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Purpose Hirschsprung's disease (HD) is uncommon in females. There are very few reports on the patients' obstetric and gynecological outcome. Hydrosalpinx causes pain and infertility. It is rare in nonsexually active teenagers. It may be because of an intrinsic disease of the fallopian tubes or secondary to surgery. Aim: to describe the relationship between hydrosalpinx and HD or its surgical approach; to report the impact of bilateral hydrosalpinx on fertility in HD. Methods The records of all females with HD since 1980 were reviewed. Only patients who reached menarche were included. Prevalence of hydrosalpinx and hydrosalpinx-free survival were compared after abdominoperineal (A) or transanal (T) surgery. Treatment for hydrosalpinx was reviewed. Results Seventeen out of 27 patients had reached menarche (Group A: 13 patients; Group T: 4 patients). Five patients in group A and none in group T presented bilateral hydrosalpinx (p=0.261). There were no statistical differences in hydrosalpinx-free survival between groups (p=0.344). Hydrosalpinx treatment: two bilateral and one unilateral salpingectomy, one pyosalpinx evacuation and one untreated. Three patients had conception desire: one has children; two are on IVF program. Conclusion An association between hydrosalpinx and HD was observed. The development of hydrosalpinx was not associated with surgical approach in our study. Females with HD should have a gynecological follow-up for the development of hydrosalpinx, which can impair fertility. Level of evidence Level III, retrospective comparative study.

Anal canal duplication (ACD) is the rarest of gastrointestinal duplications. Few cases have been reported. Most cases present as an opening in the midline, posterior to the normal anus. The aim of our revision is to contribute with eight new cases, some of them with unusual presentations: five presented as the typical form, one with a perianal nodule, and two presented as two separate orifices (anal canal triplication). Complete excision was performed in all patients with no complications. ACD is the most distal and the least frequent digestive duplication. Its treatment should be surgical excision, to avoid complications such as abscess, fistulization, or malignization. Anal canal triplication has never been described before.

To analyze our experience in the treatment of anorectal malformations (ARM) with the posterior sagittal anorectoplasty (PSARP), and our modifications through the last few years and the outcomes. We reviewed 107 cases divided into two groups: Former (F: 1994–2003) and Recent (R: 2004–2008). Type of ARM, associated anomalies, management and complications were noted. A telephone questionnaire regarding continence outcome was addressed to the 74 cases older than 3 years. According to the type of ARM, there were 53 perineal fistulas, 2 anal stenoses, 11 no fistulas, 12 rectourethral fistulas (5 rectobulbar and 7 rectoprostatic fistulas), 22 vestibular fistulas, 1 rectovesical fistulas and 6 cloacas. A total of 47 patients presented with 73 associated malformations. As much as 45 colostomies were performed, including 5 perineal fistulas, with 6 of 7 vestibular fistulas in group F and only 8 of 15 in group R. We had 19 complications of PSARP. The most frequent one was rectal mucosa prolapse in 14 (12F and 2R) and 2 wound infections (F). Continence was good in 62, poor in 3 and fair in 5. Seven out of eight children with poor or fair continence had associated malformations. All perineal fistulas can be managed without colostomy. Vestibular fistulas can be safely treated without colostomy in otherwise healthy patients without severe malformations. Overall, continence is good, and fair/poor results are related to associated malformations. Cumulative experience helps avoid colostomies and reduce complication and reoperation rates.

The intestinal damage in gastroschisis (GS) has been attributed to a narrow abdominal wall defect (AWD), among other causes, but this causal effect is difficult to prove in humans. The present experimental study was done to ascertain the damaging effect of clinically extrapolable mild and moderate constriction at the AWD on the intestine of fetuses with GS. AWDs of two different sizes were carried out in the fetal rabbit model: small-ring GS (1.5x bowel diameter, SRG) and large-ring GS (3x bowel diameter, LRG); a group of unoperated littermates served as controls. Fetal body weight, intestinal length and weight, bowel diameter and wall thickness, and histology were checked 7 days later. No statistical difference was found in body weight and bowel diameter among the groups. Intestinal length, weight, and wall thickness were significantly different in the GS groups compared to the controls, but no difference was found between the GS groups. Histology did not show venous stasis, ischemic lesions, or differences in the degree of edema between groups SRG and LRG. Mesothelial hyperplasia was seen in both GS groups. The intestinal changes in length, weight, diameter, wall thickness, and histology in GS should thus not be attributed to the diameter of the AWD.

Skin wound healing has been shown to be a different process in the fetus than in the adult animal. Some of these differences have been attributed to the unique fetal environment (i.e., amniotic fluid). The aim of the present study is to compare fetal skin healing in intraamniotic and extraamniotic wounds. A fetal rabbit model has been used in which three types of skin wounds were induced on 23-day-old fetuses in contact with either amniotic fluid or maternal peritoneal fluid. The wounds consisted of a sutured skin incision, a nonsutured incision, and an electrocautery burn. Seven days later all wounds were examined mechanically (scar resistance), biochemically (collagen and noncollagen protein concentration), and histologically. Biochemical and growth factor studies of both environments, the amniotic and the peritoneal fluids, were also conducted. The results showed excellent healing by first intention and absence of healing by second intention in both environments, greater scar resistance in the intraperitoneally positioned fetal wounds, and a higher concentration of insulin-like growth factor-1 (IGF-1) in the peritoneal fluid than in the amniotic fluid. It can be concluded that fetal skin wounds in contact with peritoneal fluid show the same healing pattern as in the natural fetal environment (amniotic fluid). The higher concentration of IGF-1 in the peritoneal fluid suggests that this growth factor, through its relationship with the growth hormone, plays a role in increasing the scar resistance of fetal skin wounds in contact with maternal peritoneal fluid.

Background/Purpose Recent experience in fetal surgery to correct myelomeningocele in humans reports an early reversion of hydrocephalus and decreased need of ventricular shunting in the first months of life; however, it has not been possible to demonstrate benefit in lower extremity function. In the present work, we have tried to ascertain the impact of cord exposure on hind limb function. Methods Fetal rabbits with myelomeningocele (group M), treated myelomeningocele (group T), and control animals (group C) were compared at birth regarding physical examination, somatosensory-evoked potentials of the hind limbs, ventricular morphometry, and spine histology. Results No major difference was found between groups M and T in the physical examination. Somatosensory-evoked potentials of the hind limbs were absent in group M and present in group T, although showing a longer latency period and decreased amplitude than controls. The area of the third ventricle was significantly larger in group M than in group C; in group T, it was also somewhat larger but not significantly so. Cord histology had evident changes in group M and minor changes in group T, which resembled normal group C cord. Conclusions Prenatal covering of the spinal cord prevents central and peripheral neurologic deterioration in this animal model of myelomeningocele.

Background/purpose: The aim of this study was to determine the role of urine exposure in gastroschisis on the pathologic and biochemical aspects. Methods: The intestines of fetal rabbits with gastroschisis (group G), gastroschisis and urethral ligation (group GL), and normal controls (group C) were studied by measuring weigh and length, intestinal diameter and wall thickness, and thickness of each intestinal layer. Number and length of villi and villi edema were evaluated. Total protein and DNA were measured in intestinal homogenate. Lactase activity and alkaline phosphatase activity were analyzed in isolated microvilli membranes. Results: Intestinal length, diameter, and wall thickness were significantly different in groups G and GL compared with C but not between groups G and GL. The same was true for the thickness of the internal muscular and serosa, villi length, and villi edema. Serosal reaction was milder in group GL than in group G, and absent in C. Total protein, lactase activity, and alkaline phosphatase activity were also significantly different in G and GL compared with C but not between G and GL. Conclusions: Urine in amniotic fluid causes an increased serosal reaction but does not account for the mucosal enzyme dysfunction.