Gemelo heterópago / Heteropagus Twins

Los gemelos parásitos o heterópagos son aquellos unidos asimétricos con partes deformes del parásito que se unen en diferentes regiones del gemelo normal (autosite), del cual depende para su soporte nutricional y crecimiento. Presentamos el caso de un gemelo heterópago con las extremidades inferiores y rudimentos de las superiores del parásito unidas al periné y glúteo derecho del autosite. Aunque no compartían órganos pélvicos, la compresión extrínseca de estructuras óseas del parásito causaron estenosis del recto, lo que obligó a la corrección quirúrgica. Los estudios por imágenes permitieron planificar la cirugía, lo cual facilitó la separación exitosa y lograr la sobrevida del paciente con muy buena calidad de vida. A pesar de ser reportados desde épocas ancestrales, aún generan gran interés debido a la variabilidad en su presentación clínica y al desconocimiento en su etiopatogénesis.

Heteropagus or "parasitic" twins are asymmetric conjoined twins in whom the tissues of a severely defective twin (parasite) are dependent at variable sites of the other intact twin (autosite) for nutritional supply and growth. Here we present the case of heteropagus twins. The parasite possessed two lower limbs and rudimentary upper limbs attached to the perineum and right buttock of the autosite. Although they did not share pelvic organs, the external compression of the parasite ́s skeletal structures caused rectum stenosis on the autosite, which required surgical treatment. Imaging studies allowed presurgical planning for the twins ́ successful separation with good survival and quality of life of the autosite. Although heteropagus twins have been described since ancient times, they still generate great interest due to the varying clinical manifestations and lack of knowledge on their pathogenesis

Síndrome de Mayer-Rokitansky- Küster-Hauser asociado con malformación anorectal / Mayer-Rokitansky-Küster-Hauser syndrome associated with anorectal malformation

La agenesia vaginal es una entidad poco frecuente que puede presentarse en forma aislada o acompañada de otras anomalías congénitas. El desconocimiento de esta asociación puede retardar su diagnóstico, causar complicaciones indeseadas y comprometer el resultado del tratamiento. Se describen tres casos clínicos con agenesia vaginal y malformación anorrectal, cuyos diagnósticos no fueron realizados en el momento del nacimiento. Aunque la coexistencia de anomalías vaginales y anorectales sean poco frecuentes, los médicos responsables de su tratamiento deben ser conscientes de su existencia y practicar examen perineal cuidadoso.

Vaginal agenesis is a rare condition. It may exist in isolation or be associated with other congenital anomalies. A lack of awareness on this association may delay diagnosis, cause undesirable complications and compromise treatment outcomes. We describe three cases of vaginal agenesis associated with anorectal malformation which were not diagnosed at birth. Although the coexistence of vaginal and anorectal anomalies is uncommon, treating physicians should be aware of them and perform a detailed perineal examination.

Torsión aislada de trompa de falopio en niñas prepúberes / Isolated torsion of the fallopian tube in premenarcheal girls

La torsión aislada de la trompa de Falopio es una causa poco común de dolor abdominal agudo. Se describen dos casos clínicos de niñas prepúberes de 10 y 13 años de edad, con manifestaciones clínicas compatibles con apendicitis aguda, en quienes se evidenció torsión de la trompa derecha con signos de necrosis en la intervención quirúrgica. La existencia de esta entidad debe ser considerada en niñas con dolor abdominal agudo para evitar la lesión irreversible de la trompa.

Isolated torsion of the fallopian tube is a rare cause of abdominal pain. We report on two premenarcheal girls, ages 10 and 13 years, who presented with clinical manifestations compatible with acute appendicitis, in whom, torsion of the right tube with signs of necrosis was evidenced during surgical intervention. This condition should be considered in all girls with acute abdominal pain to avoid irreversible damage to the tube.

Lesiones de uretra posterior secundarias a fractura pélvica tratadas a través de abordaje perineal progresivo. Breve revisión de la literatura. / Pelvic fracture posterior urethral injury repaired by progressive perineal approach: A brief review of the literature

Objetivo: Revisar los resultados operatorios de nuestros pacientes con rotura traumática de uretra posterior, tratados a través del abordaje perineal progresivo y realizar una breve revisión de la literatura. Materiales y métodos: fueron analizadas las historias médicas de 7 pacientes en edades comprendidas entre 2 y 12 años, con lesiones uretrales asociadas con fracturas pélvicas, producidas entre marzo de 2005 y enero de 2017. Después del diagnóstico de la lesión, se realizó cistostomía derivativa en los 7 casos; en 2 de ellos con lesión rectal asociada se realizó, además, colostomía tipo Hartman en el momento de la derivación urinaria. La evaluación urológica preoperatoria para la uretroplastia definitiva incluyó uretrografía anterógrada y retrograda simultánea para determinar la separación entre los segmentos uretrales. Se decidió la reparación retardada de la uretra a través de abordaje perineal progresivo con un mínimo de 6 meses posterior al trauma. Resultados: El mecanismo de producción del trauma fue arrollamiento por vehículos a motor en 6 pacientes y aplastamiento por balancín petrolero en uno. La lesión uretral fue completa en todos. Tres presentaron complicaciones quirúrgicas; 2 estenosis uretrales que mejoraron con dilataciones y una fístula uretroperineal que cerró en forma espontánea sin consecuencias. Todos los pacientes se encuentran asintomáticos; ninguno ha requerido una nueva uretroplastia. Comentarios: Nuestros hallazgos con la implementación de este abordaje terapéutico coinciden con lo reportado en la literatura.

Objective: To evaluate the postoperative outcomes of our patients with posterior urethral traumatic rupture repaired by progressive perineal approach, and to conduct a brief review of the literature. Materials and methods: The clinical records of 7 patients aged between 2 to 12 years with urethral injuries due to pelvic fracture produced between March 2005 and January 2017, were reviewed. A suprapubic cystostomy for urinary drainage was constructed in the 7 patients immediately after diagnosis; two of them had concomitant rectum injuries thus a Hartmann's colostomy was also performed in them. The preliminary urologic appraisal for definite urethroplasty included a synchronous anterograde and retrograde cysto-urethrogram to determine separation of the urethral segments. The final selection of surgical procedure was delayed urethral repair by progressive perineal approach minimum 6 months after the trauma event. Results: The mechanism of injury was, blunt trauma after being struck by a moving vehicle in 6 patients and a crash injury caused by an oil rocker in one patient. The urethral disruption was complete in all patients. Three patients developed surgical complications: 2 urethral strictures which improved with dilatation and one urethral-perineal fistula with spontaneous closure and no associated complications. All patients remain asymptomatic; no patient has required a redo urethroplasty. Commentaries: Our findings through the implementation of this therapeutic approach are consistent with those reported in the literature. Conclusions: Progressive perineal access allows performing an anastomotic urethroplasty with good clinical outcomes in most patients.

[Congenital recto-vaginal fistula associated with a normal anus (type H fistula) and rectal atresia in a patient. Report of a case and a brief revision of the literature]. / Asociación de fistula rectovaginal congénita con ano normal (Fístulas Tipo H) y atresia rectal en una paciente. Reporte de un caso y breve revisi6n de la literatura.

Congenital recto-urogenital type fistulas with a normal anus and rectal atresia, represent both anorectal malformations that are infrequently seen in clinical practice. We describe the case of a girl with an association of these two anomalies, together with a double vagina who, on her seventh day of life, expelled feces through her genitals. The malformations were corrected by means of a posterior sagittal approach, descending from the rectum to the anus without perineal dissection. The vaginal septum was resected thru the vulva. There is no evidence of recurrence of the recto-vaginal fistula.

Asociación de fistula rectovaginal congénita con ano normal (Fístulas Tipo H) y atresia rectal en una paciente. Reporte de un caso y breve revisión de la literatura / Congenital recto-vaginal fistula associated with a normal anus (type H fistula) and rectal atresia in a patient. Report of a case and a brief revision of the literature.

Las malformaciones ano-rectales del tipo de fístulas recto-urogenitales congénitas con ano normal y atresia rectal son anomalías poco frecuentes. Se discute el caso de una niña con la asociación de estas dos entidades, acompañada además de una vagina doble, quien fue llevada a la consulta a los siete días de vida por la expulsión de heces a través de los genitales. Las malformaciones fueron corregidas a través de un abordaje sagital posterior, con descenso del recto hasta el ano sin disección perineal. El tabique vaginal fue resecado a través de la vulva. En la actualidad no hay evidencia de recurrencia de la fístula recto-vaginal.

Congenital recto-urogenital type fistulas with a normal anus and rectal atresia, represent both anorectal malformations that are infrequently seen in clinical practice. We describe the case of a girl with an association of these two anomalies, together with a double vagina who, on her seventh day of life, expelled feces through her genitals. The malformations were corrected by means of a posterior sagittal approach, descending from the rectum to the anus without perineal dissection. The vaginal septum was resected thru the vulva. There is no evidence of recurrence of the recto-vaginal fistula.

[Exstrophy of rectal duplication associated with anorectal malformation and penoscrotal transposition with perineal hypospadias. A case report]. / Duplicación rectal extrofiada asociada a malformación anorrectal y transposición pene-escrotal con hipospadias perineal. reporte de un caso clínico.

We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

Duplicación rectal extrofiada asociada a malformación anorrectal y transposición pene-escrotal con hipospadias perineal: Reporte de un caso clínico / Exstrophy of rectal duplication associated with anorectal malformation and penoscrotal transposition with perineal hypospadias: A case report

Se presenta el caso de un paciente masculino quien requirió tratamiento por agenesia anorrectal con fístula rectouretral y transposición pene-escrotal con hipospadias perineal, acompañados de una masa perineal. La tumoración perineal se encontró íntimamente adherida y en continuidad al recto, lo que la hace compatible con una duplicación rectal extrofiada. La reconstrucción quirúrgica de la anomalía se realizó en etapas hasta lograr resultados funcionales y estéticos aceptables.

We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

[Two cases of Wernicke's disease]. / Dos casos de enfermedad de Wernicke.

We describe the cases of two men, one of whom was a non-alcoholic 50-year-old man who had been on hunger strike for 50 days and who was admitted to hospital with dysarthria, unstable gait, disorientation, ophthalmoplegia and ataxia. The suspected diagnosis was Wernicke's encephalopathy and treatment consisted of intramuscular thiamine shots followed by oral administration of the same drug, which produced rapid symptom resolution. In both patients, magnetic resonance imaging showed bilateral and symmetrical injuries around Silvio's aqueduct and in the periventricular region of both thalami.

Dos casos de enfermedad de Wernicke / Two cases of Wernicke’s disease

Presentamos el caso de 2 varones, uno de ellos no bebedor de alcohol y en situación de huelga de hambre desde 50 días antes, que ingresan con disartria, inestabilidad para la marcha, desorientación temporoespacial, oftalmoplejía y ataxia. Ante el diagnóstico de sospecha de encefalopatía de Wernicke, se inició en ambos casos tratamiento con tiamina intramuscular y luego por vía oral, con rápida desaparición de los síntomas. La resonancia magnética permitió constatar en ambos casos lesiones bilaterales y simétricas alrededor del acueducto de Silvio y en la región periventricular de ambos tálamos (AU)

We describe the cases of two men, one of whom was a non-alcoholic 50-year-old man who had been on hunger strike for 50 days and who was admitted to hospital with dysarthria, unstable gait, disorientation, ophthalmoplegia and ataxia.The suspected diagnosis was Wernicke’s encephalopathy and treatment consisted of intramuscular thiamine shots followed by oral administration of the same drug, which produced rapid symptom resolution. In both patients, magnetic resonance imaging showed bilateral and symmetrical injuries around Silvio’s aqueduct and in the periventricular region of both thalami (AU)

Laparoscopic-assisted correction of a retrocaval ureter.

Retrocaval ureter has been traditionally corrected surgically through an open retroperitoneal procedure; however, in recent years, the correction has been accomplished using laparoscopy. The great advantages of this procedure remain limited because of the prolonged time it takes to complete the intracorporeal suture of the ureter, besides the need for a highly experienced surgeon. We present a case in which the ureter was freed using laparoscopy, and the ureteral anastomosis was carried out extracorporeally, which notably diminished the duration of the surgery. This technique would have limited use in obese patients, patients with ureters of limited length, patients with very proximal obstructions of the ureter, and patients with risk of devascularization of both ureteral segments. However, the technique does appear effective and reproducible in adequately selected patients.

Hernia diafragmática post-traumática: reporte de un caso pediátrico / Hernia diaphragmatic traumatic: report of a pediatric case

Se presenta el caso de un paciente de siete años con ruptura diafragmática post-traumatica. Se hace una revisión de la fisiopatología, clínica, diagnóstico y tratamiento de esta entidad nosológica