Immunoglobulin G4-related retroperitoneal fibrosis / Fibrosis retroperitoneal relacionada con IgG4

Abstract Some patients diagnosed with idiopathic retroperitoneal fibrosis could be reclassified as IgG4-related disease (IgG4-RD). Classification criteria have not been uniform and prevalence of IgG4-related retroperitoneal fibrosis (IgG4-RPF) is unknown in our region. We aimed to describe IgG4-RPF frequency relying on criteria published recently and comparing clinical, histopathologic and radiologic features with non-IgG4-RPF. From January, 2005 to December, 2020, nineteen adults with histopathologic diagnosis of idiopathic retroperitoneal fibrosis were included in a dynamic retrospective cohort at Hospital Italiano de Buenos Aires. Pathology slides were reviewed and immunohistochemistry was performed and assessed for each case. We used classification criteria described in 2019 American College of Rheumatology/European League Against Rheumatism to identify IgG4-RD cases. Ten of 19 patients met criteria for IgG4-RPF. Median age was similar in two subsets (61 versus 55, p = 0.2) and both had male predominance. Three out of 10 patients (p = 0.2) featured other manifestations of IgG4-RD in the IgG4-RPF group, and periaortic fibrosis was the most significant finding in images (p = 0.01). Corticosteroids were mostly used as therapy, followed by azathioprine and rituximab. Most patients did not receive specific treatment. IgG4-RPF patients had dense lymphocytic infiltrate and 8 out of 10 showed storiform fibrosis (p = 0.02). IgG4+ cells/hpf and IgG4/IgG ratio were significantly higher (p = 0.01). Over half of the patients in our cohort met the criteria of IgG4-RPF. New criteria may harmonize the identification of IgG4-RD. As IgG4-RD may be reversible at initial stages, these findings may lead to early recognition, treatment and integral follow-up.

Resumen Muchos pacientes con diagnóstico de fibrosis retroperitoneal idiopática (FRI) pueden ser reclasificados como enfermedad relacionada con IgG4 (ER- IgG4). Los criterios diagnósticos no han sido uniformes y la frecuencia de fibrosis retroperitoneal relacionada con IgG4 en nuestra región es desconocida. El objetivo fue describir la frecuencia de ER-IgG4 en pacientes clasificados como FRI y comparar características clínicas, histopatológicas y de laboratorio con aquellos que no reunían criterios de la enfermedad. Se incluyeron 19 adultos en un estudio de cohorte retrospectiva dinámica con diagnóstico anatomopatológico de FRI, en el Hospital Italiano de Buenos Aires, desde enero de 2005 hasta diciembre de 2020. Se revisaron las biopsias y se realizó inmun ohistoquímica en cada una. Se consideró caso al paciente que reunía los criterios de la American College of Rheumatology/European League Against Rheumatism 2019. Diez pacientes reunieron criterios de ER-IgG4. La mediana de edad fue similar en ambos grupos (61 vs. 55, p = 0.2) y en ambos hubo predominio masculino. Tres de 10 pacientes (p = 0.2) tuvieron otras manifestaciones de ER-IgG4 y la fibrosis periaórtica fue el hallazgo más significativo en los estudios por imágenes (p = 0.01). Los corticoides fueron las drogas más utilizadas seguidos por azatioprina y rituximab, pero la mayoría no reci bió tratamiento específico. Todos los pacientes con fibrosis retroperitoneal relacionada con IgG4 presentaron infiltrado linfocitario denso y 8/10 fibrosis estoriforme (p = 0.01), así como las células IgG4+/hpf y ratio IgG4/ IgG fueron significativamente mayores (p = 0.01). Más de la mitad de los pacientes con FRI cumplieron criterios de ER-IgG4. Los nuevos criterios diagnósticos podrían contribuir a homogeneizar la identificación de ER-IgG4. Dado que esta enfermedad puede ser reversible en estadios tempranos, estos resultados promueven aumentar el conocimiento de la entidad para tratamiento precoz y seguimiento integral.

Exclusión renal por endometriosis profunda ureteral: a propósito de un caso / Renal exclusion due to deep infiltrating ureteral endometriosis: a case report

INTRODUCCIÓN: La endometriosis ureteral es una afección rara que afecta al 0.01-1,7% de las mujeres con endometriosis. Hasta un 30% cursa de forma asintomática y un 11,5-14,7% pueden evolucionar con falla renal. La falta de diagnóstico de la enfermedad puede terminar en una uropatía obstructiva y falla renal irreversible. Se presenta el caso de una paciente con afectación grave de la función renal secundaria a endometriosis profunda con compromiso ureteral. CASO CLÍNICO: Mujer de 35 años con endometriosis que consultó por exacerbación de los síntomas. En su estudio destaca, en la resonancia magnética, el hallazgo de endometriosis pélvica profunda y compromiso endometriósico intrínseco del uréter distal derecho, provocando una acentuada hidroureteronefrosis. El cintigrama renal demuestra acentuado compromiso de la función renal derecha, con una función relativa del 7%. Se realizaron nefrectomía total derecha y resección de enfermedad pélvica profunda laparoscópica, sin incidentes. CONCLUSIONES: La endometriosis ureteral representa un desafío diagnóstico y terapéutico. El manejo multidisciplinario entre radiólogos, ginecólogos y urólogos, mediante el diseño de una estrategia quirúrgica individualizada, es imprescindible para definir el tratamiento óptimo de estas pacientes.

INTRODUCTION: Ureteral endometriosis is a rare entity that affects 0.01-1,7% of women with endometriosis. Up to 30% of the patients are asymptomatic and 11.5-14.7% will develop renal failure. Misdiagnosis can lead to obstructive uropathy and permanent renal failure. We present the case of a patient with severe compromise of renal function secondary to deep infiltrating endometriosis with ureteral involvement. CASE REPORT: A 35-year-old woman with endometriosis presented with exacerbation of symptoms. Magnetic resonance showed deep pelvic endometriosis and intrinsic endometriotic involvement of the right distal ureter, causing a marked hydroureteronephrosis. Renal scintigram showed a severe compromise of the right renal function, with a relative function of 7%. Through laparoscopy a total right nephrectomy and resection of deep infiltrating endometriosis was performed. The patient had a satisfactory recovery in the postoperative period. CONCLUSIONS: Ureteral endometriosis presents a diagnostic and therapeutical challenge. Joint multidisciplinary management between radiologists, gynecologists and urologists through the design of an individualized surgical strategy is essential to define the optimal treatment for these patients.

Office stent placement under local anesthesia is a safe and efficient procedure for the management of multiple ureteral disorders. / La colocación de catéteres doble J con anestesia local en régimen ambulatorio es un procedimiento seguro y eficiente para el manejo de distintas patologías ureterales.

OBJECTIVE: To assess the outcomes of ureteral stent placement under local anesthesia for the management of multiple ureteral disorders. METHODS: Retrospective study of 45 consecutive ureteral stents placed under local anesthesia from January 2015 to July 2016. Inclusion criteria were hemodynamically stable patients with urinary obstruction, urinary fistula or for prophylactic ureteral localization during surgery. Five minutes before the procedure, 10ml of lidocaine gel and 50ml of lidocaine solution were instilled in the bladder. A 4.8Fr ureteral stent was placed using a 15.5Fr flexible cystoscope under fluoroscopic control. Characteristics of procedures and outcomes were analysed. RESULTS: A total of 45 procedures (33 placement, 12 replacements) were attempted in 37 patients, of which 40 (89%) were successful. There were 10 male (27%) and 27 female patients (73%) with a mean age of 58.6 years (±17.5). Main indications for stent placement were stones (37.8%), extrinsic ureteral compression (28.9%) and surgery ureteral localization (22.2%). The reasons for failing to complete a procedure were the inability to pass the guidewire/stent in 4 cases (8.8%) or to identify the ureteral orifice in 1 (2.2%). Postoperative complications occurred in 8 patients (17.8%) (7 Clavien I, 1 Clavien IIIa). No procedure was prematurely terminated due to pain. Statistical analysis did not find significant successful predictors. The outpatient setting provided a fourfold cost decrease. CONCLUSIONS: Ureteral stent placement can be safely and effectively performed under local anesthesia in the office cystoscopy room. This procedure could free operating room time, reduce costs and minimize side effects of general anesthesia.

[Laparoscopic ureterovesical repair for megaureter treatment]. / Plastia ureterovesical laparoscópica para el tratamiento del megauréter.

BACKGROUND: An alternative treatment for obstructive and refluxing obstructive megaureter with ureterovesical junction maintenance through laparoscopy is proposed. MATERIAL AND METHODS: The series consists of 8 cases, all of them studied because of prenatal hydronephrosis or febrile urinary tract infection. Seven were diagnosed with obstructive megaureter and one with obstructive refluxing megaureter. The procedure consisted in incising the stenotic portion of the ureter longitudinally and maintaining its posterior wall attached to the bladder, the anterior wall of the ureter was anastomosed transversally to the bladder mucosa in order to liberate the obstruction. RESULTS: Two of the 8 cases were women and 6 were male. They were aged between 5 months and 11 years (average age of 2.9 years). The surgical time varied between 90 and 120min, with a 48h hospital stay. The permanence of the vesical catheter and the double J stent was of 48h and 6 weeks, respectively. A female patient developed febrile urinary tract infection one week after the surgical procedure. The rest of the patients remained asymptomatic, with normal urinalysis and quarterly urine culture results. Six months after the procedure, the ultrasound showed improvement of the ureteral diameter and of the pyelocaliceal ectasia. The average follow-up was of 2 years. DISCUSSION: The handling of stenosis with longitudinal incision of the ureter and transverse anastomosis to the bladder mucosa, seems promising. The laparoscopic approach allows to identify clearly the longitude of the stenotic segment and join with relative precision such opening with the one of the vesical mucosa.

Evolución de los pacientes pediátricos con diagnóstico de hidronefrosis que consultaron al Hospital Universitario San Vicente Fundación, Medellín, Colombia, entre 1960 y 2010 / Evolution of pediatric patients diagnosed with hydronephrosis at Hospital Universitario San Vicente Fundación, in Medellín, Colombia between 1960 and 2010

Introducción: la hidronefrosis es una de las malformaciones congénitas detectadas comúnmente en la ecografía prenatal. Los casos moderados y graves frecuentemente se asocian a anomalías de la vía urinaria. Objetivo: describir las causas de la hidronefrosis, la frecuencia de su diagnóstico prenatal y la de enfermedad renal crónica (ERC) en una serie de pacientes pediátricos. Materiales y métodos: estudio descriptivo, retrospectivo de 924 pacientes atendidos entre 1960 y 2010 en un hospital universitario de Medellín. Resultados: hubo predominio de hombres (64,3%); en el 14,4% el diagnóstico fue prenatal. En el 18,3% no se encontraron anomalías urológicas asociadas. Siete de estos (4,2%) llegaron a la ERC. La estenosis pieloureteral fue la anomalía hallada con mayor frecuencia (28,6%) seguida por el reflujo vesicoureteral (21,5%) y las valvas de la uretra posterior (9,4%); el 10,2% de los pacientes llegaron a la enfermedad renal crónica; al agrupar los pacientes de acuerdo con el método utilizado para el diagnóstico de hidronefrosis, el grupo en que se hizo por urografía excretora tuvo ERC en el 11,3%, en contraste con el 8,4% en quienes el diagnóstico se hizo por ecografía renal; por otro lado. al agrupar los pacientes con diagnóstico ecográfico de hidronefrosis prenatal o posnatal, el porcentaje de ERC fue de 4,8% frente a 10,8%, respectivamente. Conclusión: el diagnóstico temprano de la hidronefrosis permite detectar anomalías urológicas susceptibles de seguimiento o corrección quirúrgica. Todavía existe mucha controversia acerca del mejor seguimiento imaginológico para estos pacientes.

Introduction: Hydronephrosis is one of the most common congenital malformations detected on prenatal ultrasounds. Moderate and severe cases are often associated with urological abnormalities. Objective: To describe the causes of hydronephrosis, and the frequency of its prenatal diagnosis and of chronic kidney disease (CKD) in a series of pediatric patients. Materials and methods: A descriptive, retrospective study of 924 patients under 18 years of age seen at a university hospital in Medellín, Colombia, between 1960 and 2010. Results: 64.3% were male. Diagnosis was prenatal in 133 (14.4%). Hydronephrosis was bilateral in 198 patients (28.5%). In 169 (18.3%) no associated urological abnormality was found; 7 of these (4.2%) reached CKD. Ureteropelvic stenosis was diagnosed in 216 (23.3%) followed by vesicoureteral reflux in 199 (21.5%) and posterior urethral valves in 87 (9.4%); 93 (10.2%) reached CKD. When hydronephrosis was diagnosed by urography, patients developed CKD in 11.3% vs. 8.4% in those whose diagnosis was made by ultrasound; frequency of CKD was 4.8% when diagnosis by ultrasound was prenatal, and 10.8% when it was postnatal. Conclusion: Early diagnosis of hydronephrosis allows the detection of urologic abnormalities susceptible to treatment. There is controversy about the best imaginological method for the follow-up of these patients.

[Ureteral valves: literature review and description of 4 new cases]. / Valvas ureterales: revisión de la literatura y descripción de 4 nuevos casos.

Ureteral valves are a rare malformation, with an incidence of 1 in 5000-8000 live births. Urological malformations are associated with 50% of cases according to the current literature. We report 4 cases treated in our hospital from 2004 to 2012. Three of the patients had renal dysgenesis, one case associated with complete urethral duplication, and another case associated with a ureteral atresia.

Cistitis eosinofílica en niños: un reporte de caso / Eosinophilic cystitis in children: a case report / Cistites eosinofílica em crianças: um reporte de caso

La cistitis eosinofílica es una entidad poco común que se caracteriza por la inflamación con infiltrado eosinofílico de la pared vesical. No es muy frecuente en la edad pediátrica con mínimos reportes de casos en la literatura. Presentamos un caso de un paciente de 24 meses, a quien se le diagnostica dicha patología, el manejo instaurado y una revisión de la literatura.

Eosinophilic cystitis is a rare entity characterized by inflammations with eosinophilic infiltration of the bladder wall. It appears infrequently in children, and there are few case reports in medical literature. This paper presents the case of a 24-month-old patient diagnosed with this condition, the treatment that has been established for it, and a review of the literature.

A cistites eosinofílica é uma entidade invulgar que se caracteriza pela inflamação com infiltrado eosinofílico da parede vesical. Não é muito frequente na idade pediátrica com mínimos reportes de casos na literatura. Apresentamos um caso de um paciente de 24 meses, a quem se lhe diagnostica dita patologia, o manejo instaurado e uma revisão da literatura. Métodos diagnósticos moleculares em tuberculoses

Fibrosis retroperitoneal idiopática: un enigma médico / Retroperitoneal fibrosis: a medical enigma

La fibrosis retroperitoneal es una enfermedad rara de causa desconocida. Fue descrita por primera vez en la literatura inglesa en 1948 por Ormond. Sin embargo, la primera descripción conocida de esta enfermedad fue realizada por el urólogo francés Albarran en 1905. Se caracteriza por una inflamación crónica e inespecífica del retroperitoneo, la cual puede atrapar y obstruir las estructuras que allí se encuentran, particularmente a los uréteres. Es frecuente que la enfermedad se detecte en etapas avanzadas, cuando se manifiesta por falla renal crónica. Los avances en las técnicas imaginológicas y el mayor conocimiento de la entidad han permitido que el diagnóstico se realice con mayor frecuencia en etapas más tempranas, cuando los pacientes aún no se encuentran obstruidos. Como resultado de las observaciones que muestran que los aneurismas inflamatorios de la aorta abdominal difieren de la enfermedad de Ormond sólo en el diámetro de la aorta inflamada, se ha sugerido que ambos síndromes representan variaciones de la misma patología, la cual se ha llamado "periaortitis crónica".

Retroperitoneal fibrosis is an uncommon disease of unclear cause. It was described for the first time in English literature by Ormond, in 1948. However, the first known description of this disease was done by the French urologist Albarran, around 1905. It is characterized by chronic nonspecific inflammation of the retroperitoneum that can entrap and obstruct retroperitoneal structures, notably the urethers. This disease frequently is detected only after severe renal failure is present. However, advances in imaging techniques and increased awareness of the disease have resulted in an earlier diagnosis in unobstructed patients. As a result of the observations that showed that the only difference between an inflammatory aneurysm of the abdominal aorta and Ormond's disease is the diameter of the vessel, it had been suggested that both syndromes represent variations of the same pathology, which has been named "chronic periaortitis".

Marsupialización ureterorrenoscópica de quiste renal peripiélico obstructivo / Flexible ureteronephoscopy for surgical treatment of a symptomatyc peripelvic renal cyst

Relatamos un caso de gran quiste peripiélico causando obstrucción de la pelvis renal y episodios recurrentes de dolor lumbar. Se utilizó ureterorrenoscopia flexible para la marsupialización interna del cisto en el sistema colector renal, con electrocoagulación del interior del cisto. El caso y la técnica se describen con detalles.

We report a case of a large peripelvic renal cyst causing obstruction of renal pelvis and episodes of flank pain. Flexible ureteronephoscopy has been used for the internal marsupialization of cyst into the renal collecting system with eletrocoagulation of the inner part of the cyst. The case and technique are described in detail.

Reconstrucción de la Unión Pielo Ureteral: 40 años de experiencia / Reconstruction of the pielo ureteral junction: experiency in 40 year

Se hace una revisión de 326 casos operados por la Técnica de Hynes Anderson en 40 años de experiencia, donde se analizan las causas más frecuentes, siendo la estenosis de la unión pielo-ureteral la causa encontrada con mayor incidencia. Las manifestaciones clínicas fundamentales en su aparición fueron el dolor lumbo abdominal y la infección urinaria. Recomendamos esta técnica por los excelentes resultados obtenidos y la buena evolución post operatoria. Solamente se le realizó nefrectomía posterior a los dos y tres años de operados a dos pacientes por atrofia hidronefrótica tardía.

We reviewed 326 cases operated using the Hynes-Anderson Technique in 40 years of experience and analyzed the most frequent causes, being the pielo-ureteral joint stenosis the most frequent. The main clinical manifestations were lumbo-abdominal pain and urinary infection. We recommend this technique for the excellent results obtained and the good post surgery evolution. Only two patients were subject of nephrectomy two or three years later because of late hydronephrotic atrophy.

Reconstrucción de la Unión Pielo Ureteral: 40 años de experiencia / Reconstruction of the pielo ureteral junction: experiency in 40 year

Se hace una revisión de 326 casos operados por la Técnica de Hynes Anderson en 40 años de experiencia, donde se analizan las causas más frecuentes, siendo la estenosis de la unión pielo-ureteral la causa encontrada con mayor incidencia. Las manifestaciones clínicas fundamentales en su aparición fueron el dolor lumbo abdominal y la infección urinaria. Recomendamos esta técnica por los excelentes resultados obtenidos y la buena evolución post operatoria. Solamente se le realizó nefrectomía posterior a los dos y tres años de operados a dos pacientes por atrofia hidronefrótica tardía(AU)

We reviewed 326 cases operated using the Hynes-Anderson Technique in 40 years of experience and analyzed the most frequent causes, being the pielo-ureteral joint stenosis the most frequent. The main clinical manifestations were lumbo-abdominal pain and urinary infection. We recommend this technique for the excellent results obtained and the good post surgery evolution. Only two patients were subject of nephrectomy two or three years later because of late hydronephrotic atrophy(AU)

Obstrucción ureteral parcial crónica por vena ovárica: Descripción de un caso y revisión de la literatura / Chronic partial uretheral obstruction by the ovarian vein: A case report and literature review

Se presenta el caso de una paciente con dolor lumbar crónico secundario a obstrucción ureteral parcial por la vena ovárica en la que se corrobora la efectividad del tratamiento quirúrgico.

We present a female patient with chronic loin pain caused by partial obstruction of the urether secondary to ovarian vein crossing. We highlight the benefit derived from surgical treatment.