Localized cystic kidney disease: a case report unveiling clinical and histopathological challenges.

Localized cystic kidney disease (LCKD) is a distinct renal disorder characterized by the presence of cysts within specific regions of the kidneys. We present a rare case of a 41-year-old African American man, who presented to our medical center with lower urinary tract symptoms and gross hematuria. The initial assessment culminated in the identification of an uncomplicated urinary tract infection, prompting the prescription of appropriate oral antibiotic therapy. On follow-up after 5 months, the patient presented with gross hematuria. Imaging studies revealed a mixed-density cystic lesion of 2.6 cm situated within the interpolar region of the right kidney. This cystic lesion exhibited intricate septations at the superior pole of the kidney. Robotic-assisted right partial nephrectomy was performed, and pathologic examination was diagnostic for LCKD. This report not only underscores the uniqueness of LCKD but also presents a comprehensive review of the existing literature that pertains to this condition. Particular emphasis is placed upon its inherent benign behavior and its marked divergence from the progressive trajectory commonly associated with other renal diseases. We also explored the incidental findings of the disease, its diverse clinical symptomatology, conceivable etiological underpinnings, and the array of diagnostic modalities used. Finally, similarities in histopathologic findings with polycystic kidney disease and other entities are discussed, underscoring the importance of accurate diagnosis and management.

Localized cystic kidney disease: a case report unveiling clinical and histopathological challenges

ABSTRACT Localized cystic kidney disease (LCKD) is a distinct renal disorder characterized by the presence of cysts within specific regions of the kidneys. We present a rare case of a 41-year-old African American man, who presented to our medical center with lower urinary tract symptoms and gross hematuria. The initial assessment culminated in the identification of an uncomplicated urinary tract infection, prompting the prescription of appropriate oral antibiotic therapy. On follow-up after 5 months, the patient presented with gross hematuria. Imaging studies revealed a mixed-density cystic lesion of 2.6 cm situated within the interpolar region of the right kidney. This cystic lesion exhibited intricate septations at the superior pole of the kidney. Robotic-assisted right partial nephrectomy was performed, and pathologic examination was diagnostic for LCKD. This report not only underscores the uniqueness of LCKD but also presents a comprehensive review of the existing literature that pertains to this condition. Particular emphasis is placed upon its inherent benign behavior and its marked divergence from the progressive trajectory commonly associated with other renal diseases. We also explored the incidental findings of the disease, its diverse clinical symptomatology, conceivable etiological underpinnings, and the array of diagnostic modalities used. Finally, similarities in histopathologic findings with polycystic kidney disease and other entities are discussed, underscoring the importance of accurate diagnosis and management.

A case of colonoscopy-induced Wunderlich's syndrome in a hemodialysis patient: coincidence or association? / Um caso de síndrome de Wunderlich induzida por colonoscopia em um paciente em hemodiálise: coincidência ou associação?

Abstract Wunderlich syndrome, or spontaneous renal hemorrhage (SRH), is a rare condition encountered in patients undergoing chronic hemodialysis (HD) usually attributed to acquired cystic kidney disease (ACKD) among other causes. In the literature, colonoscopy is associated with splenic injuries, and renal hemorrhage has not been previously described. Management can range from conservative treatment to angiographic embolization or exploration and nephrectomy. Here we report an unusual case of a 54-year-old woman HD patient who presented with SRH within a few days of colonoscopy. The reason of SRH was rupture of an ACKD cyst. We assumed that colonoscopy was a provoking factor and elaborated hypotheses for its etiopathogenesis. The patient underwent successful left nephrectomy. The importance of this case lies in the fact that colonoscopy is not always an innocent procedure in HD patients, and could be complicated by renal cyst hemorrhage.

Resumo A síndrome de Wunderlich, ou hematoma perirrenal espontâneo (HPE), é uma condição rara encontrada em pacientes submetidos à hemodiálise crônica (HD) geralmente atribuída à doença renal cística adquirida (DRCA), entre outras causas. Na literatura, a colonoscopia está associada a lesões esplênicas, e o hematoma renal não foi descrito anteriormente. O manejo pode variar de tratamento conservador a embolização angiográfica ou exploração e nefrectomia. Aqui relatamos um caso incomum de uma paciente em HD de 54 anos de idade que se apresentou com HPE dentro de poucos dias após a colonoscopia. O motivo do HPE foi a ruptura de um cisto de DRCA. Consideramos que a colonoscopia foi um fator provocador e elaboramos hipóteses para sua etiopatogenia. A paciente foi submetida a uma nefrectomia esquerda bem-sucedida. A importância deste caso reside no fato de que a colonoscopia nem sempre é um procedimento inocente em pacientes em HD, e pode ser complicada por hemorragia do cisto renal.

La laparoscopía el nuevo estándar de oro para el tratamiento de quistes renales simples sintomáticos / Laparoscopy the new gold standard for the treatment simple symptomatic renal cysts

más frecuentes del riñón. Su tratamiento consiste en la exploración quirúrgica y marsupialización. La cirugía laparoscópica permite una mejor disección, exploración e identificación de la anatomía con trauma mínimo del paciente. OBJETIVO. Mostrar la experiencia en el tratamiento quirúrgico laparoscópico mínimamente invasivo del quiste renal simple sintomático. MATERIALES Y MÉTODOS. Estudio retrospectivo del tratamiento laparoscópico transperitoneal, se evaluó la seguridad de la técnica, complicaciones perioperatorias y resultados clínicos en el transcurso de un año. Se estudió 8 pacientes: 4 mujeres y 4 hombres, con diagnóstico de quistes renales simples sintomáticos, atendidos por el Servicio de Urología del Hospital de Especialidades Carlos Andrade Marín de Quito, Ecuador. CASOS CLÍNICOS. Se registró edad, sexo, antecedentes patológicos personales, comorbilidades, historia familiar, examen físico completo, exámenes de imagen y de laboratorio. RESULTADOS. La media de la edad fue de 57 años con un intervalo entre 39 y 77 años. La indicación de cirugía fue dolor en región lumbar ipsilateral, que no respondieron a los analgésicos convencionales. El tamaño de los quistes osciló entre 7-10 cm de diámetro. El tiempo operatorio medio fue de 71 minutos sin requerir conversión a cirugía abierta. Los pacientes fueron dados de alta en los primeros cinco días del postoperatorio y los drenajes extraídos dentro del primer día. CONCLUSIÓN. El tratamiento laparoscópico de quistes renales simples sintomáticos por vía transperitoneal fue exitoso en todos los casos. El abordaje permitió la extirpación de los quistes, con menor morbilidad, y con un mejor confort postoperatorio para los pacientes.

kidney. Its treatment consists of surgical exploration and marsupialization. Laparoscopic surgery allows a better dissection, exploration and identification of the anatomy with minimal patient trauma. OBJECTIVE. To show the experience in the minimally invasive laparoscopic surgical treatment of the symptomatic simple renal cyst. MATERIALS AND METHODS. Retrospective study of transperitoneal laparoscopic treatment, the safety of the technique, perioperative complications and clinical results over the course of a year were evaluated. 8 patients were studied: 4 women and 4 men, with diagnosis of symptomatic simple renal cysts, attended by the Urology Service of the Carlos Andrade Marín Specialty Hospital of Quito, Ecuador. CLINICAL CASES. Age, sex, personal pathological history, comorbidities, family history, complete physical examination, image and laboratory tests were recorded. RESULTS. The mean age was 57 years with an interval between 39 and 77 years. The indication for surgery was pain in the ipsilateral lumbar region, which did not respond to conventional analgesics. The size of the cysts ranged between 7-10 cm in diameter. The mean operative time was 71 minutes without requiring conversion to open surgery. The patients were discharged in the first five days of the postoperative period and the drains extracted within the first day. CONCLUSION. The laparoscopic treatment of simple symptomatic renal cysts by the transperitoneal route was successful in all cases. The approach allowed the removal of the cysts, with less morbidity, and with a better postoperative comfort for the patients.

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.

Quiste renal hiperdenso. Presentación de un caso / Hyperdense Renal Cyst. A Case Report

Parejo al aumento de las exploraciones radiológicas ha ocurrido el escubrimiento de quistes renales que, en ocasiones, no cumplen los criterios de benignidad o malignidad. Dentro de esta lesiones se encuentran los quistes hiperdensos, los cuales constituyen un reto para todos los responsables del diagnóstico y tratamiento de estos pacientes. Por tales razones se decidió la presentación del caso de una paciente de 23 años de edad, con antecedentes de sicklemia, que acudió al cuerpo de guardia del Hospital General Universitario Dr. Gustavo Aldereguía Lima, por sufrir dolor lumbar en lado izquierdo, de tipo cólico, que no se aliviaba con analgésicos. Mediante estudio por tomografía axial computadoriada, simple y con contraste endovenoso, se le diagnosticó un quiste renal hiperdenso tipo II, según la clasificación de Bosniak.

Increased radiological tests have facilitated the discovery of kidney cysts, which sometimes do not meet clear criteria for benignity or malignancy. Among these lesions, the hyperdense cysts pose a challenge to all those responsible for their diagnosis and treatment. For such reasons we decided to present the case of a 23-year-old female patient with a history of sickle cell anemia, who attended the emergency department of the Dr. Gustavo Aldereguía Lima University General Hospital due to colic-like pain in the left lumbar region unresponsive to analgesics. She was diagnosed with category II renal cyst according to Bosniak classification using computed tomography with and without intravenous contrast.

Asociación de tumor renal sólido y tumor quístico: a propósito de un caso / Association between solid renal tumor and cystic tumor: a case report

Fundamento: los carcinomas de células renales surgen en su gran mayoría en el parénquima renal, existiendo un número menor de casos que aparecen de inicio en un quiste renal, denominándose carcinoma quístico renal. Objetivo: demostrar que pueden coexistir carcinomas renales sólidos y quísticos de forma independiente, aunque sea infrecuente. Caso Clínico: se presenta el caso clínico de un paciente con carcinoma quístico renal más tumor renal sólido en el parénquima, ambos con la variedad de células claras. El ultrasonido y la tomografía axial computarizada fueron los estudios que ayudaron al diagnóstico. Se le realizó nefrectomía radical en el Hospital Docente de Oncología María Curie, de Camagüey, en junio del 2013. El paciente egresó del hospital a los siete días del tratamiento quirúrgico, con seguimiento por consulta externa.

Background: renal cell carcinomas mostly appear in the renal parenchyma; there are some few cases in which they initially appear in a renal cyst so they are identified as renal cyst carcinoma. Objective: to prove that cystic and solid renal carcinomas can coexist independently, although it is infrequent. Clinical case: the clinical case of a patient who was diagnosed with renal cyst and solid renal tumor in the renal parenchyma, both originated from clear cells, is presented. Ultrasound and CAT scan helped determining the diagnosis. Results: the patient underwent a radical nephrectomy which was performed at the Maria Curie Teaching Oncological Hospital in June 2013 in Camagüey city. The patient was discharged seven days after surgery and a follow-up care was established.

Hematoma perirrenal espontâneo em paciente lúpica submetida a tratamento hemodialítico e portadora cistos renais adquiridos / Spontaneous perirenal hematoma in a lupic patient on haemodialysis treatment and with renal cysts

A hemorragia perirrenal espontânea apresenta-se mais comumente como dor súbita no flanco ipsilateral, sem história de trauma. A doença renal cística adquirida é um achado frequente em pacientes sob tratamento hemodialítico crônico. Entretanto, a hemorragia espontânea proveniente da ruptura dos cistos é uma entidade clínica rara. Descrevemos o caso de uma paciente do sexo feminino, 45 anos, portadora de hipertensão arterial sistêmica controlada há 8 anos, de insuficiência renal crônica por 15 anos e de nefrite lúpica há 2 anos, em tratamento hemodialítico três vezes por semana desde 2006, e que apresentava concomitantemente doença renal cística adquirida. Foi admitida no setor de emergência queixando-se de aparecimento súbito de dor em região toracoabdominal esquerda. Diagnosticou-se hematoma perirrenal por meio de ultrassonografia e tomografia computadorizada de abdômen. A paciente foi submetida à embolização da artéria renal esquerda, com boa evolução.

The spontaneous perirenal hemorrhage usually presents as sudden pain in the side ipsilateral, no history of trauma. Acquired cystic kidney disease is a common finding in chronic hemodialysis patients. However, spontaneous bleeding from the rupture of the cyst is a rare clinical entity. We describe the case of a female patient, 45 years old, with controlled hypertension for 8 years, chronic renal failure for 15 years and lupus nephritis 2 years ago, undergoing hemodialysis three times a week since 2006, and who presented concurrently acquired cystic kidney disease. She was admitted to the emergency department complaining of sudden onset of pain in the thoraco-abdominal left. Perirenal hematoma was diagnosed by ultrasound and computed tomography of the abdomen. The patient underwent embolization of left renal artery, with good evolution.

Uropatias obstrutivas bilaterais fetais: sinais ultrassonográficos durante a gravidez e evolução pós-natal / Fetal bilateral obstructive uropathies: ultrasound findings during pregnancy and postnatal outcomes

OBJETIVO: verificar a associação entre sinais ultrassonográficos durante a gestação e evoluções pós-natais em casos de fetos com uropatias obstrutivas bilaterais, acompanhados de forma expectante. MÉTODOS: fetos com uropatias obstrutivas bilaterais apresentando oligoâmnio grave e tórax estreito foram comparados a fetos com uropatias obstrutivas bilaterais que não desenvolveram estas alterações com relação à presença ou ausência de cistos em ambos os rins e à presença ou ausência de hiperecogenicidade de parênquima em ambos os rins. Casos em que houve óbito do neonato foram comparados com aqueles em que o neonato teve alta do berçário em relação aos mesmos aspectos ecográficos renais acima citados, à presença de oligoâmnio grave e de tórax estreito. A sensibilidade, a especificidade, os valores preditivos positivo e negativo da presença de cistos renais bilaterais, hiperecogenicidade renal bilateral, oligoâmnio grave e tórax fetal estreito para óbito do neonato foram calculados. RESULTADOS: o oligoâmnio grave e o tórax estreito foram mais frequentes (p=0,03; p<0,001) nos fetos que tiveram cistos renais bilaterais quando comparados àqueles com parênquimas renais ecograficamente normais. O óbito neonatal foi mais frequente entre os casos que tiveram oligoâmnio grave (p<0,001), tórax estreito (p<0,001) e cistos renais bilaterais (p<0,002) quando respectivamente comparados aos casos sem essas alterações. Os melhores valores de sensibilidade, especificidade, valores preditivos positivo e negativo para óbito do neonato/lactente foram obtidos com o uso do aspecto ecográfico tórax estreito, tendo sido de 81,8, 100, 100 e 79,3 por cento, respectivamente. CONCLUSÕES: Em casos de fetos com uropatias obstrutivas bilaterais acompanhados de forma expectante, os sinais ultrassonográficos mais associados ao mau prognóstico são o oligoâmnio grave, o tórax fetal estreito e a presença de cistos renais bilaterais.

PURPOSE: to verify the association between ultrasonographic signs during gestation and post-delivery evolution in fetuses with bilateral obstructive uropathies, followed up in an expectant way. METHODS: fetuses with bilateral obstructive uropathies presenting severe oligoamnios and narrow thorax have been compared with fetuses with bilateral obstructive uropathies without those alterations, concerning the presence or absence of cysts in both kidneys, and the presence or absence of parenchymal hyperechogenicity in both kidneys. Cases of neonatal death were compared with cases of neonatal discharge from the nursery, regarding the same renal echographic aspects mentioned above, the presence of severe oligoamnios and narrow thorax. The sensitivity, specificity, positive and negative predictive value of the presence of bilateral renal cysts, bilateral renal hyperechogenicity, severe oligoamnios and narrow fetal thorax for the neonatal death were calculated. RESULTS: severe oligoamnios and narrow thorax were more frequent (p=0.03; p<0.001) in fetuses with bilateral renal cysts, as compared to those with echographically normal renal parenchyma. Neonatal death was more frequent among cases with severe oligoamnios (p<0.001), narrow thorax (p<0.001) and bilateral renal cysts (p<0.002), when respectively compared with cases without those alterations. The best values of sensitivity, specificity, positive and negative predictive value for the death of neonatal/breastfeeding infants were obtained using the echographic aspect of narrow thorax, and were 81.8, 100, 100 and 79.3 percent, respectively. CONCLUSIONS: in cases of fetuses with bilateral obstructive uropathies followed up in an expectant way, the ultrasonographic signs more associated to bad prognosis are severe oligoamnios, narrow fetal thorax and presence of bilateral renal cysts.