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

Lymphangioma is a congenital abnormality of the lymphatic system detected primarily in early childhood. There are rare reports of mediastinal lymphangioma in older adults. We hereby report on a 66-year-old female patient who underwent kidney transplantation 20 years previously and who developed pathologically confirmed solitary mediastinal lymphangioma 1 year ago. Chest radiography showed a mediastinal nodule, which was not observed 2 year previously, therefore she was referred to the pulmonary division. She had no symptoms, and chest computed tomography demonstrated a 25-mm, well-defined, low-density nodule located at the anterior mediastinum. The size of the nodule had increased from 25 mm to 34 mm 1 year later, and it was completely resected via video-assisted thoracic surgery. The histological diagnosis was cystic lymphangioma. Therefore, we recommend that clinicians consider cystic lymphangioma as a possible diagnosis even in older patients with a mediastinal cystic mass that shows progressive enlargement.
Adult ; Aged* ; Congenital Abnormalities ; Diagnosis ; Female ; Humans ; Kidney Transplantation* ; Kidney* ; Lymphangioma* ; Lymphangioma, Cystic ; Lymphatic System ; Mediastinal Cyst* ; Mediastinum ; Radiography ; Thoracic Surgery, Video-Assisted ; Thorax

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

OBJECTIVETo investigate the diagnosis, treatment and surgical outcomes of ureteropelvic junction obstruction (UPJO) caused by renal crossing vessels.

METHODSThe case records of 24 patients discharged from Peking University Third Hospital between June 2001 and September 2011 with the diagnosis of UPJO caused by renal crossing vessels were reviewed .Of the 24 patients, 17 were male and 7 were female patients. The mean age was 28 years (range, 2-63 years). The mean disease duration was 22.3 months (range, 7 days to 180 months). Of which, 4 patients underwent open surgery, and the other 20 patients were treated with laparoscopic surgery. Surgical approach was decided by operative conditions: adhesion release technique, dismembered pyeloplasty or Y-V anastomosisor, with or without cut off the crossing vessels. The kind of crossing vessels was recorded, and the effect of surgery was evaluated by follow-up.

RESULTSFifteen cases were caused by oppressed renal crossing artery, 8 cases by renal crossing vein, and 1 case by 2 renal crossing arteries and 1 renal crossing vein. Among them, 11 cases were followed up successfully. Average follow-up time was 48.2 months (range, 13-120 months). Eight cases (8/11) were relieved, and 1 case (1/11) had no obvious improvement, another 2 cases (2/11) were aggravating. Among those 6 cases underwent adhesion release technique, 3 cases were relieved, 1 case had no obvious improvement, and 2 cases were aggravating. Five cases who underwent dismembered pyeloplasty was relieved significantly.

CONCLUSIONSRenal crossing artery is one of the main causes of UPJO, the crossing artery should be retained as far as possible. Crossing vessel oppression is not the only pathological cause of UPJO, so the treatment of UPJ constriction is also very important. Dismembered pyeloplasty seems to be the most efficacies treatment procedure for UPJO caused by repressed vessels, and the remission rate of adhesion release technique seems limited.

Adolescent ; Adult ; Arteries ; surgery ; Child ; Child, Preschool ; Female ; Humans ; Hydronephrosis ; congenital ; etiology ; surgery ; Kidney ; blood supply ; Kidney Pelvis ; blood supply ; Laparoscopy ; methods ; Male ; Middle Aged ; Multicystic Dysplastic Kidney ; etiology ; surgery ; Renal Artery ; abnormalities ; Treatment Outcome ; Ureteral Obstruction ; etiology ; surgery ; Young Adult

OBJECTIVE: To evaluate the safety and efficacy of transarterial nephrectomy, i.e., complete renal artery embolization, as an alternative to surgical nephrectomy. MATERIALS AND METHODS: This retrospective study included 11 patients who underwent transarterial nephrectomy due to a high risk of surgical nephrectomy or their refusal to undergo surgery during the period from April 2002 to February 2013. Medical records and radiographic images were reviewed retrospectively to collect information regarding underlying etiologies, clinical presentations and embolization outcomes. RESULTS: The underlying etiologies for transarterial nephrectomy included recurrent hematuria (chronic transplant rejection [n = 3], arteriovenous malformation or fistula [n = 3], angiomyolipoma [n = 1], or end-stage renal disease [n = 1]), inoperable renal or ureteral injury (n = 2), and ectopic kidney with urinary incontinence (n = 1). The technical success rate was 100%, while clinical success was achieved in eight patients (72.7%). Subsequent surgical nephrectomy was required for three patients due to an incomplete nephrectomy effect (n = 2) or necrotic pyelonephritis (n = 1). Procedure-related complications were post-infarction syndrome in one patient and necrotic pyelonephritis in another patient. Of four patients with follow-up CT, four showed renal atrophy and two showed partial renal enhancement. No patient developed a procedure-related hypertension. CONCLUSION: Transarterial nephrectomy may be a safe and effective alternative to surgical nephrectomy in patients with high operative risks.
Adult ; Aged ; Angiomyolipoma/therapy ; Arteriovenous Malformations/therapy ; Child ; Embolization, Therapeutic/adverse effects/*methods ; Female ; Graft Rejection/therapy ; Hematuria/etiology ; Humans ; Infarction/etiology ; Kidney/blood supply ; Kidney Diseases/surgery/*therapy ; Kidney Failure, Chronic/therapy ; Kidney Neoplasms/therapy ; Male ; Middle Aged ; Nephrectomy/adverse effects/*methods ; *Renal Artery/abnormalities ; Retrospective Studies ; Young Adult

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

Crossed fused renal ectopia is a rare anomaly and may be associated with pelvic ureteric junction obstruction (PUJO). The L-shaped fusion variety is even rarer. We report such a case with a crossed fused ectopic pelvic kidney (L-type) with PUJO and its successful laparoscopic management. Through this report we emphasize the importance of adequate preoperative imaging and intraoperative details to avoid mishaps.
Adolescent ; Female ; Humans ; Kidney/*abnormalities ; Kidney Diseases/*complications/congenital/surgery ; Kidney Pelvis/*surgery ; Laparoscopy/*methods ; Reconstructive Surgical Procedures/*methods ; Ureter/*surgery ; Ureteral Obstruction/etiology/*surgery ; Urologic Surgical Procedures/methods

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

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

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