Congenital cervico-vaginal aplasia with a functional uterus: A case report and review of literature.

Maldevelopment of the vagina and cervix is frequently accompanied by uterine aplasia or hypoplasia. Complete cervico-vaginal aplasia with a normally developing uterus is a very uncommon type of developmental failure. Failure to treat the condition can result in complications such as hematometra and hematosalpinx caused by the retrograde flow of blood into the fallopian tubes. In this case report, we describe the case of a 32-year-old woman experiencing cyclic abdominal pain and primary amenorrhea. The patient exhibited cervico-vaginal agenesis, with a functional uterus that was complicated by hematometra and bilateral hematosalpinx.

Case report: Elective management of a bicornuate uterus with hematometra, incidentally found submucosal fibroids, rectus sheath hematoma, and hydronephrosis in a resource limited setting.

INTRODUCTION AND IMPORTANCE: Bicornuate uterus is a rare type of congenital mullerian anomaly, presenting as a diagnostic challenge. Metroplasty either via an open approach or laparoscopically can be performed to definitively diagnose and treat the defect. CASE PRESENTATION: A 26-year-old female, with no known comorbids and past surgical history of endometriotic cyst excision presented with acute symptoms of left lower abdominal pain, burning micturition, and relative constipation. After clinical and radiologic investigations, the diagnosis of bicornuate was suspected. An elective exploratory laparotomy was then performed due to limited resources and skilled surgical techniques. Intra-operatively it was found that she had a bicornuate uterus with a single cervix and vagina, with the left cornuate being non-communicating with fluid suggesting hematometra. Dense adhesions were reported with drainage of 150-200 ml of free fluid upon opening the rectus sheath. Postoperatively she remained vitally stable and was discharged home. CLINICAL DISCUSSION: We report a rare case of a bicornuate uterus with double horns along with submucosal fibroids, rectus sheath hematoma, and left sided hydronephrosis. Diagnosis of bicornuate uterus is associated with diagnostic uncertainty mainly due to its rarity and nonspecific presentation. CONCLUSION: Although bicornuate is rare, it may result in complications if not attended to timely. Early diagnosis and management are necessary to minimize associated morbidity and mortality that can occur as a consequence of associated unattended pressure symptoms.

Hematometra: A Rare Case of Pelvic Pain in Females Identified with Point of Care Ultrasound.

The differential diagnosis for abdominal or pelvic pain in women of child-bearing age that present to the emergency department is broad. A rare cause of abdominal and pelvic pain is hematometra, or a collection of blood products within the uterus. While blood is normally expelled through menses, this process is disrupted in some patients due to congenital or acquired abnormalities. This can lead to progressive uterine distension and pain, which may ultimately require medical or surgical intervention. Hematometra is rare, but is a serious condition that can be diagnosed easily at bedside using point of care ultrasound.

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.

Spontaneous rupture of a nongravid uterus: a case report.

Rupture of a gravid uterus is a known complication of a cesarean hysterotomy. Uterine rupture of a nongravid uterus is usually caused by trauma, instrumentation, a pelvic mass, infection, or malignancy. Spontaneous rupture of a nongravid uterus is a rare event with only 4 cases reported in the English literature since 2011. This was the case of a healthy 52-year-old woman with a remote history of 2 cesarean deliveries and an endometrial ablation. The patient presented with severe right lower-quadrant pain. The hospital evaluation revealed a hemoperitoneum, a 5 cm endometrial complex or mass, and layering of blood product along the cesarean delivery scar. Exploration confirmed a spontaneous rupture of the previous hysterotomy. The patient was treated successfully with a total abdominal hysterectomy. Pathology report confirmed the uterine wall defect. Uterine rupture in the non-gravid uterus is a rare event. Presentation may be atypical but consistent with the diagnosis. Spontaneous uterine rupture should be considered in the nongravid patient with abdominal pain and a hemoperitoneum of unclear origin.

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.

Challenges in the diagnosis and management of Robert's uterus: Systematic review and case presentation.

Robert's uterus is a rare Müllerian duct anomaly (MDA) characterized by complete asymmetric septum dividing uterine cavity into two non-communicating parts. This study offers insights into diagnosing and management of this condition in a young patient, and a systematic literature review. The review included 19 studies from 2013 to 2023. 11.4% of women reported a history of miscarriage and 5.7% primary infertility. Dysmenorrhea was the most frequent symptom (54.3%), and 14.3% of patients had a coexisting endometriosis. The surgical interventions most commonly combine laparoscopic and hysteroscopic techniques. Robert's uterus requires heightened clinical awareness for early diagnosis, particularly in young women.

A Case of Cervical Squamous Cell Carcinoma Developing 33 Years After Conization.

We report a fatal case of early postoperative peritoneal dissemination in a patient who was diagnosed with cervical squamous cell carcinoma after laparoscopic hysterectomy for hematometra. A 73-year-old multiparous woman with pyometra and lower abdominal pain was referred to our hospital. Her medical history was remarkable for four open surgeries and conization at the age of 40 years. The cytology obtained from the mucosa of the palpated cervix was negative. The cytology and bacterial culture of the mucus collected from the uterine cavity were negative. Increasing fluid accumulation in the uterine cavity started to cause severe abdominal pain. A laparoscopy was performed. The small intestine showed extensive adhesions to the abdominal wall, which were dissected. A total hysterectomy was performed, and the uterus was placed in a collection bag, cut inside the bag, and retrieved transvaginally. Histopathological examination revealed nests of squamous cell carcinoma that replaced the entire uterine myometrium, and the tumor cells showed diffuse positivity for p16 on immunostaining. The patient was diagnosed with squamous cell carcinoma of the uterine cervix with invasion of the uterine myometrium. Three months later, the patient suffered from small bowel obstruction. A laparotomy was performed, and it revealed numerous disseminated lesions in the pelvic peritoneum and mesentery of the small intestine. Bypass surgery was performed. A biopsy of a disseminated lesion near the vaginal cuff revealed squamous cell carcinoma. The patient died within three weeks of bypass surgery.

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.

A Rare Case of Vaginal Atresia in an Adolescent Girl Presenting With Abdominal Pain.

Vaginal atresia, an infrequent congenital anomaly characterized by the absence or underdevelopment of the vaginal canal, presents significant complexities in pediatric and adolescent gynecological practice. Its diverse range of clinical presentations, including primary amenorrhea and cyclic abdominal discomfort, creates diagnostic challenges, highlighting the need for timely intervention to relieve symptoms and preserve future reproductive health. This case underscores the essential role of a collaborative, multidisciplinary approach involving pediatricians, gynecologists, and surgeons in ensuring comprehensive care and optimizing patient outcomes. In this report, we present the case of a 10-year-old female who initially presented with chronic abdominal pain, which had been occurring intermittently over the course of several months. This ultimately led to the diagnosis of hematometra secondary to vaginal atresia. Utilizing magnetic resonance imaging, we confirmed the diagnosis without exposing the patient to radiation, prioritizing her safety. A successful surgery was performed to create a working vaginal canal. The patient received careful postoperative care, and we closely followed her progress to support a smooth recovery.

Hematometra Caused by Chronic Lichen Planus in a Patient Mimicking Acute Urinary Retention: A Case Report.

The association between lichen planus (LP) with subsequent hematometra and acute urinary retention (AUR) is quite rare, and few cases reported that association. In such cases, if the patient is left untreated, then possible kidney injury and bladder wall rupture may arise from that retention. In this case, we reported a patient who is known to have a background history of long-standing LP that presented with a history and features suggestive of AUR and was found to be caused by hematometra on an ultrasound scan.

A Case Report on a Left Unicornuate Uterus With Communicating Right Rudimentary Horn Associated With Hematometra and Hematosalpinx.

Congenital uterine anomalies (CUAs) or Müllerian duct anomalies are rare and can be either complete failure or partial failure in the development of the Mullerian duct, and they have a probability to result in a condition known as the unicornuate uterus. Partial development of one of the horns results in a rudimentary horn, which may be communicating consisting of category II A or noncommunicating consisting of category II B. This report illustrates a rare case of a 23-year-old female, unmarried, nulligravida, who presented to the outpatient department with chief complaints of acute abdominal pain and dysmenorrhea associated with an average menstrual flow. Pelvic ultrasound and magnetic resonance imaging (MRI) confirmed the diagnosis of a left unicornuate uterus with communicating right rudimentary horn associated with hematometra and hematosalpinx. As a treatment option, the surgical intervention mainly involved laparoscopic excision of the rudimentary horn and right salpingectomy that was performed by aspiration of blood from the rudimentary horn of around 25cc. Then, the right hydrosalpinx was removed, followed by right salpingectomy and excision of the rudimentary horn to reduce the risk of ectopic pregnancy having an incidence of 10% for which laparoscopic or robotic-assisted removal is preferable and practicable for young girls, compared with the open procedure. The patient adhered well to the surgical intervention.

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.

Minimally invasive vaginal approach to the uterovestibular anastomosis for cervicovaginal aplasia: a case series and review of literature.

BACKGROUND: Congenital cervicovaginal agenesis (CVA) with functioning endometrium is an extremely rare mullerian anomaly. Genital tract patency and fertility preservation are the major challenges in cases of CVA. With the advances in surgical techniques, management has shifted from a radical approach-like hysterectomy towards a more conservative approach of uterine conservation. CASE PRESENTATION AND REVIEW OF THE LITERATURE: In the present study, we report our experience in managing four cases of congenital CVA with complete vaginal atresia, which were treated with a simple minimally invasive vaginal approach for UVA without using graft for neovagina creation and studied the long-term anatomic and functional result of uterovestibular anastomosis (UVA) in patients with CVA. A literature review was performed for congenital complete vaginal atresia (≤ 2 cm blind vagina), with a functioning uterus. The experience and results of this 4-year study are in accordance with the prior studies with 97% (33/34) success rate, where vestibular mucosa was used for the UVA. CONCLUSIONS: Findings of this study should encourage more gynaecologists to learn and recreate a complete vaginal approach, as no special surgical equipments are needed. Hysterectomy should only be reserved for cases, where repeated anastomosis attempts fail. This minimally invasive technique should be preferred over canalization procedures and graft, as it is associated with a higher success rate with least complications and recurrence. Conservative end-to-end anastomosis with a completely vaginal approach should be offered as the primary treatment option for CVA with total vaginal atresia.

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.

A Rare Case of Recurrent Hematometra of Unknown Etiology.

An abnormal blood collection in the uterus is referred to as hematometra. Obstruction of the genitourinary outflow system caused by earlier surgeries or congenital defects is most frequently related to this rare disorder. The symptoms of hematometra include acute pelvic pain and a history of absent menarche. Here is a case of a 42-year-old female who presented with complaints of severe lower abdominal pain, and pain during urination that was accompanied by vulval itching in June 2021. She had undergone two Caesarean sections and a myomectomy in the past. She was given three monthly injections of gonadotropin-releasing hormone (GnRH) analogue after receiving USG-guided drainage because of a diagnosis of hematometra in January 2021. However, in June 2021, she experienced a recurrence of the same symptoms, necessitating a total abdominal hysterectomy and bilateral salpingo-oophorectomy, which completely resolved the patient's complaints. For a deeper understanding of this issue, further case reporting is necessary.

Distal vaginal agenesis and differential diagnosis of other causes of hematocolpos.

Distal vaginal agenesis is an obstructive congenital malformation of the female reproductive tract. The distal part of the vagina is replaced by fibrous tissue and the outflow of the menstrual blood and secretion of the cervical glands is disabled. This congenital anomaly most often manifests during expected menarche by primary amenorrhea and cyclic lower abdominal pain caused by cryptomenorrhea with gradually increasing hematocolpos and hematometra. The dia-gnosis is based on gynecological examination and the suspected anomaly is confirmed by ultrasound examination and magnetic resonance imaging. Therapy of distal vaginal agenesis is exclusively surgical. A pull-through vaginoplasty is the method of choice for distal vaginal agenesis not exceeding 3cm. With a greater extent of agenesis and the risk of postoperative vaginal stenosis, replacement of the missing part of the vagina with other tissues or modified balloon vaginoplasty can be used. The aim of the treatment is to enable the evacuation of the menstrual blood, ensure quality sexual intercourse and the possibility of reproduction.

Reassessment of Surgical Procedures for Complex Obstructive Genital Malformations: A Case Series on Different Surgical Approaches.

The objective of this case series was to describe different uterus-preserving surgical approaches and outcomes in patients with complex obstructive Müllerian duct malformation caused by cervical and/or vaginal anomalies. A retrospective analysis was performed including patients undergoing uterovaginal anastomosis (n = 6) or presenting for follow-up (n = 2) at the Department for Gynecology at the University of Tuebingen between 2017 and 2022. Uterovaginal anastomosis was performed with a one-step combined vaginal and laparoscopic approach (method A), a two-step/primary open abdominal approach with primary vaginal reconstruction followed by abdominal uterovaginal anastomosis after vaginal epithelization (method B) or an attempted one-step approach followed by secondary open abdominal uterovaginal anastomosis due to reobstruction (method A/B). Patients presented at a mean age of 15 years. Two patients were treated by method A, four by method B and two by method A/B. Functional anastomosis was established in seven of eight patients, with normal vaginal length in all patients. Concerning uterovaginal anastomosis, the primary open abdominal approach with or without previous vaginal reconstruction seems to have a higher success rate with fewer procedures and should be implemented as standard surgical therapy for complex obstructive genital malformations including the cervix.

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.