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Mullerian ageness is commonly known as Mayer-Rokitansky-Kuster-Hauser syndrome. It occurs in 1 among 4000-5,000 females. Mullerian ducts are the anlage of primordial tissue of female reproductive tract, which is rare cause of agenesis or hypogenesis female genital tract. This case presented here is one among the rarest in this tertiary care hospital. Case History: 11-year-old female presented with complaints of fever and vague abdominal pain since 15 days. On examination, the presence of imperforate hymen was noted; USG revealed hematometra and hematocolpus. Right ovary showed a simple cyst. Left ovary was poorly indiscernible; right kidney was unremarkable while left kidney was not visualized. Vaginal atresia was noted while doing hymenoplasty; hysterectomy with salpingoophorectomy was done for hematometra with hematocolpus and hematosalpinx and the specimen was sent for HPE examination.. Conclusion: The case presented here is unique example of mullerian agenesis which presented with unusual clinical features of fever and vague abdominal pain. It is also unique in that it is very rare case which occurred in a female with early menarche and cryptomenorrhea that was incidentally diagnosed on pelvic USG. Lastly, its incidence is noted to be rare in this tertiary care teaching hospital that it is the first case of its kind reported in the last five years. Keywords: Mullerian agenesis, Haematometra, Haematocolpos imperforate hymen, MayerRokitanskyKuster Hauser syndrome, Hematosalpinx unicornuate uterus vaginal atresia.

Access to the uterine cavity can sometimes be a challenge or even impossible because of various causes such as prior surgery. We report our experience with a novel approach to this problem in 8 cases using ultrasound-guided transmural injection of dye; afterward, the cavity was successfully found in all cases by ultrasound-guided hysteroscopy following the colored epithelium.

Primary amenorrhea secondary to childhood pelvic trauma is very rare. We report two cases of adolescent girls with cryptomenorrhoea. Case 1 presented at 14 years of age with primary amenorrhea and cyclical lower abdominal pain for 1 year. She was run over by a tractor when she was three years old. Ultrasonography pelvis showed hematometra. Examination under anesthesia with diagnostic laparoscopy proceed laparotomy revealed uterine avulsion at level of isthmus. The second case presented at 13 years of age with urinary retention. Catheterization was not possible due to malpositioning of urethra so suprapubic catheterization was performed by urologists. Ultrasonography pelvis revealed hematocolpos. Detailed history revealed run over injury by a vehicle when she was two years old. Examination under anesthesia showed obstruction due to adhesions high up in vagina which were divided.

Study Objective The objective of this study was to assess short-term benefits and risks of utero-vaginal anastomosis done for cases of cryptomenorrhea due to cervical atresia with vaginal aplasia. Design Prospective study. Setting Surgical procedures were done between December 2013 and September 2015 at the department of Obstetrics and Gynecology, Cairo University Hospital. Participants Five patients who had cryptomenorrhea due to cervical atresia associated with vaginal aplasia were included. Interventions Utero-vaginal anastomoses were performed in 2 stages; a stage of McIndoe vaginoplasty and a stage of excision of the atretic cervical tissue and anastomosing the uterus to the neovagina. Follow-up was done by gynecological and ultrasound examination in a duration ranged from 12 to 36 months. Main Outcome Measures Occurrence of regular menstrual flow and relief of the severe cyclic pain. Results All patients had relief of the severe cyclic pain. Four patients had regular menstrual flow. One patient developed occlusion of the track after 1 year and needed dilatation once. Three patients developed low vaginal stenosis without occlusion of the track. One patient had rectal injury repaired without causing postoperative morbidity. Conclusion Uterovaginal anastomosis is a promising conservative management option for cervical atresia with vaginal aplasia, which has benefits but is not free of risks. Long-term follow-up is still needed to judge its feasibility. We recommend performing McIndoe vaginoplasty as a starting stage before the anastomosis preferably in a separate setting.

Cosmetic laser use has many pros and cons. The worldwide use of laser for body hair removal has led to many medical complications. Unsupervised use of the laser for hair removal in vulva may result in many problems and can merely damage the vulva, although rarely, affecting the body image. This rare and novel case report is a 21 year old virgin who presented with acute urinary retention and cryptomenorrhea due to complete synlabia secondary to unsupervised vulval laser hair removal. The urinary retention was relieved by suprapubic catheterization initially. During examination under anesthesia, the fused labia were separated by a surgical incision with drainage of hematocolpos and then, a Foley's urethral catheter was inserted. She had an uneventful recovery. We report this case to emphasize on the supervised use of laser by trained and qualified personnel for hair removal in vulva to minimize its complications.

Crytomenorrhea arises usually due to the imperforate hymen, sometimes due to vaginal septum, rarely due to cervical agenesis. Here, we present a case of primary amenorrhea resulting from cervical agenesis in a 38-year-old woman. She presented with primary amenorrhea and cyclic lower abdominal pain. She had undergone some surgery 5 years back, details of which were not available. On examination secondary sexual characters were normal, per abdomen there was an 18 weeks size firm abdominopelvic mass. Local vaginal examination showed a blind vaginal pouch. A clinical diagnosis of hematometra due to transverse vaginal septum was made. However, magnetic resonance imaging pelvis suggested hematometra with cervical stenosis. The patient was taken up for examination under anaesthesia (EUA) and exploratory laparotomy. On opening the abdomen uterus found to be enlarged with dense adhesions all around and signs of endometriosis. Extensive adhesiolysis revealed bilateral chocolate cysts of ovaries with hematosalpinges and peritubal adhesions. Hysterotomy and drainage of tarry contents were followed by an exploration of the uterine cavity. The lower pole ended blindly with no evidence of any cervix. Peroperative diagnosis of cervical agenesis leading to hematometra and endometriosis was made. A subtotal hysterectomy with bilateral salpingo-oophorectomy was done. Histopathological examination confirmed ovarian endometriosis and adenomyosis of uterus. Though reconstructive surgery for cervical dysgenesis has been successful in some cases, hysterectomy is generally recommended for cervical agenesis.

The current case series was done to evaluate the feasibility of transfundal hysteroscopy (TFH) in helping the development of new cervical canal in cases with cervical aplasia. Five cases with obstructive cervicovaginal agenesis with hematometra were included in this report. Laparoscopic-guided TFH was done in conjunction with endoscopic canalization to all cases. Additional retropubic balloon vaginoplasty (BV) was needed in three cases with associated vaginal aplasia. The hysteroscope was passed through the uterine fundus. After complete washing, the endometrial lining was inspected, and a properly located intrauterine catheter coming out from the vagina or vaginal dimple was used to drain the uterine cavity and maintain the cervical tract. The procedure was done successfully in all cases with adequate drainage of hematometria. Additional time needed for TFH was between 4 to 15 min. Second-look hysteroscopy revealed adequate canalization in all cases. The five cases had regular menstrual cycles up to the sixth postoperative month. In conclusion, TFH is a safe and feasible procedure as a harmonizing technique during endoscopic canalization of cervical atresia with or without BV in cases of obstructive Mullerian anomalies. The procedure accelerates the drainage of uterine contents and localizes the correct site of draining catheter.

BACKGROUND: Rare case of the patient having multiple developmental variations of mullerian duct: unicornuate uterus with non-communicating rudimentary horn, cervical agenesis and transverse vaginal septum with cryptomenorrhoea. AIMS: Restoration of menstrual function and restoration of reproductive function. METHODS AND MATERIAL: Patient was examined and then surgical repair of reproductiove organs was done. RESULTS AND CONCLUSIONS: Patients responded well in post-operative period. She started having normal menses without any complaints. Patients with cryptomenorrhea must be thoroughly evaluated and appropriate surgical correction of underlying congenital anamoly provides good results.

Gynatresia is the narrowing or complete occlusion of the vagina, which may be congenital or acquired. In Nigeria, acquired gynatresia is more common, usually resulting from the use of herbal pessaries. Its mere occurrence is indeed one social calamity to many. We present a case of a 37-year-old P0+1 with acquired gynatresia following insertion of herbal pessaries at a trado medical center. This was in an attempt to treat cryptomenorrhea which had developed following an abortion complicated by uterine perforation at a private hospital 20 years prior. Poor quality of medical care may push women to seek unsafe alternatives, resulting in even more harm.

Objective To discuss common pitfalls in diagnosis and management of distal vaginal agenesis, and summarize 10 years of experience among 11 patients using an interposition full-thickness graft and fibrin glue. Study design Eleven patients with distal vaginal agenesis were evaluated and managed with an interposition full-thickness graft to bridge the gap between the upper vagina and the introitus. Associated renal abnormalities and complications including infection, total lack of skin graft take, stress urinary incontinence, partial graft loss, vaginal stricture and graft uptake were all investigated. Results The mean age of the patients was 12.91 (standard deviation 1.22) years. All patients had primary amenorrhoea, cryptomenorrhea, and cyclical or constant pelvic pain. None of the patients had associated urological abnormalities, and there were no cases of infection, total lack of skin graft take, stress urinary incontinence, partial graft loss or vaginal stricture. Graft uptake was 100% in 10 of the 11 patients. Four patients have subsequently married and report a satisfactory sex life. Conclusion Accurate diagnosis of distal vaginal agenesis and careful pre-operative set-up, including evaluation of associated anomalies, bowel preparation, available vaginal stents and a multidisciplinary approach for the potential need for grafts, may be key to success.

Mullerian malformations result from defective fusion of the Mullerian ducts during development of the female reproductive system and have an incidence of 2-3%. The American Fertility Society classification of Mullerian anomalies is the most commonly utilized standardized classification. The least common form of these malformations is Herlyn-Werner-Wunderlich syndrome characterized by obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). This syndrome has been described with case reports since 1922. Early diagnosis and surgery that include drainage of fluid and resection of the vaginal septum is necessary for OHVIRA to prevent late complications (i.e. pyocolpos, chronic cryptomenorrhea). Here in we report a case of Herlyn-Werner-Wunderlich syndrome that hymen has been preserved during the operation of non-fenestrated transverse vaginal septum resection with hysteroscopy.

Hematometra following caesarean section is rare. We present here a case of 23-year-old woman who had undergone caesarean section for placenta previa. She presented with cryptomenorrhoea and hematometra after 5 months of caesarean delivery owing to adhesions in the vagina. The hematometra was drained vaginally.

Background Cryptomenorrhea at menarche is commonly due to mullerian duct anomalies. Uterine avulsion due to blunt pelvic trauma is a very rare cause of cryptomenorrhea. But so far no case of uterine avulsion has been reported due to a childhood trauma without pelvic fracture. Case A 15-year-old young girl was evaluated for delayed menarche and the diagnosis of hematometra with obstruction at the level of internal cervical os was made on radiological examination. Detailed history revealed significant childhood blunt pelvic trauma when at the age of 3 years she was run over by a tractor. But the presence or absence of pelvic fracture was never documented as no radiographs were obtained. On abdominopelvic exploration cervix was well formed. Uterine body was found to be separated from the supravaginal cervix and there was a peritoneal window in between. During surgery hematometra was drained and the continuity of the uterine outflow tract was restored. Post surgery patient started having normal periods. Summary and Conclusions Though very rare, uterine avulsion during pelvic trauma may cause cryptomenorrhea. Therefore such history should be sought in all cases of obstruction of the cervical canal because cryptomenorrhea has a much better prognosis than cervical congenital dysgenesis.

We report a case of hematometra and bilateral hematosalphinx in a 15-year-old mentally handicapped adolescent girl, complicated by vaginal agenesis over cervical atresia in the presence of an ovarian adenoma. The case was managed by abdominal hysterectomy and bilateral salphingectomy of the hematosalphinx, which had formed a tubo-ovarian mass. One ovary was preserved. This case also considers the management of the mentally handicapped patient unable to comprehend pain arising from cryptomenorrhea.

Objective To describe the surgical reconstructive options in cases of cryptomenorrhea because of an obstructed functioning uterus with upper and lower vaginal agenesis. Design Assessment of operative, anatomic, and functional results after operative reconstruction over a 1-year follow-up period. Setting University hospital and referral center for pediatric and adolescent gynecology. Patient(s) Three adolescent girls, aged 15 to 18 years with primary amenorrhea, cyclic abdominal pain, hematometra, and complete vaginal agenesis. Intervention(s) A combinative vaginal and laparoscopic approach to reconstruct a neovagina by human amniotic membranes and establish the uterovaginal continuity. Main Outcome Measure(s) Anatomic success was defined by a vaginal length ≥8 cm, and a width allowing the introduction of two fingers. Functional success was evaluated according to the restoration of menstrual cycle resolving of pain and patients'sexual satisfaction. Result(s) Neovaginal length was 9 to 12 cm and adequately wide. In all patients the uterovaginal continuity could successfully be restored. Satisfactory anatomic and functional results could be achieved, with no operative morbidity. Regular menstruation resumed with normal ultrasound findings during follow-up. Conclusion(s) In rare cases of an obstructed functioning uterus by complete vaginal agenesis, a combinative laparoscopic and vaginal surgical approach effectively restores anatomy and function, by an overall minimal operative morbidity. Human amnion provides excellent results in neovaginal reconstruction.

Although dysmenorrhea, pelvic mass or pain, genital irritation, and amenorrhea are relatively common complaints, the astute clinician needs a broad differential diagnosis to avoid missing uncommon underlying etiologies such as Mullerian anomalies and cryptomenorrhea, ovarian teratomas and torsion, labial hypertrophy, vaginal foreign bodies, dermopathies, genital ulcers, imperforate hymen, and the absent vagina. This article discusses and illustrates uncommon pediatric and adolescent gynecologic conditions that present with these common complaints.

BACKGROUND: Uterocutaneous fistula is a rare condition that may be difficult to manage. CASE: A young woman who underwent surgical intervention for cryptomenorrhea 3 years ago developed menstrual discharge from the abdominal scar. A fistulous tract leading from the infraumbilical midline scar to the uterus was demonstrated on contrast study. Genital examination revealed vaginal agenesis. A vaginoplasty was performed as the first stage. This was followed one year later by excision of the fistulous tract and establishment of cervicovaginal communication. The patient is now menstruating from the vaginal passage. CONCLUSION: This case shows that a stepwise, well-planned, and well-executed procedure can lead to a satisfactory repair of an uterocutaneous fistula.

The aim of this study was to demonstrate the clinical results of postoperative evaluation for a consecutive series of cases of imperforate hymen presenting at a tertiary medical center during an over 14-year period. We retrospectively reviewed the clinical records of 15 patients with imperforate hymen that had undergone hymenectomy between 1987 and 1998. After completing a questionnaire via a telephone interview regarding sexuality, fertility, menstrual problems, micturition and defecation after surgical correction, those patients were persuaded to come back to the hospital for further gynecological surveys during 2002. The mean postoperative follow-up was 8.5 years (range, 4–14 years). The mean age at diagnosis was 13.2 years (range, 11–16). The most common clinical symptom was cryptomenorrhea (15), followed by pelvic pain (11), palpable abdominal mass (9), urinary retention and other voiding problems (8) and problems of defecation (4). None admitted intercourse attempts before. Two patients also had uterine anomalies, but none had urinary tract or bowel anomalies. During the follow-up period, we found that the majority of patients had irregular menstrual cycles and were worried about their future fertility. Six patients suffered from dysmenorrhea; of 11 patients who began having intercourse two later delivered babies, and none complained of sexual dysfunction. After hymenectomy, the women with imperforate hymen were markedly relieved of cryptomenorrhea, and problems of micturition and defecation also greatly improved. Although complaints of irregular menstruation and dysmenorrhea gradually evolved as the center of attention during follow-up, most patients fared well in terms of fertility and sexual function.

Cervical agenesis is an extremely rare congenital Mullerian duct anomaly of female genital tract. It is a complex surgical problem and management is still controversial. Failure to manage these patients correctly may be associated with long-term psychological, sexual, reproductive health damage and suffering. We have managed two cases of cervical agenesis with cryptomenorrhea. DOI: http://dx.doi.org/10.4038/sljog.v36i1.6969 Sri Lanka Journal of Obstetrics and Gynaecology 2014; 36: 17-18

Background: Adnexal torsion is a well-recognized cause of acute pelvic pain. Isolated tubal torsion with ovarian sparing has certainly been documented, but is uncommon. Although risk factors for the latter include a menstrual period, menarche in particular is not known to predispose a patient to this event. Severe unilateral pelvic pain with first menses is more likely to herald a congenital mullerian anomaly and cryptomenorrhea, particularly when accompanied by a pelvic mass. We present a case of tubal torsion where a coincidental, yet misleading temporal relation to menarche led to a delay in laparoscopy and ultimate diagnosis.Case: KG, an eleven-year-old female, experienced severe right-sided dysmenorrhea with her first and second menses in August and September 1999 respectively. Between episodes, pain, although still present, was more tolerable and the patient never required hospitalization. Ultrasound revealed a lobulated inhomogeneous mass posterior to the uterus and extending from one normal ovary to the other (Figures). MRI further described the mass as pseudoencapsulated with inhomogeneous areas of high attenuation on T1 and T2 images (Figures). Findings were consistent with an endometrioma, but admittedly could have represented a hemorrhagic cystic mass. No definite mullerian anomaly was seen to explain advanced endometriosis, but two focal areas within the endometrial canal raised the possibility of a uterine septum. Examination of the patient (one week after presentation) was not very helpful although she was pubertal, did have a hymenal septum and was mildly tender on bimanual examination in the Pouch of Douglas. The patient had been started on continuous oral contraceptives while undergoing investigations. Pain only recurred during an episode of break-through bleeding. Ultimately she came to laparoscopy and hysteroscopy where chronic right tubal torsion and necrosis was identified with an inflammatory/hemorrhagic reaction in the pelvis (Photos). There were no identifiable fimbria of the right tube which was densely adherent distally to perirectal fat (Photo). No obvious precipitant was found. Laparoscopic lysis of adhesions and right distal salpingectomy was performed (Photo). Her uterine cavity was in fact normal (Photo)Conclusion: Whether or not this patient's right tube was originally normal will never be known. Congenital abnormalities of fallopian tubes do occur and can predispose to torsion. Nonetheless, adnexal torsion must always be kept in mind whenever a woman presents with unilateral pelvic pain. Early diagnosis is paramount in children and women of reproductive age in order to improve the likelihood of adnexal salvage and future fertility. A "gold-standard" radiological investigative tool continues to elude us. Laparoscopy, albeit more invasive, remains an invaluable procedure in this context with relatively low morbidity as compared to the consequences of delayed diagnosis.

A case is reported of a 16-year-old adolescent with cryptomenorrhea. The patient had a congenital anomaly of a bicornuate, nonfused, separate rudimentary blind uterine horns with functioning endometria, and complete cervical-vaginal agenesis. Ultrasound, laparoscopy, and minilaparotomy for unilateral salpingectomy failed to accurately identify the exact classification of the anomaly. Magnetic resonance imaging, however, accurately correlated with operative findings of exploratory laparotomy and McIndoe procedure.

Female circumcision is not unique to one country. It is performed in Egypt (until recently) Sudan Kenya Abyssinia Indonesia Malaya Eastern Mexico Peru Western Brazil among the Urabunna tribes of Australia and among the Skopizy people in Russia. In Sudan the custom is so deeply ingrained that propaganda and legislation by the Sudanese Ministry of Health did not deter people from practicing it. The type of circumcision practiced in Sudan is infibulation which involves excising the labia majora and obliterating the introitus. Immediate complications of this operation include shock hemorrhage injury to adjacent structures infection retention of urine and anemia. Late gynecological complications include infertility implantation dermoids menorrhagia dysmenorrhea cryptomenorrhea keloid scar and in rare cases vaginal calculi. Marital and psychological complications are difficult to assess. General surgical and medical procedures are used in managing the complications. Prophylaxis is evidently the best treatment; however the only means of avoiding complications altogether is abolishing the practice of female circumcision.

through the gamut of emotional problems that are created by ignorance or physical embarrassment, or by the girl's special environment. Finally the nurse becomes a court of appeal in questions concerning true anatomical or pathological irregularities. She can, by virtue of her special training, and by her personal example, prove that menstruation is a harmless physiologic function and need seldom be accompanied by psychologic disturbance, physical pain, or incapacity to work. An understanding of the normal menstrual cycle is important as a preface to a consideration of the irregularities which occur when it is beginning. The estrous or ovarian cycle begins with the rupture of a graafian follicle through the covering of the ovary, freeing the ovum, which then enters the Fallopian tube (oviduct) to be met by a sperm from the male and to be fertilized. In the meantime preparations are going on in the ovary and in the uterus for the expected pregnancy. The follicle from which the egg has ruptured now fills in with yellow cells and becomes the corpus luteum, an important organ of internal secretion during the first three months of pregnancy. The walls of the waiting uterus become deep and soft and richly supplied with blood vessels to nourish the awaited inseminated ovum. The fertilized egg itself undergoes certain changes en route through the oviduct which will enable it to attach itself to the walls of the uterus when it arrives. This is the biological cycle that nature has pla n d. However, if, as is most often the case, the ovum is not met by a sperm in the oviduct, these preparations have been made in vain. The follicle heals over, the ovary sets about readying another egg for the next cycle, the unfertilized ovum descends through the uterus and on out, and the uterus, defeated in its purpose, sheds its endometrium and begins to weep its biological tears of blood. But its frustration is not for long. The allseeing pituitary at once begins the preparations for another, and another, ovum until pregnancy can take place and the true biological purpose be fulfilled. This, the tale of a disappointed uterus, is the story which nurses will be called upon time and again to tell. It is, perhaps, the biological frustration underlying the entire phenomenon which lays it open to so many trials and tribulations. Often the sincere telling will be helpful enough to the adolescent, but, unfortunately, all the problems attending menstruation at its onset are not so easy. To give direction to our necessarily brief discussion of these graver issues, we have chosen the following headings: (1) psychic and emotional disturbances, (2) precocious cyclic bleeding, (3) delayed menarche and primary amenorrhea, (4) cryptomenorrhea, (5) oligomenorrhea, (6) menorrhagia, and metrorrhagia, (7) dysmenorrhea, and (8) intermenstrual pains and tenderness.