What urethrogram sees and a surgeon does not. / Lo que una uretrografía ve y un cirujano no.

INTRODUCTION: Urethrorrhagia is an infrequent sign in childhood. It should be distinguished from hematuria, since they have a different etiology. CLINICAL CASE: 11-year-old male patient with significant urethrorrhagia. Urinary sediment analysis: red blood cells++. Pelvic ultrasonography: fusiform anechoic image in the corpus spongiosum of the penile root. Retrograde urethrogram: normal anterior urethra, extraluminal contrast passage in the ventral aspect of the bulbar urethra. Cystoscopy: no pathological findings in the urethra or the bladder. Control retrograde urethrogram: cystic dilatation of Cowper's gland duct; Maizels' type 3 perforated syringocele. DISCUSSION: Cowper's syringocele is a rare pathology. It can occur at any stage of childhood in the form of urinary infection, obstructive voiding symptoms, or urethrorrhagia. Urethrogram is key for diagnostic purposes, since most Cowper's syringoceles are detected following urethrogram or cystoscopy. Cases with functional repercussions for the urinary system require surgical treatment. Otherwise, a wait-and-see approach is feasible.

INTRODUCCION: La uretrorragia es un signo infrecuente en la infancia que debe distinguirse de la hematuria dada la diferente etiología de las mismas. CASO CLINICO: Varón de 11 años con uretrorragia franca. Sedimento urinario: hematíes++. Ecografía pélvica: imagen anecoica fusiforme en cuerpo esponjoso de raíz peneana. Uretrografía retrógrada: uretra anterior normal, paso de contraste extraluminal ventral en uretra bulbar. Cistoscopia: sin hallazgos patológicos en uretra ni vejiga. Uretrografía retrógrada de control: dilatación quística del conducto de las glándulas de Cowper; siringocele perforado tipo 3 de Maizels. COMENTARIOS: El siringocele de Cowper es una patología infrecuente que puede debutar en cualquier momento de la infancia como infección urinaria, síntomas miccionales obstructivos o uretrorragia. La uretrografía es fundamental en su diagnóstico ya que la mayoría se objetivan por este medio o cistoscopia. Los casos con repercusión funcional del sistema urinario requieren tratamiento quirúrgico. En caso contrario podrá realizarse actitud expectante.

[Genital-urinary rhabdomyosarcoma in childhood: our experience]. / Rabdomiosarcoma genitourinario en la infancia, nuestra experiencia.

INTRODUCTION: Rhabdomyosarcoma (RSM) becomes the most common tumour of the soft tissues during the paediatric age. It represents among 2-3% of child tumours. The genital-urinary location is the second most common location, only after head and neck. The treatment is usually medical, being the surgery a mere contribution, except for the cases in which the situation is not under control, when very aggressive surgery is necessary. The aim of this study is to analyse the cases of genial-urinary RMS that have been treated in our centre and the role that surgery has in their treatment. MATERIAL AND METHODS: Retrospective study of 20 patient (7 girls and 13 boys) with a median age of 24 months (range from 1 month to 12 years) with RMS in the aurochs-genial tract who have been treated in our hospital from 1990 to 2012. The variables described are demographic, location of the primary tumour, state at diagnosis, received treatment, both medical and surgical, with greater emphasis on the kind of surgery applied and monitoring in terms of survival. RESULTS: The location of the primary tumour was: bladder (6), paratesticular (5), vagina (3) retroperitoneal space (3), lesser pelvis (2) and prostate (1). All of them received medical treatment with chemotherapy and radiotherapy following International Society of Pediatric Oncology protocol after diagnostic biopsy. Surgery, which was always used as help, was: reappraisal of biopsy (1), orchiectomy (5), tumoral resection (8) and radical surgery (cystoprostatectomy or pelvic exenteration) in 6 patients. There were 3 deaths, 2 because of the evolution of the disease and 1 because of postoperative sepsis. The survival rate is 80% with a median follow - up of 14 years. CONCLUSIONS: The RMS is the most common tumour of soft tissues in childhood and the genital-urinary location is the second most common after the parameningeal one. The treatment is multidisciplinary and the surgery has a contributing role when there is no answer to the medical treatment or when there is a residual tumour even if some patients do not respond to medical treatment and they need a radical surgery for recovery.

INTRODUCCION: El rabdomiosarcoma (RMS) constituye el tumor de tejidos blandos más frecuente en la edad pediátrica, representando el 2-3% de los tumores infantiles. La localización genitourinaria es la segunda en frecuencia tras la cabeza y cuello. El tratamiento suele ser médico, quedando la cirugía como coadyuvante, excepto en casos no controlados en que se precisan cirugías muy agresivas. El objetivo del estudio es analizar los casos de RMS de localización genitourinaria tratados en nuestro Centro y el papel que la cirugía tiene en su tratamiento. MATERIAL Y METODOS: Estudio retrospectivo de 20 pacientes (7 niñas y 13 niños) con una mediana de edad de 24 meses (rango de 1 mes a 12 años) con RMS del tracto urogenital tratados en nuestro Hospital desde 1990 hasta 2012. Se describen variables demográficas, localización del tumor primario, estadio al diagnóstico, tratamiento recibido, tanto médico como quirúrgico, con especial atención al tipo de cirugía realizada y seguimiento en términos de supervivencia. RESULTADOS: La localización del tumor primario fue: vejiga (6), paratesticular (5), vagina (3), retroperitoneo (3), pelvis menor (2) y próstata (1). Todos recibieron tratamiento médico con quimioterapia y radioterapia según protocolo de la Sociedad Internacional de Oncología Pediátrica (SIOP) previa biopsia diagnóstica. La cirugía, practicada en todos los casos como coadyuvante fue: reevaluación por biopsia (1), orquiectomía (5), resección tumoral (8) y cirugía radical (cistoprostatectomía o exanteración pélvica) en 6 pacientes. Hubo 3 fallecimientos, 2 por progresión de la enfermedad y 1 por sepsis postoperatoria. Los 17 restantes están vivos, lo que supone una supervivencia del 80% con una mediana de seguimiento de 14 años. CONCLUSIONES: El RMS es el tumor de tejidos blandos más frecuente en la infancia y la localización genitourinaria la segunda en frecuencia tras las parameníngeas. El tratamiento es multidisciplinar y la cirugía tiene un papel coadyuvante en casos de no respuesta al tratamiento médico o de tumor residual aunque hay pacientes que no responden al tratamiento médico y precisan de cirugía radical para su curación.

[Therapeutic approach in urinary tract stones in children]. / Abordaje de la litiasis en tracto urinario en niños.

PURPOSE: Kidney stone disease in children is a rare pathology, with a low incidence in Spain (1/4,500 hospitalized children). The spontaneous expulsion rate is about 34-47% which means that more of 50% of children need active treatment. Paediatric patients forming urinary stones have a high risk of recurrence, therefore, a standard diagnosis and treatment are needed. We present our experience in urolithiasis treatment in children. MATERIALS AND METHODS: We reviewed retrospectively all the patients ≤ 16 years hospitalized in our hospital with urolithiasis diagnosis from 2000 to 2013, citing treatment modality, stone-free rates and complications. RESULTS: A total of 69 patients with a mean age of 8,2 years (range 1-16 years) were treated in our hospital during that period. The main clinical presentation was pain (52%). The diagnosis was made by abdominal ultrasounds in all cases. About localization, 21 lithiasis were found in distal urether (UD), 8 in medium urether (UM), 3 in proximal urether (UP) and 13 in renal pelvis (PR). The mean size was 13 mm. 21 (30%) patients had a spontaneous expulsion of the stone, 14 (20%) patients were treated with extracorporeal shock wave lithotripsy and in 22 (32%) patients the elected therapy was ureterosopic stone fragmentation (n = 13) or removal (n = 9). No complications were observed. The overall stone-free rate was 79% (n = 55). CONCLUSIONS: Kidney stone disease in children is a rare pathology, with its own features about diagnosis and treatment, which requires medical care in a specialized center. The optimal treatment should be considered regarding the age of the patient, localization and size of the stone, as well as the team experience.

[Laparoscopic pyeloplasty in pediatric patients: our experience]. / Pieloplastia laparoscópica en la edad pediátrica. Nuestra experiencia.

The aim of this essay is to present our initial experience with laparoscopic pyeloplasty and highlight how some specific technical changes allowed us to improve our results. We performed a chart review of the patients that underwent laparoscopic pyeloplasty in our institution. We included patients older than 6 months old with proved stenosis of the ureteropelvic junction. We compared our first patients with the last ones in which we performed laterocolic approach in all left pyeloplasties and included a modification of the technique to place an external ureteric stent. We performed 13 laparoscopic pyeloplasties, 8 male patients and 5 female. There were 3 right pyeloplasties (23%) and 10 left ones (77%). We performed transmesocolic approach in 2 cases (left) and laterocolic approach in 11. Mean surgical time was 184 minutes in the first 8 cases and 142 in the 5 last ones. We had three cases of complications in the first group, two stents migrated to ureter and one postsurgical infection. In the last cases we had a postoperative bleeding. Laparoscopic approach is an effective option for pyeloplasty with similar results to those of the open approach in spite of a longer surgical time. Experience and specific surgical details allow us to reduce complication rate and surgical time.

[Ophthalmological manifestations in Fabry's disease. Four clinical cases showing deficient alpha-galactosidase-A activity]. / Manifestaciones oftalmológicas en la enfermedad de Fabry. A propósito de 4 casos con actividad deficiente de a-galactosidasa A.

CASE REPORT: Fabry's disease is an illness produced by an alteration in the catabolism of the glycosphingolipids. We report ophthalmologic findings in 4 people, detected after 113 patient evaluations from an analytical, cardiological and genetic point of view. DISCUSSION: Fabry's disease is uncommon and shows variable ophthalmologic affectation. Some patients with Fabry's disease do not present ocular affectation, while, on the other hand, healthy carriers with important ocular alterations have been described. The deposit of glycosphingolipids produces affectation at the corneal, crystalline, vascular and retinal levels. The vascular alterations affect not only the veins but also the arteries, as we report in our patients.

[Circumscribed choroidal hemangioma treated with photodynamic therapy]. / Hemangioma circunscrito de coroides tratado con terapia fotodinámica.

CLINICAL CASE: A 45-year-old male referred with a scotoma in the left eye of 2 months' evolution. A peripapillary tumor was found, with a basal diameter of 7 mm and thickness 3.4 mm. It was diagnosed as a circumscribed choroidal hemangioma. It was decided to treat the patient using photodynamic therapy. After 4 sessions and 17 months follow-up, the patient remained asymptomatic and visual acuity was stable. There was evidence of subretinal fibrosis and no subretinal fluid was found. DISCUSSION: Photodynamic therapy is a good option for the treatment of circumscribed choroidal hemangioma given the minimal damage is causes to the adjacent retina.