Childhood ACTs occur predominantly in females and almost always causes clinical signs. Complete resection is required for cure. Residual or metastatic disease carries a poor prognosis. Our results demonstrate the feasibility of a disease-specific database for obtaining meaningful clinical and outcome information.

Chondromatous hamartoma of the chest wall is an extremely rare, benign lesion that usually occurs in early infancy. It typically arises in the rib cage and produces a large mass. It is composed of a varying admixture of hyaline cartilage that has features resembling growth plate cartilage, fascicles of spindle cells, woven bone, and hemorrhagic cysts. Treatment consists mainly of surgical excision, which is usually curative. We present 3 new cases, which demonstrated interesting findings, including multicentricity, involvement of the sternum, and local recurrence, and we discuss these findings in the context of a literature review.

OBJECTIVES AND INTRODUCTION:Gastroschisis is a congenital abdominal wall defect with increasing occurrence worldwide over the past 20–30 years. Our aim was to analyze the morbidity of newborns after gastroschisis closure, with emphasis on metabolic and hydroelectrolyte disturbances in patients at three tertiary university centers.METHODS:From January 2003 to June 2009, the following patient data were collected retrospectively: (A) Background maternal and neonatal data: maternal age, prenatal diagnosis, type of delivery, Apgar scores, birth weight, gestational age and sex; (B) Surgical modalities: primary or staged closure; and (C) Hospital course: levels of serum sodium and levels of serum albumin in the two first postoperative days, number of ventilation days, other postoperative variables and survival. Statistical analyses were used to examine the associations between some variables.RESULTS:163 newborns were included in the study. Primary closure of the abdominal defect was performed in 111 cases (68.1%). The mean serum sodium level was 127.4±6.7 mEq/L, and the mean serum albumin level was 2.35±0.5 g/dL. Among the correlations between variables, it was verified that hyponatremia and hypoalbuminemia correlated with the number of days on the ventilator but not with the number of days on total parenteral nutrition (TPN); mortality rate correlated with infection. The final survival rate was 85.9%.CONCLUSION:In newborns with gastroschisis, more aggressive attention to hyponatremia and hypoalbuminemia would improve the outcome.

The sacrococcygeal teratoma (SCT) is the most frequent tumor in the neonatal period. Alpha-fetoprotein (AFP) levels fall after tumoral resection, although the time required for level normalization has not been established. The authors evaluate the time required for AFP levels to reach normal levels in 6 infants with prenatal diagnosis of SCT who underwent surgery for resection. The mean time required for AFP level normalization was 9 months. All patients were alive and 1 had neurogenic bladder. AFP has a progressive decrease, which may last 9 months in average and should not be confused with tumoral relapse.

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Objective: Report the results of laparoscopic upper-pole nephroureterectomy in infants. Materials and Methods: Six consecutive infants underwent 7 laparoscopic upper-pole nephroureterectomy. Pre and postoperative evaluation included renal sonography, voiding cystourethrogram and renal scintigraphy. All infants showed upper-pole exclusion. Surgery was performed through a transperitoneal approach with full flank position in all infants. Three or 4 ports were used according to the necessity of retracting the liver. The distal ureter was ligated close to the bladder whenever reflux was present and the dysplastic upper-pole was divided with the help of an electrocautery. Data regarding operative time, postoperative use of analgesics, time to resume oral feeding, hospital stay and tubular function were collected and analyzed. Results: All procedures were concluded as planned. Mean operative time was 135 min. One patient underwent staged bilateral upper-pole nephrectomy. There were no complications and the postoperative hospital stay was 48 hours in 5 procedures and 24 hours in 2 procedures. Pain medication was required only in the first day. Renal tubular function showed improvement in half of the cases. Conclusion: Laparoscopic partial nephrectomy is a safe and feasible procedure in infants. Due to the magnification provided by the lenses, a better vision of the structures is achieved, facilitating selective dissection of vascular upper-pole, renal parenchyma and distal ureter. This approach is less damaging to the lower pole, and is associated to low morbidity and a short hospital stay.

Intraoperative measurement of ureteral opening pressure may help to confirm that the vascular hitch procedure has relieved the pelvic obstruction, precluding the need for dismembered procedures. We believe that in some doubtful cases, with the addition of intraoperative pelvic pressure measurement, vascular hitch may be considered a safe procedure to treat selected cases of PUJO in children.

Context.—The behavior of adrenocortical tumors (ACTs) is usually difficult to establish in childhood, and the role of immunomarkers in predicting outcome has not yet been elucidated. Objective.—To investigate the relationship between clinical, pathologic, and immunohistochemical findings and prognosis in a series of children with ACTs. Patients and Methods.—Clinical data were evaluated retrospectively in 33 children with ACTs, including age at diagnosis, sex, time between first symptoms and diagnosis, clinical signs and symptoms, tumor position, and follow-up. Histologic sections were reviewed, each tumor was classified, and staging was performed according to previously published criteria. Immunohistochemical analysis of p53, Ki-67, c-Erb-B2, and Bcl-2 was performed according to previously published techniques. Results.—Sixty-four percent (n = 21) of the patients were female, and the age at diagnosis in the cohort ranged from 2 to 96 months. Virilization alone affected 70% (n = 23) of the patients, and 18 patients had stage 1 disease, 9 had stage 2 disease, and 3 each had stage 3 and stage 4 disease. Female sex and stage 1 and stage 2 disease were associated with good outcome. None of the histopathologic criteria evaluated correctly predicted outcome. Only tumors with a volume exceeding 200 mL were associated with malignant behavior. Because only a small number of tumors expressed the antigens, results of these immunohistochemical tests were considered inconclusive. Conclusion.—In this sample of pediatric ACTs, the clinical and surgical parameters are the most important prognostic factors, while the immunohistochemical markers evaluated were not predictive of outcome.