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Massive ovarian edema is a rare cause of ovarian enlargement in young women. This article describes the case in a nine-year-old girl without signs of virilization or early puberty. The patient underwent laparotomy and a right oophorectomy was performed. The final pathology was massive ovarian edema. Awareness of this rare and benign lesion in young women and pediatric patients may allow a conservative management avoiding oophorectomy in some cases.

Pneumonectomy is a rare procedure in pediatric age. In contrast to adults, children that undergo this intervention are prone to postoperative mediastinal shift, which leads to bronchial stretching resulting in severe respiratory failure. This postpneumonectomy syndrome can be corrected by inserting a prosthesis in the empty side of the chest. The authors present the case of a 9-month-old infant affected with a pleuropulmonary blastoma who underwent left pneumonectomy and prosthesis placement in the same operation.

Cystic fibrosis is the most common life-limiting recessive genetic disorder in Caucasian. It is caused by mutations of CFTR gene (cystic fibrosis transmembrane conductance regulator); at present over 500 mutations are known. Cystic fibrosis as a cause of respiratory distress in the neonate is quite rare. In neonatal period the most important clinical manifestations are meconium ileum and much rarely cholestatic jaundice. We present two cases of cystic fibrosis in newborns. In the first one, we point out the strict association between meconium ileum and cystic fibrosis. The patient underwent a surgical treatment for meconium ileum and the diagnosis was rapidly confirmed by genetic analysis and sweat test. The second one had intestinal obstruction from birth caused by meconium ileum associated with ileal atresia; besides, he developed cholestatic jaundice, severe and rapidly progressive respiratory disease. He died at 102 degrees day of age for cardiac failure. The diagnosis of cystic fibrosis, supported by typical clinical features and high level of serum trypsin, unfortunately wasn't confirmed by genetic analysis (lambda F508/neg), in addition, the sweat test wasn't reliable because an inadequate quantity of sweat was collected.

Female pseudohermaphroditism is a condition characterized by various degree of external genitalia virilization in a patient with female internal genitalia and karyotype (XX). External genitalia is masculinized congenitally when female fetus is exposed to excess androgenic environment. Fetal metabolic abnormalities, like congenital adrenal hyperplasia, are the most common causes of female pseudohermaphroditism, however there is a low incidence of gestational hyperandrogenism caused by maternal pathology. We report a case of female pseudohermaphroditism secondary to a maternal ovarian luteoma of pregnancy producing androgenic hormones. The newborn presented a severe degree of external genitalia virilization with high urogenital sinus (stage Prader V). Moreover we describe the main steps of diagnostic iter that are necessary both to exclude other causes of virilization and to study all anatomical aspects in view of the surgical correction. The operation consists in two phases of action: an early clitorisvulvoplasty according to Passerini-Glazel and a late vaginal pull-through with anterior saggital transanorectal approach (ASTRA).

Purpose: Bladder exstrophy has recently become one of the several congenital malformations detectable prenatally by fetal ultrasonography, which implies the necessity of a correct prognostic evaluation of quality of life on which therapeutic termination of pregnancy may depend within the temporal limits stated by the law. While maintaining a preeminently informative role, prenatal counseling has gained increasing relevance for the quality of life long-term prognosis in adults affected by bladder exstrophy, particularly in regard to urinary continence, and sexual and reproductive problems in male and female individuals. We report a case of bladder exstrophy diagnosed at 22 week of gestation, and comprehensively review the literature on prenatal diagnostic tools and procedures as well as long-term followup in such cases., Materials and Methods: At week 22 of gestation a woman underwent prognostic counseling due to the diagnosis of fetal bladder exstrophy made by prenatal ultrasound. After counseling the parents elected pregnancy termination. In addition, literature data were analyzed in regard to quality of life and bladder exstrophy., Results: We believe that the rarity of the prenatal diagnosis of this abnormality has resulted in the lack of a comprehensive review of necessary counseling criteria., Conclusions: We suggest what we consider to be a valid counseling approach for parents after a prenatal diagnosis of bladder exstrophy.

Purpose: Surgical access to the posterior urethra is often difficult and several surgical solutions have been proposed. We suggest an anterior sagittal transanorectal approach based on splitting the anterior rectal wall only. This alternative technique provides excellent exposure to the retrourethral region, permitting simple and safe surgery., Materials and Methods: Between 1994 and 1996 we performed surgery via the anterior sagittal transanorectal approach in 8 patients with a mean age of 9.06 years. Patients included 1 girl with a posttraumatic urethrovaginal fistula, 3 with intersex disorders (2 with mixed gonadal dysgenesis raised as boys and 1 with male dysgenetic pseudohermaphroditism with an enlarged urtricle) and 4 boys (1 with penile agenesis raised as girl, 2 with urethral duplication and 1 with prostatic rhabdomyosarcoma). The patient was placed in a knee-chest position. A midline sagittal incision was made through the anterior anorectal wall only and deepened through the perineal body to expose the posterior urethra and retrovesical space. After the pathological condition was corrected the anterior rectal wall and perineal body were reconstructed. The operation was completed with protective colostomy. In our final patient with prostatic rhabdomyosarcoma the anterior sagittal transanorectal approach was used without colostomy. Anorectal manometry was done 6 months postoperatively., Results: All patients were completely continent of stool and urine. Convalescence was unremarkable in all cases. Postoperative manometry in 7 patients revealed no differences from preoperative measurements., Conclusions: This procedure should be considered a useful alternative to other techniques for various congenital and acquired pelvic disorders.

In the female adrenogenital syndrome, the treatment of the urogenital sinus with high implanted vagina still presents a surgical challenge. The conventional technique (perineal vaginal pull-through) has been plagued by a high incidence of vaginal stenosis. A posterior sagittal transanorectal approach was proposed as an alternative to obtain an excellent exposure and thus an adequate mobilization of the vagina. But bivalving a normal rectum and anus has the potential for compromising bowel control and represents a bold maneuver, and the rectum and sphincteric mechanism must be meticulously reconstructed. In the original procedure a protective colostomy must be performed before the operation. To reduce these disadvantages, the authors made the following modifications: sagittal incision of only the anterior rectal wall (Anterior Sagittal Transanorectal Approach--ASTRA) and protective colostomy at the same time as the operation. In this way, maintaining the same excellent exposure and reducing the number of operations from three to two, we operated on 10 girls with adrenogenital syndrome: 4 with high, 3 with intermediate urogenital sinus, and 3 who had previously undergone vaginal pull-through but experienced a vaginal retraction with severe stenosis. At the time of surgery four patients were under 1 year (mean, 9.25 months) and six were from 2 to 11 years of age (mean, 6.5 years). After closing the colostomy, all patients were continent in stools and urine, and the vagina looked normal. The authors suggest using this modified approach as an alternative to the conventional operation and for those patients in whom other techniques have failed.

Neoplastic invasion of the inferior vena cava due to renal tumors (especially Wilms' tumor) is uncommon in children. The tumor thrombus, according to the aggressiveness of the original neoplasm, can extend in diverse ways, obliterate the vascular lumen completely, and even reach the right atrium. The luminal thrombus might be accompanied by the involvement of the caval wall, which requires wide vascular resection. The purpose of this paper is to present our experience with 7 children, aged 18 months and 6 years, affected by caval invasion due to Wilms' tumor. Furthermore, the diagnostic techniques and the surgical treatment in simple caval thrombosis and in associated invasion of the caval wall are described.