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.

Clinical phenotype and genotype analysis in 9 children with nephronophthisis / 中华肾脏病杂志

Objective:To investigate and analyze the clinical phenotypes and genotypes in children diagnosed with nephronophthisis (NPHP), and to provide references for clinical diagnosis.Methods:Clinical data of 9 children with NPHP diagnosed by genetic testing in the Department of Nephrology, Wuhan Children′s Hospital from April 2017 to January 2022 were retrospectively collected. The clinical characteristics and genetic test results were analyzed.Results:The median onset age was 11.2(3.4, 14.2) years old in 9 patients, including 5 females and 4 males. There were 8 cases of glomerular proteinuria, 8 cases of renal tubular proteinuria, and 7 cases of reduced urinary gravity in 9 patients. All the children had varying degrees of impaired renal function at the time of diagnosis. Seven cases entered chronic kidney disease (CKD) stage 5, 1 case entered CKD stage 3, and 1 case entered CKD stage 4 at the time of diagnosis. All the children had renal ultrasound abnormalities of varying degrees: size change (3/9), echo enhancement (8/9) and cysts (3/9). Extrarenal phenotypes were present in 3 children. Genetic test showed that 6 patients had mutation of NPHP1 gene, 1 patient had mutation of WDR19 gene, 1 patient had mutation of NPHP3 gene and 1 patient had mutation of NPHP5 gene. Conclusions:Deletion mutation of NPHP1 gene is the most common, while NPHP3, NPHP5 and extremely rare WDR19 mutations have also been found in NPHP patients. The clinical manifestations of NPHP are not typical, so it is necessary to find a specific diagnosis method in the early.

Prenatal diagnosis of Joubert syndrome caused by de novo mutations of CPLANE1 gene / 中华围产医学杂志

We report a case of fetal cerebellar vermis dysplasia diagnosed prenatally by ultrasonography. Ultrasonography of the 27-year-old woman at 20 +6 gestational weeks revealed partial separation of the cerebellar vermis (Dandy-Walker variants), unclosable upper and lower lips, and polydactyly, based on which a preliminary diagnosis of multiple fetal malformations was made. Karyotype and chromosomal microarray (CMA) analysis of the amniotic fluid showed no abnormality. After genetic counseling, amniocentesis was performed again for a whole-exome sequencing test. The results suggested that there are compound heterozygous variations of c.3435G>A(P.W1145X) and c.2941C>G(p. p981A) in the exon 19 and exon 17 of the CPLANE1 gene, which were both de novo mutations and inherited from the father and mother, respectively. The fetus was diagnosed as Joubert syndrome. Given the facial and limb deformities and a significant risk of neurological abnormalities of the fetus, the patient and her family decided to terminate the pregnancy.

Sonographic features of Joubert syndrome in first or second trimester: report of two fetuses / 中华围产医学杂志

We report two cases of Joubert syndrome initially tentatively diagnosed by prenatal ultrasound in the first or second trimester which were thereafter confirmed by whole exome sequencing (WES). Case 1 was one of the twins who presented with increased intracranial transparency (IT) and thinner brainstem at 12 +1 gestational weeks. Ultrasound at 18 +2 weeks found multiple intracranial malformations, "molar tooth sign (MTS)" at the midbrain-hindbrain junction level in the cerebral cross section, and bilateral ventriculomegaly. Enlarged and echogenic kidneys and oligohydramnios were also detected. In case 2, ultrasound image at 17 +5 weeks of gestation indicated multiple intra-and extra cranial and extracranial malformations, MTS in the midbrain-hindbrain junction plane, bilateral ventriculomegaly, unclear cavum septum pellucidum. Extracranial anomalies were bilateral multicystic enlarged kidneys, invisible bladder, and oligohydramnios. Both fetuses underwent amniocentesis, which showed normal karyotype and no copy number variation was detected. However, variation of the TMEM67 gene (c.312+5G>A at introns 2 and c.1175C>G at exon12) was detected in both fetuses by WES, supporting the diagnosis of Joubert syndrome. Selective reduction and termination of pregnancy were performed on case 1 and case 2 at 18 +5 and 19 weeks of gestation, respectively.

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.

Prenatal diagnosis and prognosis of fetal hyperechogenic kidney: a study of 65 cases / 中华围产医学杂志

Objective To investigate prenatal diagnosis and prognosis of fetus with hyperechogenic kidney.Methods Clinical data of 65 cases prenatally diagnosed with fetal hyperechogenic kidney in Peking University First Hospital between July,2009 and May,2015 were retrospectively analyzed.Results of fetal ultrasound screening and pregnancy outcomes were analyzed and Growth and development status of those babies were followed up until December,2015.Independent-sample-t,Chi-square or Fisher's exact test was applied for statistical analysis.Results Among the 65 cases,including 48(73.8％) bilateral and 17 (26.2％) unilateral,34 cases (52.3％) were diagnosed as non-isolated and 31 cases (47.7％) as isolated fetal hyperechogenic kidney.The primary associated malformations with non-isolated fetal hyperechogenic kidney included cardiac abnormality (14 cases,41.2％),urinary system abnormality (12 cases,35.2％),skeletal system abnormality (nine cases,26.5％),central nervous system abnormality (eight cases,23.5％) and Meckel-Gruber sydrome (one case,2.9％).Amniotic volume,the size and numbers of affected kidney between non-isolated and isolated groups showed no significant differences (all P＞0.05).Twenty out of the 65 cases (30.8％) received fetal karyotyping and one received non-invasive prenatal testing,and no abnormality was detected.Three cases received cord blood array comparative genomic hybridization with negative results.Pathogenic genes were found in two cases who received targeted exome capture with high throughput sequencing,including a TTC21B mutation in cord blood in one case and a HNF1β deletion mutation in peripheral blood after birth in the other.There were 23 (35.4％) terminations of pregnancy and 42 (64.6％) live births among which three died after birth.The rate of live birth was significantly higher in the isolated group than in the non-isolated group[87.1％(27/31) vs 44.1％(15/34),x2=13.101,P＜0.01].Of the fifteen live births in the non-isolated group,there were fourteen survived symptomfree except that one lost to follow-up.Of the 27 live births in the isolated group,follow up study revealed 23 symptom-free survivors,one lost,two neonatal deaths (one died of volvulus neonatorum,and the other due to unknown causes) and one death of renal and liver function failures at the age of two-and-a-half.ConclusionsFetal hyperechogenic kidney is an important prenatal ultrasound marker for congenital renal anomalies,and the prognosis of non-isolated fetal hyperechogenic kidney is poor.The current rate of abnormal karyotype in fetus with hyperechogenic kidney is very low.However,the rate of prenatal genes screening should be encouraged.

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.

Effect of Tube Current on the Pseudo-enhancement of Renal Cyst / 中国医学影像学杂志

PurposeTo evaluate the effect of tube current on the pseudo-enhancement of renal cyst by simulating the phantom model of simple renal cyst.Materials and Methods 10% glucose and iodine solution with a certain concentration was used to simulate the renal parenchymal background concentration in plain scan, moderate enhancement and maximum enhancement respectively. The diameters of the cysts were 6 mm, 10 mm and 15 mm, respectively, and the cysts were divided into three groups according to different tube current: 119 mAs (group A), 178 mAs (group B) and 297 mAs (group C) while the tube voltage were all 120 kV. Whether pseudo-enhancement exists in cyst under different conditions was determined using an increase of CT value of 10 HU as the critical value. Results In group A, there was pseudo enhancement at the 240 HU background, and it was most significant with the diameter of 6 mm, which was 21 HU. In group B, pseudo-enhancement occurred in cysts with diameter of both 10 mm and 6 mm under the background of 180 HU and 240 HU, moreover, the biggest difference was 20.4 HU and it occurred in cyst with diameter of 6 mm under the background of 240 HU. In group C, pseudo-enhancement only occurred in cyst with diameter of 6 mm under the condition of 125 HU and 240 HU background concentration. Background concentration (F=17.587, P<0.01) and cyst diameter (F=4.214,P<0.05) had greater impact on cyst pseudo-enhancement, the higher the background concentration and smaller the diameter, more significantly the pseudo-enhancement would occur. With the increase of the tube current, the CT volume dose index increased, and the pseudo enhancement value was smaller, but there was no obvious regularity of pseudo-enhancement occurrence rate in cysts with different background concentration and diameter in each group.Conclusion The increase of tube current cannot completely eliminate cyst pseudo-enhancement. High background concentration and small diameter cyst are important factors in pseudo-enhancement. However, increasing the tube current can reduce the probability of occurrence of pseudo-enhancement to some extent. For those with heavier body weight, it might be necessary to increase the tube current to improve image quality and reduce the occurrence of renal cyst pseudo-enhancement.

CT Manifestations of Renal Cell Carcinoma with Cystic Change / 中国医学影像学杂志

Purpose To explore the CT features of renal cell carcinoma with cystic change and to compare them with pathological ifndings, so as to improve its diagnostic accuracy. Materials and Methods A retrospective study was carried out in 44 cases of renal cell carcinoma with cystic change conifrmed surgically and pathologically, among which 10 were multilocular cystic renal cell carcinoma, 21 were renal clear cell carcinoma and 13 were papillary renal cell carcinoma. The CT features of these 3 kinds of diseases were analyzed comparatively. Results All of 10 cases of multilocular cystic renal cell carcinoma appeared to have multilocular cysts with thin cystic walls and septa. The mean CT value of cyst was (15.8±5.6) HU. The diameter of wall nodule was larger than 5 mm in 4 cases. The contrast-enhanced CT scan showed that the cystic walls and septa had early moderate enhancement in 8 cases. Among 21 cases of renal clear cell carcinoma, 9 presented to have multilocular cysts with thick cystic walls and septa. The mean CT value of cyst was (32.5±6.7) HU. The diameter of wall nodule was larger than 5 mm in 19 cases. The cystic walls and septa had obvious early enhancement in 20 cases. As to the 13 cases of papillary renal cell carcinoma, 4 appeared to have multilocular cysts with thick cystic walls and septa. The mean CT value of cyst was (26.1±5.6) HU. The diameter of wall nodule was larger than 5mm in 12 cases, and 12 cases appeared to have slight to moderate delayed enhancement. Conclusion The CT features of renal cell carcinoma with cystic change could be used in differential diagnosis, such as with or without pseudocapsule, mean CT value of cyst, thickness of cystic wall and septum, size, boundary, and enhancement of nodule.

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.

A clinical analysis of multilocular cystic renal cell carcinoma and its correlation with mutation of the von Hippel-Lindau gene / 中华泌尿外科杂志

Objective To discuss the diagnosis and surgical management of multilocular cystic renal cell carcinoma (MCRCC) and to evaluate the gene function of the mutation of von Hippel-Lindau (VHL) gene in MCRCC. Methods Seventeen MCRCC cases (11 men and 6 women) out of 512 cases of renal cell carcinoma from 2000 to 2010 were retrospectively analyzed. The mean age of the 17 patients was 46 years (37-61 years). Ultrasonography and CT were available in all 17 cases, and 1 case was misdiagnosed as parapelvic renal cyst. The mutation of VHL gene was detected by PCR in the specimens of can-cerous tissue and adjacent normal tissue from 11 cases of MCRCC. Results Three of 17 cases underwent nephron sparing surgery, the others underwent radical nephrectomy. One case underwent unroofing of parapelvic renal cyst, but the rapid frozen pathology of the cyst wall showed renal cell carcinoma of clear type. As a result, radical nephrectomy was eventually performed. All 17 cases were confirmed as MCRCC by eva-luating pathological characteristics, such as the cyst wall lined by single or several layers of clear tumor cells and the nuclei which were small and anachromasis. Clinical stages of all cases were T1N0M0, in which there were 14 cases with pathological T1G1 and 3 cases with pathological T1G2. All patients underwent a follow-up of 9 to 36 months (mean, 12 months) without recurrence or metastasis. Mutation of VHL gene was detected in 7 of 11 cases (64%), but all adjacent normal tissues were negative. Conclusions As a rare subtype of renal cell carcinoma, MCRCC is difficult to diagnose. CT is an essential measure in diagnosis of MCRCC preoperatively. Because of the good prognosis of reported cases, nephron sparing surgery for the treatment of MCRCC is recommended. VHL gene mutations may play an important role in the carcinogenesis of MCRCC.

Treatment experience of 36 cases of simple renal cysts / 中国基层医药

ObjectiveTo summarize the treatment of simple renal cysts experience,the advantages and disadvantages of the two surgical options. MethodsRetrospective analysis of 15 cases of renal cysts after general anesthesia in patients with laparoscopic cyst decompression in the clinical data and 21 cases of renal cysts underwent open surgery in patients with clinical data,comparative analysis of advantages and disadvantages of both types of surgery were conducted. ResultsIn this study,14 patients with laparoscopic surgery were successful,1 patient converted to open surgery,laparoscopic surgery in patients with blood loss,postoperative complications and time of staying hospital after operation postoperative hospital stay were significantly less than group of open surgery. ConclusionThe laparoscopic renal cyst decortication was a safe and effective treatment for simple renal cysts,compared to traditional open surgery in patients with trauma,less bleeding postoperative pain,rapid recovery,shorter hospital staying and so on.

Concomitant Laparoendoscopic Single-Site Surgery for Ureterolithotomy and Contralateral Renal Cyst Marsupialization

A 63-year-old woman presented with acute right-flank pain and left-flank pain. Computed tomography identified a right ureter stone and a left renal cyst. The patient underwent concomitant laparoendoscopic single-site surgery (LESS) for ureterolithotomy and renal cyst marsupialization with the use of an Alexis(R) wound retractor, which was inserted through the umbilical incision. Flexible laparoscopic instruments and conventional rigid instruments were used during LESS following a procedure similar to that used with conventional laparoscopic surgery without additional transcutaneous ports. LESS may be more efficient at treating bilateral diseases than is conventional laparoscopic surgery.

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.

Segmental Cystic Disease of the Kidney: A Case Report

Segmental cystic disease of the kidney is a rare form of cystic disease of the kidney that manifests as variable sized, numerous cysts that are localized in a segment of one kidney. Morphologically and pathologically, it is indistinguishable from autosomal dominant polycystic kidney disease except for its unilateral localization, the lack of an autosomal dominant genetic background and the progressive deterioration of the renal function. We experienced a case of surgically confirmed segmental cystic disease of the kidney in a 49-year-old patient and we report on its ultrasonographic and CT findings, along with a brief review of the relevant literature.