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

2021 ◽  
Vol 9 ◽  
Author(s):  
Mark J. C. M. van Dam ◽  
Bas S. H. J. Zegers ◽  
Michiel F. Schreuder
Keyword(s):  
Case Series ◽  
Multicystic Dysplastic Kidney ◽  
Unilateral Renal Agenesis ◽  
Mullerian Anomalies ◽  
Müllerian Anomalies ◽  
Treatment And Prevention ◽  
Solitary Functioning Kidney ◽  
Dysplastic Kidney ◽  
Uterine Anomalies ◽  
Functioning Kidney

Unilateral renal agenesis and multicystic dysplastic kidney, resulting in a contralateral solitary functioning kidney (SFK), are part of the broad spectrum of congenital anomalies of the kidney and urinary tract (CAKUT). In girls with SFK, screening for asymptomatic Müllerian anomalies of uterus and vagina is not yet routinely performed, and therefore often overlooked until clinical complications in the menstrual cycle or fertility process occur. In this case series, we report on four teenagers with congenital SFK presenting with menstrual problems due to a Müllerian anomaly. Routine peri-menarchal screening for Müllerian anomalies in girls with SFK may provide timely counseling, surgical treatment and prevention of associated complications such as endometriosis, infertility and miscarriages.

Congenital solitary functioning kidney

2015 ◽  
Author(s):  
Michiel F. Schreuder
Keyword(s):  
Renal Injury ◽  
Renal Agenesis ◽  
Multicystic Dysplastic Kidney ◽  
Kidney Donation ◽  
Adult Age ◽  
Solitary Functioning Kidney ◽  
Dysplastic Kidney ◽  
Functioning Kidney

The main cause for unilateral non-formation or non-functioning of a kidney can be found in renal agenesis/aplasia and multicystic dysplastic kidney. Even though kidney donation at adult age is considered safe, studies in recent years have shown that this may be different in congenital solitary functioning kidneys. Whether this is based on dysplasia in the remaining kidney or based on glomerular hyperfiltration damage, follow-up has shown renal injury, defined as hypertension and/or proteinuria, in up to 32% of children with a congenital solitary functioning kidney. Therefore, long-term infrequent follow-up of all patients with a congenital solitary functioning kidney seems to be indicated.

scholarly journals Solitary functioning kidney in children: clinical implications

2018 ◽  
Vol 40 (3) ◽  
pp. 261-265
Author(s):  
Veerbhadra Radhakrishna ◽  
Krishna Kumar Govindarajan ◽  
Kumaravel Sambandan ◽  
Bibekanand Jindal ◽  
BikashKumar Naredi
Keyword(s):  
Renal Function ◽  
Vesicoureteric Reflux ◽  
Posterior Urethral Valves ◽  
Multicystic Dysplastic Kidney ◽  
High Morbidity ◽  
Solitary Functioning Kidney ◽  
Dysplastic Kidney ◽  
Study Results ◽  
Functioning Kidney

ABSTRACT Introduction: Children with solitary functioning kidney (SFK) are prone to develop long term problems, which are not well represented in the literature. The extent to which the presence of associated congenital anomalies of kidney and urinary tract (CAKUT) further de-stabilize renal function is to be addressed. Objective: This study was conducted to evaluate the etiology, presentation, presence of CAKUT, and renal damage in children with SFK. Methods: All children with SFK who presented to the department of pediatric surgery from March 2014 to May 2016 were included in the study. Children with malignancy were excluded from the study. Results: Of the 20 patients with SFK, 14 (70%) had primary SFK (8 with agenesis and 6 with multicystic dysplastic kidney), 6 (30%) belonged to secondary SFK group, among them 3 had pelviureteric junction obstruction, 2 had posterior urethral valves and 1 had vesicoureteric reflux. Eight (40%) had associated CAKUT, 4 (20%) were asymptomatic while 8 (40%) had UTI and 6 (30%) had hypertension. Ten (50%) patients had reduced glomerular filtration rate (GFR) suggesting compromised renal function. Conclusion: Children with SFK have high morbidity especially when associated with ipsilateral CAKUT. Long-term periodical follow up is essential in these patients to improve clinical outcome.

Early Nephrectomy in Unilateral Multicystic Dysplastic Kidney in Children Cures Hypertension Early: An Observation

2017 ◽  
Vol 27 (06) ◽  
pp. 533-537 ◽  
Author(s):  
Vijai Upadhyaya ◽  
Manish Gupta ◽  
Laxmi Bharti ◽  
Ram Rao ◽  
Sheo Kumar ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Antihypertensive Drugs ◽  
Case Series ◽  
Multicystic Dysplastic Kidney ◽  
Time Duration ◽  
Level Of Evidence ◽  
Variable Time ◽  
Dysplastic Kidney ◽  
Diagnosis Of Hypertension

Objective Surgery is still indicated as the treatment of choice in subset of patients with unilateral multicystic dysplastic kidney (UMCDK) because of its potential complications and malignant change. The purpose of this study is to present our observation that early nephrectomy may cure hypertension early in children with UMCDK and review the literature. Materials and Methods We report here four children (two males and two females) with antenatally diagnosed UMCDK with hypertension, treated in the past 4 years. All have antenatal diagnosis of UMCDK and referred to us after their birth. Diagnosis of hypertension (blood pressure > 95th percentile) was made after 3 months of follow-up in all patients. Nephrectomy was performed in all children in variable time duration. Hypertension, urinary tract infection, and desire of parents were the main indication of surgery. Results All children showed decrease in the size of their dysplastic kidney with time, but hypertension was persistent and needed antihypertensive drugs; even increase in the doses in successive follow-up. After nephrectomy, three children who were operated early got cured having normal blood pressure within variable time duration, while a child, operated late, still had high blood pressure and needed antihypertensive drugs. Conclusion Early nephrectomy in recently diagnosed hypertension in UMCDK is advisable and can cure hypertension early. Levels of Evidence The level of evidence is IV (case series with no comparison group).

Placental malperfusion as a possible mechanism of preterm birth in patients with Müllerian anomalies

2017 ◽  
Vol 45 (1) ◽  
Author(s):  
Jovana Lekovich ◽  
Joshua Stewart ◽  
Sarah Anderson ◽  
Erin Niemasik ◽  
Nigel Pereira ◽  
...  
Keyword(s):  
Preterm Birth ◽  
Gestational Age ◽  
Placenta Previa ◽  
Preterm Births ◽  
Mullerian Anomalies ◽  
Müllerian Anomalies ◽  
Increased Risk ◽  
Uterine Anomalies ◽  
Gestational Age At Delivery ◽  
Term Births

AbstractObjective:Müllerian anomalies are associated with increased risk of miscarriage, intrauterine growth restriction (IUGR) and preterm birth. While a commonly implicated cause is restricted expansion of endometrial cavity, alternatively it could be due to abnormal placentation. We sought to examine clinical and histopathologic factors associated with preterm delivery in women with Müllerian anomalies.Study design:One hundred and eleven singleton pregnancies in 85 women were analyzed retrospectively. There were 42 pregnancies with bicornaute, 24 with unicornuate, 24 with septate, 19 with didelphys and one each with arcuate and T-shaped uterus. Primary outcomes included gestational age at delivery, placental histopathology, placenta previa and accreta.Results:Twenty-eight (25.2%) of pregnancies were delivered prior to term. Of those, only 14 (50%) were due to preterm labor or preterm premature rupture of membranes (PPROM). Histological evidence of placental malperfusion was present in 22% of all pregnancies and those delivered at an earlier median gestational age [34 (IQR 31–37) vs. 37 weeks (IQR 34–39); P=0.001]. Malperfusion was more common in preterm than in full term births (46% vs. 14%; P=0.04). Conversely, inflammation was not more common in preterm compared to term deliveries (17.9% vs. 16.9%; P=0.89). Five pregnancies had placenta previa, three of which were complicated by accreta.Conclusion:Placental malperfusion, rather than inflammation, was more commonly associated with preterm births in women with uterine anomalies.

Twin pregnancy in uteri with congenital anomalies: prenatal diagnosis by three- dimensional ultrasound

2018 ◽  
Vol 8 (1) ◽  
Author(s):  
Efraim Zohav ◽  
Sophia Leytes ◽  
Eyal Y. Anteby ◽  
Leonti Grin
Keyword(s):  
Sagittal Plane ◽  
Three Dimensional ◽  
Area Under The Curve ◽  
First Trimester ◽  
Mullerian Anomalies ◽  
Müllerian Anomalies ◽  
Uterine Anomalies ◽  
Mullerian Anomaly ◽  
First Trimester Of Pregnancy

Abstract Objectives To establish a practical and valid method for the diagnosis of twin gestation in uteri with Müllerian anomalies in the first trimester of pregnancy. Case presentation This was a prospective cohort study. Two cases with congenital uterine anomalies were followed prospectively from 7 weeks of gestation and compared to eight controls. The longitudinal to transverse uterine lumen diameter ratio (LTDR) was measured in mid-sagittal and coronal planes. The LTDR was compared between unicornuate and didelphys uteri to normal uteri controls weekly. The area under the curve for the coronal and sagittal planes were 1.000 and 0.823, respectively. For the coronal plane, an LTDR of 1.15 was selected as optimal for predicting uteri with a Müllerian anomaly, with a sensitivity of 100%, specificity of 100%. An LTDR of 1.68 in the sagittal plane was selected as optimal for predicting anomalous uteri, with a sensitivity of 100%, specificity of 73%. Conclusions LTDR ratios measured in mid sagittal and coronal planes of unicornuate and didelphys uteri with twin gestation during the first trimester of pregnancy are highly predictive of Müllerian anomalies. Early diagnosis of uterine anomalies could provide a reference to initiate earlier meticulous follow-up.

scholarly journals Obstructive mullerian anamolies: a case series

2019 ◽  
Vol 8 (7) ◽  
pp. 2954
Author(s):  
Urvashi Miglani ◽  
Poonam Laul ◽  
V. K. Kadam ◽  
Sanjeev Miglani
Keyword(s):  
Case Series ◽  
Genetic Mutation ◽  
Management Options ◽  
Renal Anomalies ◽  
Vaginal Septum ◽  
Mullerian Anomalies ◽  
Imperforate Hymen ◽  
Müllerian Anomalies ◽  
Transverse Vaginal Septum ◽  
Psychosocial Counselling

AIM of the study was to review the heterogeneous clinical presentations and management options for some of the obstructive mullerian anomalies through a case series. Background: Müllerian duct anomalies (MDAs) are a miscellaneous group of entities that result from the non-development, defective vertical or lateral fusion, or resorption failure of the müllerian ducts due to genetic mutation. 5 cases of obstructive mullerian anomaly are reviewed. Cases of OHVIRA (obstructed Hemivagina with Renal Agenesis) syndrome, transverse vaginal septum, imperforate hymen and obstructed rudimentary horn of unicornuate uterus are included. Results: We found that cyclical abdominal pain was the most common presenting complaint. There is a high incidence of associated renal anomalies. Psychosocial counselling before treatment is necessary to address the functional and emotional aspects of the patient. Surgical management was done in all patients with good postoperative outcome. Conclusion: Obstructive mullerian anomalies need to be evaluated by a meticulous examination and imaging studies to reach the diagnosis with precision. The treatment has to be tailored to the specific anomaly.

scholarly journals Management of Second Trimester Fetal Demise in a Noncommunicating Uterine Horn

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
R. Tyler Hillman ◽  
Homer G. Chin ◽  
Sheila K. Mody
Keyword(s):  
Pregnancy Termination ◽  
Uterine Horn ◽  
Imaging Studies ◽  
Fetal Demise ◽  
Operative Strategy ◽  
Mullerian Anomalies ◽  
Müllerian Anomalies ◽  
Unintended Pregnancies ◽  
Unicornuate Uterus ◽  
Uterine Anomalies

Müllerian anomalies are uncommon but when present they can increase the risk of obstetrical complications. Anomalies such as bicornuate and unicornuate uterus can also increase the surgical risks of pregnancy termination. The diagnostic approach and surgical management must be individualized for each patient so that the termination procedure is safe and preserves fertility. We present a case of a patient with a 17-week pregnancy with fetal demise in a noncommunicating right uterine horn. Laparotomy and hysterotomy were required for evacuation of the fetus. The use of appropriate imaging studies to diagnose suspected uterine anomalies and a flexible and individualized operative strategy are essential for reducing complications associated with the termination of abnormal or unintended pregnancies in women with Müllerian anomalies.

Renal agenesis

2015 ◽  
Author(s):  
Michiel F. Schreuder
Keyword(s):  
Prenatal Diagnosis ◽  
Renal Agenesis ◽  
Multicystic Dysplastic Kidney ◽  
Metanephric Mesenchyme ◽  
Ureteric Bud ◽  
Unilateral Renal Agenesis ◽  
Dysplastic Kidney ◽  
Long Term Follow Up

In renal agenesis, the kidney never forms due to an absence of interaction in the developing embryo between the ureteric bud and the metanephric mesenchyme. Most cases of renal agenesis are unilateral, for which the reported incidence is around 1 in 3000. The prenatal diagnosis of unilateral renal agenesis is based on the absence of a recognizable kidney, either at the normal or an ectopic site. However, such cases of an empty renal fossa can also be explained by an involuted multicystic dysplastic kidney, or by renal aplasia. Based on the hyperfiltration hypothesis and clinical studies, glomerular hyperfiltration can be expected, resulting in hypertension, albuminuria, and renal injury, for which long-term follow-up of all patients with renal agenesis is desirable.

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