Case Report: Uterine Anomalies in Girls With a Congenital Solitary Functioning Kidney

Dam, Mark J. C. M. van; Zegers, Bas; Schreuder, Michiel F.

doi:10.3389/fped.2021.791499

Cited by 5 publications

(3 citation statements)

References 21 publications

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“…5,6 It should be due noted that Mullerian anomalies are often associated with renal anomalies and thus should be specifically investigated during preoperative evaluation. 7 With the advances in minimal invasive surgery, primary key goal in patients with cervical agenesis has been reconstructive surgery with uterovaginal anastomosis. 8 But due to rarity of these cases, no randomized controlled trials (RCTs) or even multicentric studies have been possible to formulate a clinical plan for operative management.…”

Section: Discussionmentioning

confidence: 99%

A rare case of cervical agenesis with agenesis of the upper 2/3rd vagina with hematometra

Makwana

Patel

Halpati³

2023

Int J Reprod Contracept Obstet Gynecol

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Cervical agenesis is an extremely rare form of congenital Mullerian anomaly. Due to the rarity of the cases with different presentations ranging from cervical agenesis to dysgenesis (fragmentation, fibrous cord, obstruction of external os), along with the functionality of the uterus, there are numerous conservative surgical options involving uterovaginal anastomosis, cervical reconstruction, and cervical canalization, and total hysterectomy in cases where conservative surgical procedures fail or not feasible. In our case, the patient was a 32-year-old female with primary amenorrhea with, a history of marriage for 6 years and a history of surgery for primary amenorrhea during adolescence. During the present visit, she had severe abdominal pain due to massive hematometra with a short blind vagina. She was planned for laparoscopic Uterovaginal anastomosis but converted to abdominal hysterectomy due to the large size of uterus, lack of uterine supports, and high length of the defect between the lower end of the uterus and vaginal end. Due to the lack of proper guidelines and variations in clinical presentation, a case-based approach is required.

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Section: Discussionmentioning

confidence: 99%

A rare case of cervical agenesis with agenesis of the upper 2/3rd vagina with hematometra

Makwana

Patel

Halpati³

2023

Int J Reprod Contracept Obstet Gynecol

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“…У девочек самыми распространёнными аномалиями половых органов являются истинная однорогая матка (65%), двурогая матка с одним рудиментарным рогом (7,3%), атрофия одной маточной трубы и яичника, отсутствие или гипоплазия влагалища, удвоение влагалища [3][4][5][6][13][14][15][16]. Об истинной однорогой матке, связанной с односторонней АгП, впервые сообщил Puech в 1885 г., в 1938 г. Shumacker описал ещё 28 случаев с данной патологией [3,5,14].…”

Section: пороки развития половой системы сочетающиеся с агенезией почкиunclassified

Diseases and syndromes associated with unilateral renal agenesis in children

Romashova

2024

RPJ

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The incidence of congenital malformations is increasing annually, with a single kidney developmental disorder accounting for 4–8% of urinary system malformations. If the contralateral organ is normal, agenesis of the kidney is not clinically apparent and is usually detected during a preventive examination of the child or during an examination for combined anomalies of the genitourinary and urinary system.This pathology occurs due to a disruption of the interstitial interaction between the ureteral bud and the metanephrogenic tissue from 4th to 8th gestation weeks. By this time, the mesonephral (Wolff) ducts are already fully formed, in contrast to the paramesonephral (Müllerian) ducts, which develop only by the 5th week of the intrauterine period, that is, in the period of high risk of malformations of the urinary system. Accordingly, anomalies of the female genital system are more common than those of the male one, with concomitant agenesis of the ipsilateral kidney. Diagnosis of unilateral agenesis of the kidney is possible when performing routine antenatal screening or conducting preventive examinations at decreed dates. In girls, the most common genital anomalies are true unicornuate uterus (65%), bicornuate uterus with one rudimentary horn (7.3%), atrophy of one fallopian tube and ovary, absence or hypoplasia of the vagina, and vaginal doubling. In boys with unilateral agenesis of the kidney, the seminal vesicles, prostate gland, and testicular appendage may be rudimentary or absent. Agenesis of the kidney is a component of such genetic syndromes as OHVIRA, Kallman, Zinner, and Mayer–Rokitansky–Küster–Hauser syndromes. It is also combined with other congenital anomalies of the kidney and urinary tract and extrarenal anomalies, including mainly malformations of the gastrointestinal tract, heart and musculoskeletal system. Consequently, children with this pathology should be fully screened to rule out associated malformations and anomalies.

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“…Indeed, these disorders may predispose to recurrent urinary tract infections (rUTI), resulting in kidney impairment [4]. Uterus didelphys with obstructed hemivagina (OHVIRA) may cause acute abdominal pain, rUTI, and this condition may be associated with CAKUT [5]. Therefore, OHVIRA should be considered in young females with these symptoms and CAKUT.…”

Section: Introductionmentioning

confidence: 99%

Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart

Malanowska-Jarema,

Starczewski,

Osnytska

et al. 2023

JCM

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We present here a case of complex uterine anomaly—obstructed hemivagina with ipsilateral renal agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 13-year-old girl with a history of recurrent urinary tract infections (rUTI). In the emergency room, a trans-abdominal sonography revealed an ovarian cyst and renal agenesis, without any suspicion of vaginal obstruction. This led to a delay in the diagnosis of this uncommon anomaly. Finally, MRI findings confirmed the presence of OHVIRA syndrome. As the congenital anomalies of the kidney and urinary tract (CAKUT) are present in almost one third of cases associated with genital malformations, urologists should carefully screen patients with rUTI. The patient underwent simultaneous laparoscopy and vaginoscopy, which was in our opinion the most appropriate therapeutic decision. In this article, we are also going to discuss the role of laparoscopy in the management of OHVIRA syndrome, as well as other surgical techniques described in the literature.

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Case Report: Uterine Anomalies in Girls With a Congenital Solitary Functioning Kidney

Cited by 5 publications

References 21 publications

A rare case of cervical agenesis with agenesis of the upper 2/3rd vagina with hematometra

A rare case of cervical agenesis with agenesis of the upper 2/3rd vagina with hematometra

Diseases and syndromes associated with unilateral renal agenesis in children

Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart

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