Effect of folic acid in a modified experimental model of anorectal malformations adriamycin-induced in rats

PURPOSE: To determine the effect of a single dose of adriamycin (ADR) to induce anorectal malformations (ARMs) and determine the effect of folic acid (FA) in this model. METHODS: Ten female Wistar rats were divided randomly in two groups. Group A - ADR; Group B - FA+ADR. Dams from group B received daily, since two weeks before the pregnancy to the end of pregnancy, FA (50mg/kg) by gavage. Dams from both groups received ADR (6mk/kg) by intraperitoneal injection on gestational day (GD) 8. Their fetuses were harvested by cesarean section on GD21 and were examined looking for ARMs. The thickness of anal stratified squamous epithelium (ASSE) and intestinal epithelium (IE) were analyzed. p≤0.05*. RESULTS: 81 fetuses were harvested. The number of fetuses; number of ARMs; mean (∆%) (± SD) were determined to be, respectively: ADR - 41[29;65%(±37%)] versus FA+ADR - 40[04;16%(±36%)] (p=0.05). AMRs were significantly lower in FA+ADR group than in ADR group (p=0.05). The thickness (µm) of ASSE (± SD) and IE (± SD) were measured, respectively: ADR - [25.98(±0.74) and 19.48(±1.68)] versus FA+ADR - [24.74(±0.91) and 24.80(±0.81)] (p<0.005). The thickness of IE was significantly enlarged when FA was given (p<0.005). CONCLUSIONS: Single dose of adriamycin on D8 was able to induce anorectal malformations. Folic acid reduces the number and enlarged the IE of ARMs ADR-induced.

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.

Surco perineal en ginecología infantil: reporte de 2 casos clínicos / Perineal groove in pediatric gynecology: a report of 2 cases

Introduction: the perineal groove is a very uncommon anorectal anomaly. It is the result of an unknown embryology anomaly. The perineal groove is a wet sulcus extending from de fourchette to the anus. It is a benign pathology and tends to resolve spontaneously. Objective: We report two cases of girls diagnosed with perineal groove and their follow up. Cases reports: The first case is an 18 days old baby girl, that during her first clinical examination there was found a painless lineal lesion in the perineum from the fourchette to the anus, which created a big anxiety in her family. She was cared at the Pediatric Gynecology Unit, with local lubrication, doing well with the epithelization of it. The second case is an 8 year-old girl who consulted because, when being a child, she was diagnosed with a perineal lesion, but she didn't receive any special treatment and now she feels some aches in the perineum. Conclusions: The perineal groove is a benign entity pretty unknown by general practitioners or pediatricians. It is important to be up to date with this condition to avoid alarming misinterpretations.

Introducción. El surco perineal es una malformación poco frecuente, de etiología desconocida, consiste en una ranura en la línea media del periné desde la horquilla vulvar al ano, es benigna y tiende a la resolución espontánea. Objetivos: Reportar el diagnóstico y evolución de dos pacientes con surco perineal. Casos clínicos: Caso 1. Recién nacida de 18 días que previo al alta de maternidad se pesquisó una lesión lineal no dolorosa desde la horquilla vulvar al ano, lo que generó gran inquietud en su familia. En Ginecología Infantil se confirmó el diagnóstico, se manejó con lubricación del área genital evolucionando a la epitelización del surco. Caso 2. Niña de 8 años consultó por que en controles de salud se diagnosticó una "fisura" entre la horquilla vulvar y el ano que no fue tratada y presentaba molestias inespecíficas en el periné. Se indicó resección quirúrgica, pero paciente evolucionó satisfactoriamente con lubricación de la zona. Conclusiones. El surco perineal es una entidad benigna que debe ser conocida por médicos de atención ambulatoria debido a la alarma que pueden generar sus diagnósticos diferenciales.

OEIS complex with major cardiac malformation: a case report.

OEIS Complex is a rare congenital multisystem defect that consists of omphalocele, exstrophy, imperforate anus and spinal defects. We report a case of such complex with additional major cardiac and other multisystem anomalies which are rarely described in literature. The authors give a review of literature on this infrequent complex along with a discussion on its pathogenesis, differential diagnosis and prenatal diagnosis.