A Case Report of Hematometrocolpos in Two Adolescents: Divergent Clinical Presentations and Diagnostic Challenges.

Hematometrocolpos is an infrequent congenital anomaly (Mullerian duct anomaly) that results in an imperforate hymen, followed by accumulation of menstrual blood in the vagina and or uterus in prepubertal girls results in retrograde menstruation. This commonly manifests as abdominal pain in premenarcheal pubescent girls. We discuss the case of two adolescent girls who presented to the emergency with lower abdominal pain, constipation, back pain, and/or urinary retention. They were found to have an imperforate hymen and hematometrocolpos. The diagnosis was made with the use of a genital examination and ultrasound. Hymenotomy was performed successfully in both cases and the patients recovered completely without complications. Failure to diagnose premenstrual girls presenting with lower abdominal pain and/or retention of urine with hematometrocolpos might lead to complications like infertility, endometriosis, tubal infections, adhesions, etc.

A case of image-guided hematometrocolpos drainage requiring tissue plasminogen activator in a pediatric patient.

Hematometrocolpos (HMC) is a rare disorder that occurs when an anatomical anomaly like imperforate hymen causes menstrual blood to be retained in the uterus and vagina. There is no standard of care established for HMC beyond urgent vaginoplasty which requires a demanding post-operative course that may not be suited for all pediatric patients. This is a case report of successful use of image-guided percutaneous drainage of HMC with tissue plasminogen activator (TPA) followed by vaginoplasty in a 13-year-old female with lower vaginal atresia. Additionally, this case explores the role of menstrual suppression and the need for individualized guidelines. It emphasizes the potential of image-guided percutaneous drainage with TPA as a promising, less-invasive treatment option for pediatric HMC as well as the impact on follow-up surgery.

Urinary retention complicated by hematocolpos in an adolescent girl: Case report.

INTRODUCTION AND IMPORTANCE: This case report is intended to present an unusual cause of urinary retention by a congenital vaginal obstruction, such as 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. CASE PRESENTATION: A 12-year-old female patient, was referred to our emergency department for recurrent abdominal pain lasting approximately 90 days. She presented with intermittent dysuria and supra pubic fullness. Ultrasonography confirmed acute urinary retention and features of hematocolpos [7]. CLINICAL DISCUSSION: High index of suspicion is necessary to diagnose and treat such patients promptly. Patients usually present with cyclic pain, primary amenorrhea, incomplete emptying of urine, chronic constipation, back pain and recurrent urinary tract infections. Our patient presented with acute urinary retention. CONCLUSION: This case report highlights the importance of early recognition of hematocolpos as a potential cause of cyclic pain, and urinary symptoms. Prompt and accurate diagnosis with the appropriate surgical managements, to resolve their symptoms with successful outcomes.

Management of Hematometra and Hematocolpos in Obstructive Mullerian Anomalies by Image-Guided Drainage with Interventional Radiology: A Multi-Institutional Case Series.

STUDY OBJECTIVE: To describe cases of image-guided drainage of symptomatic hematometrocolpos from obstructive Müllerian anomalies as a temporizing measure to manage acute pain symptoms and delay definitive management of the obstructive Müllerian anomalies that require complex reconstruction METHODS: Institutional Review Board exemption from all included institutions was obtained. A retrospective case series from 3 academic children's hospitals of 8 females under the age of 21 with symptomatic hematometrocolpos due to obstructive Müllerian anomalies drained by image-guided percutaneous transabdominal vaginal or uterine drainage with interventional radiology was reviewed and described. RESULTS: Eight pubertal patients with obstructive Müllerian anomalies (6 patients with distal vaginal agenesis, 1 patient with an obstructed uterine horn, and 1 patient with a high obstructed hemi-vagina) and symptomatic hematometrocolpos are reported. All patients with distal vaginal agenesis had greater than 3 cm lower vaginal agenesis, which would usually require complex vaginoplasty and use of postoperative stents. Given their immaturity and inability to use stents or dilators postoperatively or medical complexity, they subsequently underwent ultrasound-guided drainage of hematometrocolpos with interventional radiology to relieve pain symptoms, followed by menstrual suppression. The patients with obstructed uterine horns had complex medical and surgical histories requiring perioperative planning; they also underwent ultrasound-guided drainage of hematometra as a temporizing measure to manage acute symptoms. CONCLUSION: Patients presenting with symptomatic hematometrocolpos due to obstructive Müllerian anomalies might not be psychologically mature enough to undergo definitive complex reconstruction, which requires vaginal stent or dilator use postoperatively to prevent stenosis and other complications. Image-guided percutaneous drainage of symptomatic hematometrocolpos serves as a temporizing measure by offering pain relief until patients are ready to undergo surgical management and/or to allow time for complex surgical planning.

Imperforate Hymen and Hematometrocolpos in a Female With Back Pain and Urinary Retention.

A 12-year-old female with primary amenorrhea presented to the emergency department with episodic low back pain, suprapubic discomfort, and acute urinary retention. A protruding purple mass was noted at the vaginal introitus consistent with an imperforate hymen. Point-of-care bedside transabdominal ultrasonography revealed a distended uterus containing hypoechoic material. The patient underwent formal pelvic ultrasonography, which revealed a markedly enlarged uterus containing a large number of blood products, thinned myometrium, and a distended vaginal canal consistent with hematometrocolpos secondary to imperforate hymen. Imperforate hymen is a rare congenital anomaly of the female urogenital tract, in which the hymen obstructs the vaginal opening causing a vaginal outlet obstruction. Vaginal outlet obstruction secondary to imperforate hymen may lead to retrograde menstruation with a collection of blood within the uterus and vagina, which is termed hematometrocolpos. Treatment is based on identifying and treating the underlying imperforate hymen with surgical approaches. The growing use of bedside ultrasonography allows the clinician to rapidly and accurately diagnose hematometrocolpos. The use of point-of-care bedside ultrasonography can serve as an essential tool as delayed diagnosis and treatment of this rare condition are associated with significant morbidity and lifelong infertility.

Familial occurrence of concomitant imperforate hymen and transverse vaginal septum: a case report.

The imperforate hymen and transverse vaginal septum are female reproductive tract abnormalities. Their concurrent occurrence is rare. So far, no manifestation of both anomalies within the same family has been reported in the literature. We report the first familial case of two sisters having both mentioned abnormalities. Our patient was the youngest sister of a 14-year-old, who presented with cyclic lower abdominal pain. Clinical and imaging examinations showed an imperforate hymen with hematometrocolpos. In addition, the surgery confirmed a low transverse vaginal septum, which was resected using a virginity-preserving procedure. In conclusion, the presence of both mentioned abnormalities between siblings reflects a potential genetic etiology, also in order to avoid late diagnostics of such anomalies, the imperforate hymen must be screened in the birth room.

Endoscopic Treatment of a Severe Vaginal Stenosis Following Battery Insertion in an 11-Year-Old Girl.

Acquired vaginal strictures are rare entities in children. As a result, they are generally difficult to manage and tend to recur despite appropriate initial therapy. This case study reports the staged management of vaginal stenosis following the insertion of a button battery. In this case, an 11-year-old girl experienced at 4 years old a battery insertion in the vaginal canal by her neighbor's son, who was 6-year-old at the time. Two weeks from insertion, the parents noted the foreign body discharge spontaneously. The girl had not complained of any symptoms at the time and had been asymptomatic for many years. In November 2020, she came to the emergency department reporting cramping abdominal pain accompanied by mucopurulent discharge. An abdominal ultrasound showed the presence of hematometrocolpos, and a vaginal stenosis dilation under general anesthesia was performed the following day. After 3 weeks, the stenosis was still present, preventing the passage of Hegar number 4. The girl was subjected to a vaginoscopic stenosis resection utilizing a monopolar hook passed through an operative channel. A Bakri catheter filled with 120 mL of water was left in place. After 10 days, the girl was discharged home with the Bakri inserted. Two weeks after discharge, she was reevaluated in the outpatient setting, where the Bakri was removed with no signs of residual stenosis. Acquired vaginal stenosis could be demanding to treat, particularly with the sole conservative approach. A first-line option can be the Hegar dilation. The endoscopic approach can be a second-line, minimally invasive treatment, but long-term outcomes are difficult to predict.

Abdominal swelling and obstructive uropathy due to hematometrocolpos secondary to imperforate hymen: a case report.

Imperforate hymen is an uncommon congenital anomaly of the female genital tract and can lead to the collection of blood in the vagina and the uterus. Most patients are not diagnosed until menarche when they present with symptoms such as cyclic abdominal and pelvic pain, constipation, tenesmus, back pain, and difficulties with urination in association with lack of menses. We discuss the case of an adolescent female who presented with the complaint of an increasing abdominal swelling along with the urgency and a sense of incomplete urination. She had not attained menarche. A diagnosis of hematometrocolpos was made based on computed tomography findings. Subsequent perineal examination revealed a bulging imperforate hymen. Hymenotomy was performed with complete resolution of the symptoms. This case highlights the importance of keeping a high index of suspicion for this condition in patients presenting with these symptoms and the importance of appropriate gynecologic examination.

The Herlyn-Werner-Wunderlich (HWW) syndrome - A case report with radiological review.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of female urogenital tract involving combined mullerian duct anomalies and mesonephric duct malformation characterized by uterus didelphys, obstructed hemi-vagina and ipsilateral renal agenesis which is also known as OHVIRA syndrome. It can be classified based on a completely or incompletely obstructed hemivagina. It presents soon after menarche or shows delayed presentation depending on the type. The most common presentation is lower abdominal pain, dysmenorrhea, and abdominal mass in the lower abdomen secondary to hematometra or hematocolpos. We present a 15-year-old unmarried patient with an unusual case of OHVIRA syndrome suffering from dysmenorrhea and painful mass in suprapubic region. We described the role of imaging modalities in diagnosis of the Herlyn-Werner Wunderlich syndrome with a review of literature. On USG and MRI, she had right renal agenesis with compensatory hypertrophy of the left kidney, didelphic uterus with an obstructed hemi-vagina on right side which led to marked distention of ipsilateral cervix and proximal vagina in the form of hematometrocolpos. OHVIRA syndrome can present early or late, depending on the type. In patients with uterine and vaginal abnormalities, a work-up for associated renal anomalies should be performed. The choice imaging modalities for the diagnosis of OHVIRA syndrome are ultrasound and MRI. Knowing the imaging findings of this rare condition is crucial for early diagnosis in order to prevent complications which may lead to endometriosis and infertility.

Herlyn-Werner-Wunderlich syndrome: A report of three cases in adolescents and adult woman.

We report three cases with Herlyn-Werner-Wunderlich syndrome in adolescents and young female. The objective of this report was to describe the clinical presentation, ultrasound (US) and magnetic resonance imaging (MRI) findings of Herlyn-Werner-Wunderlich syndrome. The three patients were 12, 13 and 34 years old, respectively. The reason for admission and clinical symptoms varied between the 3 patients, including menstrual cramps, vaginal bleeding, and dull pain in the hypogastric region. Nevertheless, the sonographic and MRI findings of all three cases were typical for HWWS, including didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis.

Congenital vaginal atresia: about an uncommon case.

Congenital vaginal atresia is a rare congenital abnormality of the female reproductive tract due to a failure of canalisation in the urogenital sinus. We report the uncommon case of a 14-year-old girl with a primary amenorrhea associated to a cyclical pelvic pain, in whom examination objectified a vaginal cup that replaced the introitus. Ultrasound examination and magnetic resonance imaging (MRI) revealed atresia of the lower third of the vagina. The diagnosis of partial vaginal aplasia on functional uterus was retained, the patient had a perineal vaginoplasty. The evolution was satisfactory with regular cycles and improvement of pelvic pain. The decline is three years. Congenital vaginal atresia is a rare malformation classically and clinically pictured as a primary amenorrhea with chronic cyclic pelvic pain. Diagnosis is based on clinical examination and imaging. The MRI is designed to assess the importance of atresia and guide surgical management while the surgical technique aims to restore the integrity of the utero-vaginal tract and to increase the possibility of pregnancy for these patients.

Late diagnosis of imperforate hymen with hematometrocolpos and bilateral hydronephrosis of a horseshoe kidney.

Imperforate hymen is a fairly uncommon genital disorder where the hymen completely obstructs the vaginal opening. Several authors have ruled out its co-occurrence with other congenital anomalies. In this report, we discuss the exceptional case of a late diagnosis of imperforate hymen associated with bilateral hydronephrosis of a horseshoe kidney in a 19-year-old female patient. To our knowledge, an association of imperforate hymen and horseshoe kidney has never been reported.

Virginity-sparing management of hematocolpos with imperforate hymen: case report and literature review.

Imperforate hymen results from failure of the endoderm of the urogenital sinus to completely canalize and has an incidence of 0.01% to 0.05%. This sometimes presents as a pelvic mass that compresses the bladder causing acute urinary retention. A 13-year-old girl was referred to our department with a history of primary amenorrhea, cyclic lower abdominal pain, abdominal-pelvic mass, constipation and acute urinary retention. She had an ultrasonography misdiagnosis of a huge ovarian mass before referral to our unit. On examination, the vagina was bulging and compressing the rectum. Repeat abdominal ultrasonography confirmed the diagnosis of hematometrocolpos. She underwent X-shaped hymenotomy with a favorable outcome. Diagnosis of imperforate hymen requires high suspicion index. Virginity-sparing surgery constitutes a good treatment option for cultural and religious reasons.

Hematometrocolpos Disguised as Abdominal Pain.

BACKGROUND: Hematometrocolpos caused by an imperforate hymen is a common form of vaginal outflow obstruction. This is a rare pediatric anomaly that can present with atypical or vague symptomatology, such as abdominal pain or constipation or urinary retention in the setting of amenorrhea. It is essential to obtain a gynecologic history and inquire about menstrual cycles to properly evaluate a young female with such a common complaint as abdominal pain. Failure to perform a gynecologic examination in the emergency department setting may delay diagnosis and appropriate care for this rare condition, which can lead to serious complications. CASE REPORT: This case describes a 12-year-old female who presented to the emergency department with a complaint of abdominal pain and urinary symptoms. Because of the severity of the patient's pain on abdominal examination, we obtained a computed tomography scan of her abdomen and pelvis, which showed findings consistent with hematometrocolpos. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Obstruction of the female genital outflow tract is a rare occurrence. Because abdominal pain is such a common complaint, not only in the pediatric emergency department but also in the outpatient setting, the diagnosis of hematometrocolpos may easily go undiagnosed for months or even years. Obtaining a complete gynecologic history is key when evaluating young females with abdominal pain or urinary retention. Early detection and timely management can prevent serious complications and long-term sequelae. This patient had a successful outcome and early surgical management of her hematometrocolpos which was caused by an imperforate hymen.

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.

True management of Obstructed Hemi-vagina and Ipsilateral Renal Anomaly syndrome.

Herlyn-Werner-Wunderlich syndrome is an unusual congenital anomaly of the female genitourinary system, which is described as uterine didelphys with Obstructed Hemi-vagina and Ipsilateral Renal Anomaly (OHIRA), also known as OHVIRA syndrome. Typical symptoms are pelvic pain, tenderness, pelvic mass due to blood collection in the obstructed hemi-vagina and uterus, and dysmenorrhea that usually begins shortly after menarche. Clinical suspicion is very important for diagnosis and correct management avoids both short- and long-term complications. Surgical removal of the vaginal septum is the main treatment method. Herein, we describe the evaluation and surgical management of a patient with OHVIRA syndrome who was diagnosed using magnetic resonance imaging and pelvic ultrasound.

Postoperative Course and Complications after Pull-through Vaginoplasty for Distal Vaginal Atresia.

STUDY OBJECTIVE: To report the usual postoperative course and complications after pull-through vaginoplasty for isolated distal vaginal atresia. DESIGN, SETTING, AND PARTICIPANTS: Retrospective chart review at Texas Children's Hospital of all patients who were diagnosed with isolated distal vaginal atresia and underwent pull-through vaginoplasty during the study time frame. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Postoperative complications such as vaginal stenosis or infection and postoperative vaginal diameter. RESULTS: Sixteen patients were identified and charts were reviewed. Patients were initially evaluated by pelvic magnetic resonance imaging and found to have distended hematometrocolpos with distal vaginal atresia. All patients underwent pull-through vaginoplasty with similar operative techniques. The average distance from the perineum to the level of the obstruction was 1.84 ± 1.2 cm. Two patients, both with obstructions at greater than 3 cm, experienced stricture formation postoperatively. Four patients (25%) experienced postoperative vaginitis. One patient (6.25%) experienced a postoperative urinary tract infection. Two groups (3 cm or less versus greater than 3 cm) were compared, and the presence of stricture was statistically different based on mean centimeters from perineum prior to pull-through vaginoplasty (P = .038). CONCLUSIONS: Distal vaginal atresia is managed with pull-through vaginoplasty. Atresias that extend greater than 3 cm from the perineum are at increased risk for vaginal stricture formation and should be followed to monitor for their formation. Other complications are infrequent and minor.

Fate of the uterus in 46XX cloacal exstrophy patients.

PURPOSE: Genetically female cloacal exstrophy (46XX CE) patients develop complications later in life due to their abnormal uterine anatomy, resulting in various invasive gynecologic procedures. Furthermore, they have difficulty becoming pregnant, and if they do conceive, they are unlikely to carry the pregnancy to term. We performed this review to determine the rate of gynecological complications, the fate of the uterus, and the rate of pregnancy in 46XX cloacal exstrophy patients. METHODS: All charts for 46XX CE patients treated by us were reviewed following IRB approval. Patient age at last follow-up, surgical management of the uterus, uterine complications, and pregnancies were recorded. RESULTS: The charts of all 16 of our 46XX CE patients who survived past the neonatal period were reviewed. Two patients underwent hemi-hysterectomy (HH): 1 for an atretic hemi-uterus at birth, another for abnormal uterine insertion at 3 years. A third patient initially had HH for hydrometrocolpos leading to ureteral and colonic obstruction at 14 years but she required a completion hysterectomy a year later. Four patients underwent total hysterectomy (TH) at the outset: 2 neonates for a diminutive uterus with extreme disparity in the halves, another for uterine prolapse at 1 month of age, and a fourth for hematometrocolpos at 16 years of age. Six patients reached adulthood without requiring gynecologic intervention; one of these six is now being managed at another institution. Two patients are prepubertal and one was lost to follow-up. The only patient in the series who became pregnant miscarried at 11 weeks gestation. CONCLUSION: Out of 13 post-pubertal patients 6 have retained the entire uterus and another 2 had a hemi-hysterectomy. One patient who became pregnant miscarried at 11 weeks gestation. We believe it is appropriate to avoid ablative genital surgery as far as possible and for these patients to become pregnant after detailed discussion with physicians experienced in the care of 46XX CE patients.

Malformaciones uterinas en el Hospital Central de Maracay: revisión de 1999 a 2007 / Uterine malformations in the Hospital Central of Maracay: review of 1999-2007

Conocer la incidencia de malformaciones uterinas y describir las características clínicas asociadas. Estudio descriptivo, retrospectivo y epidemiológico de 24 pacientes egresadas con el diagnóstico de malformación uterina entre los años de 1999 a 2007. Departamento de Obstetricia y Ginecología del Hospital Central de Maracay. Las pacientes en 70,8 por ciento tenían edades comprendidas entre 16 y 25 años. El 50 por ciento eran primigestas. El 33,3 por ciento presentaba diagnóstico de ingreso por presentación fetal anómala. En el 66,6 por ciento de los casos el diagnóstico se realizó por visualización directa de la estructura. En el 62,5 por ciento la malformación más frecuente fue el útero didelfo. En 50 por ciento de los casos el resultado obstétrico fue la cesárea segmentaria. En cuanto al diagnóstico de ingreso el útero didelfo registra el 62,5 por ciento de los casos ingresados. Las mujeres con anomalías uterinas se reproducen normalmente. Las malformaciones uterinas por defectos de fusión de los ductos müllerianos son las más frecuentes. Esporádicamente pueden ser sintomáticas ameritando diagnóstico diferencial y tratamiento

To know the incidence of congenital uterine anomalies and the clinical presentations. Retrospective, epidemiological, descriptive study of 24 cases of patients with congenital uterine anomalies. Department of Obstetric and Gynecology, Hospital Central de Maracay. In the 70.8 percent the age was between 16 and 25 years. The 50 percent were primipara. The 33.3 percent had personal history of abnormal fetal positions. The 66.6 percent were detected by visualization of the uterus. In 62.5 percent the most frequent malformation was the uterus didelphys. In 50 percent the obstetric performance was the cesarean section. The reproductive performance didelphic reported 62.5 percent of patients. Women’s with Müllerian anomalies demonstrate no problems with conception. However comes to medical attention when become symptomatic because of poor obstetric performance