Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 65
Filtrar
1.
Case Rep Womens Health ; 43: e00647, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39286600

RESUMEN

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital condition characterized by renal agenesis, uterine didelphys, and obstructed hemivagina. This report presents the case of a 19-year-old woman who reported lower abdominal pain and offensive vaginal discharge. Imaging revealed a didelphys uterus, two vaginas, two cervixes, hematocolpos, and an absent right kidney. Surgical intervention involved draining the hematocolpos and excising the uterine septum. After surgery, the patient successfully conceived and had a full-term pregnancy, delivering via cesarean section without complications. This case highlights the importance of early diagnosis and surgical management in preventing complications such as endometriosis and infertility. Prompt recognition and treatment are crucial for preserving fertility in patients with HWW syndrome.

2.
J Med Case Rep ; 18(1): 361, 2024 Aug 03.
Artículo en Inglés | MEDLINE | ID: mdl-39095912

RESUMEN

INTRODUCTION: Herlyn-Werner-Wunderlich syndrome , a rare Müllerian ducts congenital disease, is characterized by a diphtheritic uterus, blind hemivagina, and ipsilateral renal agenesis. Diagnosis is at young age by ultrasound and magnetic resonance imaging, and the prognosis is good. Usually, complications evolve endometriosis and secondary pelvic inflammation. CASE REPORT: A 40-year-old female patient, Brazilian, white, primigravida, diagnosed at 30 years with a didelphic uterus on ultrasound, and 4 years later, with a left ovarian endometrioma, multiple ovarian cysts, and left renal agenesis on magnetic resonance imaging. Subsequently, due to dyspareunia and a feeling of swelling, the patient underwent transvaginal ultrasound with bowel preparation, and a hematocolpos was found and Herlyn-Werner-Wunderlich syndrome was suspected; 10 years after the diagnosis she had a planned pregnancy. She presented frequent contractions following the 15th week of pregnancy and fortunately there were no complications or premature labor. Labor was inducted at 40 weeks and 6 days without progress and a cesarean section was indicated and performed without complications. Herlyn-Werner-Wunderlich syndrome often goes unnoticed, leading to inadequate treatment. Individuals with Herlyn-Werner-Wunderlich syndrome commonly face fertility issues, such as high miscarriage rate (21-33%), and obstetric complications, such as spontaneous abortions (40% risk), intrauterine growth restriction, postpartum hemorrhage, increased fetal mortality, preterm delivery (21-29%), and elevated rates of cesarean sections. In addition, there is higher susceptibility of developing endometriosis, especially with hemivaginal obstruction, and pelvic adhesions. CONCLUSION: Early diagnosis enables timely treatment and, consequently, fewer complications. Still, when these factors are absent, vaginal birth may still be possible. The true prevalence and incidence of complications related to Herlyn-Werner-Wunderlich syndrome are still unknown.


Asunto(s)
Endometriosis , Humanos , Femenino , Adulto , Endometriosis/complicaciones , Embarazo , Útero/anomalías , Útero/diagnóstico por imagen , Cesárea , Riñón/anomalías , Conductos Paramesonéfricos/anomalías , Anomalías Múltiples , Anomalías Congénitas/diagnóstico , Anomalías Congénitas/diagnóstico por imagen , Vagina/anomalías , Complicaciones del Embarazo , Enfermedades Renales/congénito , Enfermedades Renales/diagnóstico
3.
Int J Surg Case Rep ; 121: 110029, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39013247

RESUMEN

INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS) is characterized by uterine didelphys, unilateral cervical obstruction, and ipsilateral renal defects. Owing to its rarity, no standard surgical approach exists. PRESENTATION OF CASE: An 11-year-old girl with severe dysmenorrhea had a duplicated uterus, a right cervical hemorrhagic cyst, and right ipsilateral kidney agenesis, indicative of HWWS. As transvaginal surgery was challenging, we turned to laparoscopic surgery for abdominal cavity inspection and surgical assistance. A longitudinal incision was made on the right uterus, followed by inserting a catheter tube fixed to an intrauterine device (IUD) into the right cervical canal from the anterior wall of the right uterine horn. Subsequently, the right external cervical os was inverted to prevent restenosis. Postoperatively, the hemorrhagic cyst at the right cervix disappeared. The patient had no symptom recurrence 24 months after the surgery. DISCUSSION: The preoperative diagnosis for female genital malformations is complicated, and transvaginal manipulation is often difficult in adolescent girls. Laparoscopy is a valuable tool for evaluating female genital malformations, allowing for a thorough diagnosis and safe surgical treatment. In cases of female genital malformation with cervical obstruction, as in this case, reconstruction of the uterine cervix is important to prevent restenosis after surgery. CONCLUSION: In female genital malformations, laparoscopy provides a comprehensive evaluation of the malformation, assisting in a precise diagnosis and safe surgical treatment. Insertion of the catheter tube with IUD into the uterus and reconstruction of the cervix contribute to preventing restenosis.

4.
Radiol Case Rep ; 19(9): 3667-3671, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38983302

RESUMEN

Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA) also known as Herlyn-Werner-Wunderlich syndrome is a rare type of Müllerian duct anomaly with concomitant mesonephric duct anomalies. It is characterized by uterus didelphys, obstructed hemi-vagina, and commonly renal agenesis however; other types of mesonephric duct anomalies such as duplicated kidneys, dysplastic kidneys, rectovesical bands, or crossed fused ectopia have also been reported. We present a case report of a 21-year-old female patient experiencing new-onset vaginal bleeding and pelvic pain over the past month. Subsequent multi-sequence MRI revealed characteristic features indicative of OHVIRA syndrome. Clinical manifestations typically encompass pelvic pain and menstrual abnormalities but mainly depend upon the presence of communication between the hemi-uterus. This article aims to discuss the clinical presentation, types, and diagnostic utility of ultrasound and MRI in OHVIRA syndrome. Additionally, we discuss the possible management plans.

5.
Reprod Sci ; 2024 Jun 21.
Artículo en Inglés | MEDLINE | ID: mdl-38907127

RESUMEN

PURPOSE: To describe the clinical and imaging characteristics of Herlyn-Werner-Wunderlich syndrome (HWWS). METHODS: This study presented an observational case series involving consecutive patients diagnosed with HWWS, whose medical records were retrospectively reviewed. From June 2012 to December 2022, there were a total of 85 patients with HWWS enrolled in our study. We obtained the medical history, including demographic characteristics, clinical presentation, treatment, complications, and radiologic examinations performed. Patients > 18 years of age (n = 58) were recontacted. RESULT: In our analysis, 27 patients were categorised as having complete obstruction, and 58 were categorised as having incomplete obstruction. The mean age at the onset of symptoms and diagnosis of complete obstruction was significantly younger than incomplete obstruction (P < 0.05). For complete obstruction, the median time between menarche and the onset of symptoms was 2.1 years, while for incomplete obstruction, it was 5.3 years. There was a significantly lower incidence of intermittent mucopurulent discharge, irregular vaginal haemorrhage, and occasional examination findings of complete obstruction than incomplete obstruction (P < 0.05). Complete obstruction was significantly associated with dysmenorrhea and pelvic endometriosis compared with incomplete obstruction (P < 0.05). CONCLUSIONS: There are distinct clinical differences between patients with complete obstruction of the hemivagina and those with incomplete obstruction. HWWS can manifest as various combinations of uterine anomalies, communications anomalies, and renal anomalies. Early recognition and treatment can avoid complications and preserve fertility. KEYSWORDS: Herlyn-Werner-Wunderlich syndrome (HWWS); complete obstruction; incomplete obstruction; obstructed hemivagina; congenital malformation.

6.
Endocrinol Diabetes Nutr (Engl Ed) ; 71(4): 171-176, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38735678

RESUMEN

Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.


Asunto(s)
Enfermedades Renales/congénito , Riñón , Síndrome de Prader-Willi , Útero , Vagina , Humanos , Femenino , Adulto , Síndrome de Prader-Willi/complicaciones , Vagina/anomalías , Vagina/cirugía , Riñón/anomalías , Útero/anomalías , Útero/diagnóstico por imagen , Anomalías Múltiples , Hematómetra/etiología , Hematocolpos/etiología , Anomalías Urogenitales/complicaciones , Anomalías Congénitas , Dolor Abdominal/etiología
7.
J Indian Assoc Pediatr Surg ; 29(2): 180-182, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38616843

RESUMEN

Zinner syndrome, a rare congenital anomaly affecting males, is characterized by atresia of the ejaculatory duct, seminal vesicle cysts, and ipsilateral renal agenesis. This case report details a 2-year-old boy successfully treated with laparoscopic excision of a dilated vas deferens and seminal vesicle cyst. The rarity of Zinner syndrome in pediatric patients underscores the importance of understanding its diagnosis and minimally invasive surgical management.

8.
J Pediatr Adolesc Gynecol ; 37(1): 72-77, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-37558159

RESUMEN

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.


Asunto(s)
Enfermedades Renales , Anomalías Urogenitales , Didelfo Uterino , Femenino , Humanos , Riñón/anomalías , Vagina/cirugía , Vagina/anomalías , Estudios Retrospectivos , Útero/cirugía , Útero/anomalías , Imagen por Resonancia Magnética
9.
J Pediatr Adolesc Gynecol ; 37(2): 198-204, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38070700

RESUMEN

STUDY OBJECTIVE: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) includes uterine didelphys, unilateral obstructed hemivagina, and ipsilateral renal anomaly. Surgical management of this condition relies on accurate diagnosis to excise the obstructed longitudinal vaginal septum (OLVS). Vital considerations involve identifying the side affected, ipsilateral renal anomaly (IRA), thickness of the septum (TS), septal axis (SA), and distance of the septum to perineum (DSP). The study aimed to evaluate the preoperative characteristics, imaging findings, and surgical outcomes of OHVIRA. METHODS: Institutional review board approval was obtained for this retrospective chart review. ICD-10 codes identified OHVIRA cases between 2012 and 2019 at a single children's hospital. Patient demographic characteristics, magnetic resonance imaging findings, surgical management, outcomes, and complications were reviewed. Descriptive statistics were utilized. RESULTS: Twenty-six patients met inclusion criteria. Most were diagnosed at puberty (92%). Abdominal pain (50%) was the most common presenting complaint. The mean age of diagnosis was 13.2 years overall and 11.2 years for those with regular cycles vs 13.4 years for those with irregular cycles. Preoperative imaging showed predominant right-sided OLVS (50%), IRA (77%), and oblique SA (65%). All patients underwent vaginoscopy, septum resection, and vaginoplasty, except 1 who was managed with an abdominal drain as a neonate. Four required postoperative vaginal stent or Foley, with DSP greater than 5 cm in all cases. One intraabdominal abscess complication occurred. No instances of hematocolpos re-accumulation or reoperation were observed during the 3-year follow-up period. CONCLUSION: This study demonstrates that detailed preoperative planning and a systematic surgical approach lead to favorable outcomes in OHVIRA irrespective of the OLVS laterality, TS, SA, or DSP.


Asunto(s)
Anomalías Múltiples , Enfermedades Renales , Enfermedades Vaginales , Niño , Femenino , Recién Nacido , Humanos , Adolescente , Riñón/anomalías , Anomalías Múltiples/cirugía , Estudios Retrospectivos , Enfermedades Renales/diagnóstico , Útero/anomalías , Vagina/cirugía , Vagina/anomalías , Imagen por Resonancia Magnética/métodos
10.
J Pediatr Adolesc Gynecol ; 37(2): 205-208, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38122961

RESUMEN

STUDY OBJECTIVE: To compare the anatomic variation between patients with a diagnosis of an obstructed hemivagina with an anorectal malformation (ARM) and those without an ARM. METHODS: This was a retrospective chart review conducted at a single tertiary children's hospital. Patients with an obstructed hemivagina seen from 2004 to 2019 were included. RESULTS: We identified a total of 9 patients diagnosed with an obstructed hemivagina: 4 patients with a history of ARM and 5 patients without an ARM. Patients presented with obstructive symptoms between the ages of 11 and 20. Two-thirds of patients had a left-sided obstruction. All patients without an ARM had ipsilateral congenital anomalies of the kidney and urinary tract. Half the patients with a history of ARM had an ipsilateral renal anomaly, and the other half had a contralateral renal anomaly. CONCLUSION: Obstructed hemivagina occurs in patients with a history of ARM. However, unlike patients with isolated obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), patients with an ARM and an obstructed hemivagina can present with associated renal anomalies on either the ipsilateral or contralateral side. In our small case series, patients with a history of ARM had high septa and required more complex surgical management due to the inability to access the septum vaginally. Knowledge of renal anatomy and ureteral path is important because a hysterectomy may be needed to relieve the obstruction in patients with ARMs. A larger case series is needed to better characterize the spectrum of complex anomalies in patients with ARMs.


Asunto(s)
Malformaciones Anorrectales , Enfermedades Renales , Niño , Femenino , Humanos , Adolescente , Adulto Joven , Adulto , Malformaciones Anorrectales/complicaciones , Malformaciones Anorrectales/cirugía , Útero/anomalías , Vagina/cirugía , Vagina/anomalías , Estudios Retrospectivos , Enfermedades Renales/congénito , Riñón/anomalías
11.
J Hum Reprod Sci ; 16(3): 257-259, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38045505

RESUMEN

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a very rare type of Müllerian duct anomaly. Cases of OHVIRA syndrome have varied clinical manifestations. We report the case of a 15-year-old virgin with endometrial cyst and OHVIRA syndrome. Magnetic resonance imaging confirmed the diagnosis of OHVIRA syndrome. A salpingo-oophorectomy laparotomy was performed, but the patient refused to perform a septotomy or vaginal septal resection. We compared the cases of OHVIRA syndrome that we found with several other reports. The complaints of patients with OHVIRA syndrome may vary from one another, with different histories. Imaging investigations can confirm the diagnosis. The main treatment in OHVIRA syndrome is intended to alleviate symptoms that appear.

12.
Gynecol Minim Invasive Ther ; 12(3): 123-129, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37807996

RESUMEN

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare congenital defect of the Müllerian ducts characterized by uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. The aim of this systematic review is to summarize the main symptoms and presentation of the OHVIRA syndrome, as well as the different types of management, fertility, and obstetrical outcomes. A comprehensive search was performed in PubMed, EMBASE, SCOPUS, and Web of Science databases since inception to May 1, 2022, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. After duplicate records removed, the search strategy retrieved 103 articles. The full texts of 73 articles further were assessed for eligibility, and 44 studies were finally included in the systematic review. The mainstay surgical treatment of OHVIRA syndrome is usually a minimally invasive vaginal approach to remove the septum. Ultrasound-guided hysteroscopic resection and laparoscopic resection of the septum have been described as alternatives. Considering the feasibility of minimally invasive approach for the management of the syndrome, laparotomy should be avoided as much as possible and considered only in selected cases.

13.
J Pediatr Adolesc Gynecol ; 36(6): 541-544, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37451429

RESUMEN

OBJECTIVES: The objectives of the study were to compare the demographic characteristics, presenting complaints, timeliness of diagnosis, and treatments given to all patients diagnosed with obstructive hemivagina ipsilateral renal agenesis (OHVIRA) between the adolescent group (age 10-19) and the adult group (age 20 and above) and to propose a feasible screening test consisting of routine bedside ultrasound to detect renal anomalies in addition to pelvic ultrasound during the assessment of females with gynecologic complications in resource-limited settings. METHOD AND ANALYSIS: We conducted a retrospective cohort analysis of all patients with OHVIRA at our institution, Sabah Women and Children's Hospital, Malaysia, between the 2013 and 2022. Data were collected by reviewing patients' medical notes. RESULTS: There were a total of 18 patients diagnosed with OHVIRA from 2013 to 2022, aged 10-41 years old. Nine patients (50%) presented during adolescence. Most (88.9%) came with symptoms such as abdominal pain, urinary symptoms, abnormal uterine bleeding, foul-smelling vaginal discharge, and primary infertility, whereas only 2 patients (11.1%) were asymptomatic and diagnosed incidentally. Acute abdomen was more common in the adolescent group (P = .015). No significant difference was found on the side of the renal anomaly. CONCLUSION: Presenting symptoms vary and are often mimicked other gynecologic or surgical conditions, making the diagnosis difficult and delayed. Some patients were incidentally diagnosed while being managed for other problems, and it was not uncommon to have unnecessary surgery before the correct diagnosis was made. We suggest that all female patients with abdominal and pelvic complaints should be screened for renal anomaly during pelvic scan to improve diagnostic rates.


Asunto(s)
Anomalías Múltiples , Vagina , Niño , Adulto , Adolescente , Femenino , Humanos , Adulto Joven , Malasia , Estudios Retrospectivos , Vagina/anomalías , Anomalías Múltiples/cirugía , Riñón/diagnóstico por imagen , Riñón/anomalías , Síndrome , Útero/anomalías
14.
Radiol Case Rep ; 18(8): 2771-2784, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-37388267

RESUMEN

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.

16.
Medicina (Kaunas) ; 59(4)2023 Apr 04.
Artículo en Inglés | MEDLINE | ID: mdl-37109661

RESUMEN

Background: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Most cases of OHVIRA have been reported in adolescents or adults. Gartner duct cysts, including those manifesting as vaginal wall cysts, are also rare. Fetal OHVIRA syndrome and Gartner duct cysts are difficult to diagnose. Case Presentation: Here, the authors report a case of combined OHVIRA and Gartner duct cyst diagnosed prenatally by ultrasonography, along with a brief review of the relevant published reports. A 30-year-old nulliparous female was referred to our institution at 32 weeks' gestation for fetal right kidney agenesis. Detailed ultrasonographic examinations using 2D, 3D, and Doppler ultrasounds revealed hydrocolpometra, and uterus didelphys, with a normal anus and right kidney agenesis. Conclusions: When encountering female fetuses with ipsilateral renal agenesis or vaginal cysts, clinicians should be aware of OHVIRA syndrome and Gartner duct cysts and perform systematic ultrasonographic examinations for other genitourinary anomalies.


Asunto(s)
Anomalías Múltiples , Vagina , Adulto , Embarazo , Adolescente , Femenino , Humanos , Vagina/diagnóstico por imagen , Vagina/anomalías , Riñón/diagnóstico por imagen , Riñón/anomalías , Anomalías Múltiples/diagnóstico por imagen , Diagnóstico Prenatal , Feto/diagnóstico por imagen
17.
Ann Med Surg (Lond) ; 85(4): 1223-1226, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-37113823

RESUMEN

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare developmental anomaly of the female reproductive system caused by a failure of fusion during Mullerian duct development. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes HWWS. The most common presenting symptoms are dysmenorrhoea, pelvic pain, primary infertility in later years, and an abdominal mass due to hematometrocolpos. Case Presentation: A 17-year-old girl presented to the authors' department with recurrent low back pain, which was neither responsive to analgesics nor associated with urinary complaints, vomiting, or fever. Imaging techniques confirmed she had the triad of uterus didelphys, obstructed hemivagina, and right renal agenesis. Clinical Discussion: The genital system is the same for males and females before 6 weeks of pregnancy. HWWS is a rare congenital disorder as a result of the failure of fusion during Mullerian duct development. It consists of a didelphic uterus, hemivaginal septum, and unilateral renal agenesis. Conclusion: Shame and social stigma associated with virginity continue to endanger the lives of many girls in Syria. To complicate matters further, the low resources in Syria produced by war pose a difficult challenge in managing many gynecological conditions, including HWWS, like this case, in which endoscopic technologies were not available, necessitating open surgery while keeping in mind preserving hymen intactness. So, the authors indicate that preserving virginity could be conducted even though the approach is open surgery by very careful intervention and experienced surgeons.

18.
J Minim Invasive Gynecol ; 30(4): 262-263, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36754275

RESUMEN

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.


Asunto(s)
Enfermedades Renales , Menarquia , Niño , Femenino , Humanos , Riñón/anomalías , Útero/diagnóstico por imagen , Útero/cirugía , Útero/anomalías , Vagina/diagnóstico por imagen , Vagina/cirugía , Vagina/anomalías
19.
Arch Gynecol Obstet ; 308(5): 1409-1417, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-36823415

RESUMEN

PURPOSE: This study aimed to perform a systematic review of patients with Herlyn-Werner-Wunderlich syndrome (HWWS) and present the prevalence of symptoms, anatomical variants, endometriosis, surgical interventions, and short- and long-term outcomes. METHODS: A structured search was performed in PubMed, Scopus, Embase, and China National Knowledge Infrastructure, and studies published between 1 January, 2000 and 19 April, 2022 were included. The following data on HWWS were recorded: symptoms, anatomical variations, surgical interventions and short- and long-term outcomes. RESULTS: A total of 1673 patients were included in our analysis. The main symptoms were dysmenorrhea (53.8%), abnormal uterine bleeding (28.9%), and vaginal discharge (26.6%). The principal anomalies were right-obstructed hemivagina (57.3%), haematocolpos (81.7%), uterus didelphys (88.8%), and ipsilateral renal agenesis (93.1%). A majority of patients belonged to classification 1.1 (46.7%), with a blind hemivagina, and classification 2.1 (39.2%), with a small communication between two vaginas. The mainstay of treatment was vaginal septum excision (91.8%). Minimally invasive surgery (48.5%) was performed only after vaginal surgery (61.9%), and only a few patients required a second surgery (2.2%). Endometriosis was found in 9.6% of the patients. Fifty-two percent of them had ipsilateral ovarian endometriosis cysts. Pregnancy rate of these patients was 72.1%. The rate of adverse pregnancy outcomes was 22.4%. The caesarean section rate was 61.2%. CONCLUSIONS: Patients with HWWS presented with nonspecific symptoms and demonstrated various combinations of anomalies. The most common anatomical variants are classifications 1.1 and 2.1. Vaginal septum excision is effective in relieving symptoms and preventing complications, with hysteroscopic surgery as an option where there is concern about protecting the hymen from minor injury. The pregnancy rate for these patients after surgery was satisfactory, and the rate of adverse pregnancy outcomes after surgery was acceptable. We advise females with urological anomalies to be screened for Müllerian anomalies because of the close association between these two types of anomalies. Thus, HWWS contributes to the occurrence of endometriosis; however, more research is required to investigate the relationship between pelvic endometriosis and HWWS.


Asunto(s)
Anomalías Múltiples , Endometriosis , Humanos , Embarazo , Femenino , Cesárea/efectos adversos , Endometriosis/complicaciones , Endometriosis/cirugía , Riñón/anomalías , Útero/cirugía , Útero/anomalías , Vagina/cirugía , Vagina/anomalías , Anomalías Múltiples/epidemiología , Anomalías Múltiples/cirugía , Resultado del Tratamiento
20.
J Obstet Gynaecol Res ; 49(1): 350-355, 2023 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-36245420

RESUMEN

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare malformation that not only causes severe menstrual cramps shortly after menarche but can also lead to endometriosis and infection in the future. We report a case of OHVIRA successfully managed by vaginoscopic excision of the vaginal septum. A 12-year-old virgin girl presented to our hospital with dysmenorrhea and lower abdominal pain. OHVIRA was diagnosed using magnetic resonance imaging. Vaginoscopic surgery was performed for drainage of hematocolpos and excision of the vaginal septum. Vaginoscopic excision of the vaginal septum was performed using a resectoscope, without a vaginal speculum. The procedure was completed safely without injuring the hymen. This is the first case report of successful excision of the vaginal septum by vaginoscopic surgery for OHVIRA in Japan. Vaginoscopic excision may be one of the effective options for the treatment of vaginal obstruction.


Asunto(s)
Enfermedades Renales , Riñón , Femenino , Humanos , Niño , Riñón/cirugía , Riñón/anomalías , Vagina/anomalías , Endoscopía/métodos , Dismenorrea , Útero/cirugía , Útero/anomalías
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA