Hydroperitoneum: A unique complication of Supine Percutaneous Nephrolithotomy on an Ectopic Solitary Pelvic Kidney

@#A 47-year-old male complained of anuria for 2 days with elevated creatinine of 14 mg/dL on admission. Patient underwent emergent hemodialysis. Non-contrast CT showed a solitary ectopic pelvic kidney with a 2 cm. pelvolithiasis and a 1 cm upper pole calyceal stone with obstructive hydronephrosis. He therefore underwent ultrasound-guided nephrostomy tube placement. Once clinically stable, the patient underwent a multi-tract supine PCNL. Intraoperatively, the authors noted tense abdominal distention accompanied by hypotension during the procedure. A diagnosis of compartment syndrome secondary to hydroperitoneum was considered. An indwelling stent and a nephrostomy tube were placed. An abdominal pigtail drain was placed removing three liters of fluid. The patient remained intubated for 3 days. He underwent blood transfusion. He required two 2 sessions of hemodialysis postoperatively. The patient was discharged in stable condition on postoperative day 22. Hydroperitoneum is a potential complication of PCNL in ectopic pelvic kidneys. Its prompt recognition, followed by immediate aspiration of intraabdominal fluid and drain placement is life-saving.

Laparoscopic management of urinary tract calculi: uncommon but effective alternative to open surgery in certain situations

Introduction: The use of minimally invasive techniques has helped decrease the morbidity and convalescence associated with the management of urolithiasis. In this regard laparoscopy has also developed as one of the modalities. However, in comparison with other surgeries, laparoscopy for stone removal is relatively uncommon. Here we present our experience of laparoscopic stone surgeries. Materials and methods: 10 patients with large renal and ureteric calculi where planned for laparoscopic surgery. Three patients had 2-3 cm proximal ureteric calculi, 2 patients had > 3cm renal pelvic calculi, 2 patients had multiple renal calculi associated with PUJ Obstruction, 2 patients had pelvic calculi in ectopic kidneys and one patient had calculus in crossed fused ectopia. Results: All the patients had a smooth intra-operative course. Post-operative period was uneventful except for one patient with crossed fused ectopia. One of the patients with PUJ obstruction had a single residual calculus which was managed with ESWL on follow up. Conclusions: Laparoscopic approach can be an effective alternative to endourological or open procedures for stone removal. It can be utilized for stone management in urinary tract where ESWL, PCNL and ureteroscopy have failed or deemed unsuitable. It is also a good option in patients with unusual anatomy such as ectopic kidney.

A Cadaveric Study Of Variations In The Urological System

Introduction: Knowledge of anatomical variations of the urinary system is important for urological surgeriesinvolving renal transplant and radiological interpretations. When urologists and clinicians have a soundknowledge of anatomical variations, it eases management, surgical interventions and helps to reducecomplications.Advanced imaging technology is the boon for the patients requiring minimally invasive approaches for variouskidney disorders. These approaches require precise knowledge of normal and variant anatomy of the kidneys,ureters and vascular structures at the hilum of the kidney. Therefore, the objective of this study was to furnish theclinicians with the major anatomical variations of urological system.Method: Ninety adult human cadavers were examined for number, shape and position of kidneys and the uretersover a period of 5 years. Out of these, 85 were males and 15 were female cadavers.Results: Kidneys were bilaterally present in all the cadavers. Hypoplastic kidneys were seen bilaterally in 3.3%cadavers. Out of 90 cadavers, 3 showed bilateral and 6 showed unilateral lobulated kidneys. 2 cadavers showedunilateral (1 L, 1 R) incomplete double ureter. One showed bilateral and 5 showed unilateral accessory renalartery amongst 90 cadavers. Ectopic kidney was seen in one cadaver.Conclusions: Morphological variations in the kidney are very common and are clinically important for urosurgeons

Cystic fibrosis transmembrane conductance regulator (CFTR) gene abnormalities in Indian males with congenital bilateral absence of vas deferens & renal anomalies.

Background & objectives: The role of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in congenital bilateral absence of vas deferens and unilateral renal agenesis (CBAVD-URA) has been controversial. Here, we report the cases of five Indian males with CBAVD-URA. The objective was to evaluate the presence or absence of CFTR gene mutations and variants in CBAVD-URA. The female partners of these males were also screened for cystic fibrosis (CF) carrier status. Methods: Direct DNA sequencing of CFTR gene was carried out in five Indian infertile males having CBAVD-URA. Female partners (n=5) and healthy controls (n=32) were also screened. Results: Three potential regulatory CFTR gene variants (c.1540A>G, c.2694T>G and c.4521G>A) were detected along with IVS8-5T mutation in three infertile males with CBAVD-URA. Five novel CFTR gene variants (c.621+91A>G, c.2752+106A>T, c.2751+85_88delTA, c.3120+529InsC and c.4375-69C>T), four potential regulatory CFTR gene variants (M470V, T854T, P1290P, Q1463Q) and seven previously reported CFTR gene variants (c.196+12T>C, c.875+40A>G, c.3041-71G>C, c.3271+42A>T, c.3272-93T>C, c.3500-140A>C and c.3601-65C>A) were detected in infertile men having CBAVD and renal anomalies Interpretation & conclusions: Based on our findings, we speculate that CBAVD-URA may also be attributed to CFTR gene mutations and can be considered as CFTR-related disorder (CFTR-RD). The CFTR gene mutation screening may be offered to CBAVD-URA men and their female partners undergoing ICSI. Further studies need to be done in a large sample to confirm the findings.

Impaction of an intrathoracic kidney acted as a shield against herniation of the abdominal viscera in a case of right congenital diaphragmatic hernia

We describe a case of an intrathoracic kidney combined with right congenital diaphragmatic hernia (CDH) that was diagnosed at 32 weeks of gestation. Although it has been well established that a right CDH shows a poorer outcome than a left CDH, our present case showed a good outcome because there was no herniation of other abdominal viscera, except for the right kidney. Our findings in this case indicate that impaction of the intrathoracic kidney may act as a 'shield' against further herniation of other abdominal viscera into the thoracic cavity.

Abdominal Aortic Aneurysm with Right Ectopic Kidney / 日本心臓血管外科学会雑誌

We present the case of a 68-year-old woman with abdominal aortic aneurysm (AAA) complicated by right ectopic kidney, in another term, congenital pelvic kidney. The patient underwent AAA repair and right renal artery reconstruction using renal perfusion with cold Ringer's acetate, and no deterioration of renal function was observed. 3D-CT was essential diagnostic procedure in order to identify the blood supply to the ectopic kidney for planning a careful surgical technique.

A Case of Neurofibromatosis Combined with Ectopic Kidney / 소아과

Neurofibromatosis(NF) is an autosomal dominant disorder, probably of neural crest origin that affects all three germinal layers. It is a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Type 1(von Recklinghausen disease) is the most common neurocutaneous disorder among the eight subtypes. Previous reports showed various involvements in the renal organ. Renovascular hypertension is the most common major manifestation of renal involvement in this disease. However, we experienced a case of ectopic kidney concurrent with neurofibromatosis type 1. The diagnosis of neurofibromatosis had been made by typical skin manifestation on physical examination, and ectopic kidney was discovered accidentally during routine abdominal sonography. The etiological basis of this association is not clear. We report a rare case of coexisting neurofibromatosis and ectopic kidney in a 7-year-old girl with a brief review.

A Case of Intrathoracic Kidney in an Adult / 대한신장학회잡지

Intrathoracic kidney is a very rare congenital anomaly in development of the kidney. It was firstly described by Mikulics in 1922. More than 130 cases were reported in the world. Most of the patients have no clinical problem and discovered incidentally in chest X-ray. We have experienced a case of intrathoracic kidney in a 48-year-old female who was admitted due to recurrent calculous cholecystitis. Intrathoracic mass lesion was noted in the plain chest film and confirmed as intrathoracic kidney by chest computed tomography and intravenous pyleography. We report a case of intrathorcic kidney with brief review of literature.

Hypoplastic lumbar kidney with single vaginal ectopic ureter; a case report / 대한비뇨기과학회지

Lumbar kidney is one of renal ectopia which rests opposite the sacral promontory in the iliac fossa and anterior to the iliac vessels. We have experienced a case of hypoplastic kidney associated with ipsilateral vaginal ectopic urethral opening which had been discovered by abdominal exploration.

A Case of Hypoplastic Ectopic Kidney in Pelvic Cavity / 대한비뇨기과학회지

The Author's are presenting a case of hypoplastic ectopic kidney in pelvic cavity with short ureteral length and ectopic ureteral orifice in vaginal vestibulum. The size of ectopic kidney was unusually small, chest nut size, as dislocated kidney congenitally which made put more effort then usual diagnostic approach to locate it.

Ectopic Kidney (2 Cases) / 대한비뇨기과학회지

We report two cases of ectopic kidney with the review of literatures.

A Case Report of Simple Unilateral Ectopic Kidney / 대한비뇨기과학회지

Additional a case of ectopic kidney with a literatural review was presented. The terms renal ectopia describe kidneys which are congenitally located in abnormal anatomic positions. Its clinical interest is that it often confused with retroperitoneal tumor and pelvic tumor and other abdominal conditions. We experienced a case of ectopic kidney that was clinically confused with retroperitoneal tumor in 22-year old Korean male.

Ectopic Kidney / 대한비뇨기과학회지

Additional a case of ectopic kidney with a literatural review was presented. Right kidney was located at the level of 4th lumbal spine and shortening of the right ureter. Left one was normal.

Simple Unilateral Ectopic Kidney / 대한비뇨기과학회지

Two cases of ectopic kidney were reported in conjunction with review of literatures. The ectopic kidneys, measuring 13 cm-,15 cm in length of their ureter, were located between the L3 .and S1. Operative removal of the ectopic kidney disclosed aberrant arteries in their both pole. histological examination revealed normal findings.