Hematocolpos: An Uncommon Cause of Acute Urinary Retention and Hydronephrosis.

Acute urinary retention is uncommon in pediatric age and is usually associated with infection or obstruction of the urinary tract. We present the case of a 12-year-old girl admitted to the emergency room with acute urinary retention and lower urinary tract symptoms. Physical examination revealed abdominal distension and a blue-bulging mass occupying the vaginal introitus. Ultrasound confirmed the diagnosis of hematocolpos and revealed hydronephrosis, caused by compression. Kidney function and urinalysis were normal. A hymen incision and excision of redundant tissue were performed without complications. Hematocolpos is a condition caused by obstructive uterovaginal deformity. Imperforate hymen is responsible for most of the cases and usually manifests as primary amenorrhea and cyclic lower abdominal pain. The diagnosis of hematocolpos can be challenging. However, it is important to consider it in female adolescents without menarche presenting with acute urinary retention.

Imperforate Hymen: A Report of a Case With Classical Signs.

An imperforate hymen (IH) is a condition where the hymen, which is a thin membrane that partially covers the vaginal opening, completely obstructs the vaginal canal. This condition is associated with problems such as pelvic mass, cyclical abdominal discomfort, and difficulty in urination. The occurrence of IH is quite rare, with an incidence of only one in 1000-10,000 women worldwide. We discuss a classical case of primary amenorrhoea with associated complaints of urinary retention and its management by hymenotomy (cruciate incision). We also considered the risk of hymen re-closure due to the lack of estrogenization of genital tissue and offered the patient the option of vaginal molding.

Exploring the intersection of tuberous sclerosis and precocious puberty unveiled by hematocolpos.

We present the case of a 6-year-old girl who initially presented with acute pelvic pain, ultimately diagnosed with imperforate hymen leading to hematocolpos. Further investigation revealed additional clinical features including academic struggles, mood swings, and cutaneous findings, prompting consideration of a neurocutaneous syndrome. Magnetic Resonance Imaging (MRI) revealed features consistent with tuberous sclerosis complex (TSC), including radial migration lines in the subcortical white matter and an incidental arachnoid cyst. Notably, this case exhibited a unique presentation with absence of typical TSC findings such as subependymal nodules or cortical tubers. Additionally, precocious puberty, rarely associated with TSC, was observed, suggesting a potential link between hypothalamic lesions and hormonal imbalance. This case underscores the importance of comprehensive evaluation in pediatric patients presenting with seemingly unrelated symptoms, as it may unveil underlying conditions necessitating tailored management strategies.

A rare cause of amenorrhea: Hematocolpos in a post-cystocele repair patient.

Key Clinical Message: Secondary hematocolpos is a rare but serious complication after cystocele repair. This warrants multidisciplinary management (gynecology and urology) to optimize outcomes & minimize iatrogenic risks. Abstract: Hematocolpos is the term used for the accumulation of menstrual blood in the vagina. It is most commonly seen in adolescent girls but can also occur in elderly women as a result of gynecological conditions. We present the case of a 48-year-old female who presented with amenorrhea and abdominal pain following surgical repair of a cystocele. Investigation revealed secondary hematocolpos due to surgical trauma. Surgical drainage and correction of the uterus were performed, resulting in immediate pain relief. This case underscores the importance of considering hematocolpos in patients with post-surgical amenorrhea, and further research is needed to better understand its causes, risk factors, and optimal management strategies.

A Rare Case of Hematometrocolpos due to Transverse Vaginal Septum along with Distal Vaginal Atresia in an Adolescent Girl.

Primary amenorrhoea due to Müllerian malformations is rare, with 1 in 4500 cases and 2%-8% of cases presenting as infertility. Obstructive Müllerian anomalies present as hematometra and hematocolpos during puberty. Timely surgical intervention is required to relieve acute pelvic pain and restore functional anatomy. A 15-year-old girl presented to OPD with complaints of severe pain in her lower abdomen and lower back for the last 2-3 weeks, not relieving on medication. She has not attained menarche and has been having cyclical pain and low backache for 7-8 days every month for the last year. Physical examination showed a suprapubic lump with vaginal agenesis. Magnetic resonance imaging revealed hematometrocolpos due to transverse vaginal septum and distal vaginal atresia. Pull-through vaginoplasty along with complete excision of transverse vaginal septum was performed. Vaginal dilator therapy was done after the healing of the sutures. In follow-up, the patient attained menstruation with a patent vagina. Obstructive Müllerian anomalies should be identified early by detailed clinical examination and targeted investigations to prevent long-term morbidity and infertility.

Endoscopic treatement by vaginoscopy of a Herlyn-Werner-Wunderlich syndrome: A case report.

INTRODUCTION: Herlyn-Werner-Wunderlich syndrome is a rare complex congenital disorder, presenting with obstructed vagina, uterus didelphys and ipsilateral renal agenesis. Hemivaginal obstruction firstly asymptomatic, leads to symptoms after menarche such as dysmenorrhea, pelvic pain or infertility. CASE PRESENTATION: A 15-year-old patient presenting with few symptoms, transvaginal ultrasound reveals an hematocolpos, we report also typical findings of this disorder on magnetic resonance imaging. DISCUSSION: The pelvic pain caused by the hematocolpos is the main symptom that leads patients to consult often urgently, the MRI is the gold standard exam to confirm diagnosis, the treatment consists on incision the septum vaginal and leads to avoid complications. CONCLUSION: Early diagnosis of this syndrome usually leads to initiate surgical treatment in order to avoid complications.

An Incidental Diagnosis of Herlyn-Werner-Wunderlich Syndrome in a Young Female: A Case Report of a Rare Entity.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a rare genetic condition of the urogenital system, is characterized by distinctive features such as ipsilateral renal agenesis, obstructed hemivagina, and uterus didelphys. It is also referred to as Herlyn-Werner-Wunderlich (HWW) syndrome. Its clinical manifestations include dysmenorrhea, consistent abdominal pain, and infrequent periods. It is typically diagnosed after menarche. We report a case of a 20-year-old female who was admitted to the casualty ward following a road accident. She was Incidentally found to have uterine didelphys with hemorrhagic cystic lesion and left renal agenesis on ultrasonography (USG). She also had gallbladder stones, along with the findings mentioned above. Clinicians should exclude HWW syndrome in cases where uterine didelphys and unilateral renal agenesis coexist. Prompt identification and treatment of the condition can help avoid potential untoward pregnancy-related issues in the future.

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.

Managing pronounced hematocolpos in Herlyn-Werner-Wunderlich syndrome: A comprehensive case report.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare and complex congenital anomaly of the genitourinary system characterized by uterus didelphys, an obstructed hemivagina, and ipsilateral renal agenesis. It is the result of the maldevelopment of both the Mullerian and Wolffian ducts. Clinical manifestations of dysmenorrhea, dysuria, and urinary retention appear after menarche due to the accumulation of menstrual blood and distention of the obstructed hemivagina. Diagnosis of this anomaly is often delayed, and the obstructive nature of the condition is missed due to cyclic menstruation from the one canalized hemivagina. This case report represents a typical form of HWW syndrome to highlight the importance of considering this rare entity in an adolescent girl presenting with signs of dysmenorrhea and urinary complaints. A 13-year-old patient was presented at the obstetrics and gynecology clinic due to low abdominal pain and urinary retention on the fifth day of menstruation. For the last 6 months, the patient suffered progressive cyclic low abdominal pain associated with menstruation. Inspection of the external genitalia was unremarkable, and the hymenal membrane had a normal oval opening. An ultrasound examination revealed the sonographic features of the didelphys uterus and was suggestive of a right obstructed hemivagina with hematocolpos. Magnetic resonance imaging and computed tomography urography showed evidence of didelphys uterus, right blind obstructed hemivagina associated with huge hematocolpos, and ipsilateral renal agenesis. The diagnosis of HWW syndrome was established. Surgical resection of the vaginal septum was undertaken without delay, and the patient was discharged the following days.

Adolescent and Young Adult Patients with Vaginal Graft-vs-Host Disease and Hematocolpos Managed with Vaginal Stents: A Case Series.

STUDY OBJECTIVE: Vaginal stenosis can be acquired as a result of vaginal graft-vs-host disease (GVHD) in patients who have undergone hematopoietic stem cell transplant (HSCT). Little data exist to guide the management of vaginal GVHD, particularly in adolescent and young adult patients. The objective of this study was to detail the management of vaginal stenosis with lysis of adhesions and vaginal stent placement in 3 young patients with vaginal GVHD. METHODS: A retrospective chart review was done for 3 patients with vaginal GVHD causing vaginal stenosis with hematometrocolpos. All 3 were treated using vaginal stent placement. Additionally, a literature review was conducted through PubMed and Google Scholar to identify 21 case reports (with a total of 35 patients) of menstrual obstruction due to GVHD. RESULTS: Obstructive vaginal stenosis secondary to vaginal GVHD occurred in our patients at ages 15, 16, and 24 years. Resolution of hematocolpos was obtained with lysis of vaginal adhesions with vaginal stent placement in all patients, with varying regimens of systemic and topical hormones, topical corticosteroids, and dilator therapy. DISCUSSION: Vaginal stenosis secondary to vaginal GVHD should be considered in patients with a history of allogeneic HSCT presenting with amenorrhea, especially those with a diagnosis of primary ovarian insufficiency. The use of vaginal stents, along with postoperative medical and dilator management as appropriate, may prevent re-stenosis, although more information is needed regarding the efficacy of treatments.

Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series.

INTRODUCTION: There are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly. STUDY OBJECTIVE: To describe another variant of UD and compare the presentation and management across different institutions METHODS: This was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used. RESULTS: Five patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain. CONCLUSION: Diagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

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.

Himen imperforado con hematocolpos. A propósito de un caso

El himen imperforado es una malformación congénita poco frecuente reconocida como la causa más común de hematocolpos. La mayoría de las pacientes con himen imperforado son infradiagnosticadas hasta la manifestación de síntomas obstructivos en la adolescencia. Presentamos el caso de una mujer de 13 años a quien se le diagnosticó de himen imperforado con hematocolpos.

Imperforate hymen is a rare congenital malformation recognized as the most common cause of hematocolpos. Most patients with imperforate hymen are underdiagnosed until the manifestation of obstructive symptoms in adolescence. We present the case of a 13-year-old female diagnosed with imperforate hymen with hematocolpos.

Herlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal anomaly: A case report and a comprehensive review of literature.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a Müllerian duct anomaly. It is a rare clinical condition consisting of a duplicated uterus with an oblique vaginal septum that causes partial genital tract outflow obstruction. A urinary tract anomaly, most commonly renal agenesis, is usually present on the obstructed side. The diagnosis of genital tract outflow obstruction is often delayed due to the normal functioning of the unaffected side. The most frequent complications are dysmenorrhea, chronic pelvic pain, infection, infertility and endometriosis. This report describes a 17-year-old G0P0 patient with a history of severe dysmenorrhea and left-sided renal agenesis, who was admitted for complaints of foul vaginal discharge over the past 3 months that was unsuccessfully treated with antibiotics. Transrectal ultrasound revealed the presence of 2 separate hemicavities on transverse and longitudinal views. A cystic lesion with ground-glass opacities was detected between the bladder and a normal-appearing cervix, which was determined to be hematocolpos. The diagnosis of OHVIRA was made. This case highlights the importance of excluding a Müllerian anomaly in the presence of renal system abnormalities. Being aware of the type of anomalies, combinations and variants is crucial to determine the diagnosis and the best surgical approach. Ultrasound was an invaluable imaging exam to determine the type of anomaly and its complexity. Awareness of this syndrome and its variants will prevent misdiagnosis and will help to define the appropriate treatment for these patients.

Hematocolpos due to lower vaginal agenesis in an adolescent girl.

Background: Hematocolpos due to imperforate hymen is an important differential diagnosis of abdominal pain in early adolescent stage. However, hematocolpos due to lower vaginal agenesis must be considered because the management differs. Case Presentation: A healthy 11-year-old girl presented with a 2-day left lower abdominal pain history. Her breast development had begun, but she had not reached menarche. Computed tomography showed high absorptive value liquid filling the upper vaginal to uterine cavity, a pale highly absorptive fluid component suggestive of hemorrhagic ascites in the abdominal cavity on both sides of the uterus, and normal bilateral ovaries. Magnetic resonance imaging diagnosed hematocolpos due to lower vaginal agenesis. The blood clot was aspirated with a transabdominal ultrasound-guided transvaginal puncture. Conclusion: History-taking, imaging tests, and appropriate collaboration with obstetrician/gynecologist with awareness of secondary sexual characteristics were crucial in this case.

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.

Vaginoscopic Resection of Oblique Vaginal Septum in OHVIRA Syndrome Before Menarche.

OBJECTIVE: To demonstrate vaginoscopic resection of the oblique vaginal septum in a girl with Obstructed Hemi Vagina and Ipsilateral Renal Agenesis (OHVIRA) syndrome before menarche. DESIGN: Stepwise demonstration of surgical technique with narrated video footage. SETTING: OHVIRA syndrome is a rare urogenital anomaly in which patients present after menarche, with progressive dysmenorrhea and a palpable pelvic mass due to hematocolpos and hematometra on the obstructed side. Delay in diagnosis may cause endometriosis, pelvic adhesions, and infertility [1,2]. A 12-year-old, premenarchal girl with complaints of pelvic pain and an ultrasound report of right renal agenesis was referred to the urology department of our hospital. She was also found to have uterus didelphys and a loculated fluid collection behind the urinary bladder on ultrasonography and on magnetic resonance imaging (Figs. 1 and 2). A probable diagnosis of OHVIRA syndrome with mucocolpos was made [3]. INTERVENTION: Vaginoscopy showed an obstructing bulging vaginal septum on the right side. The left uterine horn was inspected by hysteroscopy. The vaginal septum was incised vaginoscopically with monopolar cautery using Collins knife (Video still 1) [4-6]. The collected mucus was drained. The right cervix and the right uterine horn were visualized by hysteroscopy. Edges of the septum were resected with a resectoscope loop (Video still 2). The hymen was not injured during the surgery. Relook vaginoscopy done after 2 months showed a normal and healed vagina. CONCLUSION: Vaginoscopic resection of the obstructing oblique vaginal septum in OHVIRA syndrome is a very simple, minimally invasive, and virginity conserving surgery. Early diagnosis and treatment can prevent future complications due to cryptomenorrhea.

Hematocolpos due to imperforate hymen: a case report and literature systematic review.

INTRODUCTION AND HYPOTHESIS: Hematocolpos is a rare condition, where menstrual blood fills the vagina, instead of being expelled, due to a series of uterovaginal pathologies, the most frequent of which is the imperforate hymen. To date, few cases of hematocolpos have been reported in the literature. METHODS: We report a case of hematometrocolpos due to imperforate hymen initially misdiagnosed as constipation and subsequently as ovarian mass; moreover, the present study undertakes a systematic review of studies on hematometrocolpos due to imperforate hymen to synthesize available knowledge on epidemiology, diagnosis, and management about this rare condition. RESULTS: A total of 35 studies, describing 61 patients, were identified. The presence of hematocolpos should be suspected in premenarchal patients complaining of low abdominal pain, abdominal swelling, and urinary retention. Genital examination disclosing a tender, pale hymen and ultrasound represent a useful tool for diagnosis. The goal of the management is to timely perform hymenotomy to drain the hematocolpos, followed by hymenectomy to prevent recurrence. Follow-up is needed to diagnose possible recurrences. CONCLUSIONS: In the case of an adolescent girl complaining of genital pain associated with primary amenorrhea, hematocolpos due to imperforate hymen should be suspected.

Surgical disorders in pediatric and adolescent gynecology: Vaginal and uterine anomalies.

Obstructive vaginal and uterine anomalies including imperforate hymen, transverse vaginal septum, and vaginal and/or cervical atresia or aplasia, might rarely present in infancy or childhood with hydrocolpos and/or hydrometra but they usually go unrecognized until presentation with amenorrhea and hematocolpos and/or hematometra in puberty. They should always be included in the differential diagnosis of a suprapubic and/or introital mass; in the latter case, vaginal vascular malformations and vaginal tumors should also be considered. Uterovaginal aplasia typically manifests with amenorrhea in puberty and needs to be differentiated from complete androgen insensitivity syndrome and gonadal dysgenesis of genetic males. Uterine fusion anomalies usually present with fertility and/or obstetrical complications in adulthood. However, a unicornuate uterus with a blind rudimentary contralateral horn containing functioning endometrium, and didelphys or septate uterus with a deviating obstructive septum might present in childhood or puberty with sequelae related with secretions or menstrual retention. This review provides a collective account of the most clinically important information about vaginal and uterine anomalies in childhood and adolescence for clinicians involved in the care of young females with the aim to provide guidance in appropriate evaluation and management.