Ectopic Kidney Research Articles

Crossed Fused Renal Ectopia with Transitional Cell Carcinoma of Kidney: A Rare Association

Crossed Fused Renal Ectopia with Transitional Cell Carcinoma of Kidney: A Rare Association

Clear cell renal cell carcinoma in crossed fused renal ectopia: A case report

Clear cell renal cell carcinoma in crossed fused renal ectopia: A case report

Makeshift Shunts in Extrahepatic Portal Vein Obstruction in Pediatric Population.

More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency. During the period 1983-2018, 138 children with portal hypertension were managed under the care of a single surgeon (VB). Of them, 134 were EHPVO. Children with EHPVO were treated with splenectomy and proximal lienorenal shunt (n = 107), splenectomy and devascularization (n = 21), and makeshift shunts (n = 6). Makeshift shunts comprised (i) side-to-side right gastroepiploic vein (Rt-GEV) to left renal vein (LRV) shunt (n = 1), (ii) superior mesenteric vein (SMV) to inferior vena cava (IVC) shunt using a spiral saphenous venous graft (n = 1), (iii) side-to-side inferior mesenteric vein (IMV) to LRV shunt (n = 2), (iv) side-to-side IMV to IVC shunt (n = 1), (v) end-to-side IMV to IVC shunt (n = 1), and (vi) side-to-side IMV to LRV shunt (n = 1) in a case of crossed fused renal ectopia. Following the creation of portosystemic shunt, a decline in portal pressure was demonstrated in all six patients. There was resolution of symptoms including hematemesis, melena, and anorectal variceal bleed. None of the patients demonstrated the features of hepatic encephalopathy. The associated portal cavernoma cholangiopathy (n = 1) also resolved following Rt-GEV to LRV shunt. Shunt patency was documented for the entire duration of follow-up (1.5-4 years) in five of six patients; the sixth patient demonstrated shunt block at 6-month follow-up but without recurrence of symptoms. Makeshift shunts offer a viable alternative to standard portosystemic shunting in pediatric patients with a nonshuntable vein. The selection of such shunts is, however, subject to surgeon's preferences and has to be individualized to local anatomy.

Surgical management of renal calculi in cases of crossed renal ectopia: A rare case series

Crossed renal ectopia is a rare congenital malformation. Only 36 cases of crossed renal ectopia with calculus have been recorded in literature, even a few cases of urolithiasis in them have been surgically managed. The majority of cases in literature have been published as single case reports. We conducted a retrospective study of all the crossed renal ectopia cases admitted and managed in our department in the past 2 years. Among them, only one was without fusion. Youngest patient was 32-year-old male with crossed left kidney with calculus in the left kidney. Both the patients underwent retrograde intrarenal surgery (RIRS). Oldest patient was 80-year-old female with a right crossed kidney with pelvic calculus, who underwent open pyelolithotomy. Post-operative period was uneventful in all cases with complete stone clearance. Patients undergoing RIRS had less post-operative pain and were discharged early compared to open pyelolithotomy. RIRS is a safe and effective modality of treatment and can be considered as the first choice for the management of calculus in crossed renal ectopia in properly selected cases. However, in cases of huge stone burden and difficult anatomy, laparoscopic/open pyelolithotomy is still an attractive option for clearing the stone in a single setting, even though it has comparatively more morbidity and longer hospital stay.

Diabetes mellitus in Kabuki syndrome 1 on a background of post-transplant diabetes mellitus.

Kabuki syndrome is a genetic disorder characterised by distinctive facial features, developmental delays, and multisystem congenital anomalies. Endocrine complications such as premature thelarche and short stature are common, whereas disorders of glycaemic control are less frequent. We describe a 23-year-old white female referred to the diabetes clinic for hyperglycaemia during haemodialysis. She was subsequently diagnosed with Kabuki syndrome based on characteristic clinical features, confirmed by detecting a heterozygous pathogenic variant in KMT2D. She was known to have had multiple congenital anomalies at birth, including complex congenital heart disease and a single dysplastic ectopic kidney, and received a cadaveric transplanted kidney at the age of 13. She had hyperglycaemia consistent with post-transplant diabetes mellitus (DM) and was started on insulin. Examination at the time revealed truncal obesity. She developed acute graft rejection and graft failure 14 months post-transplant and she was started on haemodialysis. Her blood glucose levels normalised post-graft explant, but she was hyperglycaemic again during haemodialysis at the age of 23. Given her clinical phenotype, negative diabetes antibodies and normal pancreas on ultrasound, she was assumed to have type 2 DM and achieved good glycaemic control with gliclazide. Involve clinical genetics early in the investigative pathway of sick neonates born with multiple congenital anomalies to establish a diagnosis to direct medical care. Consider the possibility of Kabuki syndrome (KS) in the differential diagnoses in any neonate with normal karyotyping or microarray analysis and with multiple congenital anomalies (especially cardiac, renal, or skeletal), dysmorphic facial features, transient neonatal hypoglycaemia and failure to thrive. Consider the possibility of diabetes as an endocrine complication in KS patients who are obese or who have autoimmune disorders.

Right Ectopic Pelvic Kidney and Bilateral Vascular Aberrations a Female Donor: A Case Report

During an abdominopelvic cavity dissection in an undergraduate gross anatomy course, a right ectopic pelvic kidney was identified in the lesser pelvis and was concomitant with bilateral vascular aberrations. The right kidney was located anterior to the sacrum and medial to the right common iliac artery bifurcation with aberrant vasculature. The left kidney was located near the traditional T12-L3 level; however, multiple aberrant arteries supplied this kidney as well. Such renal anatomy anomalies have immediate implications in anatomy and embryology education, medical imaging, abdominopelvic surgical approaches, and the field of nephrology regarding renal form, function, and pathophysiology.

Ectopic Intrathoracic Kidney due to Diaphragmatic Eventration Detected on Bone Scan.

The technetium-99m methylene diphosphonate (99mTc-MDP) whole-body bone scan along with single-photon emission computed tomography (SPECT/CT) can detect challenging soft tissue uptake patterns. We present a case of a 67-year-old female in whom the 99mTc-MDP scan, performed 3hours after injection, revealed abnormal soft tissue uptake in the right thoracic region. No functioning right kidney was seen in the right lumbar region. Hybrid SPECT/CT revealed an ectopic right kidney in the subdiaphragmatic location, accompanied by gut loops and eventration of the right-sided diaphragm. This case underscores the value of SPECT/CT in identifying and characterizing unexpected anatomical abnormalities, such as ectopic kidneys.

A rare case of a combination of ectopic kidney and medial arcuate ligament syndrome: a case report

BackgroundEctopic kidney and median arcuate ligament syndrome are both rare conditions. The clinical presentation and diagnosis of these conditions are not well studied. There are no reports on the combination of these two rare conditions.Case presentationWe report a 24-year-old woman with fever, dysuria, urinary frequency and left flank pain for two days. The primary diagnoses in the clinic were left acute pyelonephritis and left hydronephrosis due to throbbing pain in the left costovertebral angle and pyuria. However, further computed tomography showed right ectopic pelvic kidney, left renal pelvis dilatation without definite ureteral lesion, good bilateral renal contrast enhancement, and compression of the celiac axis due to obstruction by the median arcuate ligament. Chronic abdominal symptoms were reported by the patient after repeat history taking. The patient’s condition was fully explained and discussed with her and her family, but they refused further therapy. After the acute pyelonephritis began improving, the patient was discharged for follow-up at our outpatient clinic.ConclusionWe present an extremely rare case of a combination of two rare conditions: ectopic kidney and median arcuate ligament syndrome. No study to date has reported on the relationship between the two diseases. Given the rarity of the two conditions, no evidence or even a hypothesis exists to explain the possible etiology of their combination. More reports are required to enhance the understanding of these rare conditions.

Acquired ectopic kidney.

Acquired ectopic kidney.

Mini Percutaneous Nephrolithotomy in Ectopic Lumber Kidney: A Case Report

The chance of the development of hydronephrosis and the formation of stones is more in the ectopic kidney. Treatment of stones in ectopic kidneys is challenging for urologists due to abnormal location and rotation of the kidney and unfavourable local anatomy. We herein report a case of stone in a lumber kidney successfully managed by mini percutaneous nephrolithotomy (PCNL) in a prone position. A 55-year-old male presented with right lower abdominal pain. His CT scan of the KUB reveals the right kidney is in an ectopic location and has a hyperdense structure measuring about 15 mm in length, seen in the right renal pelvis with mild dilatation of the pelvicalyceal system. The patient underwent right-sided prone mini PCNL under the subarachnoid block (SAB), and complete stone clearance was achieved. There was no adverse event during or after surgery or post-operative blood transfusion. Bangladesh J. Urol. 2022; 25(2): 122-125

WITHDRAWN: Ectopic Kidney as a Clinical Manifestation of Type II MRKH Syndrome Patient: A Case Report

The article has been withdrawn at the request of the author. Bentham Science apologizes to the readers of the journal for any inconvenience this may have caused. The Bentham Editorial Policy on Article Withdrawal can be found at https://benthamscience.com/editorial-policies-main.php. Bentham Science Disclaimer: It is a condition of publication that manuscripts submitted to this journal have not been published and will not be simultaneously submitted or published elsewhere. Furthermore, any data, illustration, structure or table that has been published elsewhere must be reported, and copyright permission for reproduction must be obtained. Plagiarism is strictly forbidden, and by submitting the article for publication the authors agree that the publishers have the legal right to take appropriate action against the authors, if plagiarism or fabricated information is discovered. By submitting a manuscript, the authors agree that the copyright of their article is transferred to the publishers if and when the article is accepted for publication.

The Diagnostic Efficacy of and Requirement for Postnatal Ultrasonography Screening for Congenital Anomalies of the Kidney and Urinary Tract.

We aimed to investigate the efficacy of postnatal ultrasonography in detecting congenital anomalies of the kidneys and urinary tract in term infants without prenatal history of congenital anomalies of the kidneys and urinary tract. In this retrospective cohort study, we reviewed the records of term infants between six weeks and three months of age who underwent urinary tract ultrasonography during routine pediatric care. Congenital anomalies of the kidneys and urinary tract were detected on prenatal ultrasonography in 75 of the 2620 patients included in the study. Congenital anomalies of the kidneys and urinary tract were detected via postnatal USG in 46 (1.8%) of 2554 patients without anomalies on prenatal USG screening. The most common anomaly was hydronephrosis (69.6%). Thirty-two cases of hydronephrosis, three cases of renal agenesis, four cases of horseshoe kidney, one case of MCDK, and two cases of duplex systems which were not detected on prenatal USG were detected on postnatal USG. On the other hand, 29 (1.1%) cases with mild or moderate hydronephrosis on prenatal ultrasonography did not have hydronephrosis on postnatal ultrasonography. In our study, approximately one-third of the cases of hydronephrosis, unilateral renal agenesis, duplex systems, horseshoe kidney, and ectopic kidney were not detected in prenatal ultrasonography screening. Therefore, we believe that in addition to prenatal ultrasonography screening, postnatal ultrasonography screening of all children for urinary tract anomalies would be beneficial.

Laparoscopic Approach for Reconstructive and Ablative Procedures in Ectopic Pelvic Kidneys: A Challenge in 8 Cases.

Introduction and Objective: Laparoscopy is the most widely followed approach in ablative or reconstructive kidney surgeries. The aim of this study is to assess the utility and safety of laparoscopic approach in pelvic ectopic kidney surgeries. Methods: Between July 1, 2021 and June 30, 2022, 8 patients with pelvic kidneys; 4 with pelviureteric junction obstruction, 3 with pelvic stones, and 1 nonfunctioning kidney underwent laparoscopic pyeloplasty, pyelolithotomy, and nephrectomy, respectively. The records of all 8 patients were evaluated retrospectively for analyzing the operating time, blood loss, postoperative hospital stay, intra- and postoperative complications, surgical difficulty, and the success in completing the case laparoscopically. The patients were followed for at least 6 months to know the outcome. After pyeloplasty the improvement in function and drainage as well were recorded. Results: Of 8 cases, 6 (75%) were completed laparoscopically. One pyelolithotomy and 1 pyeloplasty patient were converted to open surgery. The median operative time was 180 (140-240) minutes, median blood loss was 100 (50-300) mL, and median hospital stay was 4 (3-6) days. One patient, who had open conversion, had Clavien grade I complication in the form of prolonged fever. Pyeloplasty patients at 6 months follow-up showed improvement in symptoms as well as function. Conclusion: The laparoscopic approach has obvious benefits in pelvic surgeries. Laparoscopy for ectopic pelvic kidneys are challenging due to abnormal anatomy of vessels and kidneys. Proper exposure of kidneys and exact identification of vessels can accomplish laparoscopic procedure in ectopic kidneys successfully with patients having nil complications and early convalescence.

VE31 - Use of augmented reality in robotic renal surgery for the treatment of postural ischemic pain in an ectopic kidney

VE31 - Use of augmented reality in robotic renal surgery for the treatment of postural ischemic pain in an ectopic kidney

Case Report – Incidentally Detected Subdiaphragmatic Renal Ectopia in Dimercapto Succinic Acid Scan (DMSA) – A Rare Occurrence

Kidney lies outside renal fossa, it is called ectopia. The common cause for renal ectopia is failure of ascend during organogenesis. Renal ectopia is not uncommon; however, high renal ectopia is extremely rare. We report a case of male infant with Edward syndrome.

Pelvi-Ureteric Junction Obstruction In A Malrotated Ectopic Kidney With Concomitant Lithiasis – A Rare Clinical Scenario

Kidney lying outside renal fossa is referred as ectopic kidney. Hereby we are presenting a rare case of Pelviureteric junction obstruction in a malrotated ectopic kidney with concomitant lithiasis in a 16 year male. He presented with pain in lower abdomen intermittently. On CT scan, a left malrotated ectopic kidney was located in the pelvis near the midline with a 10 mm stone in the lower pole, and having obstructed pattern drainage with moderate hydronephrosis. Open Anderson-Hynes dismembered pyeloplasty was done. Thorough clinical approach with delineating aberrant anatomy of ectopic kidneys and their vascular supply with consideration of unusual functional data on conventional functional renogram is necessary for intervening in malrotated ectopic kidney with concomitant lithiasis.

Pelvic Ectopic Kidney Prevalence and Pressure Changes During Cloacal Exstrophy (Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome) Closure

To assess the prevalence of pelvic ectopic kidneys (PEK) and compare renal parameters of the PEK to the orthotopic kidney following pubic bone approximation. In four Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Syndrome (OEIS) patients undergoing second-stage repair with known pelvic and orthotopic kidneys, changes in the renal pelvis pressure (RPP), peak systolic velocity (PSV), and resistive index (RI) were measured in the pelvic and orthotopic kidneys following pubic bone approximation. A paired t-test was performed for analysis. Prevalence was tabulated using a multi-institutional retrospective review. Mean rise in RPP was +26.0 mmHg in the PEK and +10.3 mmHg in the orthotopic kidney (P = .55). One patient had immediate increase in RPP of 66mmHg in the PEK (7mmHg in the orthotopic kidney) which decreased to +17 mmHg in the PEK upon release of the pubic approximation stitch. Mean change in PSV was +67.7 cm/s in the PEK compared to - 25.7 cm/s in the orthotopic kidney (P = .09). Mean change in renal RI was +0.06 in PEK compared to -0.01 in the orthotopic kidney (P = .29). Among 80 OEIS patients, 24 (30%) had a PEK. 3 (4%) had a solitary PEK. During second-stage OEIS closure, we witnessed patterns of higher change in RPP, PSV, and RI of the PEK compared to the orthotopic kidney. Furthermore, PEKs were found in nearly a third of our OEIS patients. Real-time RPP monitoring, especially those with solitary PEK, may aid in the management of OEIS patients during the perioperative period.

Incidence of genitourinary anomalies in congenital scoliosis: systematic review and meta-analysis.

The main objective of this study was to assess the overall incidence of genitourinary anomalies in patients with congenital scoliosis by providing the highest level of evidence. The secondary objective was to look for associations and trends influencing the incidence. A meta-analysis using PubMed, Embase, Scopus, and the Cochrane Collaboration Library database was carried out. We included studies focusing on patients with congenital scoliosis and genitourinary anomalies. The main outcome was the incidence of genitourinary anomalies in congenital scoliosis. We also collected the following data: patient gender, type of deformity (formation, segmentation, or mixed), deformity location, and associated anomalies. We included cohort studies. Data was extracted from published reports and combined using Review Manager 5.4. The quality of the included studies was assessed independently by two authors using the Methodological Index for Non-Randomized Studies (MINORS) criteria. A total of eight cohort studies were included from a pool of 2781 patients. The incidence of genitourinary anomalies associated with congenital scoliosis was 22.91% (95% CI 13.39-32.43%). The incidence of surgically treated genitourinary anomalies was 13.92% (95% CI 4.54-23.31%). There were no differences related to gender (male 49.3% versus female 50.7%; p > 0.05). There were no differences regarding the type of deformity. The incidences of associated intraspinal, cardiac, musculoskeletal and craniofacial anomalies were 33.30%, 17.60%, 27.77% and 19.83% respectively. The most frequent genitourinary anomalies were: unilateral kidney (111/388); renal ectopia (50/367); obstructive uropathy (30/201), horseshoe kidney (30/313) and undescended testicle (16/180). The incidence of genitourinary anomalies associated with congenital scoliosis was 22.91%, and 13.92% were surgically treated. Unilateral kidney was the most common genitourinary abnormality. There were no differences between genders and deformity types. It is important to consider the association between genitourinary anomalies and intraspinal or musculoskeletal anomalies.

Supernumerary Kidney Associated With Other Genitourinary and Cardiovascular Anomalies In A dysmorphic Infant: A Rare Case Report

The supernumerary kidney, also known as the accessory kidney, is one of the rarest congenital anomalies of the genitourinary system without sufficient epidemiological studies. It is defined as having more than two kidneys with each kidney having its own vasculature, collecting system, and capsule. To date, there are few cases documented in literature worldwide. We hereby present the first reported supernumerary kidney case in a preterm 2-month-old infant with dysmorphic features associated with an ectopic kidney and coarctation of the aorta. The coexistence of cardiovascular anomalies and genitourinary anomalies indicates a shared embryologic origin and genetic linkage, underscoring the importance of screening both systems when anomalies are identified in either. Newborn screening ultrasonographic examinations are crucial for detecting congenital anomalies, especially in infants with pertinent medical history or physical features, leading to better outcomes through timely diagnosis and intervention.

Laparoscopic Heminephrectomy in Crossed Fused Renal Ectopia with Complex Cyst

Laparoscopic Heminephrectomy in Crossed Fused Renal Ectopia with Complex Cyst