Joaquim Murray Bustorff-Silva

Adrenocortical tumors in Brazilian children : Immunohistochemical markers and prognostic factors

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.

Laparoscopic upper-pole nephroureterectomy in infants

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.

WILMS TUMOR IN A PRENATALLY DIAGNOSED MULTICYSTIC KIDNEY

Although conservative treatment of multicystic dysplastic kidney is generally accepted, there are reports of complications when such a kidney is retained, particularly malignant degeneration.1.2 We report on a neonate with Wilms tumor in a prenatally diagnosed multicystic dysplastic kidney. To our knowledge this is the fifth case of Wilms tumor arising in a multicystic dysplastic kidney but only the second diagnosed prenatally.

Epignathus: Report of a case with successful outcome

Epignathus is an extremely rare form of teratoma that arises from the palate or pharynx in the region of the basisphenoid (Rathkes pouch). This condition is associated with a high mortality rate caused by severe airway obstruction in the neonatal period, thus requiring prenatal planning and prompt surgical treatment after birth. The authors describe a case of a giant epignathus that was successfully resected followed by an uneventful recovery.

Evolution of critically ill patients with gastroschisis from three tertiary centers

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.

Cross-sectional study comparing different therapeutic modalities for cystic lymphangiomas in children.

OBJECTIVE: Here, we describe our experience with different therapeutic modalities used to treat cystic lymphangiomas in children in our hospital, including single therapy with OK-432, bleomycin and surgery, and a combination of the three modalities. METHODS: We performed a retrospective, cross-sectional study including patients treated from 1998 to 2011. The effects on macrocystic lymphangiomas and adverse reactions were evaluated. Twenty-nine children with cystic lymphangiomas without any previous treatment were included. Under general anesthesia, patients given sclerosing agents underwent puncture of the lesion (guided by ultrasound when necessary) and complete aspiration of the intralesional liquid. The patients were evaluated with ultrasound and clinical examinations for a maximum follow-up time of 4 years. RESULTS: The proportions of patients considered cured after the first therapeutic approach were 44% in the surgery group, 29% in the bleomycin group and 31% in the OK-432 group. These proportions were not significantly different. Sequential treatment increased the rates of curative results to 71%, 74% and 44%, respectively, after the final treatment, which in our case was approximately 1.5 applications per patient. CONCLUSION: The results of this study indicate that most patients with cystic lymphangiomas do not show complete resolution after the initial therapy, regardless of whether the therapy is surgical or involves the use of sclerosing agents. To achieve complete resolution of the lesions, either multiple operations or a combination of surgery and sclerotherapy must be used and should be tailored to the characteristics of each patient.

Does staged closure have a worse prognosis in gastroschisis

INTRODUCTION: Correction of gastroschisis can be accomplished by primary or staged closure. There is, however, no consensus regarding the best approach or criteria to favor one method over the other has been established. OBJECTIVE: To compare the outcome of primary and staged closure in newborns with gastroschisis using intravesical pressure (IVP) as the decision criterion. PATIENTS & METHODS: We prospectively analyzed 45 newborns with gastroschisis. An IVP with a threshold of 20 cm H2O was used to indicate primary or staged closure, and the outcomes between the two methods were compared. RESULTS AND DISCUSSION: Newborns in whom primary closure was feasible were born at a lower gestational age. There was no significant difference in the frequency of complications, time to begin oral feeding, length of parenteral nutrition or length of hospital stay. Compared with previous reports, our data showed higher rates of prenatal diagnosis and cesarean delivery, a lower average birth weight, a higher rate of small gestational age babies and a more frequent association with intestinal atresia. Conversely, our data showed a lower rate of postoperative necrotizing enterocolitis and a lower average length of hospital stay. CONCLUSION: No significant difference was observed in the outcome of newborns who underwent primary closure or staged closure of gastroschisis when using an IVP below 20 cm H2O as the criterion for primary closure.

Effect of fundoplication on the gastric emptying of liquids

To evaluate the effect of fundoplication on the gastric emptying (GE) of liquids, the authors studied 96 male Wistar rats divided into three main groups: group E (early postoperative), formed by 32 rats that received physiological saline as a test meal and whose gastric emptying was evaluated 8 days after surgery; group L (late postoperative), which received the same test meal but was evaluated 29 days after surgery; and group G (glucose), which received 5% glucose in water and was studied 8 days after surgery. Each group was subdivided in two subgroups of 16 animals: in one (atropine), the animals received intravenous (I.V.) atropine sulfate (0.3 mg/100 mg rat weight) 60 minutes before GE test; the other subgroup (controls) received I.V. physiological saline. In both subgroups 8 animals had been submitted to fundoplication and 8 to sham operation. Every test meal, containing 6 mg% red phenol, was infused by gravity through a metallic catheter. Gastric retention was determined by measuring the concentration of the marker in the liquid recovered from the stomach 10 minutes after infusion. In the animals of group E, fundoplication increased the gastric emptying of physiological saline, both in the control and the atropine subgroups. In the L group, gastric retention values were similar in fundoplication and sham-operated rats, suggesting an adaptation of the stomach to the fundoplication. In the G group, fundoplication enhanced GE among the control animals, but not among those receiving I.V. atropine sulfate. These results support the importance of gastric emptying studies in every patient to be submitted to fundoplication.

Monitoring intravesical pressure during gastroschisis closure. Does it help to decide between delayed primary or staged closure

Introduction and objective: Correction of gastroschisis may be accomplished by either primary or staged closure or even delayed primary closure after the use of a preformed silo. However, there is neither a consensus on the best approach nor established criteria to favor one method over the other. The aim of this paper was to investigate the role of intravesical pressure (IVP) as a tool to prevent abdominal compartment syndrome in newborns undergoing correction of abdominal wall defects. Methods: We retrospectively analyzed 45 newborns with gastroschisis in whom trans-operative intravesical pressure was used to choose between primary or staged closure. A threshold of 20 cm H2O was used and the outcomes between the two methods were compared. Results: In 24 children delayed primary closure was achieved while the remaining 21 underwent staged reduction and closure. There was no difference in the frequency of complications, time to begin oral feeding, length of parenteral nutrition or length of hospital stay between the children of the two groups. The incidence of temporary oliguria or anuria, averaged 33% and it was similar in both groups of children. Conclusion: The data here presented suggests that monitoring intraoperative IVP during correction of gastroschisis may help to select children in whom staged closure is necessary, keeping their complication rate and overall outcome similar to that of children undergoing delayed primary closure. Further prospective studies should investigate more deeply the correlation between type of closure and the development of a compartment syndrome.

Mesenchymal stromal cells from adipose tissue attached to suture material enhance the closure of enterocutaneous fistulas in a rat model

BACKGROUND AIMS Surgical treatment for enterocutaneous fistulas (EF) frequently fails. Cell therapy may represent a new approach to treatment. Mesenchymal stromal cells (MSCs) have high proliferative and differentiation capacity. This study aimed to investigate whether MSCs could adhere to suture filament (SF), promoting better EF healing. METHODS MSCs, 1 × 10(6), from adipose tissue (ATMSCs) were adhered to a Polyvicryl SF by adding a specific fibrin glue formulation. Adhesion was confirmed by confocal and scanning electron microscopy (SEM). A cecal fistula was created in 22 Wistar rats by incising the cecum and suturing the opening to the surgical wound subcutaneously with four separate stitches. The animals were randomly allocated to three groups: control (CG)-five animals, EF performed; injection (IG)-eight animals 1 × 10(6) ATMSCs injected around EF borders; and suture filament (SG): nine animals, sutured with 1 × 10(6) ATMSCs attached to the filaments with fibrin glue. Fistulas were photographed on the operation day and every 3 days until the 21st day and analyzed by two observers using ImageJ Software. RESULTS Confocal and SEM results demonstrated ATMSCs adhered to SF (ATMSCs-SF). The average reduction size of the fistula area at 21st day was greater for the SG group (90.34%, P < 0.05) than the IG (71.80%) and CG (46.54%) groups. CONCLUSIONS ATMSCs adhered to SF maintain viability and proliferative capacity. EF submitted to ATMSCs-SF procedure showed greater recovery and healing. This approach might be a new and effective tool for EF treatment.