Prenatal diagnosis of ectopic kidney: Evaluation of characteristics, additional anomalies and urinary complications.

OBJECTIVE: To assess the characteristics, additional structural anomalies and postnatal urinary outcome of the cases diagnosed with fetal ectopic kidneys in the prenatal period. STUDY DESIGN: Cases having fetal ectopic kidneys, detected from a total of 14,617 pregnant women examined by routine detailed (Group 1) or indicated (Group 2) obstetric ultrasonography (USG) in a tertiary perinatology unit were analyzed. The prevalence of the cases, time of the diagnosis, sidedness of the affected kidney, anatomical location, origins of blood supply, additional urinary or extraurinary anomalies, and urinary complications during the postnatal follow-up period were investigated. RESULTS: We have detected 33 fetuses with ectopic kidneys in our cohort. The prevalence of fetal ectopic kidney was 0.22 %, with a median (min.-max.) diagnosis time of 21.3 (17.6-34) weeks. In the group in whom indicated USG was performed, the time of diagnosis was later compared to routine detailed USG (p = 0.04) group. There was no difference in terms of gender [male, (n = 14), female (n = 19), p = 0.38] and the sidedness of the ectopic kidneys (p = 0.38). The location of ectopic kidneys was most frequent in the iliac fossa (n = 20, 60.6 %) and in the lateral pelvic areas (n = 13, 39.3 %). The blood supply origin of ectopic kidneys was the common iliac artery in 22 (66.6 %), whereas the aorta in 11 cases (33.3 %). There was an additional urinary anomaly in 8 cases (24 %), an extraurinary structural anomaly, most commonly cardiac, and/or a soft marker for aneuploidy were presented in 16 cases (48 %). The most common urinary complication in the postpartum period was vesicoureteral reflux (n = 5). CONCLUSION: Ectopic kidney in the prenatal period is a rare structural anomaly that can equally affect both genders and both kidneys. Prenatal diagnosis is important for the diagnosis of additional anomalies and follow-up of postnatal complications.

Traumatic injury of an ectopic kidney with iatrogenic ureteral injury and endovascular control of aberrant renal vessel.

The management of traumatic injuries in patients with ectopic kidneys presents special challenges. There is a paucity of literature regarding optimal strategies for renal salvage. We describe a case of a patient who presented in hemorrhagic shock after a motor vehicle collision. On initial operative exploration, he was found to have a large retroperitoneal mass. Subsequent imaging demonstrated a large retroperitoneal hematoma and an ectopic kidney. The patient was successfully treated with a combination of open renorrhaphy and endovascular angioembolization. This case demonstrates the importance of a multidisciplinary approach to treating these complex injuries.

A case report on pyelovesicostomy of PUJO of pelvic crossed fused renal ectopia with single ureter.

INTRODUCTION: A pyelovesicostomy is a rare procedure that offers an alternative to difficult pyeloplasty or failed pyeloplasty in the case of PUJO of pelvic renal ectopia. In this case report, we utilized pyelovesicostomy for a dilated renal pelvis of a crossed fused ectopic pelvic kidney with a single ureter. CASE PRESENTATION: This is a 19-year-old male patient who received a diagnosis of pelvic ectopic kidney disease of 4 years duration. He was on a periodic DJ-stent exchange every 3 months at a different hospital. Upon presentation, the patient complains of pain in the right lower quadrant and a decrease in urine output. On physical examination, a palpable mass is detected in the right lower quadrant. The laboratory reveals an elevated creatinine level of 2.3 mg/dl. He was admitted with a diagnosis of acute kidney injury and a crossed fused ectopic pelvic kidney with Pelvic-Ureteric Junction Obstruction and a migrated Double-J stent. Initially, a semi-rigid ureteroscope guided the migrated DJ-stent, which was successfully removed and a new one was inserted. Later, an open surgery with a pyelovesicostomy was performed. Subsequently, the patient improved and was discharged. DISCUSSION: Pyelovesicostomy is an ideal treatment option for pelvic ureteral junction obstruction in congenital pelvic kidneys. The pyelovesicostomy should not be prone to strictures at the anastomotic site as long as it makes wide anastomosis, and the free reflux that results from the surgery shouldn't impair renal function. CONCLUSION: Pyelovesicostomy is feasible, safe, and a potential treatment option for congenital-ectopic pelvic kidney with ureteric obstruction.

Laparoscopy-assisted ultrasound-guiding percutaneous nephrolithotomy for the treatment of stones in pelvic ectopic kidney: A case report.

Background: Pelvic ectopic kidney is a kind of congenital malformations of urinary system due to the failure of ascent from the pelvis to the abdomen during development. Case presentation: A 39-year-old man was referred to the urology department of our hospital for abdominal pain for half a month. Radiographic studies revealed stones in pelvic ectopic left kidney. Laparoscopy-assisted ultrasound-guiding percutaneous nephrolithotomy (PCNL) was performed. Postoperative plain film revealed that the patient achieved a stone-free status. Conclusions: Laparoscopy-assisted ultrasound-guiding PCNL is a safe alternative for the treatment of stones in pelvic ectopic kidney with a high stone-free clearance.

Autosomal dominant polycystic kidney disease with ectopic unilateral multicystic kidney: a case report.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder and the fourth cause of death of end-stage renal disease. The disease has a prevalence of 1:400-1:1000 accounting for 10% of patients on dialysis. In most ADPKD patients, bilateral kidneys are similarly affected, with numerous fluid-filled cysts arising from different nephron segments. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic. CASE PRESENTATION: We report a case of a 46-year-old Ghanaian male patient who presented with left flank pain and hematuria with high BP and deranged renal function. Abdominal ultrasonography showed both kidneys to be larger than normal and had multiple cysts of varying sizes with the right kidney located in the right iliac fossa. Follow up Abdominopelvic computer tomographic scan (CT-Scan) without contrast showed enlarged kidneys with the renal parenchyma replaced by innumerable cyst of varying sizes. The right kidney was ectopically located in the right aspect of the pelvis. A diagnosis of ADPKD with right pelvic ectopic multicystic kidney was made. He was put on antihypertensives, analgesia for the left flank pain and to have follow up at the urology and nephrology departments. CONCLUSION: In most ADPKD patients, bilateral kidneys are similarly affected. Only a few cases of ADPKD with ectopic unilateral multicystic kidney have been reported. It has been observed that the deterioration of their kidney function seemed to be quicker than their age- and sex-matched controls and siblings especially when the ectopic kidney is dysplastic.

Crossed fused renal ectopia diagnosed in an adult: Case report.

INTRODUCTION AND IMPORTANCE: Crossed fused renal ectopia is one of the rare congenital anomalies in which a kidney is found on the side opposite to its normal anatomical location and is fused with the contralateral kidney. Most patients are asymptomatic and diagnosed incidentally. CASE PRESENTATION: In this case report we present a 60 year old woman diagnosed with a left to right crossed fused renal ectopia with fusion of the left kidney to the inferior pole of the right kidney. The patient presented with vague abdominal pain and diagnosis was made with abdominal ultrasound and CT urography. CLINICAL DISCUSSION: Though usually asymptomatic, a number of anomalies and complications can be associated with crossed fused renal ectopia including obstruction, stone disease, and tumors. Evaluation and diagnosis usually requires cross sectional imaging. Patients who are asymptomatic and without complications can be managed conservatively. CONCLUSION: Crossed fused renal ectopia is one of the rare congenital anomalies which can be associated which needs cross-sectional imaging for adequate evaluation. Associated anomalies and complications should be carefully looked for since management depends on it.

Expanding the Clinical Features of Schimke Immuno-Osseous Dysplasia: A New Patient with a Novel Variant and Novel Clinical Findings.

Schimke Immuno-Osseous Dysplasia (SIOD) (MIM:242900) is an ultra-rare autosomal recessive pan-ethnic pleiotropic disease. Typical findings of this syndrome are steroid-resistant nephrotic syndrome, cellular immunodeficiency and spondyloepiphyseal dysplasia and facial dysmorphism. Biallelic variants in the SMARCAL1 gene cause SIOD. The five-and-half-year-old female patient was evaluated because of short stature, dysmorphism, hypercalcemia, hypophosphatemia and elevated FSH levels. Karyotype analysis and array-CGH testing were normal. Clinical Exome Sequencing was performed via next-generation sequencing to analyze genes associated with hypophosphatemia. No pathogenic variant was detected. The subsequent detection of proteinuria during her follow-up for cross-fused ectopic left kidney ultimately facilitated the diagnosis of SIOD, although no obvious spondyloepiphyseal dysplasia was detected. Re-analysis of CES revealed a novel homozygous c.2422_2427+9delinsA pathogenic variant in the SMARCAL1. One hundred twenty-five SIOD cases from 38 literature reporting SMARCAL1 gene pathogenic variants were reviewed to investigate whether hypercalcemia, hypophosphatemia and elevated FSH levels had been previously reported in SIOD patients. This review revealed that this was the first time these findings had been reported in a SIOD patient. This report expands not only the phenotypic but also genotypic spectrum of SIOD.

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 3 hours 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.

Ectopic Kidney with an Ectopic Ureter Masquerading Adnexal Mass: A Case Report and Literature Review.

An ectopic kidney is a rare congenital defect that is often asymptomatic, but can be incidentally discovered during imaging examinations. Moreover, the morphological characteristics and laboratory features of ectopic kidneys are nonspecific, which may lead to misleading diagnostic approaches, particularly when there are additional factors, such as infection, obstruction, or other anomalies. A 43-year-old female presented with a mass in the left adnexal area. She had septate uterus and a history of congenital urinary incontinence. Ultrasound and MRI findings indicated that the mass was a cyst originating from the ovary. However, it is possible that the lump was derived from the urinary system. To confirm the diagnosis, laparoscopy was performed, followed by pathological examination, which confirmed the presence of an ectopic kidney with a single-system ectopic ureter. The patient underwent nephroureterectomy, and her symptoms successfully resolved, leading to a favorable prognosis. This case report highlights a rare case involving an ectopic kidney with a vaginal ectopic ureter that initially presented as an adnexal cyst and caused urinary dribbling. This case emphasizes the importance of early recognition and accurate diagnosis in women with similar symptoms.

Evaluation of Ectopic Kidney Located at the Deep Subcutaneous Region of the Abdominal Wall: Role of Diuretic Renography with 99mTc-DTPA.

An ectopic kidney is often found inadvertently during CT, ultrasonography, MRI, or urologic physical examination. Ectopic kidneys usually occur in the pelvis. A pelvic ectopic kidney may be misinterpreted for a pelvic tumor by less experienced physicians and surgeons. We present an extremely rare case of ectopic kidney in the deep subcutaneous region of the abdominal wall and associated with the additional abnormality of spina bifida. MRI found an ectopic kidney but failed to identify ureteropelvic drainage. Diuretic renography with 99mTc-diethylenetriaminepentaacetic acid showed normal functioning and identified nonobstructive ureteropelvic drainage of the ectopic subcutaneous kidney.

Right-Sided Ectopic Intrathoracic Kidney Associated With Symptomatic Bochdalek Hernia in an Adult Indian Female: Case Report and Review of Literature.

Bochdalek hernia is an inherited posterior lateral defect in the diaphragm that allows the abdominal organs to herniate into the thoracic cavity. In addition to being the most prevalent variety of congenital diaphragmatic hernia (CDH), it is also the type that is observed on the left hemithorax the majority of the time. Ectopic kidney is an uncommon condition, and the occurrence of ectopic intrathoracic kidney is even more uncommon, accounting for only a few of all the cases of renal ectopias. The occurrence of intrathoracic kidney associated with Bochdalek hernia is infrequent among adult individuals and is typically an incidental finding. A 52-year-old obese female patient presented to the pulmonology outpatient unit and reported experiencing the symptoms of coughing, wheezing, and difficulty in breathing since three years. A chest radiograph revealed an elevated dome of the diaphragm on the right side. A computed tomography (CT) of the chest revealed a defect in the posterior aspect of the right hemi-diaphragm with herniation of the right kidney and retroperitoneal fat into the right hemi-thorax. CT urography showed normal size and enhancement of the intrathoracic kidney with prompt excretion of contrast into the pelvicalyceal system. With regard to the small size of the hernia and considering the absence of complications on CT urography, a conservative treatment was proposed to the patient. The patient was followed up every year. There was no occurrence of renal complications during the follow-up period. When evaluating patients with 'elevated hemi-diaphragm' or thoracic 'mass', it is essential to check for the presence of intrathoracic kidney to avoid undesirable surgical procedures and image-guided biopsies.

Outcomes of robot-assisted laparoscopic pyeloplasty among pediatric patients with complex renal anatomy: A retrospective comparative study.

INTRODUCTION: Robot-assisted laparoscopic pyeloplasty (RALP) has been increasingly utilized in the treatment of pediatric ureteropelvic junction obstruction (UPJO) with reported success rates of >95%. Complex renal anatomy can make some cases challenging to reconstruct. OBJECTIVE: To evaluate outcomes of children undergoing RALP with aberrant renal anatomy and compare it to those with simple renal anatomy. METHODS: An IRB approved prospective registry was queried to retrospectively identify all patients who underwent robotic pyeloplasty at our institution from 2012 to 2022. Patients undergoing re-do pyeloplasty were excluded. Complex anatomy was defined as horseshoe kidney, ectopic/pelvic kidney, duplex collecting system, fully bifid renal pelvis and severe malrotation (≥180°). A comparative analysis of baseline demographics, pre-operative clinical/radiological characteristics, intra and post-operative details, and long-term success was performed between those patients with complex anatomy and those without. RESULTS: Of 405 total robotic pyeloplasty's, 375 patients (378 total pyeloplasty; 353 simple, 22 complex) met inclusion criteria. 27 re-do were excluded from analysis. The complex pyeloplasty cohort included 9 horseshoe kidneys, 8 duplex collecting systems, 3 ectopic/pelvic kidneys and 2 kidneys with severe malrotation. There was no difference in age (58 vs 31 months; p = 0.38), procedure time (203 vs 207 min; p = 0.06), length of stay (1.4 vs 1.3 days; p = 0.99), or success (91.6% vs 100%; p = 0.24) between the simple and complex groups. Etiology of obstruction differed significantly between groups - high insertion was more common (3.9% vs 18.2%, p = 0.02) and intrinsic narrowing was less common (60.1% vs 36.4%, p = 0.04) in patients with complex anatomy. A multivariate logistic regression was adjusted for age, gender, etiology of obstruction, preoperative differential renal function and post-operative complications and found no difference in success between complex and simple RALP. DISCUSSION: The findings showed no significant differences in age, procedure time, length of hospital stay, or success rates between the two groups. Specifically, the success rates were 91.6% for the complex group and 100% for the simple group (p = 0.24), indicating comparable efficacy. However, the etiology of obstruction varied significantly, with high ureteral insertion more common in the complex anatomy group (18.2% vs. 3.9%, p = 0.02) and intrinsic narrowing less common (36.4% vs. 60.1%, p = 0.04). Despite these differences, multivariate logistic regression, adjusted for confounders, confirmed no difference in success rates between the groups. CONCLUSION: RALP is a safe and efficacious approach in patients with complex anatomy with success rates comparable to index patients. High ureteral insertion does appear to be more common in patients with complex anatomy undergoing pyeloplasty.

The Discovery of an S-shaped Kidney in a Patient With Prostate Cancer: A Rare Finding.

Crossed fused renal ectopia (CFRE) constitutes a rare congenital anomaly of the urinary tract, typically characterized by its predominantly asymptomatic nature and frequent incidental discovery. This case report delineates the clinical profile of a 56-year-old male admitted to our Prostate Cancer Outpatient Clinic due to elevated prostate-specific antigen (PSA) levels, ultimately leading to the diagnosis of prostate cancer. The patient was asymptomatic, with no family or surgical background. Notably, a fused ectopic kidney was incidentally identified during the staging process involving abdominal computed tomography (ACT) scanning. Remarkably, no additional abnormalities of the urinary tract or renal dysfunction manifested in this specific case. The significance of this report lies in the underscored emphasis on the importance of employing precise imaging techniques and tailored management strategies for patients harboring such anatomical variations.

An Unusual Case of Inguinal Hernia With the Left Ovary and an Ectopic Left Pelvic Kidney in a 37-Year-Old Woman: A Unique Clinical Encounter.

Gynecological components, including ovaries, fallopian tubes, ligaments, and the uterus, are seldom found within hernial sacs. The occurrence of groin hernias containing elements of female genitalia is not well-documented. This case report presents a 37-year-old woman with a unique clinical scenario involving an inguinal hernia containing the left ovary and an associated ectopic left pelvic kidney. The patient's clinical history, characterized by left inguinal pain and swelling, is detailed, including relevant reproductive and medical background. The diagnostic journey encompasses ultrasound and contrast-enhanced computed tomography, revealing the left-sided inguinal hernia with the left ovary. The report emphasizes the challenges posed by the coexistence of inguinal hernia, ovarian involvement, and ectopic pelvic kidney. A multidisciplinary approach is highlighted, encompassing surgical and medical considerations. Surgical intervention involves left-sided inguinal hernioplasty, with a particular focus on fertility preservation through the careful repositioning of the ovary. Postoperative care and considerations for successful recovery are discussed. In conclusion, this case report sheds light on the intricacies of managing a complex clinical presentation, providing insights into diagnostic, surgical, and postoperative aspects. The rarity of such cases underscores the need for ongoing research and collaborative discussions within the medical community.