Obstructed hemivagina with ipsilateral renal anomaly with ureteric remnant in a prepubertal female / Philippine Journal of Obstetrics and Gynecology

@#Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn–Werner–Wunderlich syndrome, is a rare Müllerian duct anomaly that is characterized by a triad of uterus didelphys, unilateral obstructed vagina, and ipsilateral renal agenesis. Patients with this anomaly usually present after menarche with abdominal pain or a pelvic mass which worsens over some time. Herein, presented is a case of a 12‑year‑old nulligravid with cyclic pelvic pain and palpable pelvic mass after her menarche. A series of diagnostic tests were done which were compatible with the diagnosis of OHVIRA with an associated finding of ectopic ureteral insertion into the obstructed hemivagina and nonfunctional urinary bladder. The patient underwent diagnostic hysteroscopy, cystoscopy, and full resection of the longitudinal vaginal septum as well as drainage of hematocolpos, which are the main treatment of the patient with OHVIRA.

Signo cabeza de cobra: en relación con ureterocele y cálculo vesical / Cobra Head Sign: in relation to Ureterocele and vesical calculus

Imagen: Signo de la Cobra (flecha #1) hallazgo compatible con ureterocele, y en su interior imagen hiperecoica con sombra acústica posterior en relación con cálculo (flecha #2)...(AU)

Ureterocele intravesical derecho (signo de la cabeza de cobra) / Intravesical right ureterocele (Cobra head sign)

Paciente de 29 años de edad, sexo masculino, sin antecedentes clínicos ni quirúrgicos de importancia. Acude al servicio de emergencia con dolor en la fosa iliaca derecha (escala de dolor EVA 6/10). Examen físico: abdomen suave, depresible, doloroso a la palpación en la fosa ilíaca derecha, signos de McBurney dudoso y Rovsing positivo. Se sospecha apendicitis aguda por lo cual se solicita una tomografía multicorte simple y contrastada de abdomen y pelvis que reporta dilatación quística del uréter distal izquierdo en su segmento intravesical, que es compatible con un ureterocele. Diagnóstico diferencial del dolor en la fosa ilíaca derecha: incluye algunos menos comunes como el ureterocele que no debe ser soslayado. (AU)

Clinical Characteristics of Ureteral Duplication in Children

PURPOSE: Ureteral duplication is a relatively common congenital urinary tract abnormality that can be associated with various clinical problems such as vesicoureteral reflux (VUR), hydronephrosis, and ectopic ureters. The purpose of this study was to analyze the clinical characteristics of pediatric patients with recently diagnosed ureteral duplication and to identify any differences from those described in previous reports.METHODS: We retrospectively reviewed the clinical characteristics and course of pediatric patients who were diagnosed with ureteral duplication between January 2008 and June 2017.RESULTS: A total of 32 pediatric patients were diagnosed with ureteral duplication during the study period. The male to female ratio was 1:2.2. Twenty-seven patients (84.4%) were first diagnosed with ureteral duplication at less than 3 months of age, and 26 (81.3%) were first diagnosed by prenatal ultrasonography. Four of the 32 patients were diagnosed with bilateral ureteral duplication, for a total of 36 occurrences of ureteral duplication. In 17 occurrences of complete ureteral duplication (47.2%), other urinary tract anomalies were also found; namely, ureterocele (7), VUR (11), and ectopic ureter (5). However, none of the patients with incomplete ureteral duplication had ureterocele or VUR.CONCLUSION: With the advent of routine prenatal ultrasound, ureteral duplication is being diagnosed earlier than was previously possible, enabling timely treatment of the various accompanying urinary tract anomalies. Multicenter studies are needed to establish guidelines for standardized evaluation and treatment of ureteral duplication.

Ureterocele prolapsado: un diagnostico para tener en cuenta ante una masa vulvar en una lactante. A proposito de un caso / Prolapsed ureterocele: a diagnosis to be considered for a vulvar mass in an infant. Case report

El ureterocele es una malformación del sistema urinario relativamente frecuente en niños; sin embargo, el prolapso del ureterocele a través de la uretra es una presentación, en extremo rara. La urorresonancia magnética es la prueba de elección para el diagnóstico definitivo. El tratamiento inicial de un ureterocele prolapsado consiste en la descompresión de la masa. Presentamos el caso clínico de una lactante de 7 meses con duplicación del sistema colector y ureterocele ectópico derecho, que apareció como una masa vulvar.

Ureterocele is a relatively common malformation of the urinary system in children; however, the ureterocele prolapse through the urethra is an extremely rare presentation. Urological nuclear magnetic resonance is the test of choice for definitive diagnosis. Initial treatment of a prolapsed ureterocele involves decompression of the mass. We report the case of a 7-month-old infant with duplication of the collecting system and right ectopic ureterocele appearing as a vulvar mass.

Manejo endoscópico de litiasis en ureterocele / Endoscopic management of lithiasis in ureteroce

Introducción: El ureterocele corresponde a una dilatación sacular del uréter distal, la cual puede ser congénita o ectópica. Ocurren más frecuentemente en mujeres que hombres, presentándose de manera bilateral en el 10 por ciento de los casos. Su importancia radica en que son factores de riesgo tanto para infección como para la formación de cálculos. No existe consenso en el tratamiento de cálculos asociados a ureterocele, si bien el tratamiento endoscópico pareciera ser el de elección.(AU)

Introduction: The ureterocele corresponds to a saccular dilation of the distal ureter, which may be congenital or ectopic. They occur more frequently in women than men, presenting bilaterally in 10 pertcent of cases. Their importance lies in the fact that they are risk factors for both infection and stone formation. There is no consensus in the treatment of stones associated with ureterocele, although endoscopic treatment seems to be the one of choice Clinical Case: A 69-year-old patient was referred for a long-term illness characterized by repeated episodes of UTI, associated with 2 hematuria events. An imaging study was performed (UROTAC), and a large left ureterocele was documented with 3 cm stones in its interior

Cecoureterocele, reporte de un caso / Cecoureterocele, a case report

El cecoureterocele es un ureterocele ectópico, malformación congénita del uréter distal en su desembocadura a la vejiga, que da lugar a un saco membranoso ciego que protruye por la uretra hasta el exterior. Presentamos a una niña de cinco meses y 6 kg de peso que acude para evaluación y manejo de una masa de color violáceo que protruye por el introito vaginal. Al examen físico, buen estado general. FC 134 lpm, TA 93/56 mmHg, FR 38 x´, T 36o C, Sat 02 96 %. Ecografía renal: Riñón izquierdo con una imagen líquida parahiliar compatible con doble sistema colector incompleto con dilatación del segmento proximal. Ecografía vesical: imagen con contenido líquido denso relacionada con el sistema urinario y compatible con ureterocele. Gammagrafía renal DMSA: área hipocaptante en polo superior del riñón izquierdo. Renograma isotópico DTPA: Retención del trazador en pelvis renal izquierda. Corrección quirúrgica consistente en nefrectomía parcial con escisión del polo superior del riñon izquierdo, separación y sección distal del uréter dilatado y resección del ureterocele. Evolución postoperatoria satisfactoria con alta a los dos días de la intervención. El cecoureterocele es una rara malformación de la vía urinaria habitualmente asociada a otras malformaciones corregibles quirúrgicamente. Recomendamos considerar esta posibilidad, en el diagnóstico diferencial de toda masa que protruye por la vagina.

A cecoureterocele is an ectopic ureterocele, congenital anomaly of the distal ureter at the uretero-vesical junction, consisting in a closed membranous pouch which protrudes through the urethra into the vagina. We present a five months old, 6 kg girl, admitted for assessment and management of a dark red mass protruding through the vagina. On examination, good general health. BP 93/56, HR 134 bpm, RR 38x´, T 36o, O2 Sat 96 %. Renal ultrasound: parahiliar liquid shadow in the left kidney sugesting a duplicated incomplete collecting system with a dilated proximal segment. Bladder ultrasound: dense liquid shadow related to the urinary tract sugesting a ureterocele. DMSA renal gamma scan: poor captation in the upper pole of the left kidney. DTPA isotopic renogram: Retention of the tracer in the left renal pelvis. Surgical correction consisting in a left upper pole partial nephrectomy, separation and distal section of the dilated ureter and resection of the ureterocele. Good postoperative course, discharged two days later. A cecoureterocele is an uncommon congenital malformation of the urinary tract commonly associated with other surgically correctable malformations. We recomend to consider this posibility in the differential diagnosis of any mass protruding through the vagina.

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

Ultrasonography of Congenital Hydronephrosis

Hydronephrosis is the most common urogenital anomaly with the potential for obstructive process. And ultrasonography, as a first-line imaging modality, is a useful tool for evaluation of prenatal and postnatal hydronephrosis, even though evaluation of renal function is still needed. In this article, we review the common causes of congenital hydronephrosis and the sonographic findings of those diseases.

case of dupable duple duplicity and duplexity

Duplication anomalies are quite common with ureteral duplication anomalies being the most frequent. Despite the relatively frequent incidence of a horseshoe kidney and duplication anomalies in any individual patient, the combination of horseshoe kidney and bilateral ureteric duplication is a very rare entity and very few cases have been reported to date. We present a case of a patient with novel combination of horse shoe kidney and congenital renal anomalies and their sequelae

Ureterocele ectópica em cão: relato de caso / Ectopic ureterocele in a dog: a case report

Relataram-se o quadro clínico, o diagnóstico, o tratamento e o acompanhamento de um cão com ureterocele ectópica e hidronefrose/hidroureter associados. Após as informações obtidas nos exames laboratoriais e de diagnóstico por imagem, o animal foi submetido à ureterocelectomia e à neoureterostomia. O cão apresentou evolução favorável após a conduta terapêutica. Apesar de pouco frequente, a ureterocele deve ser considerada como diagnóstico diferencial em animais jovens com histórico de incontinência urinária.

The clinical features, diagnosis, treatment and monitoring of a dog with an ectopic ureterocele and concomitant hydronephrosis/hydroureter were reported. After the information obtained in laboratory tests and imaging diagnosis, the animal was submitted to ureterocelectomy and neoureterostomy. The dog presented a favorable evolution after the therapy. Although infrequent, the ureterocele should be considered a differential diagnosis in young animals with history of urinary incontinence.

Pediatric ureteroceles: diagnosis, management and treatment options

The aim of the study was to evaluate clinical characteristics of ureteroceles particularly for diagnostic and treatment challenges. Data about patients treated for ureterocele in the two hospital clinics during 1996-2009 are retrospectively evaluated. There were 12 girls and 7 boys. Symptomatic urinary tract infection was found in twelve cases. Ureterocele was associated with duplex systems in eleven cases. Vesicoureteral reflux was detected in 4 patients. Bladder diverticulum complicated with ureterocele in 1 patient. Ultrasonography diagnosed ureterocele in 12 patients. Renal scarring was detected in 6 patients at the side of ureterocele. Fifteen patients showed varying degrees of hydro-ureteronephrosis. Surgical therapy included upper pole nephrectomy in 3 cases. Bladder level reconstruction was performed in 11 cases. Five patients were treated only by endoscopic incision. In the follow up period 4 patients showed long term urinary tract infections whereas 3 of them were treated endoscopically. Postoperative reflux was still present in two patients who were treated by endoscopic incision. Ureterocele diagnosis and treatment show challenges. Urinary tract infection is important marker for urinary system evaluation. Preoperative management generally depends on a combination of diagnostic methods. Endoscopic incision needs serious follow up for postoperative problems

Ureterocele bilateral em adulto jovem / Bilateral ureterocele in young adult

Ureterocele é uma dilatação cística do ureter intravesical. A maioria das ureteroceles é diagnosticada no útero ou imediatamente após nascimento durante exames de ecografia na pesquisa de malformações renais. Severas infecções do trato urinário são a apresentação pós-natal mais comum das ureteroceles, mas podem, raramente, sofrer um prolapso e agudamente obstruir a saída da bexiga. Ocorre mais frequentemente em crianças, entretanto quando encontrada em adultos geralmente está associada à unilateralidade, sendo a sua bilateralidade incomum. Nós apresentamos um relato de caso de uma paciente feminina adulta com ureterocele bilateral e com histórias de infecções do trato urinário por repetição (AU)

Ureterocele is a cystic enlargement of the intravesical ureter. Most ureteroceles are diagnosed in the uterus or immediately after birth in ultrasonographic scans searching for renal malformations. Serious infections of the urinary tract are the most common post-natal presentation of ureteroceles, and they may seldom prolapse and obstruct the bladder outlet. It occurs more frequently in children, but it also appears in adults, usually unilaterally, bilaterality being uncommon. Here we describe the case of an adult female patient with bilateral ureterocele with a history of repeated urinary tract infections (AU)

Prolapsing ureterocele in two infant boys

Ureterocele prolapse is a rare complication that obstructs the bladder outlet. This disease rarely presents in infant boys. In this case report, we present two 2.5 and 5-month-old infant boys with suspected posterior urethral valve diagnosis. Sonography demonstrated significant bilateral hydroureteronephrosis and unilateral interavesical ureterocele in both our patients. Voiding cystourethrography showed a filling defect in the posterior urethra associated with severe unilateral reflux. The diagnosis of prolapsing ureterocele should be considered whenever there is a ureterocele associated with bilateral uropathy

Incidence and outcome of congenital anomalies of the kidney and urinary tract detected by prenatal ultrasonography: a single center study / 소아과

PURPOSE: This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography METHODS: There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. RESULTS: The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. CONCLUSION: CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

Paraurethral Cyst Discovered in Misdiagnosed Urethral Diverticulum of an Adult Female / 대한비뇨기과학회지

A paraurethral cyst is a rare congenital or acquired abnormality in females that is characterized by a round, yellow or orange-colored cystic mass on either side of the urethral meatus. We report a patient who presented with frequent, postvoiding dribbling. The important differential diagnostic considerations in infants and adults are an ectopic ureterocele, urethral diverticulum, cystocele and paraurethral tumors. Intravenous pyelography, voiding cystourethrogram, cystourethroscopy are essential for differentiating these lesions. This case of a paraurethral cyst was treated with complete excision.

Hidronefrosis prenatal en doble sistema excretor / Hidronefrosis prenatal in double system excretor

Con la introducción del ultrasonido materno-fetal en el seguimiento sistemático del embarazo normal se detectan anomalías congénitas en el 1 por ciento de los fetos, el 20 por ciento de las cuales corresponden al tracto urinario. Se realizó el estudio de recién nacidos y lactantes con hidronefrosis prenatal, cuyos ultrasonidos materno-fetales demostraron la presencia de un doble sistema excretor. Para este propósito todos los recién nacidos y lactantes con hidronefrosis detectada prenatalmente, y doble sistema excretor diagnosticado antes o después del nacimiento, fueron seguidos mediante ultrasonografía renal evolutiva, uretrocistografía miccional, gammagrafía estática o dinámica y, en algunos casos, mediante urograma excretor. Hallamos doble sistema excretor en 7 de los 182 pacientes (3,8 por ciento) con anormalidades del tracto urinario diagnosticadas antes del nacimiento. El diagnóstico fue prenatal solo en uno de los fetos (31 semanas de embarazo). La dilatación hidronefrótica fue ligera en dos fetos y grave en cinco. La hidronefrosis obedeció a diferentes causas. Se realizó nefrectomía polar superior en los casos de ectopia ureteral y de uréter superior obstruido, reimplantación en un ureterocele, y en otro se comprobó la ruptura espontánea mediante endoscopia. Los restantes pacientes se trataron sintomáticamente. La hidronefrosis detectada antes del nacimiento mediante ultrasonografía materno-fetal puede estar asociada a un doble sistema excretor. La dilatación hidronefrótica asociada a un doble sistema puede deberse a diferentes causas, y es necesario estudiar cuidadosa y sistemáticamente a estos niños y tratarlos adecuadamente, porque cada uno puede necesitar una conducta diferente.

With the introduction of the maternofetal ultrasound in the systematic follow-up of normal pregnancy, congenital anomalies are detected in 1 percent of the fetuses, 20 percent of which correspond to the urinary tract. The newborns and infants with prenatal hydronephrosis were studied. The maternofetal ultrasound showed a double excretory system. To this end, all the newborns and infants with prenatally detected hydronephrosis and double excretory system diagnosed before or after birth were followed up by evolutive renal ultrasonography, micturition urethrocystography, static or dynamic scintigraphy and, in some cases, by excretory urogram. Double excretory system was found in 7 of the 182 patients (3.8 percent) with abnormalities of the urinary tract diagnosed before birth. Diagnosis was prenatal in just one of the fetuses (31 weeks of pregnancy). Hydronephrotic dilatation was mild in two fetuses and severe in five. Hydronephrosis had different causes. Upper polar nephrectomy was performed in those cases of ureteral ectopy and obstructed upper ureter, reimplantation in one ureterocele, whereas in another it was confirmed the spontaneous rupture by endoscopy. The rest of the patients were symptomatically treated. The hydronephrosis detected before birth by maternofetal ultrasonography may be associated with a double excretory system. Hydronephrotic dilatation associated with a double excretory system may have different causes, and it is necessary to study carefully and systematically these children and to treat them adequately, since each of them may need a different conduct.

Morbilidad de las Uropatías Congénitas Obstructivas del Tractus Urinario Superior más frecuentes en Matanzas / Morbidity of the Congenital Obstructive Uropathies of the Upper Urinary Tract more frequently in Matanzas

Se revisa la morbilidad de las más frecuentes anomalías congénitas del Sistema Urinario en la provincia de Matanzas, Cuba, en un período de 30 años. Se encuentra que las más frecuentes malformaciones Urológicas son el Reflujo Vésico Ureteral, la Hidronefrosis Congénita, por compromiso de la unión pielo ureteral, la Estenosis Urétero Vesical y el Ureterocele. Se analizan la incidencia de estas patologías, edad, sexo, raza, síntomas más frecuentes, técnicas quirúrgicas utilizadas y las patologías asociadas

We review the morbidity of the more frequently congenital anomalies of the Upper Urinary System in the province of Matanzas, Cuba, in a period of 30 years. We found that the more frequently urological malformations are the vesico–urethral Reflux; the congenital hydronefrosis for the compromise of the skin-urethral joint; vesico-urethral stenosis and ureterocele. We analyze the incidence of these pathologies, age, sex, race, and more frequent symptoms; used surgical techniques and associated anomalies.