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An important issue is how well can the physician enrique heart attack cheap plavix 75mg visa, usually a pathologist pulse pressure 20 order plavix discount, date the endometrium blood pressure 34 weeks pregnant discount plavix 75mg on line. When the same observer viewed 63 endometrial biopsies on two 126 separate occasions arrhythmia when lying down order 75mg plavix, there was exact agreement in only 15 (24%). Disagreement of more than two days in the dating of the endometrium occurred in six instances (10%). A further problem in establishing luteal phase defect as a clinical entity is the finding in most studies of an out-of-phase biopsy in approximately 20% to 30% of 125, 127 normal cycles and repetitive lags in more than one normal cycle of approximately 5%. In a series of 1492 128 biopsies in 1055 women reported by Balasch, there were 26 biopsies in conception cycles. With an in-phase biopsy, 15 of 20 pregnancies went to term but 4 out of 6 pregnancies with out-of-phase biopsies also went to term. Similarly, the term pregnancy rates were almost identical in women treated and untreated for luteal phase 129 defect. There is a window of endometrial hormonally-induced development during which successful embryo transfer with in vitro fertilization can occur. The window extends over a 6-day period, and this indicates that precise synchronization of the endometrium and the embryo may not be needed for successful implantation. Given the uncertainties surrounding the diagnosis of luteal phase defect, it becomes difficult to justify putting women through the expense and possible pain of endometrial biopsy. In common practice, again because of the discomfort, the difficulties of precise dating, and the expense associated with endometrial biopsy, attention has turned to measurements of serum progesterone levels as a means of diagnosing, if not luteal phase defect, then at least a “hormone deficiency. Frequently a diagnosis of “hormone deficiency” is made based on an isolated and not always well timed progesterone level of less than 10 ng/mL. It is also common practice to utilize such a finding as a rationale for treating as if a luteal phase defect were present. Daily progesterone measurements taken throughout the luteal phase could provide strong evidence for a luteal phase defect if the values are low, but such frequent sampling is impractical. An assessment of various approaches for progesterone measurements concluded that the best correlation with endometrial histology is achieved with 3 progesterone levels obtained randomly during days 5–9 after ovulation (normal equal to or greater than a sum of 30 ng/mL or a pooled concentration, 130 a less expensive alternative, greater than 9 ng/mL). Most important, however, is the impressive evidence documenting poor correlation between progesterone 117, 118, 119, 120, 121 and 122 measurements and endometrial histology. Other methods of diagnosis include the basal body temperature chart, ultrasonography of follicular size, and salivary progesterone measurements. All have been 130, 131 studied, and all demonstrate poor correlations with multiple progesterone levels and endometrial histology. Gonadotropin treatment is expensive, has the potential for causing hyperstimulation of the ovaries, and is associated with an increased incidence of multiple births. Because of these effects, gonadotropin treatment is seldom, if ever, used for this indication. Clomiphene citrate is the first choice of many clinicians for the treatment of luteal phase defect. The only significant risk is a 5% (twice normal) chance of multiple births, essentially all twins. The initial dose is 50 mg a day for 5 days starting on day 3, 4, or 5 of the cycle ( Chapter 30). Because there is a suspected deficiency of progesterone in luteal phase defect, exogenous progesterone has been utilized. A vaginal suppository compounded by a pharmacist and containing 25 mg of progesteroe is inserted bid starting approximately 2–3 days after ovulation. Treatment is maintained until menstruation occurs or th through the 10 week of a pregnancy. Once pregnancy is diagnosed, a switch can be made to weekly injections of 17-hydroxyprogesterone caproate (250 mg) through the 10th week of pregnancy. Progesterone in a gel that adheres to the vaginal mucosa (Crinone) is available in a prefilled vaginal applicator, applied with one 8% (90 mg progesterone) application daily. Vaginal administration accomplishes targeted delivery to the uterus without producing high circulating levels. Using progesterone therapy, success rates of approximately 50% have been acheved, but good controlled studies are lacking. In our view, this is an argument in favor of clomiphene because of a significant disadvantage associated with progesterone therapy. Progesterone supplementation prolongs the luteal phase and can delay the onset of menses. This is not a problem for the clinician, but for the couple the disappointment at the time of delayed menses or a negative pregnancy test is profound. Dopamine agonist treatment has been reported to correct luteal phase defect associated with hyperprolactinemia, but its value in women with normal prolactin levels has not been demonstrated. In a subgroup of patients with unexplained infertility, high normal prolactin levels, and expressible galactorrhea, treatment with 135 bromocriptine enhanced fertility compared to similar women treated with pyridoxine. If galactorrhea is present, even if the prolactin is normal, ovulatory dysfunction 136 responds well to dopamine agonist therapy. In the absence of galactorrhea, a prolactin elevation may be subtle (such as an increase in nocturnal peaks), and this could explain occasional good responses to dopamine agonist treatment. In evaluating any therapy it is important to keep in mind that pregnancies can occur without treatment in women who are diagnosed as having luteal phase defect. Many physicians dispense with the diagnostic evaluation of hormone adequacy and proceed to treatmentith clomiphene citrate. It is a reasonable argument that there may be subtle hormonal abnormalities that cannot be diagnosed with current technology but that can be successfully treated by stimulating the ovaries. Moreover, there is a theoretical advantage in having more than one oocyte in the fallopian tube at the time of fertilization. Randomized placebo-controlled studies support the efficacy of this approach (reviewed with references in Chapter 30). By contrast, some have not found a benefit for the use of clomiphene citrate in unexplained 137 infertility. We do not object to the empirical use of clomiphene, provided there is a clear understanding of the potential side effects and the uncertain efficacy of the medication. Luteinized Unruptured Follicle On occasion, a corpus luteum will form despite the failure of release of the oocyte. Initially it was thought that this problem could be identified at laparoscopy by 138 visualizing an absence of the ovulatory stigma, but now it is apparent that the stigma can be epithelialized rapidly and thus obscured from view. Even if ultrasonography is performed daiy, the collapse of the follicle can be missed, and a corpus luteum refilled with blood can be mistaken for a persistent follicle. Whereas ovulation usually occurs in the normal cycle when the follicle is 17–23 mm in diameter, some fertile women ovulate from a smaller follicle. In the evaluation of unexplained infertility, a single ultrasound examination in the late follicular phase is of value to detect endometrial polyps and myomas. Because inhibition of prostaglandin synthesis 139, 140 and 141 can cause a luteinized unruptured follicle, patients should be cautioned to avoid the use of nonsteroidal antiinflammatory agents. Mycoplasma Mycoplasma, a pleuropneumonia-like organism, has been implicated as a possible cause of recurrent abortion and salpingitis. A markedly higher prevalence of T 142 mycoplasma (ureaplasma urealyticum) was detected in the cervical mucus and semen of infertile couples compared with a group of fertile couples. Treatment with doxycycline decreased the number of couples with mycoplasma and also was associated with pregnancy in 15 of 52 couples (29%), all of whom had had primary 143, 144 infertility of at least 5 years duration. However, a series of reports from England agreed with these findings in only one respect. They confirmed that treatment with doxycycline could eliminate mycoplasma from the genital tract of the majority of individuals. There was no difference, however, in the frequency of either T strain or Mycoplasma hominis between infertile and fertile couples. In a double-blind study, treatment with doxycycline for 28 days had no effect on the rate of conception, 144 and e English group suggested that culturing for mycoplasma in the routine investigation of infertility was unrewarding. Since those early publications, a number of studies have estaished the widespread distribution of ureaplasma urealyticum in both fertile and infertile populations. Some have found higher colonization in infertile couples, whereas others have found no relationship between the organisms and infertility. In a study that received a great deal of media attention, it was reported that 60% of males who were culture positive for 145 ureaplasma urealyticum and were cleared of infection by antibiotic treatment achieved a pregnancy.
Manually hypertension nos definition generic plavix 75mg visa, using standard multiplanar reformats blood pressure medication hydralazine discount plavix express, using a small reconstruction field of view encom- the technique for which is shown in Figure 1 blood pressure zanidip buy plavix 75 mg low price. Using a software-based facilitating workflow with matrix in order to optimize spatial resolution zartan blood pressure medication buy genuine plavix. For relative image reconstruction, recon- may be used, the interpreter analyzing the imaging struction interval should be spaced at #10% intervals must confirm the accuracy of the generated annular across the acquired portion of the cardiac cycle. For Alternatively, if available on the employed scanning a summary of recommendations please see Table 5. Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. Freehand contour: the annular contour is traced axis dimensions inform on the eccentricity of the manually with the cursor. Importantly, manual caliper measurements should not be used to All techniques commonly provide the annular area 2 2 assess overall annular dimensions instead of con- in either [mm ]or[cm]. Polygon tools may underestimate the annular the annular contour should be drawn along the blood- perimeter when compared to cubic spline interpola- pool tissue interface, carefully avoiding presence of tion, given the straight line connection of the seg- any tissue within the contour and avoiding any mentation points. In case of annular contour detection as well as freehand contour tech- calcification, irrespective of crescent or protruding niques may, depending on the software platform, distribution, the contour should be drawn in a har- result in jagged contours, artificially increasing the monic fashion as if no calcium is present, as this aids reported perimeter. This by software algorithms, although methodology differ annular dynamism has significant implications for Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. The lower row shows an example of septal hypertrophy with smaller annular dimensions in systole than diastole, in part due bulging of the basal septum (dashed blue line) into the annulus during systole. The annular anatomy should Image quality can be rated using a subjective, quali- be reviewed throughout the available portion of the tative grading scale as good, fair and poor (non- cardiac cycle and the phase yielding the largest di- diagnostic) (Table 4). Care should be taken to identify a reconstruc- aortic valve is associated with increased risk of par- tion phase with adequate image quality, ie. In clinical practice, phases with inadequate depiction of the annular the description of annular (within annular plane) and contour, i. Increased image noise Annular and sub-annular calcification should be Mild motion artifacts, double contours or stair-step artifacts described as crescent/flat/adherent or protruding as Poor (non-diagnostic) Too low contrast attenuation Excessive image noise well as its relation to the aortic cusps. The region Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. Systolic measurements are preferred for measurement and calculation of device sizing Strong Area and perimeter measurements are preferred for sizing of the aortic annulus over isolated 2 dimensional measurements and Strong should be provided in the report Landing zone calcification Annular and subannular calcification should be qualitatively described regarding morphology and extent as well as relation to Strong the aortic valve cusps. Valve morphology Number of cusps should be stated, and if a bicuspid valve is present, its morphology should be classified. Strong the presence of a median raphe and the absence/presence of calcification of this should be mentioned Strong the aortic annulus size should be measured and reported in bicuspid aortic valves as for tricuspid aortic valves. Low coronary ostial height (<12 mm) from to commissure in parallel to the annular plane using the annulus and sinus of Valsalva mean diameter <30 three caliper measurements in [mm]. There is no recommendation as to threshold at which the procedure should be consid- whether these measurements are to be performed in ered contra-indicated, given the relatively low spec- systole or diastole. Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. Dependent on insti- circle but the implications are even greater than ex- tutional preferences, typically views are either pected owing to the non-circular geometry of the centered on the right coronary cusp, or use pre- annulus. When and implanting physician appreciate the differences identified projections result in cranial or caudal and are aware of the parameter being used for device angulation >25 , alternate angulations should be sizing of specific devices. In clinical practice, balloon Annular and sub-annular calcification, particularly expandable devices are largely sized on the basis of when protruding into the lumen, can result in annular area, with self-expandable devices relying on increased risk of both annular rupture and para- perimeter. Protruding landing zone calci- also reflect different risk profiles of these devices and fication below the non-coronary cusp has been shown the geometric implications of sizing with the different tobemost closely-associatedwithannularinjury,with variables. Sizing based on manually assessed short this effect amplified when combined with aggressive and long axis diameters have been shown to be less annular oversizing (58). The term ‘oversizing’ is a generic term as quent adverse event but is associated with a high oversizing can be calculated based on any measure- mortality if it occurs (58,59). In addition, the depth of calcification within the eter or perimeter derived diameter (55). The minimal luminal diameter along both the right and left iliofemoral system should be provided including the anatomical location to the level of the Strong expected puncture site All areas of >270 calcification in the iliofemoral arteries should be reported Strong Calcification located anteriorly at the site of probable puncture should be reported. Strong the report should include a clear description of all vascular pathologies including aneurysms, dissection, and occlusions. Strong Coronary arteries Reporting of the coronary arteries for severity of coronary artery disease can be considered in appropriately selected patients, if image quality is of Strong diagnostic quality the presence and course of anomalous coronary arteries should be reported. Protruding nod- complications have fallen with improved pre- ules of calcification are considered to connote higher procedural screening with major vascular complica- risk than flat or mural calcification. An increased no reference is made to current devices and vessel depth of implantation is the most frequently identi- diameter requirements. While early reports septumcanbemeasuredinthecoronalplane,atthe described an increased risk at a sheath:diameter longest point between the annular level and the ratio $1. However, routine assessment devices which are typically smaller with an of semi-membranous septum length has not seen increasing prevalence of expanding sheath designs, widespread adoption into clinical routine. Vascular complications are inde- the minimal diameter of the vasculature between the pendently associated with increased morbidity and aortic valve and the right and left common femoral Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. This can be performed degree of ascending aortic calcification should be either manually on multiplanar reformats using a noted—the latter relevant for potential cross- double-oblique technique, or using semi-automatic clamping. Further relevant pathologies include post-processing using centerline placement and abnormal elongation and kinking, dissections, aneu- curved multiplanar reformats. If transfemoral access is performed to ensure accurate vessel tracking, and not feasible, depending on local practice, subclavian appropriate intra-luminal location of the centerline. Due to more favorable angula- and appropriate windowing used to correct for this tion, an left-sided approach is preferred for where necessary. Finally, if a transcaval approach is measurements are performed perpendicular to the to be considered, the presence, size and level of long axis of the vessel at the location of the maximum calcification free windows of the aortic wall adjacent stenosis. Transverse source images allow no more to the inferior vena cava should be reported (80). Extend and distribution of description of all vascular pathologies including an- calcifications of the iliofemoral vasculature should be eurysms, dissection, and occlusions. Beta-blockade must be used can be assessed on transverse source images, but with caution and nitroglycerin is contra-indicated, evaluation is facilitated using a volume rendered with the absence of these associated with reduced displayfrommultipleviewingangles,suchasanterior- diagnostic accuracy (9). Rele- obtainedshouldbereviewedcarefullyforincidental vant findings should be reported, ideally with findings. This is best performed by trained radiolo- detailed location in relation to fluoroscopically iden- gists, either as a combined or separate report ac- tifiable anatomical landmarks, such as the level of the cording to local practices. Please refer presence of ascending aortic aneurysm and the to Table 7 for a summary of recommendations. Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. Broadly, bioprosthetic valves can be categorized as stented the dashed yellow line indicates the orientation of the long axis views in the lower row, aligned with the center of the surgical (rigid scaffold), stentless surgical (no rigid cusps. The extent of leaflet thickening can be graded on a scaffold), and transcatheter heart valves. Please refer to Table 8 for a summary of dality to echocardiography in patients where there is recommendations. The anatomical orientation of the cylinder is recommended to maximize image quality and to being dictated by the orientation of the surgical valve. Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. When using reference charts careful adherence edge] 3 Aortic annular dimension to the measurement technique employed in the a Overall image quality [good, fair, poor] respective papers is required to allow for accurate b When assessed (systole/diastole, reconstruction phase) c Annular area [mm ]2 estimation of the valve size. Descargado para Anonymous User (n/a) en Consejeria de Sanidad de Castilla y Leon(Sacyl) de ClinicalKey. Lancet 2015;385: aortic valve stenosis randomized to transcatheter society of cardiovascular computed tomography 2485–91. Boehm T, Husmann L, Leschka S, Desbiolles L, ment or surgical aortic valve replacement for high Marincek B, Alkadhi H.
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Simple univariate linear regression against age Transthoracic echocardiographic measurements were performed for was then used to construct the presented nomograms normalized each of the studies as per available published guidelines arrhythmia heart failure cheap plavix online. Staessen blood pressure medication nifedipine purchase plavix 75mg with mastercard, Department of Cardiology lennox pulse pressure test kit order plavix line, Catholic University Leuven arteria labialis superior buy generic plavix on line, personal communication. An extensive chest wall contusion was initially observed but he was otherwise stable for 48 hours. In the last 24 hours, his pain has increased and changed in character, with radiation to his back and with associated shortness of breath. The arterial pulse is bounding (hyperdynamic) with a large amplitude and a brisk upstroke that rises to a single systolic peak. In this patient, an arterial “runoff” lesion such as aortic regurgitation is suggested. In many cases of acute aortic regurgitation, this arterial pulse contour is not seen, as adaptive hemodynamic changes have not had time to occur. This is consistent with significant mitral or aortic valvular regurgitation with an increased stroke volume associated with an increased preload and an increased velocity of contraction due to a reduced afterload. The normal location and size of the impulse suggest that these changes are acute, i. In many cases of acute valvular regurgitation, the apical impulse is also normal in contour, as adaptive changes have not had time to occur. This results in a rapid rise in ventricular pressure that shortens the duration of the murmur and decreases its frequency. The fact that this murmur is short suggests that it is due to increased flow across a non- stenotic aortic valve, as it occurs only during maximum ejection, and may be related to turbulence alone. It likely represents an accentuated P2, consistent with some degree of pulmonary hypertension. The early diastolic murmur is also likely due to early closure of the mitral valve. While mitral stenosis must be considered, the absence of both the first heart sound and an opening snap is evidence against this diagnosis. Proceed 37-12 Answer: In the lower lung fields, there are inspiratory and expiratory crackles bilaterally, reflecting pulmonary congestion. Question: What non-invasive laboratory procedure is likely to help further define this patient’s diagnosis? Since aortic regurgitation was suspected, a color flow study was also carried out. This correlates well with the bedside finding of a soft and/or inaudible first heart sound. Transesophageal echocardiography provides very useful information regarding the aortic root. The following transesophageal study specifically defines a dissecting hematoma as the etiology of this patient’s acute aortic regurgitation. Additional injections confirmed severe aortic regurgitation and revealed normal coronary arteries. The left ventricular pressure tracings revealed a marked and early rise in left ventricular diastolic pressure, exceeding that of the left atrium. Pulmonary pressure tracings revealed moderate pulmonary hypertension, the result of the sudden changes in left ventricular pressure. Vasodilator therapy to reduce afterload and beta blockade to reduce aortic shearing forces were initiated prior to surgery. Serious cardiac and great vessel injury may be overlooked in patients with trauma, in part because clinical manifestations may be delayed from the time of the traumatic event. These injuries may range from myocardial contusion to laceration or rupture of the aorta or the cardiac valves. The result is a marked elevation of left ventricular diastolic pressure and a limited forward stroke volume. This may produce a striking difference in the clinical presentation of the acute and chronic forms of this disease. A diagram of simultaneous hemodynamic, echocardiographic and phonocardiographic features of acute aortic regurgitation follows. This results in premature closure of the mitral valve, as illustrated by the M-Mode echocardiographic tracing (broken arrow). This premature closure results in a low frequency diastolic flow rumble as shown on the apex phonocardiogram (double arrows). The most helpful clinical features that differentiate acute and chronic aortic regurgitation follow. The arrows point to a dissecting hematoma in the ascending and transverse aorta, visible through the thin aortic adventitia. The arrow points to a dissecting hematoma between the adventitia and the media of the aorta. Angiography further defines the dissection, confirms the aortic regurgitation and reveals normal coronary arteries. Initial medical treatment designed to reduce afterload and aortic shearing forces is often indicated. Advances in medical care have made it possible for people with Marfan syndrome to live a normal lifespan if they are diagnosed and treated properly. Marfan syndrome most often affects the heart, blood vessels, bones, joints, and eyes. Connective tissue holds all parts of the body together and helps control how the body grows. Because connective tissue is found throughout the body, Marfan syndrome features can occur in many different parts of the body. Most often the condition af- fects the heart, blood vessels, bones, joints, and eyes. Marfan syndrome features can appear at any age—including in infants, teens, and older adults—and they can get worse as people age. If you suspect that you or a family member may have Marfan syndrome, find a doctor who is knowledgeable about the condition. Ideally, the diagnostic process should be coordinated by a medical geneticist (a doctor who specializes in genetic conditions). It is possible to have some Marfan syndrome features, but not enough for a confirmed diagnosis. The only way to know for sure is to be checked by a doctor who understands Marfan syndrome. You can download the family health history kit from our website to compile the details, including: • Past illnesses, operations, and hospitalizations • Medications • Reasons you think you or your family member might have Marfan syndrome • Family members who have, or might have, Marfan syndrome • Family members who died of a heart or vascular problem How is Marfan syndrome diagnosed? A Marfan syndrome diagnosis can often be made after exams of several parts of the body by doctors experienced with connective tissue disorders. The evaluation includes: • A detailed medical and family history, including information about any family member who may have the disorder or who had an early, unexplained, heart-related death • A complete physical examination You should also have tests to identify Marfan features that are not visible during the physical exam, including: • Echocardiogram. This test looks at your heart, its valves, and the aorta (blood vessel that carries blood from the heart to the rest of the body). It is possible for you to have one or more features of Marfan syndrome, but not enough for you to have a Marfan syndrome diagnosis. You may need additional exams by other doctors and additional genetic testing to see if you have a disorder that is related to Marfan syndrome. The use of genetic testing for the diagnosis of genetic disorders can be very complicated. Input from a geneticist or genetic counselor may be necessary to achieve a full understanding of the capabilities and limitations of genetic testing for Marfan syndrome. Here are some situations in which genetic testing may be helpful: • A child who does not have outward Marfan features, but has a parent with Marfan syndrome, should be monitored on an ongoing basis if the genetic test for the child is positive. More information can be found in our resource on genetic testing for Marfan syndrome. Many people with Marfan syndrome features (whether they have a diagnosis or not) need medical treatment and follow-up care. There may be concerns about passing the disorder to children, as well as worries about genetic implications for siblings. The parents and siblings of a child diagnosed with Marfan syndrome may feel sadness, anger, and guilt. It is important for parents to know that nothing that they did caused the child to have the condition.
However blood pressure garlic order 75 mg plavix mastercard, rarely pulse pressure norms discount plavix 75 mg line, the uterus may be normal hypertension diagnosis jnc 7 purchase plavix american express, but lacking a conduit to the introitus class 4 arrhythmia drugs order plavix master card, or there may only be rudimentary, bicornuate cords present. If a partial endometrial cavity is present, cyclic abdominal pain may be a complaint. Because of the similarity to some types of male pseudohermaphroditism, it is worthwhile to demonstrate the normal female karyotype. Because the ovaries are not müllerian structures, ovarian function is normal and can be documented with basal body temperatures or peripheral levels of progesterone. The exact cause of müllerian agenesis is unknown; however, likely causes are mutations of the gene for antimüllerian hormone or the gene for the antimüllerian hormone receptor. The underlying mechanism would be unwanted exposure to antimüllerian hormone activity. Thus far, no activating mutations have been reported, 65 in contrast to inactivating mutations that cause persistence of müllerian structures. A 66 mutation has been identified in galactose-1-phosphate uridyl transferase in daughters with müllerian agenesis and their mothers. This is different from classic galactosemia; however, it is postulated that increased intrauterine exposure to galactose because of this error in galactose metabolism can be a biologic basis for müllerian agenesis. High-galactose feeding of pregnant mice delays vaginal opening in female offspring. In this group of patients with müllerian agenesis, oocyte depletion (and premature ovarian failure) may be more common. Approximately one-third of patients have urinary tract abnormalities, and 12% or more have skeletal anomalies, most involving the spine, although absent digits and syndactyly (webbing or fusion of fingers or toes) can occur. Renal tract abnormalities include ectopic kidney, renal agenesis, horseshoe kidney, and abnormal collecting ducts. When the presence of a uterine structure is suspected on examination, ultrasound can be utilized to 67, 68 depict the size and symmetry of the structure. Extirpation of the müllerian remnants is certainly not necessary unless they are causing a problem such as uterine fibroid growth, hematometra, endometriosis, or symptomatic herniation into the inguinal canal. Because of the difficulties and complications experienced in surgical series, we favor, when possible, an alternative to the surgical construction of an artificial vagina. Beginning first in a posterior direction, and then after 2 weeks changing upward to the usual line of the vaginal axis, pressure with commercially available vaginal dilators is performed for 20 min daily to the point of 71 modest discomfort. Utilizing increasingly larger dilators, a functional vagina can be created in several months. Plastic syringe covers can be used instead of the expensive commercial glass dilators. An easier and very effective technique is to hold the dilator in place with a tight garment, maintaining pressure by sitting on a 72 racing bicycle seat (mounted on a special stool or even on a bicycle). In patients who are unwilling or unable to undergo the dilatation program, the Vecchietti operation applies a traction device either transabdominally or by 73 laparoscopy. Operative treatment should be reserved for those women in whom the Frank method is unacceptable, or fails, or when a well-formed uterus is present and fertility might be preserved. One recommendation is to perform an initial laparotomy to evaluate the 74 cervical canal; if the cervix is atretic, the uterus should be removed. If it is the relatively simple problem of an imperforate hymen or a transverse vaginal septum, surgery is indicated. Most have recommended against trying to preserve fertility in the presence of complete vaginal agenesis. The morbidity subsequent to this surgery argues for removal of the müllerian structures at the time of construction of a neovagina. Patients with a transverse vaginal septum, which is a failure of canalization of the distal third of the vagina, usually present with symptoms of obstruction and urinary frequency. A transverse vaginal septum can be accompanied by abnormalities of the upper reproductive tract; e. Distal obstruction of the genital tract is the only condition in this category that can be considered an emergency. Delay in surgical treatment can lead to infertility due to inflammatory changes and endometriosis. Diagnostic needling should be avoided because a hematocolpos can be converted into a pyocolpos. Reassurance and support are necessary to carry a patient through these procedures. Problems with body image and sexual enjoyment can be avoided, and, although infertile, a full and normal life as a woman can be achieved. Furthermore, genetic offspring can be achieved by collection of oocytes from the genetic mother, 76 fertilization by the genetic father, and placement into a surrogate carrier. An analysis of 34 surrogacy live births resulting from oocytes retrieved from 58 women with congenital absence of the uterus and vagina could find no indication of inheritance in a dominant fashion, making surrogate pregnancy a reasonable option for 77 patients with this disorder. Androgen Insensitivity (Testicular Feminization) Complete androgen insensitivity (testicular feminization) is the likely diagnosis when a blind vaginal canal is encountered and the uterus is absent (also discussed in Chapter 9). This is the third most common cause of primary amenorrhea after gonadal dysgenesis and müllerian agenesis. Pseudohermaphrodite means that the genitalia are opposite of the gonads; thus, the individual is phenotypically female but with absent or meager pubic and axillary hair. Failures in male development can be considered a spectrum with incomplete forms of androgen insensitivity being represented by some androgen response. Transmission of this disorder is by means of an X-linked recessive gene that is responsible for the androgen intracellular receptor (see Chapter 9 for a discussion of the androgen receptor defect). A female child with inguinal hernias because the testes are frequently partially descended. These patients appear normal at birth except for the possible presence of an inguinal hernia, and most patients are not seen by a physician until puberty. Growth and development are normal, although overall height is usually greater than average, and there may be an eunuchoidal tendency (long arms, big hands, and big feet). The breasts, although large, are abnormal; actual glandular tissue is not abundant, nipples are small, and the areolae are pale. More than 50% have an inguinal hernia, the labia minora are usually underdeveloped, and the blind vagina is less deep than normal. Rudimentary fallopian tubes are composed of fibromuscular tissue with only occasional epithelial lining. After puberty, the testis displays immature tubular development, and tubules are lined by immature germ cells and Sertoli cells. In 50 reported cases, there were 11 malignancies, 15 adenomas, and 10 benign cysts: a 22% incidence of malignancy and a 52% incidence of 78 35, 79, 80 and81 neoplasia. More recent series indicate a lower overall incidence of gonadal tumors, about 5–10%. Therefore, once full development is attained after puberty, the gonads should be removed at approximately age 16–18, and the patient should receive hormone therapy. This is the only exception to the rule that gonads with a Y chromosome should be removed as soon as a diagnosis is made. There are two reasons: first, the development achieved with hormone treatment does not seem to match the smooth pubertal changes due to endogenous hormones, and second, gonadal tumors in these patients have not been encountered prior to puberty. Removal of gonadal 82 tissue can be accomplished by a skilled operator through the laparoscope, reserving the option of laparotomy if the gonads are inaccessible. When testicular feminization was first studied, it was found that the urinary 17-ketosteroids were normal, and it was suggested that there might be a resistance to androgen action rather than an absence of androgens — a congenital androgen insensitivity. Indeed, the plasma levels of testosterone are in the normal to high male range, and the plasma clearance and metabolism of testosterone are normal. Thus, these patients produce testosterone, but they do not respond to androgens, either their own or those given locally or systemically. Therefore, the critical steps in sexual differentiation, which require androgens, fail to take place, and development is totally female. Because antimüllerian hormone is present, development of the müllerian duct is inhibited, hence the absence of uterus, tubes, and upper vagina. Cases of incomplete androgen insensitivity (one-tenth as common as the complete syndrome) represent individuals with some androgen effect.
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