Horseshoe kidney and retrocaval ureter are uncommon congenital anomalies of the genitourinary system that are easily diagnosed by typical imaging features. Both anomalies presenting in one patient is a rare disease characterized by isthmus of horseshoe kidney between the abdominal aorta and inferior vena cava. The clinical diagnosis and treatment of horseshoe kidney with retrocaval ureter remain a challenge. Here, we reported a case of a 44-year-old man with the two anomalies who was preoperatively diagnosed by unenhanced computed tomography scanning immediately after retrograde pyelography. The literatures on such combined anomalies are reviewed and the diagnostic evaluation and surgical management of this rare entity are discussed.
Adult ; Humans ; Kidney ; abnormalities ; surgery ; Male ; Ureter ; abnormalities ; surgery

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare congenital anomaly. Excision of the obstructed vaginal septum is the treatment of choice for symptom relief and the preservation of reproductive capability. A 14-yr-old girl complained of persistent vaginal spotting following each menstruation. Pelvic magnetic resonance imaging revealed a uterus didelphys with left hematocolpos and ipsilateral renal agenesis. Instead of conventional transvaginal excision of the vaginal septum, we used hysteroscopic excision under transabdominal ultrasonographic guidance to preserve the integrity of the hymen. The postoperative course was uneventful, and clinical symptoms were completely resolved after this intervention. Resectoscopic excision of the vaginal septum was found to be easy, safe, effective, and appropriate for young women as it preserved hymen integrity. We believe that this is the first Korean report on the use of a hysteroscopy for vaginal septum resection in a patient with uterus didelphys with obstructed hemivagina.
Adolescent ; Female ; Humans ; Hysteroscopy/*methods ; Kidney/abnormalities ; Urogenital Abnormalities/surgery ; Uterus/*abnormalities ; Vagina/*abnormalities/*surgery/ultrasonography

Abnormalities, Multiple ; surgery ; Adolescent ; Child ; Female ; Humans ; Kidney ; abnormalities ; Kidney Diseases ; Retrospective Studies ; Uterus ; abnormalities ; surgery ; Vagina ; abnormalities ; surgery ; Young Adult

OBJECTIVETo explore the surgical strategy for ectopic kidney and evaluate the clinical outcomes.

METHODSFrom January 2000 to October 2009, 35 cases of ectopic kidney were treated surgically in our hospital. Definite diagnoses were established in all the cases by ultrasound, intravenous urography (IVU), cystoscope, CT, magnetic resonance urography (MRU) and radionuclide imaging before the surgery. In these patients, 26 had ipsilateral ectopic ureteral orifice (including 5 with bilateral duplicated kidneys and ureter), 9 had moderate or severe hydronephrosis (including 3 with ectopic kidney calculi), and 24 had dysplastic kidney (24/35). All the patients underwent operations, including 26 with ectopic nephrectomy, 7 with ectopic ureterovesical reimplantation, and 3 with ectopic renal pelvis incision.

RESULTSThe clinical effect was satisfactory in all the cases during the follow up of 7 to 29 months.

CONCLUSIONAppropriate surgical approaches according to the concurrent deformities and complications can achieve good clinical results in patients with ectopic kidneys.

Abnormalities, Multiple ; diagnosis ; surgery ; Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Kidney ; abnormalities ; surgery ; Nephrectomy ; methods ; Ureter ; abnormalities ; surgery ; Young Adult

BACKGROUNDIn the past several decades we have seen multiple advances in the reconstruction for girls born with vaginal agenesis. This study aimed to evaluate the technical feasibility, anatomical and functional outcomes of one-stage laparoscopic and gasless laparoscopic vaginoplasty with sigmoid colon for the patients of vaginal agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome).

METHODSWe did a retrospective review of a total of 150 women with Mayer-Rokitansky-Kuster-Hauser syndrome treated at Beijing Anzhen Hospital, Capital Medical University from March 2006 to August 2010. The patients were divided into the CO2 pneumoperitoneum laparoscopic group and the abdominal wall lift of gasless laparoscopic group. Sigmoid colon vaginoplasty approaches were performed in all of the patients. The surgical techniques, perioperative results, complications, anatomical and functional outcomes of vaginoplasty were recorded.

RESULTSAll procedures were performed successfully. Significant differences in the operative time and intraoperative blood loss existed in the laparoscopic vaginoplasty group compared with the gasless laparoscopic vaginoplasty group. The patients who underwent sigmoid colon vaginoplasty had good cosmetic results without the problem of excessive mucus production. The postoperative complications were minimal. During a mean follow-up of 15.6 months, no stenosis or shrinkage was encountered. The subjective sexual satisfaction rate with the surgical outcomes in all patients was 83.3%.

CONCLUSIONSLaparoscopic or gasless laparoscopic vaginoplasty with sigmoid colon are effective and feasible approaches for women with congenital vaginal agenesis. The procedures have satisfactory anatomical and functional results.

46, XX Disorders of Sex Development ; surgery ; Abnormalities, Multiple ; surgery ; Adult ; Colon, Sigmoid ; surgery ; Congenital Abnormalities ; Female ; Humans ; Kidney ; abnormalities ; Laparoscopy ; methods ; Mullerian Ducts ; abnormalities ; Pneumoperitoneum ; Postoperative Complications ; Retrospective Studies ; Somites ; abnormalities ; Spine ; abnormalities ; Uterus ; abnormalities ; surgery ; Vagina ; abnormalities ; surgery ; Vaginal Diseases ; surgery ; Young Adult

Adrenal Gland Neoplasms ; surgery ; Adrenalectomy ; methods ; Adult ; Female ; Humans ; Kidney ; abnormalities ; Laparoscopy ; Multiple Endocrine Neoplasia Type 2a ; surgery ; Pheochromocytoma ; surgery

Rectovestibular fistula is the most common type of anomaly found in a female newborn with anorectal malformation. However, when the baby is found to have two orifices in the introitus, rectovaginal fistula is much less common and suspected. The rare differential diagnosis of Müllerian agenesis, a condition in which the rectum shifts anteriorly and the vagina is absent, is seldom considered. In many cases, the diagnosis of Müllerian agenesis is made only during definitive anorectoplasty. In view of its impact on management, a proper examination under anaesthesia, imaging studies and a diagnostic laparoscopy may be required to confirm the presence or absence of Müllerian structures in such patients. We herein describe a patient with the rare coexistence of VACTERL association and Müllerian agenesis, and discuss the management of anorectal malformations in female patients with Müllerian agenesis.
Abnormalities, Multiple ; diagnosis ; Anal Canal ; abnormalities ; surgery ; Anorectal Malformations ; Anus, Imperforate ; complications ; diagnosis ; surgery ; Child ; Diagnosis, Differential ; Esophagus ; abnormalities ; Female ; Heart Defects, Congenital ; complications ; Humans ; Infant, Newborn ; Kidney ; abnormalities ; Laparoscopy ; Limb Deformities, Congenital ; complications ; Mullerian Ducts ; abnormalities ; Rectal Fistula ; diagnosis ; Rectum ; abnormalities ; surgery ; Spine ; abnormalities ; Trachea ; abnormalities ; Vagina ; abnormalities

Two examples of the rare case of complete duplicated collecting system with lower pole ureteropelvic junction obstruction are described. Ureteropelvic junction obstruction (UPJO) and duplicated collecting systems seldom occur in combination. Complete duplication of the ureter may be asymptomatic or recognized when complications develop as a result of reflux into the lower pole ureter or obstruction of the upper pole with an ectopic ureterocele. It is difficult to choose an optimal therapy due to the high variability in function, degree of obstruction, damage and potential for regeneration in growing kidneys. The diagnosis and management of UPJO of the lower pole in complete duplicated collecting systems are discussed.
Case Report ; Female ; Human ; Infant ; Infant, Newborn ; *Kidney Pelvis ; Kidney Tubules, Collecting/*abnormalities/surgery ; Male ; *Ureteral Obstruction/*complications/diagnosis/surgery ; Ureterostomy

An adenocarcinoma arising in a horseshoe kidney (HK) is rare. The case of a forty five-year-old male patient, presenting with a recurrent, painless hematuria, is reported. On investigation the patient was found to have a horseshoe kidney, with an adenocarcinoma in the left hemi-kidney, which was treated surgically, with a hemi-nephrectomy, of the involved part, being performed to excise the tumor. A brief review of the relevant literature is also presented.
Carcinoma, Renal Cell/*complications/pathology/surgery ; Human ; Kidney/*abnormalities/radiography ; Kidney Neoplasms/*complications/pathology/surgery ; Lymph Node Excision ; Male ; Middle Aged ; Nephrectomy/methods

Renal cell carcinoma associated with fused ectopic kidneys has rarely been reported in the literature. Here we report the first case of robot-assisted heminephrectomy for chromophobe renal cell carcinoma in an L-shaped fused ectopic kidney. The present case report highlights the importance of three-dimensional vision and enhanced maneuverability with the EndoWrist technology of the robotic surgical system for precise dissection. This report also highlights the importance of preoperative contrast-enhanced computed tomography with three-dimensional arterial reconstruction for surgical planning.
Carcinoma, Renal Cell/radiography/*surgery ; Female ; Humans ; Kidney/*abnormalities/radiography ; Kidney Neoplasms/radiography/*surgery ; Middle Aged ; Nephrectomy/*methods ; Robotic Surgical Procedures/*methods ; Tomography, X-Ray Computed