Forming a stone in pelviureteric junction obstruction: cause or effect?

ABSTRACT Objectives To investigate a possible causal relationship for stone formation in pelviureteric junction obstruction and to outline management options. Materials and Methods A literature search and evidence synthesis was conducted via electronic databases in the English language using the key words pelviureteric junction obstruction; urolithiasis; hyperoxaluria; laparoscopic pyeloplasty; flexible nephroscopy; percutaneous nephrolithotomy, alone or in combination. Relevant articles were analysed to extract conclusions. Results Concomitant pelviureteric junction obstruction (PUJO) and renal lithiasis has been reported only scarcely in the literature. Although PUJO has been extensively studied throughout the years, the presence of calculi in such a patient has not received equal attention and there is still doubt surrounding the pathophysiology and global management. Conclusions Metabolic risk factors appear to play an important role, enough to justify metabolic evaluation in these patients. Urinary stasis and infection are well known factors predisposing to lithiasis and contribute to some extent. The choice for treatment is not always straightforward. Management should be tailored according to degree of obstruction, renal function, patient symptoms and stone size. Simultaneous treatment is feasible with the aid of minimally invasive operative techniques and laparoscopy in particular.

Anomalías nefro-urologicas en el riñón contralateral de pacientes con displasia renal multiquística unilateral / Urological and nephrological abnormalities in the contralateral kidney in patients with unilateral multicystic dysplastic kidney

La displasia renal multiquística unilateral (DRMU) es la enfermedad quística renal más frecuente en pediatría. En estos pacientes, la función renal depende exclusivamente del riñón único funcionante contralateral. El objetivo de este trabajo retrospectivo fue determinar la prevalencia y tipo de anomalías nefro-urológicas en el riñón contralateral funcionante de 103 pacientes con DRMU atendidos en el Hospital Nacional de Pediatría Prof. Dr. Juan P. Garrahan entre 1995-2008. Se analizaron sólo aquellos niños que tenían ecografía renal y vesical, cistouretrografía miccional (CUGM) y centellograma renal con ácido dimercaptosuccínico (DMSA-Tc99m). Por ecografía se encontraron anomalías en el riñón contralateral funcionante en el 18.5%. Las más frecuentes fueron dilatación de la vía urinaria (6.8%) y aumento de la ecogenicidad (6.8%). La CUGM detectó la presencia de reflujo vesicoureteral en el riñón contralateral en 11 niños (10.7%), la mayoría leve a moderado. El centellograma renal con DMSA-Tc99m demostró escaras en 12 niños (11.6%). Los resultados muestran que el 23.3% de los pacientes con DRMU tienen alguna anomalía nefro-urológica en el riñón único funcionante contralateral.

Rim em ferradura com cistos e ureter único: relato de caso / Horseshoe kidney with cysts and a single ureter: a case report

O rim em ferradura é a mais comum de todas as anomalias de fusão, ocorrendo em aproximadamente 0,25 por cento da população geral. O rim em ferradura com ureter único é uma rara anomalia. Um paciente do sexo masculino de 60 anos foi admitido para investigação rotineira de triagem. Sua história familiar era negativa para doenças renais e o exame físico foi considerado normal. A tomografia computadorizada revelou um rim em ferradura atípico com cistos e a reconstrução tridimensional na tomografia computadorizada mostrou a presença de um único ureter. O paciente encontra-se assintomático após dois anos de seguimento. Apresentamos um raro caso de paciente portador de rim em ferradura com cistos e ureter único diagnosticado incidentalmente.

Horseshoe kidney is the most common of all renal fusion anomalies, occurring in approximately 0.25 percent of the general population. Horseshoe kidney with only a single ureter is a rare anomaly. A 60-year-old man was admitted to hospital for routine health screening. His family history was negative for kidney diseases, and there was no abnormality in his physical examination. A computed tomography (CT) scan revealed an atypical horseshoe kidney with cysts and three-dimensional spiral CT reconstruction showed the presence of a single ureter. The patient has since been followed up for two years without any signs of clinical disease. We report a rare case of a patient with a horseshoe kidney with cysts and a single ureter that was diagnosed incidentally.

Malignant Rhabdoid Tumor of the Kidney Combined with Multicystic Dysplasia in a 5-year-old Child

Multicystic dysplastic kidney (MCDK) is a relatively common developmental anomaly in infants and children and has a good prognosis. In contrast, a malignant rhabdoid tumor of the kidney (MRTK) is one of the most lethal neoplasms of early life. However, the presentation of such a lethal tumor combined with multicystic dysplasia has not been reported to date. In this report, we describe a case of MRTK in a 5-yr-old girl who also had multicystic dysplasia. She was previously diagnosed with MCDK at birth due to a huge palpable mass on the right side of the abdomen. The right kidney was extensively replaced by numerous grossly dilated, variable-sized cysts. Microscopically, the tumor cells show a diffusely infiltrative growth pattern, which revealed large non-cohesive, round-to-polygonal tumor cells with vesicular nuclei. Some tumor cells had eccentric nuclei and large, round, eosinophilic cytoplasmic inclusions. There were metanephrons present, with the central ureteric bud and peripheral branches surrounded by condensing mesenchyma, immature glomeruli, and metaplastic cartilage in the adjacent parenchyma. To our knowledge, this is the first combined case of the two aforementioned diseases and this case may, in fact, suggest a new disease entity.

Clinico-pathological profile of 22 cases of cystic renal dysplasia.

Renal dysplasia is one of the major renal developmental anomaly characterized by abnormal structural organization and development of metanephric elements. It is usually detected antenatally or in early childhood. The kidney may be multicystic, aplastic, hypoplastic or duplex. We studied 22 cases of cystic renal dysplasia diagnosed over a period often years to identify the spectrum of morphological changes in dysplastic kidney, with special emphasis on mesenchymal changes. Clinical, radiological and gross morphologicalfeatures were noted. Microscopic features were studied in detail, including the epithelial and mesenchymal changes. Twenty-one of the 22 cases studied were children. One case was a 21-year-old adult, which is a rare age at presentation. Male to female ratio was 1.1:1. One of our patients had contra-lateral ureteric stenosis, a rare anomaly reported with renal dysplasia. Ten patients, all autopsy cases, had multi-system congenital anomalies. As cystic renal dysplasia is not a hereditary disease, it must be differentiated from polycystic kidney disease. Other differential diagnoses are cystic nephroma and cystic partially differentiated nephroblastoma. Histopathological examination is the final diagnostic tool since radiological features alone may not be sufficient to exclude other cystic renal lesions. Cartilage may not be seen in all cases of renal dysplasia. Once diagnosed, other associated anomalies should also be looked for.

Neonatal Hirschsprung disease with multicystic dysplastic kidneys presenting as multiple gastrointestinal perforations.

Intestinal perforation as a presentation of Hirschsprung disease is rare, occurring mostly in infants less than 3 months of age, usually those with long-segment disease. Hirschsprung disease may also be associated with other anomalies, that complicate the management and prognosis. Identifying Hirschsprung disease as a cause of perforation will thus help in deciding the site of colostomy and looking for associated anomalies. We report a case of Hirschsprung disease with multiple intestinal perforations and bilateral multicystic kidney disease.

Rim displático multicístico: atualização / Multicystic dysplatic kidney: update

Rim displásico multicistico (RDM) é a anomalia renal cística mais freqüente na infância. Tem sido detectado por meio de ultra-sonografia pré-natal com a melhoria dos equipamentos ecográficos e com a observação detalhada da anatomia fetal. Nos últimos anos, a abordagem dessa anomalia tem mudado de cirúrgica para conduta mais conservadora. Neste estudo foram revisados os principais aspectos concementes ao RDM, com ênfase no tratamento conservador e suas possíveis complicações. Independentemente do tipo de conduta adotada, se manejo conservador ou cirúrgico, é importante ressaltar que os pacientes devem ser acompanhados com ultra-sonografia e medidas de pressão arterial por tempo prolongado, tendo em vista o pouco conhecimento acumulado, até o presente momento, acerca da evolução natural do rim multicístico.

Multicystic dysplastic kidney (MCDK) is the most common cystic anomaly diagnosed in childhood. With the improvement of the resolution of ultrasonography and detailed fetal scanning, MCKD has had its antenatal detection increased. In the last decade, the management has changed from surgical to a conservative approach. In this study, the main aspects concerning MCDK were described, emphasizing conservative management and its possible complications. Independently of the chosen management, whether conservative or surgical, it is important to point out that the patients should be followed with serial ultrasonography and blood pressure measures for a prolonged time due the incomplete knowledge of the clinical course of MCKD.