Vaginal Pouch Research Articles

A 43-year-old man with hematometra: case report and literature review.

In this case report, we present a 43-year-old man (XY) with azoospermia and typical male appearance, at Tanner stage 5 of sexual development, who presented with severe colicky abdominal pain accompanied by nausea. A pelvic CT scan revealed a pear-shaped structure in the pelvic cavity, located entirely behind the bladder, measuring 106*44cm with fluid accumulation inside it, extending into the right inguinal canal. There was also evidence suggesting the formation of the upper and mid-third part of a vagina, terminating in the prostatic gland. The patient underwent laparoscopic surgery for the removal of the uterus and the left gonad. The patient had a uterus with hematometra and a blind vaginal pouch measuring 4 centimeters at the end of the uterus, extending posteriorly behind the bladder to the apex of the prostate, containing old blood. Hormonal analysis showed serum estradiol < 5.0 pmol/L (11-44pg/mL), free testosterone at 1.57 ng/ mL(male reference range: 2.5-20 ng/mL), testosterone at 0.56 ng/mL (2.27-10.30),FSH at 44.8 mIU/L (0.95-11.95 mIU/L), LH at 20.4 mIU/L(0.57-12.07), and DHEA-SO4 at 199.0µg/mL (139.7-484.4µg/mL). Currently, the patient is under the care of a urologist and is receiving weekly treatment with hCG medication. He reports normal sexual function, including intercourse, orgasm, erection, and ejaculation.

FRI412 A Case of 46,XX Gonadal Dysgenesis With Mayer-Rokitansky-Küster-Hauser Syndrome And Primary Hypothyroidism

Abstract Disclosure: S. Al Allawi: None. R. Osman: None. Introduction: The association between Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and gonadal dysgenesis is rarely reported in the literature. Coexistence of hypothyroidism with this condition is scarce and clinical correlation remains unclear. We describe a case of this rare clinical entity in conjunction with primary hypothyroidism. Clinical case: A 27-year-old Female with a medical history of primary hypothyroidism was referred to our clinic for evaluation of primary amenorrhea. She was born with a female phenotype. Growth was appropriate, but puberty was delayed with self-reported late pubarche at age 19. She never had a menstrual cycle, and had received hormonal replacement therapy (HRT) for a few months but failed to trigger menarche. However due to financial constraints, treatment was not continued. She was diagnosed with hypothyroidism since birth, for which she is currently maintained on levothyroxine 225 mcg daily. On physical exam, blood pressure was 126/72 mmHg, pulse rate 83 BPM, height 5’9”, BMI 34 kg/m2. No facial dysmorphisms or skeletal deformity were apparent. She had normal pubic and axillary hair distribution, breast budding consistent with Tanner Stage II. Genital examination revealed normal external genitalia and a blind vaginal pouch. Hormonal workup revealed hypergonadotrophic hypogonadism with estradiol &amp;lt; 0.5 pg/mL and post-menopausal range follicle-stimulating hormone (FSH) 36.7 mIU/mL. Thyroid stimulating hormone (TSH) 2.19 uIU/ml (nl 0.45-4.5 uIU/mL) and anti-TPO antibodies 10 IU/mL (nl 0-34 IU/mL). Prior thyroid ultrasound reported a small thyroid gland. Fluorescence in situ hybridization (FISH) analysis revealed 46XX karyotype. Abdominal and pelvic ultrasound showed normal kidneys with a non-visualized uterus and ovaries. Pelvic MRI confirmed the absence of both and showed a blind end vaginal pouch, leading to the conclusion of the coexistence of gonadal dysgenesis with MRKH. She was then placed on transdermal estradioltherapy. Conclusion: The association between gonadal dysgenesis (the most common cause of primary amenorrhea) and MRKH syndrome (the second most common cause) is infrequent. HRT remains the only therapeutic option. It aims to trigger the development of secondary sexual characteristics and prevent osteoporosis by allowing appropriate bone mineral accrual. Unfortunately, infertility remains the most significant consequential issue; hence the availability of good caring and psychological counseling should not be underestimated. The co-existence of likely non-immune primary hypothyroidism is even less frequent. To our knowledge, our case is the second reported case of this trilogy. Given the paucity of data, the association of this condition with primary hypothyroidism cannot be well established and can be coincidental. More studies are needed to determine if this condition has a higher prevalence of hypothyroidism. Presentation: Friday, June 16, 2023

An adolescent girl presenting with primary amenorrhea: A case report of complete androgen insensitivity syndrome

Abstract Complete androgen insensitivity syndrome (CAIS) is an X-linked recessive disorder characterized by distinct clinical features. Key diagnostic indicators include well-developed breasts, absent axillary and pubic hair growth, and unambiguous female external genitalia. Additional clues can be obtained from the patient’s medical history, such as a previous inguinal hernia and the absence of Müllerian structures. Testosterone levels in CAIS patients typically exceed the reference range for females and fall within the normal to elevated range for males in the corresponding age group. The conversion of testosterone to estrogen plays a crucial role in promoting appropriate breast development. In CAIS, the Müllerian structures are typically absent due to the secretion of the anti-Müllerian hormone, leading to their regression and the presence of a blind vaginal pouch. This case report presents the diagnosis and management of a 15-year-old girl with CAIS who initially presented with primary amenorrhea. The patient underwent bilateral laparoscopic gonadectomy, followed by hormone replacement therapy involving estradiol administration. Plans for future treatment include vaginal dilatation to facilitate penetrative intercourse. The case emphasizes the importance of conducting a thorough physical examination in patients with primary amenorrhea, as it aids in early detection and facilitates appropriate management decisions. By recognizing the clinical clues and implementing a comprehensive management approach, healthcare professionals can effectively address the challenges associated with CAIS and provide appropriate care to affected individuals.

Integrative taxonomy and revision of the Neotropical Biomphalaria tenagophila (Mollusca: Planorbidae) complex taxa

ABSTRACT Morphological and molecular studies of the taxa of the Biomphalaria tenagophila complex were conducted on specimens from Argentina, Brazil, Paraguay, Peru, Uruguay and Venezuela. The morphological analysis included aspects of the shell, mantle, excretory organs and reproductive system. DNA barcode, ABGD and mPTP were used to group specimens into Operational Taxonomic Units (OTUs) and in attempt to solve species boundary delimitation problems within the Biomphalaria tenagophila complex. Population analyzes were also performed based on the cytochrome C oxidase I (COI) and 16S genes. The seminal vesicle, extension of the oviduct in relation to the distal ovispermioduct, vaginal pouch, aspect of the penial complex, aspect of the prepuce diaphragm, diaphragm papilla, extension of the penial sheath in relation to the prepuce and prepuce dimensions are essential for the determination of the taxa of the Biomphalaria tenagophila complex. An updated molecular phylogeny for Biomphalaria based on COI, 16S and ITS2 genes is presented and recover Biomphalaria tenagophila complex as monophyletic. The morphological, population and phylogenetic data observed in an integrated way define taxonomic questions, such as the proposition of a new status for B. guaibensis, which integrates into the Biomphalaria tenagophila complex. http://www.zoobank.org/urn:lsid:zoobank.org:pub:478145E5-D4A5-4128-AA80-DB6A69164E1E

A CASE REPORT OF DISCORDANCY IN MRKH SYNDROME IN MONOZYGOTIC TWINS

A case report of a 15 year old monozygotic twin with primary amenorrhea with the other twin attaining menarche 6 months back. The patient had developed good secondary sexual charectaristics but on examination the child had blind vaginal pouch with a infantile nodule of uterus in per rectal examination . diagnosis of MRKH was made The unique part of this case is that the other monozygotic twin is absolutely normal with no urogynecological , skeletal , cardiac abnormalities. This is a rare presentation in monozygotic twins with MRKH syndrome. Case reports like these aim to throw a new light in the research of etiopathogenesis of MRKH syndrome

Adolescent Female Genital Tract Congenital Anomalies in Northern Ghana

Objective: To ascertain the types of adolescent female genital tract congenital anomalies and their management in the Tamale Teaching Hospital in Northern Ghana.Methods: A cross-sectional study of adolescent female genital tract congenital anomalies that were managed at the Tamale Teaching Hospital from 1st January 2010 to 31st December 2012.&#x0D; Results: There were 19 cases of adolescent female genital tract congenital anomalies during the study period. The commonest female genital tract congenital anomaly was imperforate hymen 7(36.84%). Other congenital anomalies included transverse vaginalseptum 4(21.05%), vaginal atresia 2(10.53%), vaginal agenesis 4(21.05%), didelphus uterus 1(5.26)% andbicornuate uterus 1(5.26%). The management of the adolescent female genital tract congenital anomalies included surgical procedures in 14 (73.68%), dilation of vaginal pouch, counseling and psychological support in 6 (31.57%).Conclusion: While in resource limited settings, initial workup and management of adolescent female genital tract congenital anomalies may be done without sophisticated equipment, management of more complex cases are usually more challenging requiringreferral to more appropriately staffed and equipped centres.

Examining the shape and size of female and male genitalia in snakes using three-dimensional geometric morphometrics

Abstract Quantification of genital variation in males and females can inform our understanding of likely copulatory interactions and evolution of genital diversity. However, no studies have quantified genital shape variation within a single snake species or examined the shape and size of both the vaginal pouch and hemipenes. Here, we examine the shape and size of the genitalia of female and male diamondback water snakes, Nerodia rhombifer, using a three-dimensional automated landmark geometric morphometric approach on models of the lumen of the vaginal pouch and inflated hemipenes, applying these techniques for the first time to the genital shape of vertebrates. Vaginal pouch shape is significantly associated with body size and reproductive status. As females grow larger and become reproductive, the vaginal pouch enlarges, widens and becomes more bifurcated. In reproductive males, the shape of the hemipenes is also significantly associated with body size. As males grow larger, the hemipenes enlarge and widen; their bifurcation becomes more defined and the spines at the base become more prominent. Vaginal pouch and hemipenial centroid size are isometric with respect to body length. The centroid sizes of the hemipenes and vaginal pouch are not significantly different from one another, hence the genitalia match in size. Reproductive females and males covary in the degree of bifurcation and size of their genitalia. We demonstrate the utility of three-dimensional analysis in studies of the shape of soft tissues and advocate its use in future studies of genitalia.

Divergent Genital Morphologies and Female-Male Covariation in Watersnakes.

Genital evolution can be driven by diverse selective pressures. Across taxa we see evidence of covariation between males and females, as well as divergent genital morphologies between closely related species. Quantitative analyses of morphological changes in coevolving male and female genitalia have not yet been shown in vertebrates. This study uses 2D and 3D geometric morphometrics to quantitatively compare the complex shapes of vaginal pouches and hemipenes across three species of watersnakes (the sister taxa Nerodia fasciata, N. sipedon, and a close relative N. rhombifer) to address the relationship between genital morphology and divergence time in a system where sexual conflict may have driven sexually antagonistic coevolution of genital traits. Our pairwise comparisons of shape differences across species show that the sister species have male and female genitalia that are significantly different from each other, but more similar to each other than to N. rhombifer. We also determine that the main axes of shape variation are the same for males and females, with changes that relate to deeper bilobation of the vaginal pouch and hemipenes. In males, the protrusion of the region of spines at the base of the hemipene trades off with the degree of bilobation, suggesting amelioration of sexual conflict, perhaps driven by changes in the relative size of the entrance of the vaginal pouch that could have made spines less effective.

46, XY 5-Alpha Reductase Deficiency in a 36-Year-Old Phenotypic Female Patient

46, XY 5-Alpha Reductase Deficiency in a 36-Year-Old Phenotypic Female Patient

Histological aspects of the penial complex and vagina of taxa of the Biomphalaria tenagophila complex (Mollusca: Planorbidae)

Histological studies were carried out with 49 specimens; 18 Biomphalaria tenagophila guaibensis, 19 B. t. tenagophila and 12 B. occidentalis, collected in 10 municipalities in the states of São Paulo and Rio Grande do Sul, Brazil. The study considered histological aspects of the vagina, vaginal pouch and penial complex. In this study, we describe, for the first time, the histological features of the vagina, vaginal pouch and penial complex of B. t. tenagophila, B. t. guaibensis and B. occidentalis. The lining epithelial tissue and muscular tissue of the vagina, vaginal pouch and penial complex show a similar pattern in B. t. tenagophila, B. t. guaibensis and B. occidentalis, except for the intermediate muscular layer of the prepuce, which distinguishes B. t. guaibensis from the other two taxa. Histological studies on the penial complex and vagina region of other Biomphalaria species are necessary to confirm the taxonomic value of these two regions, in order to contribute to the group’s systematics.

Identification of a Novel Mutation in an Iranian Family With 17-β Hydroxysteroid Dehydrogenase Type 3 Deficiency: A Case Series

Background: We presented the clinical and genetic features of a male ambiguity due to 17-beta-hydroxysteroid dehydrogenase 3 (17B-HSD3) deficiency. Methods: The proposita was an 11-year-old girl and the first child of a consanguineous family. The external genitalia were completely female and had a short vaginal pouch. She had palpable gonads in her inguinal area and underwent bilateral gonadectomy at the age of two. At the age of 10, she was referred to our clinic for more evaluation. In pelvic sonography, uterine and ovarian were not seen. Her karyotype was 46, XY, and her LH and FSH levels were elevated, and three of the patient’s aunts and one of the mother’s aunts had similar signs. Conclusions: We identified a novel homozygous missense variation (c.731T&gt;A, p. Ile244Lys) in the HSD17B3 gene. This alteration changes Isoleucine to Lysine in exon 10.

Pituitary Stalk Interruption Syndrome in a 40-Year-Old Female With Absent Uterus and Ovaries

Background: A patient with multiple pituitary hormone deficiencies due to a rare congenital pituitary defect also presented with an absent uterus and ovaries. Coexisting Müllerian agenesis was suspected, however hormone replacement unraveled a different story. Case Presentation: A 40-year-old female presented with generalized body weakness and euvolemic hyponatremia. She also had cold intolerance, constipation, primary amenorrhea and dyspareunia. As a child, she was worked up for short stature but was lost to follow up. Physical examination showed Tanner stage 1 breast and pubic hair, and absence of axillary hair. She had no facial anomalies, visual abnormalities or anosmia. Hormone panel was consistent with panhypopituitarism: ACTH 5.69 pg/mL (<46 pg/mL), cortisol 1.9 ug/dL (4.30-22.40 μg/dL), TSH 3.375 uIU/mL (0.55-4.78 uIU/mL), FT4 0.51 ng/dL (0.55-4.78 uIU/mL), FT3 1.79 pg/mL (2.30-4.20 pg/mL), LH <0.07 mIU/mL (1.9-12.5 mIU/mL), FSH 0.95 mIU/mL (2.5-10.2 mIU/mL) and IGF-1 40.8 ng/dL (109-284 ng/mL). Bone age was delayed (16-year-old) but with most ossification centers fused. Pituitary MRI showed hypoplastic anterior pituitary, ectopic posterior pituitary and absent pituitary stalk suggestive of Pituitary Stalk Interruption Syndrome (PSIS). Low estradiol 19.35 pg/mL (19.5-144.2 pg/mL) in the setting of low LH and FSH was compatible with hypogonadotrophic hypogonadism. She had female-range serum testosterone level <0.007 ng/mL (0.1209-0.5946 ng/mL) and karyotype of 46XX. Transvaginal ultrasound revealed a blind vaginal pouch with absent uterus, fallopian tubes and ovaries; hence Müllerian agenesis was also considered. Hormonal replacement with prednisone, levothyroxine and conjugated equine estrogen was started. Secondary osteoporosis was treated with alendronate, calcium and vitamin D on top of estrogen therapy. Six months after initiation of estrogen, there was appearance of a small anteverted uterus and atrophic right ovary. Müllerian agenesis was ruled out and hypogonadotropic hypogonadism was proved to be the cause of the initial absence of the uterus and ovaries on imaging. Conclusion: Increased awareness of PSIS is important since early and accurate diagnosis is crucial for timely initiation of hormone replacement. This case also demonstrates the need for reassessment after hormonal replacement in patients with severe estrogen deficiency and apparent Müllerian agenesis.

Laparoscopic uterovaginal anastomosis in patients with congenital cervicovaginal atresia: An institutional experience with 23 patients

ObjectiveTo introduce our experience of laparoscopic uterovaginal anastomosis and operative outcomes following this technique and update the clinical management of congenital cervicovaginal atresia. Study designBetween March 2015 and January 2019, twenty-three patients with congenital cervicovaginal atresia underwent laparoscopic uterovaginal anastomosis and Luohu procedure. Their clinical characteristics, surgical outcomes and follow-up data were retrospectively reviewed. ResultsThe mean ± SD age of the cases was 16.4 ± 4.0 (11–26) years. All patients had a history of primary amenorrhea and cyclical abdominal pain, and the average delay in diagnosis from first symptoms was 33.5 ± 38 (1–156) months. Most of the patients had 1−3 cm long vaginal pouch. The operative procedure lasted 125 ± 32 (80–190) min. The average vaginal length at 1 month was 7.9 ± 1.3 (range 6–9) cm. All patients showed resumption of menstruation. The patients were followed for a mean of 27 ± 13 (12–56) months. During the follow-up, cervical stenosis did not occur in any of the cases. ConclusionLaparoscopic uterovaginal anastomosis with Luohu procedure provided a minimally invasive, safe, and effective surgical option for the patients with congenital cervicovaginal atresia. The technique is uncomplicated, easy to learn and perform, and provides a functional and anatomic satisfactory result. No special surgical instruments are required with this technique.

Vaginal ultrasound-guided Pouch of Douglas robotic entry in a live ovine model and human female cadaveric specimens.

We aimed to determine whether intraoperative ultrasound is a feasible tool for visualization of the pouch of Douglas (POD) to facilitate a safe vaginal entry for direct robotic vaginal trocar insertion for pelvic floor surgery. Endovaginal ultrasound-guided needle insertion of a trocar into the POD was performed in six fresh frozen female cadavers and a live sheep animal model. Using an endovaginal probe the POD was identified as a fluid-filled space clear of bowel or adhesions, then a Veress needle was also used to confirm POD localization. Access to the POD was achieved using a robotic trocar designed for this purpose. The animal study was approved by the Ethics Committee of Asaf-Harofe hospital. Direct visualization during laparoscopy in cadavers and open cadaveric dissections confirmed safe POD entry and accurate trocar placement. This method was found feasible in the development of a safe vaginal entry in both the animal and cadaveric model, possibly negating the need for laparoscopic umbilical observation.

Mayer-Rokitansky-Küster-Hauser syndrome with rare findings of inferior crossed-fused renal ectopia and Gartner's duct cyst: a video case report

To describe the treatments of a patient using the laparoscopic Davydov's method for Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and ureteral reimplantation for hydronephrosis and hydroureter. Surgical video article. A consent form from the patient was obtained as appropriate; the nature of the study did not necessitate ethics committee approval. There were no conflicts of interest. University hospital. A 28-year-old woman who presented at our gynecology department with the symptoms of primary amenorrhea and difficult intercourse. She had repaired congenital rectovestibular fistula and imperforate anus at the age of 8. At physical examination, she had a phenotypically normal vulva with a vaginal small pouch (0.5 cm). Magnetic resonance imaging of the pelvis revealed normal ovaries, a primordial uterus, absence of vaginal canal, and a 4.0 × 4.2 × 4.0 cm cystic structure posterior to the bladder. Magnetic resonance urography showed right to left renal crossed-ectopia with inferior fusion, and hydronephrosis and hydroureter from the superior kidney with Grade Ⅳ vesicoureteral reflux. Karyotype was 46, XX. Saline solution 300 mL was injected into the rectovesical space with an infusion of diluted adrenaline (1:200,000). The goal of this injection was to aid in the identification of tissue planes and reduce blood loss. The space between urethra/bladder and rectum progressively was dissected. Blunt dissection was performed initially with digital separation of tissues. Then, an 8-cm-long neovaginal vault of about 3 cm in diameter was created. The mobilized peritoneum was pulled downward with eight Vicryl sutures and connected to the vaginal epithelium. By cystoscope, we found the left orifice but could not find the right orifice of the hydroureter. Then we ligated the hydroureter by 2-0 absorbable suture near the cyst and cut off the hydroureter, and then incised of all the layers at the top of the bladder to make a bladder flap. We placed the 5 Fr double J stent in the hydroureter and the bladder and anastomozed with the ureteral stump (3-0 Vicryl). Then we removed the cyst laparoscopically. We performed a purse-string stitch to create the apex of the neovagina by taking posterior serosa of the bladder, the pelvic peritoneum between the ovary and rectum, primordial uterus, and anterior rectal serosa. Measurement of the final canal length, sexual function (Female Sexual Function Index), and degree of hydronephrosis. Three days later, we started to change the vaginal mold and the patient was advised to wear it day and night for the first postoperative month. The vaginal mold had to be worn each night until normal sexual intercourse was possible. Findings confirmed the cyst was Gartner's duct cyst. One year after the surgery, the final canal length was 9 cm and Female Sexual Function Index score was 28. The ultrasound showed that the degree of hydronephrosis of upper moiety was mild. The distal Wolffian ducts in the female are absorbed but may persist as vestigial remnants (Gartner's duct cysts). A few cases of the combined urogenital-Wolffian anomalies are reported; most of them are associated with the anomalies of müllerian duct fusion, such as Herlyn-Werner-Wunderlich syndrome (uterus didelphys, obstructed hemivagina, and mesonephric duct anomalies). The embryogenesis of the combined anomalies is not completely understood. With comprehensive preoperative assessments, laparoscopic surgery could be a safe and effective treatment to these cases.

Perineal hypospadias repair with preservation of a coincidental vagina or perineal utricle in boys with disorders of sex development

An enlarged utricle in patients with proximal hypospadias or disorders of sex development (DSD) is common. The utricle orifice is usually in the posterior urethra near the verumontanum, but in exceptional cases located on the perineum. Concurrence of a perineal hypospadias and perineal utricle or vagina is also known as male vagina, pseudovagina, or blind vaginal pouch. The utricle in such cases is usually excised either before or concomitant with hypospadias repair. The authors developed an alternative approach in which the vagina or perineal utricle is retained during hypospadias repair and report on the results in four patients. To report a novel technique for perineal hypospadias repair while retaining a concurrent vagina or perineal utricle. Between 1999 and 2014, four neonates presented with perineal hypospadias. In all patients, a second perineal opening providing access to either an enlarged utricle or a vagina was identified. Karyotype in peripheral blood was in two patients 46,XY and in the other two 45,X/46,XY of which one showed a complex mosaicism in gonadal tissue. No genetic cause was identified on DNA evaluation in the two patients with 46,XY DSD. All patients were raised as boys. Hypospadias repair was performed in two stages at prepubertal age. During the second stage of surgery, performed between the age of 1.5 and 5 years, the vagina or utricle orifice was incorporated into the neo-urethra, resulting in a retained 'built-in' vagina or utricle. Surgical procedures were uneventful, and patients remained asymptomatic during a mean postoperative follow-up of 8.5 (range 2-13.5) years. One patient was lost to follow-up after the age of 8 years. At their last visit, the remaining patients, at the age of 4, 15, and 17 years, were able to void in standing position without dribbling. Both adolescent patients reported erections without ejaculations and identified themselves as males without signs of gender dysphoria. Hypospadias repair in boys with perineal hypospadias while leaving a male vagina or perineal utricle in situ has not been reported previously, and the study's preliminary results are favorable. One of the benefits of this approach is that inadvertent injury to adjacent anatomic structuressuch as urethral sphincter, neurovascular bundles, ureters, vas deferens, and rectum is avoided. The main rationale for adopting this conservative approach however is to minimize genital tissue removal in children with a not yet definite gender identity, which will certainly facilitate unforeseen future gender reassignment surgery.

Application of Saline Infusion Sonocolpography in Diagnosis and Treatment of Perforated Transverse Vaginal Septum

Transverse vaginal septum (TVS) is a particularly rare vaginal anomaly, and diagnosis is often difficult in a genital examination. We herein present a case of perforated TVS for which successful diagnosis and treatment were achieved using a new technique referred to as saline infusion sonocolpography. A 32-year-old female presented with primary infertility. Speculum examination revealed a blind vaginal canal with two pinpoint perforating holes. Foley catheters with inflated balloon were inserted into the two apertures, and then normal saline was injected through the catheters to distend the vaginal pouch. This procedure of saline infusion sonocolpography revealed the uterine cervix and vaginal pouch and permitted diagnosis of perforated TVS of the upper vagina. The septum was excised and a normal cervix was ascertained. The patient had no complication such as agglutination of the vagina postoperatively. This case suggests that saline infusion sonocolpography may be useful for diagnosis and treatment of TVS.

SAT-213 Late Diagnosis of Complete Androgen Insensitivity

BACKGROUND: Complete androgen insensitivity (CAIS) is a rare cause of primary amenorrhea and is usually diagnosed in menarche. Patients with CAIS are phenotypically female with a 46, XY karyotype, have elevated testosterone, LH, and FSH levels, and have little or no virilization due to androgen receptor gene mutations. The recommended treatment is orchiectomy due to the increased risk of gonadal malignancy and post-operative hormonal therapy. CLINICAL CASE: A 31-year-old female with no past medical history presented for evaluation of primary amenorrhea. She was born full term with a normal IQ, and no developmental delays or disabilities. The patient had no menstrual periods, minimal breast development, and sparse facial, axillary, and pubic hair growth. She reported having a shallow vaginal canal and absence of a cervix. All of her siblings had normal sexual development with no reproductive or gynecological disorders, and have been able to conceive without any problems. Review of systems was negative for vision changes, galactorrhea, abdominal pain, urinary or defecation problems however positive for headaches. Patient identified as a lesbian. On physical exam the patient had normal vital signs, height of 164 cm and 86 kg (calculated BMI 32); increased muscular tone; no voice deepening; no abdominal pain; sparse hair in the pubic region, upper lip, chin, and bilateral axillae; and Tanner stage 2 breasts. Genitourinary exam was notable for large labia majora, clitoromegaly, palpable testes in inguinal canal, blind vaginal pouch, and absent uterus and cervix. Labs showed normal estradiol (17pg/mL; male 5-52pg/mL), elevated testosterone (273ng/dL; male 249-836ng/dL, female <45ng/dL), LH (24.5 mIU/mL; male 1.7-8.6mIU/mL), and FSH (39.3mIU/ml; male 1.5-12.4mIU/mL), negative urine pregnancy test, normal prolactin, normal thyroid function tests, and a 46, XY karyotype. Imaging demonstrated absent uterus and ovaries, a short blind-ending vagina, and bilateral testes in the inguinal canals. Given elevated FSH, LH and testosterone levels with virilization, the patient was diagnosed with CAIS and underwent bilateral orchiectomy. After surgery, the patient was started on estrogen hormonal replacement therapy. CONCLUSION: Early diagnosis of CAIS is important as this helps guide pharmacotherapies, surgery, malignancy surveillance, genetic counseling, and psychosocial support; all of which help determine a patient’s sexual identity. This case is interesting because the patient was diagnosed far past menarche in her fourth decade, which proved to be psychologically distressing given the patient was unaware of the presence of testes. As androgen insensitivity syndrome presents on a spectrum and any intervention is dependent on phenotype and patient preference, further research is needed to provide evidence-based guidelines regarding therapeutic intervention in any patient with CAIS.

Ultrasound-guided follicle aspiration at time of laparotomy in a patient with Mayer-Rokitansky-Küster-Hauser syndrome

To demonstrate a unique case of direct ultrasound-guided follicle aspiration at time of a laparotomy in a patient with Mayer-Rokitansky-Küster-Hauser Syndrome. Video presentation. Academic fertility center PATIENT(S): A 32-year-old gravida 0 with Mayer-Rokitansky-Küster-Hauser Syndrome and bilateral ovarian masses presented as a fertility preservation consult from gynecologic oncology due to the possibility of bilateral oophorectomy. Due to the appearance and size of the left ovary oncology planned to perform an exploratory laparotomy and left oophorectomy, with possible right oophorectomy. The patient and her partner desired embryo cryopreservation with plans for future use in a gestational carrier. She had previously undergone vaginal dilator therapy, however her ovaries were inaccessible transvaginally due to their cephalad location and small caliber of the vaginal pouch. The plan was made to proceed with controlled ovarian stimulation and concurrent ultrasound-guided follicle aspiration of the right ovary at the time of laparotomy following left oophorectomy. Ultrasound-guided follicle aspiration invivo at time of laparotomy. Successful controlled ovarian stimulation, oocyte retrieval and embryo cryopreservation. The patient underwent a long agonist protocol and received a total of 2,525 units of gonadotropin with a peak estradiol of 3,264 pg/ml. She required a total of 9 days of stimulation. The normal right ovary responded as expected, and the left ovary remained unchanged. Following laparotomy and left oophorectomy, direct application of the transvaginal ultrasound probe was used to aspirate all visible follicles on the right side invivo. Twenty-four oocytes were retrieved, 15 were mature and 5 blastocysts were cryopreserved. Final pathology of left ovary returned as serous cystadenoma. The right ovary was examined by gynecologic oncology prior to and following retrieval and was thought to be normal and remained in situ. Although the approach described here is not feasible in most cases, this video demonstrates a unique and successful fertility preservation technique by direct ultrasound-guided follicle aspiration invivo at the time of laparotomy in a Mayer-Rokitansky-Küster-Hauser Syndrome patient and to our knowledge, is the first description of its kind. This retrieval would have otherwise been limited by lack of access transvaginally and limited visualization transabdominally. This combined approach should be considered in future patients with müllerian anomalies and similar complicating factors necessitating laparotomy.

EP26.05: Accuracy of transvaginal sonography in a series of patients operated of endometriosis

To determine the accuracy of transvaginal sonography (TVS) in pre-surgery evaluation of endometriosis. Taking into account the consensus opinion from the International Deep Endometriosis Analysis (IDEA) group, we performed a retrospective study collecting data from TVS exams carried out on 32 female candidates prior to the surgical procedure for endometriosis. Four specialised sonographers performed exams; the average time between ultrasound and surgery was 4'8 months. We evaluate the presence or the absence by TVS of endometrioma and deep infiltrating endometriosis (DIE) in anterior compartment and posterior compartment: rectovaginal septum (RVS), posterior vaginal fornix (PVF), uterosacral ligament (USL), rectosigmoid (RS) and Pouch of Douglas obliteration (POD obliteration). Data was assessed and compared with surgical and histological findings by calculating sensitivity, specificity and accuracy. Due to lack of data, DIE lesions in anterior compartment, RVS and PVF were collected but not evaluated. 32 women, average age of 36,2 years old, underwent TVS and surgery for endometriosis. The sensitivity, specificity and accuracy of TVS for the diagnosis of endometrioma and DIE were 91% (95%CI 76-100), 79% (95%CI 37-100), 84% (95%CI 70-98) and 75% (95%CI 46-100), 95% (95%CI 83-100), 87,5% (95%CI 74-100), respectively. In 30% of endometriomas TVS revealed association with DIE. Results concerning DIE and POD obliteration are expressed in table1. TVS is an accurate and non-invasive test for detection and pre-surgical description of endometriomas and DIE, contributing to the management plan for surgery. Our results resemble those published in prospective studies. Further evaluation of data in our centre would be necessary in order to obtain more valuable outcomes. EP26.05: Table 1. Sensitivity, specificity and accuracy values for DIE and POD obliteration