Pelvic pain in patients with complex mullerian anomalies including Mayer–Rokitansky–Kuster–Hauser syndrome (MRKH), obstructed hemi-vagina ipsilateral renal anomaly (OHVIRA), and complex cloaca

Schall, Kathy; Parks, Melissa; Nemivant, Samantha M.; Hernandez, Janett; Weidler, Erica M.

doi:10.1016/j.sempedsurg.2019.150842

Cited by 5 publications

(3 citation statements)

References 28 publications

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“…However, patients with complex cloacal malformation or Müllerian anomalies often require multiple reconstruction surgeries. Mental health problems including anxiety and depression, infertility, and pain management for long-term pelvic pain, pain associated with menstruation, urination, and intercourse, are important issues requiring multidisciplinary team care [ 31 , 32 ].…”

Section: Discussionmentioning

confidence: 99%

Hydrometrocolpos in Infants: Etiologies and Clinical Presentations

2022

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Hydrometrocolpos (HMC) is a rare condition where fluids or secretions accumulate in the vagina (hydrocolpos) or up to the uterus (hydrometrocolpos). This case series study reports three infants with different etiologies and presentations of HMC and aims to review literature for proper workup upon initial diagnosis. The first neonate antenatally presented with a huge cystic mass. HMC secondary to imperforate hymen was proved, and hymenotomy was performed at 2 days of age. The second participant presented with persistent urogenital sinus and hematopoietic chimerism, possibly due to transfusion from her twin brother via placenta anastomoses. At 2 months of corrected age, she had difficult defecating, and sonogram revealed HMC with normal appearance of uterus and ovaries. Regular follow-ups and surgical reconstruction will be conducted before puberty. The third patient had cloacal malformation and multiple congenital anomalies at birth. Vesicovaginal fistula-related HMC was detected and managed with surgical drainage in the neonate stage. The girl began menstruation with dysmenorrhea at 12 years. The image studies demonstrated hematometrocolpos secondary to left-side hemivaginal septum, uterine didelphy, and ipsilateral renal agenesis, indicating Herlyn–Werner–Wunderlich syndrome. HMC can be diagnosed easily via sonogram. Careful external genitalia examinations help to identify persistent urogenital sinus or cloacal malformation. Occasionally, the HMC may be part of syndrome manifestations or associated with sex chromosome anomalies. Clinicians may conduct surveillance of renal, cardiac, and skeletal systems as well as chromosome study for early diagnosis and management.

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Section: Discussionmentioning

confidence: 99%

Hydrometrocolpos in Infants: Etiologies and Clinical Presentations

2022

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“…Unilateral genital tract obstruction with ipsilateral renal anomaly (UGTOIRA) syndrome is a rare congenital urogenital anomaly involving unilateral genital tract obstruction with different uterine malformations and ipsilateral renal agenesis; in the literature, it is also known as Herlyn-Werner-Wunderlich (HWW) syndrome, obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, obstructed uterovaginal duplication, oblique vaginal septum (OVS) syndrome, and other terms ( 1 - 5 ). Over the past hundred years, the common clinical manifestations of HWW syndrome have been considered the symptoms of poor blood drainage (periodic lower abdominal pain or blood dripping after menstruation) combined with infection, endometriosis, and pelvic adhesions ( 1 - 10 ). The anatomical abnormalities of most cases are classic double uterine body, double cervix, and OVS; unilateral hematocolpos, hematometra, and hematosalpinx; ovarian endometriosis cyst, pelvic endometriosis; and ipsilateral renal agenesis.…”

Section: Introductionmentioning

confidence: 99%

Diverse ultrasound image features of unilateral genital tract obstruction with ipsilateral renal anomaly syndrome on genitourinary system segmental sequential ultrasound screening and the accuracy of ultrasonic diagnosis

Zhang,

Liu,

Liu

et al. 2023

Quant Imaging Med Surg

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Background Unilateral genital tract obstruction with ipsilateral renal anomaly (UGTOIRA) syndrome is a rare congenital urogenital anomaly, characterized by different combinations of uterine abnormalities, unilateral cervical-vaginal obstruction, and ipsilateral renal abnormalities. Timely and correct diagnosis is critical. In this study, we analyzed the diverse ultrasound image features of UGTOIRA syndrome on genitourinary system segmental sequential ultrasound screening (SSUS) and the accuracy of ultrasonic diagnosis. Methods The data from 59 patients with UGTOIRA syndrome over the last decade were analyzed retrospectively, which included ultrasound presentations and diagnoses of abnormalities of the uterus, cervix, and vagina, as well as the conditions associated with the bilateral fallopian tubes, ovaries, pelvis, kidneys and ureters, bladder, and urethra. Results All 59 patients (100%) were found to have ipsilateral renal agenesis, and the diagnostic accuracy of ultrasound was 100%. The uterus was correctively diagnosed as complete bicorporal uterus in 42 cases (71.2%), bicorporal septate uterus in 7 cases (11.9%), complete uterus septate in 8 cases (13.6%), partial uterus septate in 1 case (1.7%), and unilateral isthmus atresia in 1 case (1.7%). The cervix was correctively diagnosed as septate cervix in 28 cases (47.5%), double cervix in 17 cases (28.8%), unilateral small cervix in 5 cases (8.5%), unilateral obliterated cervical orifice in 5 cases (8.5%), partial development of the unilateral cervix with a blind end in 3 cases (5.1%), and unilateral completely undeveloped cervix appearing as a normal cervix in 1 case (1.7%). The vagina was correctively diagnosed as oblique vaginal septum (OVS) in 45 cases (76.3%), and normal vagina in 13 cases (22.0%) via preoperative ultrasound. In 1 case (1/59, 1.7%) of high OVS with ipsilateral obliterated external cervical orifice, the OVS was missed by preoperative ultrasound. The ultrasound diagnostic accuracy rates of uterine malformation, cervical malformation, vaginal malformation, hematoma location and volume, uterine communication, and cervical communication were respectively 100%, 100%, 98.3%, 100%, 100%, and 100%. The ultrasonic detection rate for a hole on the OVS was 0%, and the missed diagnosis rate was 100%. Conclusions SSUS can be used to accurately evaluate uterine, cervical, and vaginal malformation, obstruction site, location and volume of keratocele, cervical or uterine communication, and ovarian endometriosis cyst in patients with UGTOIRA syndrome and ipsilateral urinary system malformation. However, ultrasound is unable to identify a hole on the OVS.

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“…Congenital anomalies of the reproductive tract resulting in Müllerian outflow tract obstruction (OTO) are rare and quite varied in terms of anatomy, presentation, and treatment [ 1 ], requiring the expertise of many medical and surgical specialties. OTO in patients without associated anomalies of the external genitalia usually presents unexpectedly during adolescence with cyclical or recurrent abdominopelvic pain and primary amenorrhea [ 2 , 3 ]. In individuals with associated early-diagnosed and reconstructed congenital anomalies, such as cloacal malformation and cloacal exstrophy, there is usually awareness by caregivers and healthcare providers of the potential for developing OTO in adolescence.…”

Section: Introductionmentioning

confidence: 99%

An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy

Sack

Speck

Hryhorczuk

et al. 2022

JCM

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People with cloacal malformation and 46,XX cloacal exstrophy are at risk of developing Müllerian outflow tract obstruction (OTO). Management of OTO requires expertise of many medical and surgical specialties. The primary presenting symptom associated with OTO is cyclical and later continuous pain and can be initially quelled with hormonal suppression as a temporizing measure to allow for patient maturation. The decision for timing and method of definitive treatment to establish a patent outflow tract that can also be used for penetrative sexual activity and potential fertility is a complicated one and incredibly variable based on patient age alone. To understand the management approach to OTO, we put forth five phases with associated recommendations: (1) caregiver and patient education and evaluation before obstruction; (2) presentation, diagnosis, and symptom temporization; (3) readiness assessment; (4) peri-procedural management; (5) long-term surveillance. This review will emphasize the importance of interdisciplinary team management of the complex shared medical, surgical, and psychological decision making required to successfully guide developing patients with outflow obstruction secondary to cloacal malformations and cloacal exstrophy through adolescence.

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Pelvic pain in patients with complex mullerian anomalies including Mayer–Rokitansky–Kuster–Hauser syndrome (MRKH), obstructed hemi-vagina ipsilateral renal anomaly (OHVIRA), and complex cloaca

Cited by 5 publications

References 28 publications

Hydrometrocolpos in Infants: Etiologies and Clinical Presentations

Hydrometrocolpos in Infants: Etiologies and Clinical Presentations

Diverse ultrasound image features of unilateral genital tract obstruction with ipsilateral renal anomaly syndrome on genitourinary system segmental sequential ultrasound screening and the accuracy of ultrasonic diagnosis

An Interdisciplinary Approach to Müllerian Outflow Tract Obstruction Associated with Cloacal Malformation and Cloacal Exstrophy

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