Renal Cell Carcinoma Arising from Isthmus of Horseshoe K.

The most common congenital renal fusion anomaly is the horseshoe kidney (HSK) occurring in about 1 in 600-700 individuals in the Indian population. HSKs are associated with problems such as renal stones, obstruction of uretero-pelvic junction causing stasis, and infection due to ectopic location of the kidneys, malrotation of the kidneys, and vascular changes. In general, normally developed kidneys have more incidents of renal cell carcinoma (RCC) as compared to HSKs. The major issue arises during surgery of HSK due to their altered anatomy and aberrant blood supply. We present a case of HSK with RCC located in the isthmus of a 43-year-old woman.

An Unusual Case of Torticollis: Split Cord Malformation with Vertebral Fusion Anomaly: A Case Report and a Review of the Literature.

We describe the exceptional case of spinal cord malformation, associating neurenteric cyst, and cervical vertebral malformation, initially presenting as torticollis. A 4-month-old child presented with torticollis to the right since birth. A cervical spine X-ray revealed suspicious findings of fusion anomaly, and a cervical spine CT showed extensive segmentation-fusion anomaly with an anterior and posterior bony defect in the C1-6 vertebrae. A cervical spine MRI revealed extensive segmentation-fusion anomaly with an anterior bony defect, and the spinal cord split forward and backward at the C3 level, showing two hemicords. The anterior half of the hemicord and dural sac extended to the right inferior side, towards the upper blind end of esophageal duplication, and the posterior half joined the hemicord at the back and C6 level. After multidisciplinary collaboration, follow-up and conservative treatment were planned. At 12 months, he had developmental delay, and torticollis showed little improvement. No neurological abnormalities have been observed. The patient plans to undergo surgery for the cervical spine fusion anomaly. Cervical spine X-rays should always be performed when assessing a patient with torticollis to rule out cervical vertebral segmentation anomalies, despite the rarity of the condition.

Long-Term Follow-Up of Laparoendoscopic Single-Site Partial Nephrectomy for Nonfunctioning Moieties of Renal Duplication and Fusion Anomalies in Infants and Children.

Objectives: To report on long-term follow-up and outcomes of infants and small children who have undergone laparoendoscopic single-site (LESS) partial nephrectomy to manage upper urinary tract duplication and fusion anomalies. Materials and Methods: A retrospective review was performed evaluating outcomes of pediatric patients who underwent LESS partial nephrectomy for upper urinary tract duplication and fusion anomalies from January 2012 to July 2015, by a single surgeon at a tertiary pediatric referral center. Demographic and perioperative data were reviewed as well as follow-up imaging. Descriptive statistics were used for analysis. Results: A total of 18 patients were identified. Additional access was used for two patients to complete the procedure. Median follow-up was 39 months. No short-term complications were encountered. Three patients were noted to have abnormal postoperative ultrasounds: two perinephric fluid collections and one atrophy of the remaining ipsilateral moiety. One fluid collection resolved completely, and the other decreased in size. Complete atrophy of the ipsilateral moiety occurred. No secondary procedures were required. Conclusions: LESS partial nephrectomy is safe for renal duplication and fusion anomalies with good long-term outcomes. Although it can be safely performed, it is a technically demanding procedure and has been discontinued in favor of robotic partial nephrectomy.

Crossed fused renal ectopia in children: a review of clinical profile, surgical challenges, and outcome.

INTRODUCTION: Crossed fused renal ectopia is a rare congenital malformation, wherein both kidneys are present unilaterally, with the ureter of the crossed kidney opening into the bladder on the contralateral side. It has varied presentation from incidental detection to renal impairment. In this largest series of crossed fused renal ectopia, the authors assessed the clinical profile of these children and also attempted to shed light on the challenges in the surgical management. METHODS: It is a retrospective study conducted from January 2009 to July 2018, among patients evaluated for crossed fused ectopia, in the Department of Pediatric Surgery, Christian Medical College, Vellore. Electronic medical records of 36 children were reviewed. Imaging modalities and operative interventions were recorded. Results were analyzed. RESULTS: A total of 36 children were found to have crossed fused ectopia, with male preponderance. Most children presented within one year of age with urinary tract infection being the commonest cause. The most common associated anomaly was anorectal malformation. Ultrasound abdomen and pelvis, micturating cystourethrogram, and radionucleotide scans were the imaging modalities preferred for diagnosis and follow-up. Left-to-right ectopia was more common with inferior ectopic variant being the most common. The predominant urological problems include pelviureteric junction obstruction, vesicoureteric junction obstruction, and vescioureteric reflux. Ureteric re-implant was the most common surgery performed followed by pyeloplasty. Pelvi-pelvostomy, uretero-ureterostomy, bladder augment with Mitrofanoff, and other procedures were performed for select cases. CONCLUSION: Crossed fused renal ectopia is a challenging entity which requires individualized management plans based on the predominant urological anomaly and the functional status. Surgical options are diverse and are guided toward the symptomatic urological problem with focus on preserving the renal function. The long-term prognosis is good in these children.

Pancake Kidney Found Inside Abdominal Cavity: Rare Case with Literature Review.

The cake or lump kidney is a rare form of fusion. The pancake kidney is always found in the pelvic cavity, in the greater or lesser pelvis. However, we report a case of pancake kidney which was found inside abdominal cavity while investigating 32-year-old male for lower limb pain. CT scan with IV contrast reveals 'fused mass situated in the right side of abdominal cavity with course uncrossed ureters opening separately into the urinary bladder found incidentally with patient work up'. Pancake kidney in this case was managed conservatively without any complication. This malformation is usually present in pelvic cavity, so blood supply is almost constant but in rare cases might be different and follows location of this anomaly.

Pancake Kidney With Obstructed Moiety: A Rare Renal Fusion Anomaly.

Renal fusion abnormalities are rare. Even more rare is pancake kidney. We present a case of a 28-year-old male with symptomatic obstruction of a non-functioning moiety of a pancake kidney. He underwent ureterectomy with a finding of only atretic renal parenchyma at exploration. He recovered well and had resolution of his pain at 3-month follow-up.

Renal cell carcinoma in a horseshoe kidney

@#Horseshoe kidney is a rare form of congenital renal malformation. It occurs in 0.25 to 3% of the population and is usually asymptomatic. Occurrence of symptoms is usually related to infection, lithiasis, and rarely, malignancy. Presented is a case of a 62-year old male with a one-year history of occasional painless hematuria associated with epigastric discomfort. On physical examination, a palpable mass was noted on the right periumbilical area, 10cm x 10cm in size and was non-tender. CT-scan with IV contrast showed 10cm x 7cm x 12cm mass on the right side of a horseshoe kidney. He underwent Radical Nephrectomy, right with Isthmusectomy. Post-operative course was unremarkable. Histopathology result showed Renal Cell Cacrcinoma, clear cell type.. Although malignancy was present in an anomalous kidney, the prognosis is the same as with normal kidneys. To date, this is the first reported case of malignancy on a horseshoe kidney in the Philippines.

Hallazgo anatomopatológico en un paciente adulto con una malformación renal congénita o anomalía de fusión / Pathological finding in an adult patient with a congenital renal malformation or fusion anomaly

Se describe el caso clínico de un paciente adulto con antecedentes de alcoholismo e hipertensión, quien falleció fuera de la institución hospitalaria y al realizarle la autopsia se encontró una ectopia renal cruzada con fusión, así como un único sistema excretor.

The case report of an adult patient with a history of alcoholism and hypertension who died outside of the hospital institution is described, and when carrying out the autopsy a cross renal ectopia with coalition, as well as only one excretory system were found.

Lumbar disc herniation in a patient with congenital vertebral body anomaly: a case report.

Lumbar disc herniation is characterized with low back and leg pain resulting from the degenerated lumbar disc compressing the spinal nerve root. The etiology of degenerative spine is related to age, smoking, microtrauma, obesity, disorders of familial collagen structure, occupational and sports-related physical activity. However, disc herniations induced by congenital lumbar vertebral anomalies are rarely seen. Vertebral fusion defect is one of the causes of congenital anomalies. The pathogenesis of embryological corpus vertebral fusion anomaly is not fully known. In this paper, a 30-year-old patient who had the complaints of low back and right leg pain after falling from a height is presented. She had right L5-S1 disc herniation that had developed on the basis of S1 vertebra corpus fusion anomaly in Lumbar computed tomography. This case has been discussed in the light of literature based on evaluations of Lumbar Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). This case is unique in that it is the first case with development of lumbar disc herniation associated with S1 vertebral corpus fusion anomaly. Congenital malformations with unusual clinical presentation after trauma should be evaluated through advanced radiological imaging techniques.

Lumbar Disc Herniation in a Patient With Congenital Vertebral Body Anomaly: A Case Report

Lumbar disc herniation is characterized with low back and leg pain resulting from the degenerated lumbar disc compressing the spinal nerve root. The etiology of degenerative spine is related to age, smoking, microtrauma, obesity, disorders of familial collagen structure, occupational and sports-related physical activity. However, disc herniations induced by congenital lumbar vertebral anomalies are rarely seen. Vertebral fusion defect is one of the causes of congenital anomalies. The pathogenesis of embryological corpus vertebral fusion anomaly is not fully known. In this paper, a 30-year-old patient who had the complaints of low back and right leg pain after falling from a height is presented. She had right L5-S1 disc herniation that had developed on the basis of S1 vertebra corpus fusion anomaly in Lumbar computed tomography. This case has been discussed in the light of literature based on evaluations of Lumbar Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). This case is unique in that it is the first case with development of lumbar disc herniation associated with S1 vertebral corpus fusion anomaly. Congenital malformations with unusual clinical presentation after trauma should be evaluated through advanced radiological imaging techniques.