Anestesia total intravenosa libre de opioides en un paciente con síndrome de Joubert: caso clínico / Opioid free total intravenous anesthesia in Joubert syndrome: Case report

Joubert syndrome (JS) is a rare autosomal recessive disorder characterized by abnormal eye movements, respiratory pattern abnormalities, anatomical airway alterations, mental retardation and hypoplasia/aplasia of the cerebellar vermis confirmed by magnetic resonance imaging. This case report describes the successful management of a patient with JS operated of cholesteatoma under 100% opioid-free total intravenous general anaesthesia. We also provide a brief review of JS, its anaesthetic implications and opioid-free anaesthesia (OFA) technique.

El síndrome de Joubert (SJ) es una enfermedad autosómica recesiva poco frecuente caracterizada por trastornos oculares, respiratorios, alteraciones anatómicas de la vía aérea, retraso mental e hipoplasia/aplasia del vermis cerebeloso constatada mediante resonancia magnética. Presentamos un caso exitoso de paciente con SJ operado de colesteatoma bajo anestesia general endovenosa total 100% libre de opioides. Asimismo, realizamos una breve revisión del SJ, sus implicaciones anestésicas y de la técnica de anestesia libre de opioides.

Simultaneous treatment of parapelvic renal cysts and stones by flexible ureterorenoscopy with a novel four-step cyst localization strategy

ABSTRACT Objective: To assess the safety, feasibility, and efficacy of simultaneous treatment of parapelvic renal cysts and stones by flexible ureterorenoscopy with a novel four-step cyst localization strategy in selected patients. Patients and Methods: We retrospectively reviewed 11 consecutive cases of parapelvic renal cysts with concomitant calculi treated by flexible ureterorenoscopy and laser lithotripsy (FURSL). Marsupialization was performed subsequently with holmium: YAG laser in our institution. Fragmentation was used to manage renal stones and a novel four-step cyst localization strategy was applied in each case for marsupialization. Results: There were no intraoperative complications. Two cases of cystitis were reported postoperatively. The mean operative times of FURSL and marsupialization were 23.6 ± 3.9 minutes and 29.1 ± 9.7 minutes, respectively. During marsupialization, seven patients underwent the first two steps of the new strategy, two patients underwent three steps and two patients underwent all four steps. The mean reduction in hemoglobin level was 4.7 ± 1.7 g / L (range 3-8 g / L). The mean length of hospital stay was 1.2 ± 0.4 days. During a mean follow-up duration of 18 months, all cases remained stone-free and there was no stone recurrence. Parapelvic cysts became undetectable in eight cases and decreased in size by at least half in three cases. Conclusion: With appropriate patient selection, FURSL and marsupialization with a four-step cyst localization strategy is feasible, safe, and effective in treating parapelvic renal cysts with concomitant calculi.

Acquired cystic kidney disease in allograft with long-standing poor function / Doença renal cística adquirida em um enxerto cronicamente disfuncional

Abstract Acquired Cystic Kidney Disease (ACKD) is regarded as a common late condition of end stage renal damage and expresses its most important features when associated with long term hemodialysis. ACKD is also widely known as a premalignant lesion. Its occurrence in chronically rejected renal allografts is rare and its frequency and behavior in this setting are not well known. Herein we report a case of ACKD in a long standing nonfunctional allograft (215 months) which is not associated with malignancy and briefly review the related literature.

Resumo A doença renal cística adquirida (ACKD) é considerada uma condição tardia relacionada à doença renal crônica terminal e manifesta-se de modo mais evidente no contexto de hemodiálise de longo prazo. ACKD é amplamente reconhecida como lesão pré-maligna. Sua ocorrência em enxertos renais cronicamente rejeitados é rara, de modo que a frequência e o comportamento da entidade nesse cenário não estão bem documentados. Relatamos a ocorrência de ACKD em um aloenxerto renal não funcionante sem malignidade após 215 meses de transplante e brevemente revisamos a literatura relacionada.

Safety and feasibility of radiofrequency ablation for treatment of Bosniak IV renal cysts

ABSTRACT Purpose To describe our initial experience with radiofrequency ablation (RFA) of Bosniak IV renal cysts. Materials and Methods From 2010 to 2014, 154 renal tumor cases were treated with percutaneous thermal ablation, of which 10 cases (6.4%) from nine patients were complex renal cysts and were treated with radiofrequency ablation. Results All complex cysts were classified as Bosniak IV (four women and five men; mean age: 63.6 yrs, range: 33–83 years). One patient had a single kidney. Lesion size ranged from 1.5 to 4.1cm (mean: 2.5cm) and biopsy was performed on four cysts immediately before the procedure, all of which were malignant (two clear cell and two papillary carcinoma). Mean volume reduction of complex cysts was 25% (range: 10–40%). No patients required retreatment with RFA and no immediate or late complications were observed. The follow-up of Bosniak IV cysts had a median of 27 months (interquartile range [IQR], 23 to 38) and no recurrence or significant loss of renal function were observed. Conclusions Mid-term follow-up of the cases in our database suggests that image-guided percutaneous RFA can treat Bosniak IV cysts with very low complication rates and satisfactorily maintain renal function.

Laparoscopic Management of a Renal Hydatid Cyst Masquerading as Simple Renal Cyst.

Hydatid cyst is a parasitic disease caused by the tapeworm Echinococcus granulosus. Hydatid cyst disease mostly involves the liver and the lung, while renal involvement is rare. Hydatid disease may present in unusual ways and lead to diagnostic diffi culty and management problems. A high index of suspicion for hydatid disease should be maintained while evaluating complex cystic renal masses. Herein we present an interesting case who was diagnosed to have a simple renal cyst on contrast-enhanced computed tomogram, which turned out to be a renal hydatid cyst. Despite modern imaging methods, isolated renal hydatid disease might still cause diagnostic dilemma and hydatid cysts can be found in unusual localization. Th is case was managed laparoscopically.

Reporte de un caso de Hipernefroma en paciente con enfermedad renal poliquística autosómica dominante / Case report of Hypernefrom in patients with autosomal dominant polycystic kidney disease

La presencia de carcinoma de células renales durante la evolución de una enfermedad renal poliquística autosómica dominante es muy rara y poco reportada en la literatura internacional. Se reporta un caso de enfermedad renal poliquística asociado con carcinoma de células renales. La forma de presentación fue un síndrome doloroso lumbar, hematuria macroscópica, pérdida de peso, síndrome febril agudo, eritrosedimentación acelerada y aumento de volumen de una imagen de aspecto heterogénea en el riñón izquierdo. En el acto quirúrgico se comprobó presencia de infiltración de la cápsula renal, uréter y arteria renal no infiltrada; en el estudio patológico macroscópico sin infiltración de la vena o arteria renal, ni uréter y en el microscópico, presencia de tejido neoplásico en el parénquima renal.

The presence of renal cell cancer during the evolution of an Autosomal Dominant Polycystic Kidney Disease is very rare and little reported in international Literature. An associated case of polycystic kidney disease with renal cell cancer is reported. The form of presentation was a lumbar painful syndrome, microscopic hematuria, loss of weight, acute febrile syndrome, accelerated eritrosedimentation and increase of volume of a heterogeneous image of aspect in the left kidney. In the surgical act the presence of infiltration of the renal capsule, ureter and not infiltrated renal artery was verified; in the macroscopic pathological study without infiltration of the vein or renal artery, ureter and in the microscopic one, there was presence of neoplastic tissue in the renal parenchyma.

Nefrectomía parcial laparoscópica: técnica y resultados / Laparoscopic partial nephrectomy: technique and results

Introducción: La nefrectomía parcial laparoscópica (NPL) es una alternativa mínimamente invasiva para el tratamiento de los tumores renales. Presentamos la experiencia inicial del autor. Material y Método: Estudio prospectivo descriptivo de 17 pacientes con diagnóstico de tumor renal sometidos a NPL en forma consecutiva por un mismo cirujano, entre septiembre de 2001 y julio de 2005. La edad promedio fue de 54,5 años (r: 27-76); 12 (70,6 por ciento) pacientes con diagnóstico de hipernefroma (66,7 por ciento), 3 (17,65) con diagnóstico de quiste renal complejo Bosniak III, y 2 (11,76 por ciento) Bosniak IV. El tamaño promedio al TAC fue 2,8 cms (2-4,2 cms); 8 lesiones del tercio medio, 6 del tercio inferior y 4 del tercio superior. Se realizaron 12 tumorectomías y 5 polectomías, se realizó abordaje transperitoneal puro en 10 (58,8 por ciento) pacientes, mano asistido en 2 (11,76 por ciento) pacientes, y lumboscópico en 5 (29,4 por ciento) pacientes. Resultados: El tiempo operatorio promedio fue 156,6 minutos (r: 75-210); el sangrado promedio fue 214,7 ml (r: 50-600); el control vascular se realizó mediante clampeo arterial en 10 (58,8 por ciento) pacientes y clampeo de pedículo en 1 (5,9 por ciento). El tiempo de isquemia caliente promedio fue de 27,8 min. (r: 18-40). En 6 (35,3 por ciento) pacientes el control vascular se realiza sin isquemia. Como técnica de hemostasia se utilizó surgicell más uno a tres puntos, en 14 casos con nudo simple y en 3 con técnica de Hemoloc. En un caso el margen quirúrgico macroscópico fue dudoso por lo que se decidió nefrectomía total. Hubo 2 (11,76 por ciento) complicaciones intraoperatorias. Se transfundieron 2 (11,76 por ciento) pacientes. El promedio de hospitalización fue de 4 días (2-10). El análisis anatomopatológico informó 14 casos de carcinoma de células claras, 1 metástasis de cáncer de tiroides, 1 hipernefroma quístico y 1 divertículo caliciliar...

Introduction. Laparoscopic partial nephrectomy (LPN) is a minimally invasive alternative for the treatment of renal tumors. We present our initial experience. Methods. Prospective analysis of 17 patients, with renal tumors operated laparoscopically between September 2001 and July 2005. Mean age was 54,5 years (27-76). 12 (70,6 percent) patients had diagnosis of hypernephroma. Three (17,65 percent) patients had diagnosis of renal cyst Bosniak III and 2 (11,76 percent) renal cyst Bosniak IV. Mean tumor size by computed tomography was 2,8 cm (2-4,2). Localization of tumors was upper third (4), middle third (8) and lower third (6). Twelve lumpectomies and 5 polectomies were performed. Surgical approach was transperitoneal, hand assisted and lumboscopic in 10, 2 and 5 cases, respectively. Results. Mean operative time was 156,6 minutes (75-210), mean blood loss was 214,7 ml (50-600). Vascular control was performed arterial in 10 (58,8 percent), hilar in 1 (5,9) patient and in 6 patients vascular control was achieved with any ischemia. Mean warm ischemia time was 27,8 min (18-40). Different haemostatic methods were employed in the series. Probable positive surgical margin in frozen section was indication for total nephrectomy in one patient. There were 2 intraoperative complications. Two patients received transfusions. Mean hospital stay was 4 days (2-10). Final pathology reported clear cell carcinoma, thyroid cancer metastases, cystic hypernephroma and caliceal diverticulum in 14, 1, 1 and 1 cases, respectively. All patients with malignant lesions remain without recurrence at a mean follow-up of 12 months (1-35). Conclusion. LPN is an alternative in nephron sparing surgery for renal cancer. It represents a technical challenging procedure and requires high skill in intracorporeal laparoscopic suturing. Results presented herein are encouraging but further follow-up is mandatory.

Cystic renal cell carcinoma arising from multilocular cystic nephroma of the same kidney

Multilocular cystic nephroma is an uncommon benign entity grouped among the cystic non-genetic diseases. It is characterized by variable-sized, non-communicating cysts separated by irregular thin walled septa. Though multilocular cystic nephroma is usually considered a benign lesion, malignant changes in the cysts should not be overlooked.

Laparoscopic renal surgery: Ramathibodi Hospital experience.

OBJECTIVE: To report the authors' early experience of laparoscopic renal surgery for benign and malignant renal conditions. MATERIAL AND METHOD: Laparoscopic renal surgery was performed on 24 patients with benign and malignant renal conditions between July 2004 and February 2005. The patient characteristics and perioperative data including operative time, blood loss, analgesic requirement, complications, duration of postoperative drain removal, length of hospital stay, and duration to return to normal activity were all recorded. RESULTS: Laparoscopic simple nephrectomy was performed in 15 patients with nonfunctioning benign renal conditions. Three operations of hand-assisted laparoscopic radical nephrectomy and one of partial nephrectomy were performed for large and small renal cell carcinoma, respectively. Transitional cell carcinomas were managed by retroperitoneoscopic nephrectomy or hand-assisted approach in 3 cases. For a case of severe inflammatory renal condition, hand-assisted approach was used for treatment. Laparoscopic renal cyst decortication was performed in one case. In the laparoscopic simple nephrectomy group, the mean operative time was 126 +/- 38.3 minutes. The median (range) estimated blood loss was 100 (50-500) mL, and one patient required conversion to open surgery because of renal vein injury. In three cases of hand-assisted laparoscopic radical nephrectomy, the operation time was 315, 325 and 150 minutes and the operative blood loss was 500, 1000 and 200 ml, respectively. In cases of hand-assisted laparoscopic partial nephrectomy, the operation time and the operative blood loss were 220 minutes and 350 ml, respectively. In three cases of transitional cell carcinoma, the operation time was 120, 140 and 150 minutes and the operative blood loss was 100, 150 and 150 ml. The surgical margins of all resected specimens for malignant tumors were negative and no major complication was recorded Simple renal cyst decortication was successfully performed within 90 minutes of operation time and bleeding 50 ml. In cases of severe inflammatory renal condition performed by hand-assisted approach, the operative time was 250 minutes and the operative blood loss was 250 ml. CONCLUSION: Laparoscopic renal surgery is a safe and efficacious approach for resection of benign nonfunctioning kidneys and malignant renal tumors.

Hipertensäo arterial associada a cisto renal único / Arterial hypertension associeted to single cyst of kidney

Cisto renal único pode provavelmente causar hipertensäo arterial por compressäo da vasculatura renal levando a uma isquemia tecidual segmentar com ativaçäo do sistema renina-angiotensina. Uma mulher de 41 anos com um cisto renal, situado no polo inferior esquerdo, apresentava pressäo arterial de 170 x 110 mmHg sem medicaçao e de 150 x 100 mmHg com agentes anti-hipertensivos, sendo de difícil controle. Reduçäo da PAD em 15,5 por cento após 3 dias de captopril 50 mg, duas vezes ao dia. Removido o cisto renal sua pressäo arterial tornou-se normal (120 x 85 mmHg) persistentemente e sem uso de medicaçäo anti-hipertensiva após dois anos e meio de seguimento.

Cirugía laparoscópica en quistes renales simples: un avance / Laparoscopic surgery in simple renal cysts: an advance

Los quistes renales simples sintomáticos requieren de un tratamiento quirúrgico. Con la introducción de la vía laparoscópica como un nuevo método de abordaje en cirugía urológica, se ha abierto una nueva modalidad terapéutica para estos casos evitando así la cirugía convencional por lumbotomía.En el período marzo 1993 - junio 1994 se intervinieron cinco pacientes, cuatro mujeres y un hombre con quistes renales simples por vía laparoscópica cuya técnica se describe en detalle. Hubo una conversión a cirugía abierta por adherencias secundarias a cirugía previa. Debido a los buenos resultados obtenidos sin morbimortalidad y a las ventajas evidentes, se propone esta nueva técnica quirúrgica como una alternativa en el manejo de quistes renales simples sintomáticos

Cirugía del quiste renal por videolaparoscopia / Renal cyst surgery by videolaparoscopy

Los quistes renales son las lesiones más frecuentes del rinón, requiriendo del tratamiento en escasas ocasiones. Se presentan 3 pacientes portadores de quistes renales sintomáticos, quienes fueron tratados por videolaparoscopia. En todos los casos se logró la extracción completa de la membrana quística con adecuada marsupialización. No hubo morbilidad y todos los pacientes fueron dados de alta precozmente. La cirugía laparoscópica constituye una laternativa para aquellos pocos quistes renales con indicación quirúrgica

Cisto renal gigante: apresentaçäo de um caso / Giant renal cyst: report of a case

Os autores apresentam um caso de cisto renal gigante, solitário, de tamanho incomum. Observa-se uma incidência de 50% acima dos 50 anos na populaçäo geral, sendo unilaterais, de origem congênita e acometem de preferência o rim esquerdo. Seu diagnóstico diferencial fica facilitado com o emprego da urografia excretora, arteriografia renal seletiva, cintilografia renal e ultrassonografia. O tratamento dos cistos renais volumosos é sempre cirúrgico, näo só pela sintomatologia associada ao tamanho da tumoraçäo, assim como pela possível associaçäo com neoplasia malígna e a possibilidade de ruptura por traumatismo

Quiste renal simple / Simple renal cyst

Se presentan dos casos clínicos portadores de quiste renal simple con seguimiento clínico de tres años y medio cada uno. El primer caso corresponde a un lactante de 3 meses de vida con un quiste renal simple supurado que evolucionó con hipertensión arterial severa que no respondía al tratamiento médico. El segundo, un recién nacido de 20 días cuyo diagnóstico se efectuó mediante ecografía en el quinto mes de embarazo. El tratamiento se realizó en ambos pacientes extirpando el riñón afectado, obteniéndose normalización de la presión arterial, en el primero de ellos, dos meses después de la operación. Actualmente los niños están asintomáticos y su crecimiento y desarrollo son adecuados

Cisto peripiélico / Peripelvic cyst

Säo descritos dois casos de cistos peripiélicos e discutidas as implicaçöes clínicas e terapêuticas dos mesmos