Distal ureteral atresia – an embryological dilemma

2022 ◽  
pp. 205141582110481
Author(s):  
Subramanya Kattepura ◽  
Attibele Mahadevaiah Shubha ◽  
Kanishka Das
Keyword(s):  
Preoperative Diagnosis ◽  
Renal Dysplasia ◽  
Urogenital Sinus ◽  
Multicystic Dysplastic Kidney ◽  
Level Of Evidence ◽  
Case Presentation ◽  
Contralateral Kidney ◽  
Urinary Tract Anomaly ◽  
Dysplastic Kidney ◽  
Associated Malformations

Introduction: Distal ureteral atresia is a rare urinary tract anomaly generally associated with ipsilateral renal dysplasia and abnormalities such as multicystic dysplastic kidney, hydronephrosis and megaureter in the contralateral kidney. Despite burgeoning investigation modalities, definitive preoperative diagnosis of this condition is rarely feasible, also the embryological disarray of events that result in the development of this anomaly and the associated malformations is not clearly understood. Case presentation: We hereby report two cases of distal ureteral atresia and discuss the diversity in their presentations, diagnosis, atypical associations and management and review the possible embryological mal-development. Conclusion: Distal ureteral atresia with urogenital sinus as in Case 1 has not been documented so far and a plausible embryological explanation is deduced regarding its occurrence. The course of the affected kidney following timely and adequate relief of obstruction in Case 2 is depicted, highlighting the eventual management. Level of evidence: Not applicable

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).

Association of Wilms tumor in multicystic dysplastic kidneys: case report and review of the literature

2021 ◽  
pp. 205141582110240
Author(s):  
Darian Andreas ◽  
Richard D Glick ◽  
Jonathan D Fish ◽  
Carolyn Fein Levy ◽  
Jordan S Gitlin
Keyword(s):  
Case Report ◽  
Antenatal Diagnosis ◽  
Wilms Tumor ◽  
Multicystic Dysplastic Kidney ◽  
Review Of The Literature ◽  
Level Of Evidence ◽  
Renal Anomalies ◽  
Dysplastic Kidney ◽  
Increased Risk ◽  
Dysplastic Kidneys

Multicystic dysplastic kidney is a rare urinary anomaly characterized by multiple non-communicating cysts resulting in a non-functional kidney. In addition to association with hypertension and contralateral renal anomalies, children with multicystic dysplastic kidney have an increased risk of Wilms tumor. Cohort studies and systematic reviews are hampered in estimating the true risk of this association due to the rarity and infrequent reporting of the condition. We present a case of a 2-year-old male child with an antenatal diagnosis of multicystic dysplastic kidney undergoing surveillance ultrasonography who presented with a symptomatic Wilms tumor. Level of evidence: Not applicable for this multicentre audit.

Abnormal contralateral kidney in unilateral multicystic dysplastic kidney disease

1995 ◽  
Vol 25 (4) ◽  
pp. 275-277 ◽  
Author(s):  
K. Kaneko ◽  
Y. Suzuki ◽  
Y. Fukuda ◽  
K. Yabuta ◽  
T. Miyano
Keyword(s):  
Kidney Disease ◽  
Multicystic Dysplastic Kidney ◽  
Contralateral Kidney ◽  
Dysplastic Kidney ◽  
Multicystic Dysplastic Kidney Disease

scholarly journals Pathophysiological clinical features of an infant with hypertension secondary to multicystic dysplastic kidney: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Keisuke Sugimoto ◽  
Takuji Enya ◽  
Kensuke Joh ◽  
Kohei Miyazaki ◽  
Tomoki Miyazawa ◽  
...  
Keyword(s):  
Left Kidney ◽  
Multicystic Dysplastic Kidney ◽  
Wall Thickening ◽  
Tubular Atrophy ◽  
Renal Masses ◽  
Renal Hypoplasia ◽  
Case Presentation ◽  
Post Surgery ◽  
Dysplastic Kidney

Abstract Background The association of hypertension with congenital renal hypoplasia has been established. We report a case of an infant who underwent nephrectomy for hypertension. Case presentation Magnetic resonance imaging for the mother revealed fetal renal masses, and fetal multicystic dysplastic kidney was suspected. Following birth, the baby developed hypertension. Numerous investigations revealed that the left kidney was non-functional, and she was initiated on benazepril hydrochloride. However, because the drug response was poor, the left kidney was removed at the age of 7 months. Examination of the renal specimen revealed abrupt transition from normal to atrophic cortex with lobar atrophy and cysts. Tubular atrophy, marked abnormal blood vessels with wall thickening, gathered immature glomeruli, and parenchymal destruction were observed. Renin was partially localized in the proximal tubules and the parietal epithelium of the Bowman’s capsule in the immature glomeruli. We speculated that an abnormal vascular structure and irregular renin localizations may be the cause of hypertension. Serum renin and aldosterone levels gradually reduced post-surgery, reaching normal levels on the 90th postoperative day. A long follow-up is needed due to the possibility of the child developing hypertension in the future. Conclusion This is a case of an infant with MCDK, which discusses the clinicopathological features based on the pathophysiological analysis, including renin evaluation.

High Activation of the AKT Pathway in Human Multicystic Renal Dysplasia

Pathobiology ◽  
2020 ◽  
Vol 87 (5) ◽  
pp. 302-310
Author(s):  
Alexia Apostolou ◽  
Brice Poreau ◽  
Loris Delrieu ◽  
Julien Thévenon ◽  
Pierre-Simon Jouk ◽  
...  
Keyword(s):  
Mesangial Cells ◽  
Renal Dysplasia ◽  
Mtor Pathway ◽  
Multicystic Dysplastic Kidney ◽  
Renal Development ◽  
Glomerular Mesangial Cells ◽  
Dysplastic Kidney ◽  
Cell Proliferation And Differentiation ◽  
Identical Manner ◽  
Multicystic Renal Dysplasia

Multicystic renal dysplasia is a congenital cystic anomaly of the kidney caused by abnormal metanephric differentiation with immature tubules. It is surrounded by mesenchymal collars and islands of immature mesenchyma present between the cysts. The PI3K-AKT-mTOR signaling pathway is a key regulator involved in cell growth, proliferation, motility, survival, and apoptosis. Activation of the PI3K-AKT-mTOR pathway results in the survival and proliferation of tumor cells in many cancers. The aim of this study is to analyze the topographic expression of phospho-AKT, phospho-mTOR, and phospho-70S6K in renal development and in the multicystic dysplastic kidney (MCDK). A total of 17 fetal kidneys of development age from the first to the third trimester and 13 cases of pathological kidneys with MCDK were analyzed by immunohistochemistry in order to evaluate the expression of phospho-AKT (S473), phospho-mTOR, and phospho-70S6K. Phospho-AKT and phospho-mTOR were expressed early in renal development and in an identical manner for every structure derived from the ureteric bud, such as collecting ducts and urothelium. Phospho-p70S6K was expressed early in the urothelium and in glomerular mesangial cells. Later, their expressions differed according to the needs of cell proliferation and differentiation over time by becoming more selective. In MCDK, phospho-AKT, phospho-mTOR, and phospho-70S6K have the same profile: a high cytoplasmic expression in cystic epithelium, loose mesenchyma, and primitive tubes. This study demonstrates the essential and specific role of the PI3K-AKT-mTOR pathway in the formation of cysts in multicystic renal dysplasia.

scholarly journals Association of fetal unilateral multicystic kidney disease with other urinary tract anomalies

2009 ◽  
Vol 66 (9) ◽  
pp. 733-737
Author(s):  
Sonja Pop-Trajkovic ◽  
Aleksandar Ljubic ◽  
Vladimir Antic ◽  
Milan Trenkic
Keyword(s):  
Diagnostic Procedures ◽  
Multicystic Dysplastic Kidney ◽  
Urogenital System ◽  
Urinary System ◽  
Contralateral Kidney ◽  
Multicystic Kidney ◽  
Gynecology And Obstetrics ◽  
Dysplastic Kidney ◽  
Operative Findings ◽  
The University

Background/Aim. Multicystic dysplastic kidney represents a disorder in the fetus development presented prenatally of postnatally, this deserving special attention due to a risk of additional anomalies in children with this disorder. The aim of this study was to determine the incidence and type of common anomalies of the urinary system in the prenatal diagnosis of unilateral multicystic dysplastic kidney, and point out the necessity of postnatal diagnostic procedures in order to evaluate the state of the urinary system. Methods. This retrospective-prospective study encompassed 38 cases of prenatally diagnosed unilateral fetal multicystic dysplastic kidney, presented to the Council for Fetal Anomalies from the Institute for Gynecology and Obstetrics of the Clinical Centre of Serbia and the University Children's Clinic within a three-year period. Associated anomalies were revealed by autopsy findings when pregnancy was terminated, ie resumed with clinical and operative findings of born children. Results. In every case of terminated pregnancy and death after birth the autopsy revealed additional renal or exstrarenal anomaly which were not prenatally detected. Postnatal evaluation of survived children with unilateral multicistic disease revealed that 31,4% of them have an anomaly of the contralateral kidney, 26,3% anomaly of the ipsilateral side, 13,2% anomaly of the lower portions of the urinary system and the same percent an additional extrarenal anomaly. The surgery was performed in 73,6% of children, more than half of the interventions were related to extrarenal anomaly. In 17% of children the kidney function was deteriorated after surgery. Conclusion. Children suffering from unilateral multicystic dysplastic kidney have a greater chance of exhibiting an anomaly of the contralateral kidney and the urinary system in general. Therefore, they require thorough examination, both prenatally and postnatally. We propose obligatory serial professional prenatal ultrasound examinations, followed by postnatal ultrasound, isotope scan, and we especially emphasize the need for performing urinary cystouretherography, bearing in mind the high incidence of the vesicoureteral reflux of the contralateral kidney. In addition to nephrectomy, cytoscopy and colposcopy also need to be performed for the purpose of discovering possible hidden anomalies of the urogenital system.

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