum, a surgical excision of the septum is required only if the young woman desires such a procedure • Some maintain the longitudinal vaginal septum and have no issues with sexual activity or vaginal delivery • Most have two cervices, so a Papanicolaou smear must be obtained from both.
  78. 78. • The septumshould be excised by “wedging” out the fibrous septal tissue. Great care is necessary to avoid the bladder above, the rectum below. • Once the septal tissue is excised, the normal vaginal mucosa from one vagina is sewn to the other vagina to create a smooth vaginal mucosa and close the resulting defect. Excision of the septal tissue removes the fibrous tissue to avoid dyspareunia
  79. 79. Thank you

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