Oris how many images can u take today I Mena what us ur limit so that u convert it into structures notes

Convert it into well structured notes . Don't skip anyy data DELAYED PUBERTY Puberty is said to be delayed when the breast tissue and/or pubic hair have not appeared by 13-14 years or menarche appears as late as 16 years (Table 5.3). The TABLE 5.3: Causes of delayed puberty. Hypergonadotropic hypogonadism Gonadal dysgenesis-45,XO Pure gonadal dysgenesis-46,XX, 46,XY Ovarian failure-46,XX Hypogonadotropic hypogonadism Constitutional delay Chronic illness, malnutrition Primary hypothyroidism Isolated gonadotropin deficiency (Kallmann's syndrome) Intracranial lesions-tumors: Craniopharyngioma, pituitary adenomas Eugonadism Anatomical causes (Ch. 4) Müllerian agenesis Imperforate hymen Transverse vaginal septum Androgen insensitivity syndromenormal upper age limit of menarche is 15 years. It is more common in boys than in girls. Delayed Menarche Before the onset of menarche, pubertal (Tanner stage 1 through 5) changes are breast budding followed within few months by the appearance of pubic hair. Breast budding is the earliest sign and menarche is the latest sign of puberty. Onset of menarche is related to body composition (ratio of body fat to total body weight) rather than total body weight. Moderately obese girls between 20% and 30% above the ideal body weight have earlier onset of menarche. Malnutrition is known to delay the onset of puberty. Diagnosis Details of history taking and physical examination are done. Examination of secondary sexual characters: Mature: To evaluate for Müllerian agenesis/dysgenesis. Asynchronous development of breasts, pubic hair → androgen insensitivity syndrome. Immature secondary sexual characters: Serum FSH, PRL, TSH, T Chapter 5: Puberty-Normal and Abnormal ↑FSH: Karyotype for gonadal dysgenesis/premature gonadal failure. Low/normal FSH sellar CT/MRI normal constitutional/chronic illness/malnutrition. Abnormal sellar CT/MRI→ hypopituitarism/CNS tumor TSH:↑ TSH hypothyroidism. Treatment Treatment is directed according to the etiology. Assurance, improvement of general health and treatment of any illness may be of help in nonendocrinal causes. Cases with hypogonadism may be treated with cyclic estrogen. Unopposed estrogen 0.3 mg (conjugated estrogen) daily is given for first 6 months. Then combined estrogen and progestin, sequential regimen is started (Ch. 6). Cases of hypergonadotropic hypogonadism should have chromosomal study to exclude intersexuality. HEAVY MENSTRUAL BLEEDING IN PUBERTY (MENORRHAGIA) Menstrual abnormality in adolescents are common. The periods may be heavy, irregular or scanty initially.Eventually, the majority of these teenaged girls establish a normal cycle and are fertile. Important Causes of Heavy Menstrual Bleeding (Menorrhagia) Abnormal uterine bleeding (0) (95%): Anovulatory cycles unopposed estrogen endometrial hyper-plasia prolonged and heavy periods. Endocrine dysfunction Polycystic ovary syndrome (PCOS) Hypothyroidism or hyperthyroidism. Hematological Idiopathic thrombocytopenic purpura Von-Willebrand's disease Leukemia. Pelvic tumors Fibroid uterus. Sarcoma botryoides. Estrogen producing ovarian tumor. Pregnancy complications (abortion). gnosis mosis is made by careful history taking and through cal examination. Evaluation is especially indicated if the menstrual aterval is <22 days or >44 days, lasts longer than one week or the bleeding is too heavy that anemia develops. Investigations Investigations are planned according to the clinical diagnosis. Investigations include, routine hematological examination, including bleeding time, clotting time, platelet count. Thyroid profile (TSH, TT), coagulation parameters [PT, PTT, factor VIII and von Willebrand factor (VWF)], and imaging study of the pelvis by ultrasonography or MRI to exclude pelvic pathology may be needed. Examination under anesthesia (EUA) and uterine curettage may be needed to exclude any pelvic pathology (pregnancy complications). The curetted material is sent for histopathological study. Management The girl needs adequate explanation, reassurance and psychological support. Rest and correction of anemia are helpful in majority of the cases. Therapy with hematinics or even blood transfusion may be needed. In refractory cases, progestogens, such as medroxyprogesterone acetate or norethisterone 5 mg thrice daily is given till bleeding stops. Usually, the bleed-ing is controlled within 3-7 days. Medication is continued for 21 days. The condition usually becomes normal fol-lowing 2-3 courses and then normal cycles resume. In emergency, conjugated equine estrogen 20-40 mg IV is given every 6-8 hours. Once the bleeding is controlled combined oral pills are started (Flowchart 5.1). GnRH analogs can be used for short-term. Regular menstrual cycle will be established once the hypothalamic-pituitary-ovarian axis is matured. POINTS

Pointwise. a 124 Chapter 11: Pelvic Infection Laparoscopy helps to aspirate fluid or pus for micro-biological study from the fallopian tube, ovary or pouch of Douglas. emphasized, one should not wait for the repon instead treatment should be started empirically. The the cervical and urethral discharge and secretion from and anaerobic). the Bartholin's gland, and laparoscopic or laparotom collection of pus from the fallopian tubes. The materia are to be subjected to Gram stain and culture (aerob materials for identification of organisms are fron Microbial diagnosis is difficult. But as alread Sonography: It is of limited value. Dilated and fluid-mass are suggestive of PID. It may be employed where clinical examination is difficult or is not informative because of acute tenderness or obesity. MRI is also useful. filled tubes, fluid in the pouch of Douglas or adnexal C ال Lo Clinical features Pain Acute salpingitis (Table 13.3) Gram stain of the discharge may be positive for gram negative intracellular diplococci of N. gonorrhoe Bacteriologic diagnosis of Chlamydia trachomatis is dis cult. However, the status of the sexual partner is the sing most important clue to the diagnosis of chlamydial infe tion. If the woman has a stable sexual relationship wi an asymptomatic man, the clinical manifestations unlikely to be due to chlamydial infection. white cell count, if exceeds 30,000/mL is significant Culdocentesis: Aspiration of peritoneal fluid and its in acute PID. Bacterial culture from the fluid is not informative because of vaginal contamination. Investigations are also to be extended to male partner and smear and culture are made from urethral secretion. Diagnosis The anatomic diagnosis of infection to the upper genital tract is made from the following clinical features (Table 11.2). TABLE 11.2: Clinical features of acute PID. Fever >38°C Bilateral lower abdominal tenderness with radiation to the legs Abnormal vaginal discharge Abnormal uterine bleeding Deep dyspareunia Cervical motion tenderness On bimanual Adnexal tenderness/mass examination Raised ESR Differential Diagnosis (Table 11.3) The clinical condition may be confused with: Appendicitis Disturbed ectopic pregnancy Torsion of ovarian pedicle, hemorrhage or rupture of ovarian cyst Endometriosis Diverticulitis Urinary tract infection. The two conditions-acute appendicitis and distur bed ectopic pregnancy must be ruled out, because be the conditions require urgent laparotomy whereas ac salpingitis is to be treated conservatively. TABLE 11.3: Clinical features of acute salpingitis, acute appendicitis and disturbed ectopic.ACUTE PELVIC INFECTIONS Pelvic inflammatory disease (PID) Following delivery and abortion Following gynecological procedures Following intrauterine devices (IUD) Secondary to other infections-appendicitis. PELVIC INFLAMMATORY DISEASE (PID) Definition Pelvic inflammatory disease (PID) of the upper genital tract, is a spectrum of infection and inflammation of the(endometrium), fallopian tubes, ovaries, pelvic peri-toneum and surrounding structures (parametrium). It is attributed to the ascending spread of microorganisms from the cervicovaginal canal to the contiguous pelvic structures. The clinical syndrome is not related to preg-nancy and surgery. The terminology is currently used to express the specific organ pathology. Thus infection may include any or all of the following anatomic sites and it is described as Endometrium (endometritis), tubes (salpingitis), ovary (oophoritis), Myometrium (myometritis), uterine serosa and broad ligament (parametritis), and pelvic peritoneum (peritonitis) or tubo-ovarian abscess. PID may be: (A) Acute (duration <30 days); (B) Subclinical; (C). Chronic (Mycobacterium tuberculosis or Actinomyces) Epidemiology The incidence of pelvic infection is on the rise due to the rise in sexually transmitted infections. The incidence varies from 1-2% per year among sexually active women. About 85% are spontaneous infection. The remaining 15% follow some procedures, including endometrial biopsy, uterine curettage, inser-tion of IUD and hysterosalpingography. Two-thirds are restricted to young women of less than 25 years and the remaining one-third limited among 30 years or older. Acute PID is the ascending infection from the vagina and the cervix in majority (99%) of cases. It is rare in women not having menstruation (premenarchal girls, postmenopausal or the pregnant women). PID may be due to transperitoneal spread of infections from a perforated appendix or intraperitoneal abscess. RISK FACTORS FOR PID ⚫ Sexually active teenagers Multiple sexual partners Absence of contraceptive pill or barrier method use Previous history of acute PID IUD users (not with LNG-IUS) Lower socioeconomic status Husband/sexual partner with urethritis or STI Genetic predisposition Protective Factors Contraceptive practice Barrier methods, especially condom, diaphragm with spermicides (p. 473). aspects. Oral steroidal contraceptives have got two preventive The Produce thick mucus plug preventing ascent of sperm. C S P Decrease in duration of menstruation, creates a upper tract. shorter interval of bacterial colonization of the Monogamy or having a partner who had vasectomy. Others Pregnancy Menopause Vaccines: Hepatitis B, HPV (p. 306) Postcoital washing (urethra, genital skin) Primary prevention: Reduction of STL with the use safe sex practices and with the use of condoms. Secondary prevention: Universal screening women at high risk for chlamydia and gonorrhe Treatment of women with infections and their se partners. Increased education and awareness needed to prevent recurrent infection. Microbiology Acute PID is usually a polymicrobial infection caused organisms ascending upstairs from downstairs. The primary organisms are sexually trarismitted limited approximately to N. gonorrhoeae in 30%, Chlam trachomatis in 30% and Mycoplasma hominis in 10% The Secondary Organisms Aerobic-nonhemolytic Streptococcus, E. coli, Streptococcus and Staphylococcus. Anaerobic-Bacteroides species-fragilis and Peptostreptococcus and Peptococcus. Mode of Affection The classic concept is that the gonococcus ascend to affect the tubes through mucosal continuity a contiguity. Reflux of menstrual blood along with gonococci the fallopian tubes is the other possibility. Mycoplasma hominis probably spreads across the ametrium to affect the tube. The secondary organisms probably affect the through lymphatics. Rarely, organisms from the gut may affect the directly. Pathology The involvement of the tube is almost always bilater usually following menses due to loss of genital defens The pathological process is initiated primarily endosalpinx, There is gross destruction of the ep cells, cilia and microvilli. In severe infection, it all the lavers of the tube and produces acute in tory reaction, Tubes become edematous and hyper The exfoliated cells along with the exudate pour in abdominal ostium is closed by the indrawing lumen of the tube and agglutinate the mucosa watery producing hydrosalpinx or purulent p edematous fimbriae and by inflammatory adhesions uterine end is closed by congestion. The closure of the ostia results in pent up of the exudate inside the Depending upon the virulence, the exudate ing pyosalpinx, The organisms spontaneously die 2-3 weeks. As the serous coat is not muchstructures are not so dense, in fact flimsy, unlike pyo-genic or tubercular infection. On occasions, the exudate pours through the abdominal ostium to produce pelvic peritonitis and pelvic abscess or may affect the ovary (the organisms gain access through the ovulation rent) producing ovarian abscess. A tubo-ovarian abscess is thus formed (Fig. 11.1). Chlamydia remain in the fallopian tube for several months. Repeat infections with Chlamydia causes severe tubal damage and increase the risk of ectopic pregnancy. Clinical Features Symptoms Patients with acute PID present with a wide range of non-specific clinical symptoms. Bilateral lower abdominal and pelvic pain which is dull in nature. The onset of pain is more rapid and acute in gonococcal infection (3 days) than in chlamydial infection (5-7 days) There is fever, lassitude and headache Irregular and excessive vaginal bleeding is usually due to associated endometritis Abnormal vaginal discharge which becomes purulent and or copious Nausea and vomiting Dyspareunia Pain and discomfort in the right hypochondrium due to concomitant perihepatitis (Fitz-Hugh-Curtis syndrome) may occur in 5-10% of cases of acute salpingitis. The liver is involved due to transperitoneal or vascular dissemination of either gonococcal or chlamydial infection. Laparoscopic examination reveals inflamed liver cap-sule with classic violin string adhesions to the parietal peritoneum and beneath the diaphragm. Signs The temperature is elevated to beyond 38.3°C.. Abdominal palpation reveals tenderness on both the quadrants of lower abdomen. The liver may be enlarged and tender (perihepatitis). Vaginal examination reveals: (a) Abnormal vaginal discharge which may be of purulent; (b) Congested external urethralmeatusoropeningsofBartholin'sducts through whichpusmaybe seenescapingoutonpressure; (c) Speculum examination shows congested cer-vix with purulent discharge from the canal and (d) Bimanual examination reveals bilateral tenderness on fornix palpation, which increases more with move-ment of the cervix (cervical motion tenderness). There may be thickening or a definite mass felt through the fornices. Chapter 11: Pelvic Infection 1 Clinical Diagnostic Criteria of Acute PID (CDC-2015) Minimum criteria Lower abdominal tenderness or Adnexal tenderness or Cervical motion tenderness Additional criteria for diagnosing PID Oral temperature >38°C Mucopurulent cervical or vaginal discharge Abundant WBCs on saline microscopy of cervical secretions Raised C-reactive protein Elevated ESR Laboratory documentation of positive cervical infection with Gonorrhea or C. trachomatis Definitive criteria Histopathologic evidence of endometritis on biopsy Imaging study (TVS/MRI) showing evidence of thickened fluid filled tubes, ± free pelvic fluid or tubo-ovarian complex. Laparoscopic evidence of PID (see below). Although initial treatment can be made before bacte-riologic diagnosis of C. trachomatis or N. gonorrhoeae infection, such a diagnosis emphasizes the need to treat sex partners. Investigations Identification of organisms: The materials are col-lected from the following available sources: Discharge from the urethra or Bartholin's gland Cervical canal Collected pus from the fallopian tubes during laparoscopy or laparotomy. The material so collected is subjected to Gram stain and culture (aerobic and anaerobic). The findings of gram-negative diplococci is very much suggestive of gonococcal infection. The detection of C. trachomatis is difficult (for diagnosis, p. 140). Treatment for C. trachomatis should be started from the clinical diagnosis. Blood: Leukocyte count shows leukocytosis to more than 10,000 per cu mm and an elevated ESR value of more than 15 mm per hour. The results correlate with the severity of the inflammatory reactions of the fallopian tubes as seen on laparoscopy. Serological test for syphilis should be carried out for both the partners in all cases. Laparoscopy: Laparoscopy is considered the "gold standard. While it is the most reliable aid to support the clinical diagnosis but it may not be feasible to do in all cases. It is reserved only in those cases in which differential diagnosis includes salpingitis, appendicitis or ectopic pregnancy. Nonresponding pelvic mass needs laparoscopic clarification. Laparoscopic Findings and Grading of PID Mild: Tubes-edema, erythema, no spontaneous purulent exudates and tubes are freely mobile Moderate: Gross purulent material present, erythema and edema, marked; tubes may not be freely movable, and fimbria stroma may not be patent Severe: Pyosalpinx or inflammatory complex, abscess 'Violin string' like adhesions in the pelvis and around the liver suggests chlamydial infection (Fig. 10.5).Laparoscopy helps to aspirate fluid or pus for micro-biological study from the fallopian tube, ovary or pouch of Douglas. emphasized, one should not wait for the repon instead treatment should be started empirically. The the cervical and urethral discharge and secretion from and anaerobic). the Bartholin's gland, and laparoscopic or laparotom collection of pus from the fallopian tubes. The materia are to be subjected to Gram stain and culture (aerob materials for identification of organisms are fron Microbial diagnosis is difficult. But as alread Sonography: It is of limited value. Dilated and fluid-mass are suggestive of PID. It may be employed where clinical examination is difficult or is not informative because of acute tenderness or obesity. MRI is also useful. filled tubes, fluid in the pouch of Douglas or adnexal C ال Lo Clinical features Pain Acute salpingitis (Table 13.3) Gram stain of the discharge may be positive for gram negative intracellular diplococci of N. gonorrhoe Bacteriologic diagnosis of Chlamydia trachomatis is dis cult. However, the status of the sexual partner is the sing most important clue to the diagnosis of chlamydial infe tion. If the woman has a stable sexual relationship wi an asymptomatic man, the clinical manifestations unlikely to be due to chlamydial infection. white cell count, if exceeds 30,000/mL is significant Culdocentesis: Aspiration of peritoneal fluid and its in acute PID. Bacterial culture from the fluid is not informative because of vaginal contamination. Investigations are also to be extended to male partner and smear and culture are made from urethral secretion. Diagnosis The anatomic diagnosis of infection to the upper genital tract is made from the following clinicals

Complications of PID Immediate Pelvic peritonitis or even generalized peritonitis Septicemia-producing arthritis or myocarditis. Late Dyspareunia Infertility rate is 12%, after two episodes increases to 25% and after three raises to 50%. It is due to tubal damage or tubo-ovarian mass Chronic pelvic inflammation is due to recurrent or associated pyogenic infection (25%) Formation of adhesions or hydrosalpinx or pyosalpinx and tubo-ovarian abscess Chronic pelvic pain and ill health Increased risk of ectopic pregnancy (6-10 fold). Treatment with: triosis litis ract infection Essential steps in the prevention are: Community-based approach to increase public health awareness. Prevention of sexually transmitted infections with the knowledge of healthy and safer sex. Liberal use of contraceptives. Routine screening of high-risk population. The principles of therapy are: citis and diste ut, because bo ny whereas a To control the infection To prevent infertility and late sequelae To prevent reinfection. Follow up examination of women within 48 to 72 hours for evaluation of response. urbed ectopic wer abdomen resent Outpatient Therapy Apart from adequate rest and analgesic, antibiotics are to be prescribed even before the microbiological report is available. In most cases, the infection is polymicrobial in nature, therefore, combination of antibiotics should be prescribed. Antimicrobial coverage includes: N. gonorrhoeae, C. trachomatis, streptococci, E. coli and anaerobes. Outpatients antibiotic therapy for acute PID is given in Table 11.4. Antibiotic therapy within 72 hours of onset of symptoms is protective against infertility or ectopic pregnancy. rise even wi emperature domen more All patients treated in the outpatients are evaluated after 48 hours and if no response, are to be hospitalized. Inpatient Therapy The patients are to be hospitalized for antibiotic therapy in the conditions as mentioned in Table 11.5. y be felt thi x extending Douglas erine cavity POD see autor of Obstemi The patient is urged to take bed rest. Oral feeding is restricted. Dehydration and acidosis are to be corrected by intravenous fluid. Fibroid torsi tion tenden Intravenous antibiotic therapy is recommended for at least 48 hours but may be extended to 4 days, if necessary (Table 11.6). TABLE 11.4: Outpatient treatment of PID (CDC-2015) Chapter 11: Pelvic infection 1 Ceftriaxone 250 mg IM single dose От Cefoxitin, 2 g IM single dose and probenecid, PO single done Or Injection Cefotaxime IV Plus 2. Doxycycline 100 mg PO bid for 14 days with or without Metronidazole 500 mg PO bid for 14 days 3. Minimum criteria: Empirical treatment of PID should be initiated. Antibiotic therapy within 72 hours of onset of symptoms, is protective against infertility or ectopic pregnancy TABLE 11.5: Indications for hospitalization (CDC-2015). Suspected tubo-ovarian abscess Severe illness, vomiting, temperature >38°C Uncertain diagnosis-where surgical emergencies, leg appendicitis) cannot be excluded Unresponsive to outpatient therapy for 48 hours Intolerance to outpatient oral antibiotics Coexisting pregnancy Patient is known to have HIV infection TABLE 11.6: Inpatient antibiotic therapy (CDC-2021). Parenteral regimen A Cefoxitin 2 g IV every 6 hours for 7 days Plus Doxycycline 100 mg PO BID for 14 days Parenteral regimen B Clindamycin 900 mg IV every 8 hours Plus Gentamicin 2 mg/kg IV (loading dose), followed by 1.5 mg/kg IV (maintenance dose) every 8 hours Aztreonam (2 g IV slowly) is similar to aminoglycoside and can be used. It has no renal toxicity and but is more expensive. Alternative parenteral regimen Ampicillin-sulbactam 3 g IV every 6 hours 3-5 days Plus Doxycycline 100 mg orally BID for 14 days Women with an IUD as outpatient therapy, keeping IUD in situ may be tried. If there is no improvement IUD should be removed. However, no regimen is uniformly effective for all patients. Moreover superiority of one regimen over the is not yet established. other in respect to initial response or subsequent fertility Improvement of the patient is evidenced by remi-ssion of temperature, improvement of pelvic tenderness, normal white blood cell count and negative report on bacteriological study. Women need to be admitted if not responding to medical therapy. Indications of Surgery The indications of surgery are comparatively less. The unequivocal indications are:Generalized peritonitis Pelvic abscess Tubo-ovarian abscess which does not respond (48-72 hours) to antimicrobial therapy/or there is rupture To prevent reinfection: The following formalities are to be rigidly followed to prevent reinfection: Educating the patient to avoid reinfection and the potential hazards of it The patient should be warned against multiple sexual partners To use condom The sexual partner or partners are to be traced and properly investigated and treated

Common Variations of Vaginal Maldevelopments Agenesis of vagina Hypoplasia Failure of vertical fusion Failure of lateral fusion. Etiological factors for Müllerian malformations are not clearly understood. The probable causes are polygenic, multifactorial, teratogens or environmental. DES related abnormalities are rare these days. Pathology of Müllerian Malformation It may be due to failure of formation of the vaginal plate or due to its failure of canalization or cavitation. Vertical fusion defects result in failure of fusion of the Müllerian system with urogenital sinus. It may also be due to incomplete or segmental canalization of the vagina. Disorders of lateral fusion are also due to failure of the two Müllerian ducts to unite. Lateral fusion defects are the most common. A wide range of anomalies can occur. Anomalies may be symmetric, asymmetric, obstructed or nonobstructed. This may result in double uterovaginal canals (See Fig. 4.7). Transverse vaginal septa (Fig. 4.5) are due to the defect in fusion or canalization of the urogenital sinus and the Müllerian ducts. About 45% occur in the upper vagina, 40% in midvagina and 15% in the lower vagina. Septum located in the lower vagina is often complete and the signs and symptoms are simi-lar to that of imperforate hymen (cryptomenorrhea). Ultrasonography is a useful investigation to detect hematometra, hematocolpos, and also urinary tract malformations. The principles of surgical treatment are the same. Septum in the upper vagina is often per-forated. Incision of a complete (imperforate) septum becomes easy when the upper vagina is distended. This reduces the risk of injury to adjacent organs. Otherwise abdominovaginal approach is made. Longitudinal septum of the vagina may be present when the distal parts of the Müllerian ducts fail to fuse (fusion failure). It may be associated with double uterus -Hematosalpinx -Hematometra Hematocolpos -Vaginal transverse septum at different levels Fig.4.5 Hematocolpos, hematometra and hematosalpinxare se due to transverse vaginal septum. Hematometra develops puberty, Cyclic lower abdominal pain is a common presentation and double cervix. It may be asymptomatic and need no treatment. But it may cause dyspareunia or ma obstruct delivery. In such circumstances, the septum to be excised. Results of surgery are good in terms achieving pregnancy. The pregnancy continuation rate is approximately 20% for a septum in the uppe 1/3 of vagina. For the middle third it is around 40 Septum in the lower third has 100% pregnanc continuation rate. Rarely an adolescent during her menses may prese with pain in the vagina and the pelvis of one side. examination, the vagina is patent and cervix is norm A mass is palpated on one side of the vaginal wall a extending up to the pelvis. Obstructed hemivagina almost always associated with ipsilateral renal agenes The triad of: uterus didelphys, obstructed hemivag and ipsilateral renal anomaly is known as OHVIL syndrome. Surgical correction of longitudinal va septum is done in obstructed cases. Sonographs guided excision may be needed (Figs. 4.6A to C). The differential diagnosis of a female with pri amenorrhea with absence of vagina includes: va agenesis, transverse vaginal septum, imperf hymen, or androgen insensitivity syndrome. Investigation for the diagnosis includes imag with 3D ultrasonography for the presence of and ovaries. MRI is superior for diagnosis of muller agenesis, MRI can detect any rudimentary mullen component. Laparoscopic evaluation may be need cases where imaging is not helpfulPARTIAL AGENESIS OF UPPER VAGINA A segment of vagina may be atretic in the upper-third. It hary inal rate is often associated with hypoplasia or even absence of cer-vix (cervicovaginal agenesis). Uterus may be normal and functioning or malformed. Primary amenorrhea (crypto-menorrhea), hematometra, hematocolpos, cyclic lower abdominal pain and presence of lower abdominal mass (as felt per abdomen or per rectum) point to the diagnosis. Imaging studies with USG or MRI can make the diagno-sis. Conventional treatment is hysterectomy. Currently, abdominovaginal approach is made to establish commu-nication between the uterovaginal canal above and the newly created vagina below. Prosthesis is used to prevent restenosis. The result is, however, not always satisfactory though successful pregnancy and live birth have been reported. When hysterectomy is considered, ovaries should be conserved. This gives the benefit of endogenous estro gen. Assisted reproductive technology would be the option, when desired, using a surrogate uterus. Complete Agenesis Complete agenesis of the vagina is almost always asso-ciated with absence of uterus. Some women may have rudimentary uterine horns. Some rudimentary horns are functional as they contain endometrial lining. There is menstruation, and the gonads are the healthy ovaries. The tubes are usually normal. The patient is phe notypically fernale, with normal fernale karyotype pattern (46,XX). The entity is often associated with urinary tract (40%) and skeletal (12%) malformation. This is called Mayer-Rokitansky-Küster-Hauser syndrome. The patient usually seeks advice for primary amenorrhea and dyspareunia. The other associated organ malformations are: Urinary tract: renal agenesis, pelvic kidney, ureteral duplication. The skeletal anomalies: congenital fusion or absence of vertebrae. Other anomalies are: cardiac defect and hearing loss. Management of Cases with Mullerian Agenesis The aim of the treatment is to create neovagina for future sexual function. Women needs to be counselled that motherhood could be possible with adoption or using her own eggs with assisted reproductive technology (ART) or gestational carrier. Other option is uterine transplantation. Treatment options are: (1) Nonsurgical and (2) Surgical. Objective of treatment is to create a neovagina:vaginal dilators for a period of 6-12 months. It is used daily for 15-30 minutes. It takes for 3-6 months or longer. Presence of a vaginal dimple (1 cm) is often seen. This method (Frank, 1938) is a simple and effective one. Surgical methods: The aim is to create a space surgi-cally between the bladder and urethra in front and the rectum with the anal canal behind, extending up to the level of pelvic peritoneum. The neovagina such crated is lined by amnion, split thickness skin graft, buccal mucosa, peritoneum, or a synthetic tissue graft. The patient has to use a mould for a number of months to maintain the vaginal space.She is advised to use vaginal dilators on a regular basis (weekly) to maintain the neovagina if she is not having vaginal intercourse. Various procedures of vaginal reconstruction (vagino-plasty) are done. Mcindoe Reed procedure (1938): A space is created digitally berween the bladder and the rectum and anal canal behind. Split thickness skin graft is used over a mold. This mold is kept in this neovaginal spare. Williams vulvovaginoplasty (1976): A vaginal pouch is created from skin flaps of labia majora in the midline. This is not done these days. Vaginoplasty with amnion graft (Chakraborty, Konar, 2004). Complications of vaginoplasty: During surgery-vesico-vaginal fistula (VVF), rectovaginal fistula (RVF), infection, and bleeding are the important ones. Dyspareunia, reste-nosis are the common late complications. ASSOCIATED ABNORMALITIES Associated abnormalities with: Vesicovaginal (congenital) fistula is formed when the Müllerian eminence ruptures into the vesicourethral part of the cloaca instead of the pelvic part of the urogenital sinus (Fig. 3.2D). Rectovaginal (congenital) fistula when the Müllerian eminence opens in the dorsal segment of the endodermal cloaca. Persistent urogenital sinus with various irregularities of urethral and vaginal orifices in the sinus. ASRM MULLERIAN ANOMALIES CLASSIFICATION 2021 (ASRM MAC-2021) The task force of ASRM and SRS classifies Mullerian anomalies into nine categories (See Figs. 4.7 A to 1). Unlike the AFS classification, anomaly categories are identified as descriptive terminologies: Mullerian agenesis Cervical agenesis Septate uterus Longitudinal vaginal Unicornuate uterus Bicornuate uterus septum Transverse vaginal Uterus didelphys septum Complex anomalies Incidence of Müllerian abnormalities: It varies betwee 3% and 4%. The incidence is found to be high in woma suffering from recurrent miscarriage or preterm dellwer (5-20%). Abnormalities of the Uterus (ASRM MAC-2021) Mullerian agenesis/Cervical agenesis, Unicornuans uterus (Figs. 4.7A to C): These abnormalities are due to failure of development (complete or partial) of one both the Müllerian ducts. Uterus didelphys (Fig. 4.7D): These anomalies are to failure of midline fusion (varying degree) of both the ducts. Bicornuate uterus, Septate uterus (Figs. 4.7E and F These anomalies are due to failure of resorption of midline septum to a varying degree. Septate uterus should have an endometri septum >1 cm depth as measured from the bicornual line with the leading edge of the septun having an angle of <90°. (Fig. 4.7G) Septum may be assessed by any technique: ultra sonography (2D/3D), MRI, sonohysterogram combined hysteroscopy-laparoscopy or hysteros copy, combined with imaging of external contour uterus. Bicornuate uterus has an external fundal indenta tion of >1.0 cm. Vaginal septum (transverse/longitudinal): This due to failure of fusion or canalization of urogenit sinus and/or the Mullerian ducts at varying level and degree. Complex anomalies: Where developmental abnor malities of the uterus, cervix, tubes and the vagina are associated in different combinations and with varying degree. vagina, cervix, fallopian tubes and urinary system mus that are beyond the common theory of Müllerian devel With this classification, associated anomalies of the be documented separately. There are many anomalies opment. Types of fusion anomalies (Figs. 4.7A to 1) Arcuate (18%): The cornual parts of the utens remains separated. Arcuate/normal uterus has depth of indentations or equal to 1 cm and the angle is 9 (Fig. 4.8D)]. Uterus didelphys (8%): There is complete lack of fus of the Müllerian ducts with a double uterus, double cer and a double vagina (Fig. 4.7D). Bicornuate uterus (26%); There is varying degrees fusion failure of the muscle walls of the two ducts. Uterus bicornis bicollis: There are two uterine cavite with double cervix with or without vaginal septum Uterus bicornis unicollis: There are two uterine caviti with one cervix. The horns may be equal or one horm in U on Clini As pr the ab MULE A MO Mülleria atrophic D Uterus longitud Serosal indentar Bicomuatemay be rudimentary and may have no communication with the developed horn (Figs. 4.7E and 4.8F). manifestation (asymptomatic patient). Otherwise she may Septate uterus (35%): The two Müllerian ducts are fused together but there is persistence of septum in between the two either partially (subseptate) or completely (Figs. 4.7G and 4.8A to C). Unicornuate uterus (10%): Failure of development of one Müllerian duct (Figs. 4.7C and 4.8E). Clinical Features As previously mentioned, depending on the extent of the abnormality, the condition may not have any clinical MULLERIAN AGENESIS CERVICAL AGENESIS A Müllerian agenesis Cervical agenesis Müllerian agenesis with atrophic uterine remnant Distal cervical agenesis B Unicornuate with distal uterine remnant with functional endometrium. Unicornuate with associated atrophic uterine remnant UTERUS DIDELPHYS Uterus didephys and longitudinal septum D Serosal indentation >1 cm Uterus didelphys and +/-longitudinal vaginal septum of variable length BICORNUATE UTERUS Chapter 4: Congenital Malformation of Female Genital Organ 41 have many symptoms. The symptoms ares Amenorrhea due to uterine hypo plasia/aplasia or obstructive anomaly. Gynecological Infertility and dyspareunia are often related in association with vaginal septum. Dysmenorrhea, cyclic or noncyclic pain in hicornuate uterus or due to cryptomenorrhea (pent up menstrual blood in rudimentary hornMenstrual disorders: Hematometra is seen in cases with obstructive anomalies. Menorrhagia is due to increased surface area in bicor-nuate uterus and abnormal uterine bleeding (AUB) may be present. Primary amenorrhea women with uterine agenesis Primary amenorrhea with pelvic pain, when uterus Is present. Pain is often due to complete obstruction of the genital tract (at the levels of vagina, cervix, or lower uterus) Dysmenorrhea with pelvic pain (cyclic or noncyclic): Unilateral obstructive anomaly. Obstructive anomalies may cause hematometra. This may lead to retrograde menstruation and endometriosis. Longitudinal vaginal septum may be associated with septate, didelphys or bicornuate uterus. Obstetrical Midtrimester miscarriage which may be recurrent. Poor obstetric outcomes: recurrent pregnancy loss (RPL). Risk of RPL ranges from 6% to 38%. Rudimentary horn pregnancy may occur due transperitoneal migration of sperm or ovum from the opposite side. Cornual pregnancy (ectopic) inevitably ends in rupture around 16th week. Uterine horn pres nancy may cause surgical emergencies due to ruptu uterus (89%) and associated maternal morbidity and mortality.Figs. 4.8A to F: (A) Hysteroscopic; (B) Ultrasonographic; (C to F) Hysterographic diagnosis of congenital malformations of uterus. Cervical incompetence. Increased incidence of malpresentation-transverse lie in arcuate or subseptate, breech presentation in bicornuate, unicornuate or complete