Uterine Remnants Research Articles

Meeting Report: First State-of-the-Art Meeting on Uterus Transplantation.

Meeting Report: First State-of-the-Art Meeting on Uterus Transplantation.

Diagnosis and Management of Ovarian Tumor in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome.

In the most recent publications on Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, the uterine remnants and ovaries in patients may develop uterine remnant leiomyoma, adenomyosis, or ovarian tumor, and this can lead to problems in differential diagnosis. Here we summarize the diagnosis methods and available interventions for ovarian tumor in MRKH syndrome, with emphasis on the relevant clinical findings and illustrative relevant case. According to the clinical findings and illustrative relevant case, with the help of imaging techniques, ovarian tumors can be detected in the pelvis in patients with MRKH syndrome and evaluated in terms of size. Laparoscopy could further differentiate ovarian tumors into different pathological types. In addition, laparoscopic surgery not only is helpful for the diagnosis of MRKH combined ovarian tumor, but also has a good treatment role for excising ovarian tumor at the same time. Moreover, laparoscopic removals of ovarian tumor can be considered as a safe and reliable treatment for conservative management.

Evaluation of Mayer-Rokitansky-Küster-Hauser syndrome with magnetic resonance imaging: Three patterns of uterine remnants and related anatomical features and clinical settings.

To characterize the anatomical features and clinical settings of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and correlate them with patterns of uterine involvement. Pelvic magnetic resonance images and medical records of 92 MRKH patients were retrospectively reviewed. Patients were subgrouped by uterine morphology: uterine agenesis, unilateral rudimentary uterus and bilateral rudimentary uteri. Uterine volume, presence of endometrium, location of ovary, endometriosis and pelvic pain were compared among groups. The mean uterine volume was 33.5 ml (17.5-90.0 ml) for unilateral uterine remnants, and 16.1 ml (3.5-21.5 ml) for bilateral uterine rudiments (p<0.01). The incidence of presence of endometrium (100% vs. 22%, p<0.001), haematometra (56% vs. 3%, p<0.001) and ovarian endometriosis (22% vs. 3%, p<0.01) was significantly increased in the group of unilateral rudimentary uteri as compared with the group of bilateral uterine remnants. Thirty-one patients (38%) showed ectopic ovaries. Pelvic pain was more common in individuals with unilateral rudimentary uterus than those who had no (56% vs. 5%, p<0.01) or bilateral uterine remnants (56% vs. 14%, p<0.05). MRKH patients with different patterns of uterine involvement may have differentiated anatomical features and clinical settings. • Rudimentary uteri, especially bilateral rudimentary uteri, were quite common in MRKH syndrome. • Uterine remnants can be relatively large, especially the unilateral rudimentary uterus. • Presence of endometrium and related complications are not rare in MRKH syndrome. • Existence of endometrium and complications are more frequent in unilateral rudimentary uterus.

Acute Torsion of Paraovarian Cyst and Ipsilateral Uterine Remnant Leiomyoma in a Patient with Mayer–Rokitansky–Küster–Hauser Syndrome

Acute Torsion of Paraovarian Cyst and Ipsilateral Uterine Remnant Leiomyoma in a Patient with Mayer–Rokitansky–Küster–Hauser Syndrome

Laparoscopic Diagnosis and Management of Bilateral Uterine Remnants

Laparoscopic Diagnosis and Management of Bilateral Uterine Remnants

Comment on case report entitled: adenomyosis of uterus with adenomyoma of fallopian tube.

To the Editor I read an interesting case report titled “adenomyosis of uterus with adenomyoma of fallopian tube” by Ganitha et al that appears in the recent issue of your journal. While reading this, I was pondering that it is adenomyoma in rudimentary horn of uterus instead of adenomyoma of fallopian tube. It may be due to wrong interpretation by pathologist as sample sent might be labeled as uterus with fallopian tube and rudimentary horn may not be mentioned. Although the development of leiomyomas from uterine remnant is a rare finding, it is still possible and it should be considered in the list of differential diagnosis of pelvic masses with mullerian dysgenesis [1].

Acute torsion of uterine remnant leiomyoma with Mayer-Rokitansky-Küster-Hauser syndrome

To report a case of acute abdomen secondary to torsion of uterine remnant leiomyoma and ipsilateral adnexa in a woman with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and known history of bilateral uterine remnant leiomyomas. Case report. Tertiary care center. A 40-year-old nulligravid woman with MRKH syndrome and a known history of bilateral uterine remnant leiomyomas with a surgical abdomen. right salpingo-oopherectomy, excision of right and left hemiuteri with pedunculated leiomyomas, and left salpingectomy. Definitive therapy with preservation of premenopausal state. First known case of acute surgical presentation secondary to torsion of uterine remnant leiomyoma and ipsilateral adnexa in a woman with MRKH syndrome and bilateral voluminous uterine remnant leiomyomas without any plan for elective surgical intervention. Elective surgical removal of uterine remnant leiomyoma for women with MRKH syndrome can prevent the complication of torsion as well as prevent ovarian resection in premenopausal women. Torsion should be considered in the differential diagnosis in a woman with MRKH syndrome and known history of leiomyomas who presents with acute abdomen.

Manejo laparoscópico de miomatosis uterina en pacientes con Síndrome de Rokitansky. Reporte de un caso y revisión de la literatura

Introducción: el síndrome de Mayer-Rokitansky-consulta a la Unidad de Endoscopia Ginecológica de Küster-Hauser (SMRKH) es un espectro de anomalías de los conductos müllerianos caracterizado por la la ausencia congénita de útero y vagina en mujeres fenotípicamente normales, con cariotipo 46 XX, con un estado endocrino normal. Muchas de estas mujeres poseen remanentes uterinos. La presencia de miomas en dichos remanentes es un hallazgo de muy escasa ocurrencia por lo que se reporta el caso.Objetivo: describir la técnica quirúrgica laparoscópica y revisar el diagnóstico clínico y el diagnóstico diferencial.Materiales y métodos: se presenta el caso de una mujer con diagnóstico previo de síndrome de Mayer-Rokitansky-Küster-Hauser (SMRKH), quien consulta a la la Unidad de Endoscopia Ginecológica de la Clínica del Prado en Medellín, institución privada de tercer nivel de complejidad y que es centro de referencia para manejo laparoscópico de pacientes con dolor pélvico crónico del departamento de Antioquia, Colombia. La ecografía reporta una masa sólida de ocho centímetros en la pelvis. Durante la laparoscopia se encuentra un mioma en uno de los remanentes uterinos. Se realiza miomectomía y se resecan ambos remanentes por laparoscopia. Se realizó una búsqueda sistemática de la literatura a través de Medline en la página de Pubmed utilizando los siguientes términos: “Mayer-Rokitansky-Hauser una mujer con diagnóstico previo de síndrome de Syndrome”, “leiomyoma” y “laparoscopy”.Resultados: se encontraron 25 artículos en los últimos 20 años de los cuales 17 estaban relacionados con el tema de manera directa; de estos, 2 incluían el manejo con laparoscopia.Conclusiones: la presencia de leiomioma en un remanente uterino de una paciente con MRKH es poco frecuente. El caso aquí presentado es el segundo reportado en Colombia y el primero manejado por vía laparoscópica. En pacientes con síndrome de Mayer-Rokitansky-Küster-Hauser y masas pélvicas, la laparoscopia es una opción diagnóstica y terapéutica por considerar.

Mayer-Rokitansky-Kuster-Hauser Syndrome: Diagnosis with MR Imaging

To evaluate the diverse magnetic resonance (MR) imaging findings of the pelvis in women with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. This retrospective review had institutional review board approval with waiver of informed consent. Between 2001 and 2011, 215 female patients with MRKH syndrome attended clinics, and 66 underwent pelvic MR imaging (age range, 14-40 years; median age, 19 years). One reviewer reviewed MR images for presence, site, volumes, and differentiation into layers (myometrium, junctional zone, and endometrium) of uterine remnants. Ovarian volumes and positions were assessed. Vaginal length was measured. Rudimentary uteri were found in 61 patients (92%); 54 were bilateral, and seven were unilateral. All uterine buds were located laterally in the pelvis and had a constant caudal relationship with their paired ovary. Mean uterine volume was 6.4 mL (range, 0.4-80.2 mL), and 18 uteri had a volume greater than 10 mL. Twenty-four uterine buds (21%) showed differentiation into more than one layer. Two uteri contained intraluminal blood, and two showed signs of adenomyosis, indicating functioning endometrial tissue; these patients had cyclical pain. Bilateral ovaries were present in 54 patients; ovaries were ectopic in 27 patients. Twenty-two patients had no discernible vagina (dimple or less). Of the 44 patients with a vagina, the mean length was 2.0 cm (range, 1.0-6.5 cm). Rudimentary uteri are common in patients with MRKH syndrome. They can be relatively large and have functioning endometrium, which can be associated with pain. Uteri have a constant caudal relationship to ovaries. Ovaries are commonly ectopic, and this must be recognized in patients undergoing fertility treatment. Online supplemental material is available for this article.

Surgical Pearls: Laparoscopic Removal of Uterine Remnants in Patients with Mayer-Rokitansky-Küster-Hauser Syndrome

BackgroundFemales with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome may require surgical removal of uterine remnant(s) which can be accomplished with a laparoscopic approach, described in this case series. CasesNine females with MRKH and pelvic pain were treated with laparoscopic resection of uterine remnants without major complication. The following management recommendations are offered: (1) preoperative evaluation for urinary tract anomalies and postoperative cystoscopy; (2) medial traction of the remnant to allow adequate exposure of the pelvic sidewall; (3) awareness of possible anomalous vascular supply to uterine remnant; (4) individualized management of associated endometriosis; (5) careful use of surgical terminology, avoiding use of the word hysterectomy. Summary and ConclusionLaparoscopic removal of uterine remnant(s) is safe and effective.

A Spayed Female Cat with Squamous Cell Carcinoma in the Uterine Remnant

A 7-year-old spayed female domestic short-haired cat presented with dysuria and hematuria that had been unresponsive to medical therapy. Imaging tests such as ultrasonography, urethrocystography and computed tomography revealed a pelvic mass compressing the urethra. Based on histological examination of the mass following surgical resection, the cat was diagnosed squamous cell carcinoma (SCC) derived from the uterine remnant. After surgery, dysuria was resolved, but on instead, urine and fecal incontinence were observed. Then, about four months after surgery, recurrence of the mass and the symptoms was observed. Consequently, the cat was ultimately euthanized. This is the first report of SCC arising from the uterine remnant in a spayed female cat.

Mayer–Rokitansky–Kuster–Hauser Type-B Anomaly with MURCS Association and Gonadal Dysgenesis

The Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome affects 1 out of 4,500 women [1]. It is a malformation of the female genitals due to interrupted embryonic development of the mullerian (paramesonephric) ducts in otherwise chromosomally, phenotypically, and endocrinologically normal female. It is second to Turner’s syndrome as a cause of primary amenorrhea and was described by Mayer (1829), Rokitansky (1838), Kuster (1910), and Hauser and Schreiner (1961) in various literature studies, which was later designated as MRKH syndrome. MRKH syndrome is broadly subdivided into type A (typical) having symmetric uterine remnants and normal fallopian tubes and type B (atypical) with asymmetric uterine buds and abnormally developed fallopian tubes and other organ system anomalies. Gonadal agenesis or dysgenesis is a chromosomal aberration with a separate spectrum of anomalies having an overall incidence of about 1:2,500 live birth females, half of which have a mosaic pattern. These patients have multiple somatic abnormalities like short stature, broad chest, webbed neck, low hair line, sometimes low I.Q., cardiac abnormalities (1/3 cases), and renal abnormalities (35–70 %). They usually have pre-pubertal female genitalia, bilateral streak gonads, but usually normal uterus and vagina (Figs ​(Figs1,1, ​,22). Fig. 1 Photograph showing webbing of neck Fig. 2 Photograph showing the absence of left thumb and rudimentary right thumb Case Report An 18-year-old girl with primary amenorrhea attended the O.P.D. with normal I.Q. (1st year college student) and without such family history. On examination, her height is 154 cm, weight—42 kg, with webbing of neck, telecanthus; and her left thumb was absent and right thumb rudimentary. Systemic examination was normal. Breasts were well developed and axillary and pubic hairs were present. On perineal examination, there was a hymenal fringe and blind vagina. Uterus was absent on per rectal examination. Routine examination of blood, ECG, and chest X-ray reported normal. X-ray cervical spine showed fusion of the cervical vertebrae (Klippel Feil Syndrome). Serum FSH, LH, TSH, and testosterone were normal. USG [02/10/07] showed right-sided crossed fused ectopic kidney with normal corticomedullary differentiation, uterus absent, and normal gonads. Diagnostic laparoscopic [04/10/07] finding was asymmetric mullerian nodule [rt > lt] with rudimentary fallopian tubes, and both ovaries were normal with signs of ovulation. Karyotyping was Turner’s mosaics pattern (46XX/45XO). Diagnosis of MRKH type-B with gonadal dysgenesis was made. Vaginoplasty was done on 12/10/07 after proper counseling as parents wanted to arrange marriage. Post-operative period was uneventful.

Patients with disorders of sex development (DSD) at risk of gonadal tumour development: management based on laparoscopic biopsy and molecular diagnosis

Study Type--Therapy (case series) Level of Evidence 4. What's known on the subject? and What does the study add? In some individuals with disorders of sex development (DSD), gonadal tumour risk is increased. The individual risk is estimated based on the molecular diagnosis and the age and approaches 30% in the high-risk group. In the past, early gonadectomy has been advised for all individuals with 46XY DSD. Gonadectomy clearly represents an overtreatment for many individuals with 46XY DSD. Thus, further clinical indicators of individual tumour risk are urgently needed. The present study provides a comprehensive description of gonadal morphology, as seen during laparoscopy. For the first time, laparoscopic features, molecular diagnosis and histopathological findings are presented in a comprehensive context. The present study adds a detailed morphological description of the variability found in different subgroups of 46XY DSD. As three of four detected tumours were microscopic, early diagnosis by inspection appears unfeasible. Biopsy, gonadopexy and precise localisation of the gonad will potentially allow for gonadal preservation in well-defined clinical situations. • To investigate the role of laparoscopy for the early detection of gonadal tumours, with emphasis on gonadal preservation, in patients with 46XY disorders of sex development (DSD). In patients with DSD, gonadectomy is frequently recommended and depending on the age and the molecular diagnosis, an increased gonadal tumour risk exists and undesired hormone effects may arise. However, gonadectomy is irreversible and impacts considerably on body image. It represents an overtreatment for some patients and should be considered after a comprehensive diagnostic evaluation. Laparoscopy is an important technique, because it is able to retrieve small gonads and allows guided biopsies. • We performed laparoscopic assessment of the gonads in 40 patients with various 46XY DSD. • In all, 77 gonads were evaluated, images were analysed and compared with histological findings. • Laparoscopic procedures included gonadectomy, biopsy, laparoscopic orchidolysis or the Fowler-Stephens procedure as well as the removal or splitting of uterine remnants. • In all, 19 patients underwent gonadectomy and tumours were discovered in four. • Three patients had only microscopic evidence of tumour, in one the tumour was diagnosed intraoperatively. • In 21 patients, biopsies were taken and the gonads preserved. • Laparoscopic biopsy and gonadopexy was performed in six patients with complete androgen insensitivity syndrome (CAIS). • Laparoscopy and biopsy detected three microscopic tumours, one tumour was macroscopically evident. • In CAIS, gonadopexy improved the visibility of the gonads on postoperative ultrasonography. This procedure facilitated the examination of the gonad at follow-up. • In complete gonadal dysgenesis, a highly variable morphology of the gonads was found. Laparoscopy improved exposure of gonads and Müllerian structures, and facilitated biopsies and organ-preserving procedures.

Vaginal agenesis or distal vaginal atresia associated with anorectal malformations

BackgroundMayer-Rokitansky-Küster-Hauser (MRKH) syndrome is defined as Müllerian aplasia with vaginal agenesis and uterine remnants. It is commonly associated with renal and sometimes vertebral anomalies. The MRKH syndrome or distal vaginal atresia is sometimes associated with anorectal malformations. The purpose of this study was to describe 7 girls with vaginal agenesis or distal vaginal atresia and an anorectal malformation and review the literature. MethodsSeven patients with vaginal agenesis or distal vaginal atresia and anorectal malformation were operated on at 3 pediatric surgical centers in Madrid, Helsinki, and Stockholm. Case records were reviewed, and the previous literature was searched. ResultsSix patients had a reconstruction of the anorectum and vagina during the first year of life. In one case, the vagina was replaced at the time of a redo posterior sagittal anorectoplasty at the age of 11 years. The 4 patients with vaginal agenesis had a sigmoid colovaginoplasty. The 3 patients with a distal vaginal atresia had a vaginal pull-through. Four of the patients needed laxatives or enemas for mild constipation at last follow-up. Short-term gynecologic problems were minor in all patients. ConclusionVaginal reconstruction at the time of anorectoplasty results in good short-term outcome. For vaginal agenesis, a primary colovaginoplasty is suggested to be the preferred technique to replace the vagina.

Uterine remnants and pelvic pain in females with mayer-rokitansky-kuster-hauser syndrome

Objective: To assess the association between pelvic pain and uterine remnants and review the management of pelvic pain in females with Mayer-Rokitansky-K€ uster-Hauser (MRKH) syndrome. Design: Retrospective cohort. Setting: Department of Obstetrics and Gynecology at a tertiary referring medical center. Patients: Forty-eight females with MRKH presenting from 1997 to 2011 with anatomy confirmed by magnetic resonance imaging (MRI). Interventions: None. Main Outcome Measure: Prevalence Of uterine remnants and the association of uterine remnants with pelvic pain in females with MRKH. Results: Of the 48 females with MRKH, 23 (48%) had uterine remnants and 22 (46%) had pelvic pain. Presence of endometrium was associated with pelvic pain (RR 5 2.3; 95% CI 5 1.2-4.7) in females with MRKH. Of the females with MKRH and pain, 9/22 had laparoscopy, with endometriosis seen in 5/9 of the uterine remnants at stages higher than are usually seen in teenagers (56%). Nine patients with pain and uterine remnants (8 with endometrium, 1 without) had laparoscopic removal of uterine remnants with resolution of pain. Conclusions: Given the high prevalence of uterine remnants in females with MRKH, anatomic evaluation with MRI should be considered when assessing the etiology of pelvic pain. Presence of endometrium within uterine remnants, and subsequent endometriosis, in females with MRKH may be associated with pelvic pain necessitating surgical or medical management.

Laparoscopic Removal of Uterine Remnants in Teens With Mayer-Rokitansky-Kuster-Hauser Syndrome and Pelvic Pain

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital anomaly of the Mullerian tract characterized by agenesis of the upper 2/3 of the vagina, cervix and uterus seen in 1/4,500 females. In those with pelvic pain, the differential diagnosis includes the presence of a functioning uterine remnant, which occurs in 7-10%. MRI is the gold standard for evaluation of these anatomic variants. Our objective is to review a series of patients with MRKH syndrome requiring surgical removal of uterine remnants for pelvic pain.

Leiomioma uterino en paciente con síndrome de Mayer-Rokitansky-Küster-Hauser: reporte de caso

Objetivo: el síndrome de Mayer-Rokitansky-Küster-Hauser (MRKH) es una malformación congénita del útero y la parte superior de la vagina en las mujeres que muestran características sexuales secundarias normales y cariotipo 46 XX. Puede ocurrir aplasia completa del útero en presencia de dos cuernos rudimentarios o hipoplasia uterina simétrica o asimétrica acompañada por aplasia de uno de los dos cuernos. Es poco frecuente la aparición de tumores en los rudimentos uterinos. Este caso se presenta con el objetivo de hacer una revisión de la literatura respecto a la asociación de leiomioma uterino y síndrome de MRKH.Materiales y métodos: se presenta el caso y se realiza búsqueda a través de MEDLINE en la página de PubMed con los términos "leiomyoma" y "Mayer-Rokitansky-Küster-Hauser syndrome". Se obtuvieron 8 referencias, de las cuales 7 se identificaron como pertinentes y se incluyeron en la revisión. Además, se hace una búsqueda manual de publicaciones relacionadas con la patología de base.Conclusión: el hallazgo de un leiomioma en el remanente uterino en el síndrome de MRKH es muy poco frecuente, con apenas unas cuantas publicaciones en la literatura médica. En esta paciente, con la impresión diagnóstica de leiomioma uterino, se prefirió realizar laparotomía dado el tamaño del tumor, donde se reportó la patología de biopsia por congelación y la definitiva como leiomioma.

The use of pelvic peritoneum in the construction of a vagina: 10 cases.

Ten cases of Rokitansky-Ktister-Hauser syndrome were treated by using the pelvic peritoneum to construct a vagina. To bring the pelvic peritoneum down the canal to the vulva, peritoneal mobilization was performed by the vulvar and abdominal routes. Each operation's difficulty depended upon the amount of the uterine remnant and the presence of urinary tract anomalies. The constructed vaginas were reported effective for coitus.

Haemangiosarcoma in the uterine remnant of a spayed female dog

A 11-year-old, female, spayed greyhound was presented with a haemorrhagic discharge from the vulva. Clinical examination, vaginoscopy and a computed tomography scan showed an irregular egg-sized mass in the region of the cervix and uterine stump. An endoscopic grab biopsy (incisional) suggested a malignant mesenchymal tumour. Following this, surgical excision of the cranial vagina, cervix and the uterine remnant was performed. The final diagnosis of haemangiosarcoma was based on histological examination of the larger excisional biopsy specimen and was confirmed by positive immunolabelling of the neoplastic endothelial cells for the von Willebrand factor.

Case report and surgical solution for a voluminous uterine leiomyoma in a woman with complicated Mayer-Rokitansky-Küster-Hauser syndrome

To describe a case of leiomyoma in a patient with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Case report. Department of Obstetrics and Gynecology, "Sacra Famiglia Hospital," Novafeltria, Italy. A 47-year-old woman with MRKH syndrome with lower abdominal pain and pelvic tumescence. Surgery. Results on ultrasound scan and magnetic resonance imaging. A pelvic leiomyoma originating from uterine remnant; resection of the mass was performed. Even though magnetic resonance imaging is useful for the diagnosis of leiomyoma of the rudimentary uterus in MRKH syndrome, surgery might be the only definitive solution. Removal of the opposite uterine remnant could also be proposed.