OHVIRA-syndrome (Obstructed hemivagina with ipsilateral renal anomaly) as differential diagnosis of acute lower abdominal pain.

INTRODUCTION: OHVIRA-syndrome (obstructed hemivagina, ipsilateral renal agenesis/anomaly) is a rare Mullerian duct anomaly that can lead to complications in pubescent children. CASE REPORT: We report a case of a 13-year-old patient with acute right-sided lower quadrant abdominal pain who was referred for exclusion of appendicitis. As a result of the examination (transvaginal ultrasound scan and gynecological examination), a female genital tract anomaly was suspected in the form of obstructed hemivagina with hematocolpos and hematometra. The MRI scan showed hematocolpos and hematometra on the right side, uterus didelphys accompanied by right-sided renal agenesis, consistent with OHVIRA-syndrome. Excision of the vaginal septum was performed and the accumulated old menstrual blood, as represented by hematocolpos and hematometra, was evacuated. Postoperative recovery was uneventful. CONCLUSION: The early surgical management of this rare Mullerian duct anomaly is important in order to prevent longterm complications. This malformation should be considered in the differential diagnosis of acute lower abdominal pain in pubescent girls. KEY WORDS: Abdominal Pain, Genital Anomaly, Obstructed Hemivagina, Renal Anomaly.

Intrauterine adhesions combined with Robert's uterus: a case report and literature review.

PURPOSE: To summarize the clinical characteristics and surgical option of Robert's uterus. METHODS: We reported a rare case of Robert's uterus with severe uterine adhesion with successive laparoscopic and hysteroscopic surgery. To our knowledge, such a case has not been reported previously. We also performed a systematic literature review from the PubMed, Embase, and Cochrane databases. RESULTS: Our patient with Robert's uterus with severe uterine adhesions was successfully treated with hysteroscopic septal resection and hysteroscopic adhesiolysis, and the intractable dysmenorrhea disappeared after the hysteroscopic septal resection. In our study, we analyzed the selected 22 reported cases, 10/22 cases (45.5%) were diagnosed before age 20; 20/22 cases (90.91%) experienced dysmenorrhea, 19/22 cases (86.36%) were with hematometra. 5/22 cases (22.73%) underwent re-operation or a third surgery before diagnosis and management. CONCLUSION: Robert's uterus, a rare congenital abnormality of Mullerian duct development, consists of an oblique septum and non-communicating asymmetrical uterine hemi-cavity. The main symptoms are the presence of hematometra and severe dysmenorrhea. Septal resection is the main surgical procedure; however, the rarity and difficulty obtaining a pre-operative diagnosis lead to a high rate of misdiagnosis and second surgery.

[Urinary Retention Due to Hematocolpos].

Hematocolpos is an obstruction of the menstrual flow due to an anomaly of the genital tract, with imperforate hymen being the most common one. It is an uncommon condition, with a prevalence of 1:1000 - 1:16 000. It is usually an asymptomatic condition until the onset of menarche, when there is accumulation of blood in the vagina (hematocolpos) or in the uterus (hematometra). A rare symptom of hematocolpos could be urinary retention. Our case report is about a 12-year-old girl, with no menarche and Tanner stage M4/P5, who was admitted to our emergency department with abdominal pain and urinary retention. A brief review of this rare condition is presented, in order to remind clinicians about its existence, so that it can be diagnosed and treated as quickly as possible.

O hematocolpos consiste na obstrução do fluxo menstrual por uma anomalia do trato genital, sendo a mais frequente o hímen imperfurado, uma patologia pouco comum (prevalência de 1:1000 ­ 1:16 000). Geralmente é uma condição assintomática até a menarca, quando ocorre acumulação de sangue na vagina (hematocolpos) ou no útero (hematometra). Um sintoma raro de hematocolpos é a retenção urinária. Apresentamos o caso de uma adolescente de 12 anos, sem menarca e com estadio sexual de Tanner M4/P5, que recorreu ao nosso serviço de urgência com dor abdominal e retenção urinária. Com o intuito de sensibilizar para esta patologia rara, fizemos uma breve revisão teórica orientada para o rápido diagnóstico e tratamento da mesma.

Uterine didelphys with one cervix obscured by blind hemivagina: a lesson in rarity.

A 14-year-old girl presented with increasing cyclical pain, scanty menses, pelvic mass and absence of the left kidney. With both radiological and clinical examinations (examination under anaesthesia), diagnosis of bicornuate uterus with single cervix could be made while on laparotomy, and it turned out to be uterine didelphys, with one cervix obscured by blind hemivagina with haematometra and haematocolpos in the left horn, for which hemihysterectomy was done. Post procedure the patient was relieved of cyclical pain and is menstruating properly.

Acute abdominal pain caused by hematometra in an adolescent female: a case report.

BACKGROUND: Hematometra is a pathologic collection of blood in the uterus. It is a rare condition that is most commonly associated with congenital anomalies or prior surgical procedures causing an obstruction of the genitourinary outflow tract. We present an unusual case of hematometra in a healthy and active adolescent female with no prior risk factors. This is a rare and important case report due to the complexity of diagnosis when a young female presents with an acute abdomen. In addition, for a patient who presents with no prior risk factors for hematometra, such as in our patient, the diagnosis and workup may become overly complicated, adding strain to patient care and health care cost. To the best of our knowledge and based on an extensive literature search, there has not been a reported case of hematometra in an adolescent female without any aforementioned risk factors. CASE PRESENTATION: Our patient is a healthy 18 year-old white woman with no significant prior medical or surgical history. Her only medication was depot medroxyprogesterone acetate use for contraception. She presented to a local emergency department with acute abdominal pain, accompanied by emesis and nausea. Workup with ultrasonography showed uterine distention most likely caused by hematometra, although no obvious cause was noted. She was treated with dilation and curettage; she was also advised to discontinue depot medroxyprogesterone acetate use. She was symptom free without recurrence of hematometra at 6-month follow-up. CONCLUSIONS: Due to the high prevalence of abdominal pain, this case report has a wide breadth of implications for health care providers ranging from general family practitioners to emergency room physicians and obstetricians/gynecologists. This case report provides potential future advancement in management and differential diagnosis in adolescent females presenting with acute abdominal pain. In addition, the use of depot medroxyprogesterone acetate contributing to or causing hematometra cannot be ruled out in our patient and warrants further investigation.

Unique Presentation of Hematometrocolpos Mimicking Cauda Equina Syndrome: Severe Back Pain and Urinary Incontinence in an Adolescent Girl.

BACKGROUND: Imperforate hymen with hematometrocolpos in adolescent females is a rare pediatric condition. Classical presentation includes abdominal pain or a pelvic mass in female patients with primary amenorrhea. Atypical complaints and reluctance among emergency physicians to perform genital examination in the emergency department or the pediatric emergency department (PED) may delay correct diagnosis. CASE REPORT: We report a unique, cauda equina syndrome-like presentation of hematometrocolpos secondary to imperforate hymen in a 13-year old, previously healthy girl with primary amenorrhea. In the PED, the unusual clinical presentation of severe back pain and urinary incontinence initially mimicked cauda equina syndrome and led to delayed correct diagnosis. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: The novelty of this case is a cauda equina-like presentation of imperforate hymen secondary to hematocolpos. This report illustrates the highly variable clinical presentation of this rare gynecological pediatric entity. It underlines the importance of considering this rare condition in the differential diagnosis of severe upper or lower back pain alongside voiding abnormalities including urinary retention and incontinence in adolescent females with primary amenorrhea. Above all, the importance of performing a thorough history and genital examination in this subgroup early in the investigation process in the PED emerges from this case. Essentially, excellent clinical judgment and genital examination by the emergency physician may minimize unnecessary radiological investigations and ultimately, accelerate correct diagnosis and expedite appropriate surgical treatment. However, not only pediatric and adult emergency physicians, but also pediatricians and general practitioners should be aware of this entity and its diverse clinical presentation.

Unique Case of Imperforate Hymen.

BACKGROUND: Imperforate hymen typically presents in adolescence with pain, hematocolpometra and primary amenorrhea. This case documents a previously unreported etiology for an atypical presentation with a history of recent menstruation. CASE: A female adolescent presented with symptoms of urinary retention and leg pain. She reported a history of irregular, painful menses. Clinical examination revealed a pelvic mass and imperforate hymen. Sonography was consistent with hematocolpometra. Before a planned hymenectomy, the patient began to pass dark blood through a fistulous opening in her vulva. Hymenectomy resulted in complete resolution of the pain and hematocolpometra. SUMMARY AND CONCLUSION: Identification of the fistulous tract explained the patient's history of menstrual bleeding despite an imperforate hymen. Spontaneous rupture of hematocolpometra through a fistulous tract to the vulva is a previously unreported atypical presentation of imperforate hymen in a "menstruating" adolescent with pain and a pelvic mass.

Cervicovaginal atresia with hematometra: restoring menstrual and sexual function by utero-coloneovaginoplasty.

BACKGROUND: Cervicovaginal atresia is a rare Mullerian anomaly. The management of cervicovaginal atresia has evolved from historical recommendations of hysterectomy to various reconstructive procedures more recently. The latter carries a risk of significant morbidity and unknown fertility. We present our experience in the management of this complex anomaly. METHODS: Twenty patients with cervicovaginal atresia were operated in our hospital from January 2004 through December 2013. The details of their anatomical variations and functional outcomes were analyzed. RESULTS: Eighteen out of twenty patients had cervical agenesis. Two patients had cervical hypoplasia. All patients underwent utero-coloneovaginoplasty. Post operatively, all patients have regular menstrual cycles. One patient is married, sexually active and has satisfactory coital function. One patient had a bowel anastomotic leak that required a diversion ileostomy. Two patients developed mild stenosis. One patient has mild neovaginal mucosal prolapse. No patient has developed pyometra. CONCLUSION: Patients with cervicovaginal atresia need to be counselled about the various reconstructive options available and the potential risks. Social and economic factor play a significant role in determining the plan of management. For patients from conservative societies, utero-coloneovaginoplasty provides a safe conduit for the passage of menstrual flow and coitus, at the cost of permanent infertility.

Hematoureter due to endometriosis.

OBJECTIVE: To report the laparoscopic management of a rare case of hematoureter due to endometriosis in a young woman with multiple genitourinary anomalies. DESIGN: Video demonstration of a surgical technique and review of genitourinary endometriosis. SETTING: Hospital. PATIENT(S): A 17-year-old nulliparous woman with multiple genitourinary anomalies presented with pelvic pain and unilateral retroperitoneal mass. The patient had uterine didelphys, a history of left nephrectomy, and partial ureter resection as an infant. She had a partial resection of a left transverse vaginal septum due to hematocolpos at age 12. A preoperative magnetic resonance imaging (MRI) scan revealed a left retroperitoneal mass with extension to the paravesical region, reaccumulation of the hematocolpos behind the partially resected left transverse vaginal septum, and a dilated left uterine horn with hematometra. INTERVENTION(S): Laparoscopic management of hematoureter due to intrinsic endometriosis. MAIN OUTCOME MEASURE(S): Intraoperative findings showed uterus didelphys with dilated left horn, normal right horn, and normal right and left fallopian tubes and ovaries. The left transverse vaginal septum was resected vaginally, and the hematocolpos and hematometra drained. The left uterine horn and cervix were laparoscopically resected. The left-side serpiginous retroperitoneal mass was dissected from the pelvic sidewall, ligated, and transected, with spillage of thick, brown liquid. The pathology of the mass wall was smooth muscle and transitional epithelium consistent with ureter, in addition to hemorrhage and glandular structures consistent with endometriosis. Endometriosis was also present in the serosa of the left uterine horn. Thus, the left retroperitoneal mass was the left ureter remnant, which acquired endometriosis and collected menstrual debris, resulting in hematoureter. CONCLUSION(S): Two major pathologic types of ureteral endometriosis have been described: intrinsic, as occurred in this patient, and extrinsic. Women with müllerian anomalies, vaginal obstruction, or imperforate hymen are at higher risk of endometriosis. Prior urogenital surgery can further complicate and distort the anatomy. Thus, a preoperative understanding of the patient's urogenital anomalies is important to consider the differential diagnoses and anticipate surgical needs.

Hysterectomy: treatment for secondary infertility.

Infertility is a distressing condition but extenuating circumstances sometimes make the choice of treatment seem paradoxical. Here is discussed a challenging case of a 30-year-old woman with no living child and secondary infertility who presented with a large abdominal mass and severe abdominal pain, sequelae of previous obstructed labour. There was complex management dilemma. She was young, had no living child, had undergone vesicovaginal fistula (VVF) repair and vaginoplasty yet was wanting fertility; however she was distressed with the abdominal pain and desired a complete cure. Both she and her spouse were counselled about the high possibility of failure if repeat attempt at vaginoplasty was made, and possible damage to the VVF repair. Finally, decision of exploratory laparotomy with total abdominal hysterectomy was taken after counselling the couple about adoption as an option for childbearing.

Himen imperforado en paciente de 17 años: presentación de un caso / Imperforate hymen in patient with 17 years old: a case

El himen imperforado es una patología relativamente rara que consiste en la ausencia del orificio central del himen por el que drenan las secreciones cervicales y uterinas. Son anomalías congénitas del desarrollo de los genitales externos y muy poco frecuentes en las niñas. Se trata una adolescente de 17 años de edad, de raza negra que acude a consulta refiriendo dolor en bajo vientre y amenorrea primaria, atendida en la consulta de ginecología de urgencia del Hospital Sefwi Asafo. Cuba–Ghana; donde fue intervenida quirúrgicamente de forma electiva, con evolución satisfactoria (AU)

Imperforate hymen is a relatively rare disease that is the absence of the central orifice of the hymen by draining cervical and uterine secretions. These are congenital and developmental anomalies of the external genitalia and very rare in girls. A 17-year-old, black girl, who comes to the clinical office complaining of pain in lower abdomen and primary amenorrhea, was attended at the gynecology emergency consultation in Hospital Sefwi Asafo. Cuba-Ghana where she was underwent with surgery, with satisfactory outcome

Imperforate hymen presenting as chronic low back pain.

Imperforate hymen in an adolescent usually presents with cyclic abdominal pain or with pelvic mass associated with primary amenorrhea. We present a 13-year-old girl with chronic lower back pain of 6 months' duration as the only complaint. On physical examination, multiple trigger points were detected in the quadratus lumborum and gluteus medius muscles bilaterally. Abdominal ultrasound revealed hematometrocolpos secondary to an imperforate hymen. Hymenectomy was performed, with complete resolution of the back pain. Myofascial pain syndrome with a viscerosomatic reflex is a possible explanation for the clinical presentation of our patient.

Imperforate hymen with elevated serum CA 125 and CA 19-9 levels.

OBJECTIVE: To report the clinical characteristics of 14 patients with imperforate hymen and their levels of tumor markers (CA 19-9 and CA 125). STUDY DESIGN: Fourteen patients with imperforate hymen who followed-up between September 2006 and September 2010 in the Department of Obstetrics and Gynecology, Dicle University School of Medicine, Diyarbakir, Turkey, were evaluated retrospectively. The clinical features and the management of the patients are discussed. RESULTS: The mean age of the patients was 13.8 years. All patients had primary amenorrhea and pelvic pain. The most common clinical symptoms were cryptomenorrhea in 14 patients, pelvic pain in 11, palpable abdominal mass in 6, voiding difficulties in 7, and defecation problems in 2. In 6 patients with palpable pelvic mass, the mean + standard deviation values of tumor markers were as follows: CA 125, 84.0 +/- 23.7 and CA 19-9, 162 +/- 189. One week after surgery we measured CA 125 and CA 19-9 levels once again. The postoperative mean CA 125 level was 13.8 +/- 3.6, and the mean postoperative CA 19-9 level was 17.5 +/- 3.5. Preoperative levels of CA 125 and CA 19-9 were significantly higher than those of the postoperative period (p < 0.001 for both comparisons). Six patients were treated by T-shaped incision and 8 patients by a central surgical incision through the hymenal membrane. CONCLUSION: Diagnosis of imperforate hymen is very important before undergoing surgery in a different clinic. Many patients have seen several doctors before receiving a clear diagnosis and have had tumor markers evaluated because the presence of pelvic mass in patients suggests the possibility of a gynecologic malignancy. Imperforate hymen is one of the benign conditions that increase serum CA 125 and CA 19-9 levels and which is not listed in the classical medical textbooks. These markers are not needed for the diagnosis.

Spontaneous uterine rupture secondary to recurrent haematometra from cervical stenosis.

Cervical stenosis is a challenging condition that often recurs despite intervention. Multiple therapeutic options have been described, but a clearly effective and reliable treatment method has yet to be identified. Patients with recurrent stenosis are at risk of developing severe complications such as chronic pelvic pain and infertility. We describe a case of congenital cervical stenosis with secondary haematometra in which repeated cervical dilatation, hysteroscopic canalisation and administration of medications to retard endometrial development were unsuccessful in relieving the obstruction and preventing re-accumulation of menstrual blood. Total hysterectomy was eventually mandated by spontaneous rupture of the haematometra.

Útero unicorne con cuerno rudimentario no comunicante en la adolescencia / Unicornuate uterus with noncommunicating rudimentary horn in adolescence

El útero unicorne es una rara malformación uterina que resulta del desarrollo anormal de uno de los conductos de Müller. Se manifiesta habitualmente con dismenorrea, dispareunia y dolor pélvico que aparece pocos meses después de la menarquia, aunque existen casos de presentación tardía o asintomática. Los médicos deben tener en cuenta la importancia de esta rara anomalía congénita del aparato genital femenino, especialmente poco después de la menarquia porque sin un diagnóstico y tratamiento precoz, las pacientes pueden desarrollar endometriosis, infertilidad, y complicaciones graves. Presentamos el caso de una paciente de 15 años con dolor pélvico crónico originado por un útero unicorne con un cuerno rudimentario no comunicante (AU)

Unicornuate uterus is a rare uterine anomaly resulting from the abnormal development of one of the Müllerian ducts. This abnormality usually manifests with dysmenorrhea, dyspareunia and pelvic pain appearing a few months after menarche, although there are late or even asymptomatic presentations. Clinicians should be aware of the importance of this rare congenital anomaly of the female genital tract, especially when symptoms occur shortly after menarche, because without early diagnosis and treatment, patients carrying this anomaly may develop endometriosis, infertility, and severe complications. We report the case of a 15-year-old girl with chronic pelvic pain caused by a unicornuate uterus with a noncommunicating rudimentary horn (AU)