Ana Catarina Fragoso

Transvesicoscopic Cohen ureteric reimplantation for vesicoureteral reflux in children: A single-centre 5-year experience

PURPOSE To evaluate our results with a new method of intravesical ureteric reimplantation using laparoscopic pneumovesicum in children. MATERIALS AND METHODS Seventy-two patients (mean age 4.2 years, range 0.5-20 years) with primary vesicoureteral reflux (VUR) underwent a laparoscopic transtrigonal ureteric reimplantation with CO(2) pneumovesicum. Ports were inserted suprapubically - 5mm for the camera and two 3-5-mm working ports. Having mobilized the ureter(s) intravesically, a submucosal tunnel is created and ureteric reimplantation performed with 5/0 and 6/0 absorbable sutures. Bladder drainage was maintained for 2-3 days postoperatively. Patients were followed up with clinical assessment and renal ultrasonography+/-voiding cystourethrogram. RESULTS Ninety percent had VUR grade > or =3. A total of 113 ureters were reimplanted. The mean operative time was 82min for unilateral and 130min for bilateral reimplantation. Four cases (6%) were converted. Three patients presented with temporary ureteric dilatation without symptoms on follow-up renal ultrasound. Seven patients had postoperative urinary tract infection without persistent reflux on cystography. Follow-up cystogram was performed in 50 patients (81 ureters). Reflux persisted in four patients (8%). CONCLUSIONS Laparoscopic ureteric reimplantation with CO(2) pneumovesicum is technically feasible with a high success rate (92%). The role of this new technique in the treatment of VUR remains to be determined.

Abdominal inflammatory myofibroblastic tumor a clinicopathologic study with reappraisal of biologic behavior.

BACKGROUND AND PURPOSE Inflammatory myofibroblastic tumor (IMT) is a proliferative lesion of controversial nosology and uncertain prognosis. In an attempt to acquire further understanding of pathogenesis and biologic behavior, we surveyed abdominal IMTs managed over the last 12 years at a single institution. METHODS Intra-abdominal IMTs treated between 1995 and 2007 were reviewed concerning demographic, clinical, and pathologic features as well as therapeutic management and outcome. All specimens were reevaluated by histologic examination and immunohistochemistry. RESULTS There were 7 patients (4 males; age range, 28 days to 14 years). Five lesions were located in alimentary tract: 1 gastric presenting with bleeding, 1 hepatic presenting with a thoracic wall mass, 1 pancreatic and 2 colonic presenting with obstructive symptoms. One splenic IMT was found incidentally. The remaining case arose from the adrenal gland and presented with a palpable mass. The gastric and adrenal IMTs had evidence of a previous or concomitant infectious setting. Five lesions were excised. The pancreatic IMT underwent a drainage procedure followed by steroid administration, and the hepatic lesion received antibiotics. Histopathology revealed characteristic findings of IMT. Expression of anaplastic lymphoma kinase was negative in all cases. At a median follow-up of 6 years (range, 3-15), all children were asymptomatic with no recurrences. The hepatic and pancreatic IMT displayed complete and near total regression, respectively. CONCLUSION A benign behavior of abdominal IMTs was observed even in patients not undergoing surgical excision. Although IMT remains a surgical disease, a conservative approach may be reasonable in select cases.

Minimal access surgery in the management of pediatric urolithiasis

PURPOSE In contrast to adult patients, a relatively large number of open surgical procedures are still needed in the treatment of urolithiasis in children. Since almost all open surgical techniques may be reproduced by minimal access surgery (MAS), there is a rationale to apply the latter in the management of pediatric urolithiasis. Our study aimed to assess the feasibility and outcome of MAS in the treatment of pediatric urinary calculi. MATERIALS AND METHODS The charts of patients with urolithiasis submitted to MAS between 1994 and 2007 were retrospectively reviewed. The inclusion criteria were contraindication for and failure of lithotripsy or endourology techniques. Demographic data, lithiasis characterization (location, dimension, composition), predisposing factors (anatomic or metabolic) and surgical approach (technique and outcome) were evaluated. RESULTS Fifteen consecutive patients (eight girls, seven boys) with a median age of 108 months (range: 10-297) were elected for MAS. Eleven (73%) children had associated urogenital malformations and three (20%) presented metabolic abnormalities. A total of 17 procedures were performed laparoscopically: three nephrolithotomies (one transperitoneal, two by retroperitoneoscopy), four pyelolithotomies (retro), three ureterolithotomies (trans) and seven cystolithotomies (suprapubic approach). Five patients underwent concomitant correction of urological anomalies (three calyceal diverticula, one obstructive megaureter, one ureteropelvic junction obstruction). Complete removal of calculi was accomplished in 14 (82%) procedures. There were two perioperative complications (one intraperitoneal vesical perforation and one perivesical urinoma). At a median follow up of 4 years (range: 1 month to 11 years), four patients have developed recurrence. CONCLUSIONS Minimal access surgery is an effective and safe approach for urolithiasis in children who are not candidates for minimally invasive modalities. Individualized application of different techniques may solve virtually all cases. In selected cases, the role of MAS as first-choice therapy deserves consideration.

The multifactorial origin of respiratory morbidity in patients surviving neonatal repair of esophageal atresia

Esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) occurs in 1 out of every 3000 births. Current survival approaches 95%, and research is therefore focused on morbidity and health-related quality of life issues. Up to 50% of neonates with EA ± TEF have one or more additional malformations including those of the respiratory tract that occur in a relatively high proportion of them and particularly of those with vertebral, anal, cardiac, tracheoesophageal, renal, and limb association. Additionally, a significant proportion of survivors suffer abnormal pulmonary function and chronic respiratory tract disease. The present review summarizes the current knowledge about the nature of these symptoms in patients treated for EA ± TEF, and explores the hypothesis that disturbed development and maturation of the respiratory tract could contribute to their pathogenesis.

Ectopic pancreas and foveolar hyperplasia in a newborn: a unifying etiopathogenesis for gastric outlet obstruction.

Gastric outlet obstruction in infants usually is attributable to infantile hypertrophic pyloric stenosis (IHPS). Rare causes, such as foveolar hyperplasia (FH) (1–3) or ectopic pancreas (4,5), have been reported. In this case we describe a newborn infant with gastric outlet obstruction related to coexisting ectopic pancreas and FH who was being treated with prostaglandin (PG) E1. A unifying etiopathogenesis for gastric outlet obstruction is proposed.

Abnormal Sonic hedgehog signaling in the lung of rats with esophageal atresia induced by adriamycin

Background:Abnormal lung development was recently described in the rat model of esophageal atresia and tracheoesophageal fistula (EA-TEF). Since in this condition the ventral-to-dorsal switch of Shh expression in the foregut is disturbed, the present study tested the hypothesis that this abnormal expression at the emergence of the tracheobronchial bud might be translated into the developing lung.Methods:Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle from E7 to E9. Three groups were studied: control and adriamycin-exposed with and without EA-TEF. Embryos were recovered and the lungs were harvested and processed for reverse transcription polymerase chain reaction and immunofluorescence analysis of the Shh signaling cascade.Results:Shh signaling was downregulated at the late embryonic stage of lung development (E13) in embryos with EA-TEF. Throughout the subsequent stages of development, the expression of both Shh and its downstream components increased significantly and remained upregulated throughout gestation. Immunofluorescent localization was consistent with these findings.Conclusion:Defective Shh signaling environment in the foregut is present beyond the emergence of lung buds and probably impairs lung development. Later in gestation, lungs exhibited a remarkable ability to upregulate the Shh cascade, suggesting a compensatory response. These findings may be relevant to understand pulmonary disease suffered by children with EA-TEF.

Lung maturity in esophageal atresia: Experimental and clinical study.

INTRODUCTION Esophageal atresia and tracheoesophageal fistula (EA-TEF) survivors suffer respiratory morbidity of unclear pathogenesis. Defective lung morphogenesis has been described in the rat model. This study examined fetal lung growth and maturity in rats and patients with EA-TEF. METHODS Pregnant rats received either adriamycin or vehicle. Control and adriamycin-exposed lungs, with and without EA-TEF, were weighed and processed for RT-PCR, DNA quantification, immunofluorescence and immunoblot analysis of TTF1, VEGF, Sp-B, and α-sma. Twenty human lungs were also processed for immunofluorescence and Alcian-blue staining. RESULTS Lungs from fetuses with EA-TEF (E21) showed decreased total DNA; FGF7 and TTF1 mRNA expressions were upregulated at E15 and E18, respectively. Protein expression and immunofluorescent distribution of maturity markers were similar. Lungs from stillborns with EA-TEF showed decreased epithelial expression of Sp-B and VEGF whereas those from newborns tended to have less Sp-B and more VEGF and mucous glands. DISCUSSION The lungs of rats with EA-TEF were hypoplastic but achieved near-normal maturity. Stillborns with EA-TEF exhibited an apparently disturbed differentiation of the airway epithelium. Newborns with EA-TEF demonstrated subtle differences in the expression of differentiation markers, and increased number of mucous glands that could influence postnatal respiratory adaptation and explain some respiratory symptoms of EA-TEF survivors.

Defective upper gastrointestinal function after repair of combined esophageal and duodenal atresia.

BACKGROUND Both esophageal atresia (EA) and duodenal atresia (DA) involve deficient anti-reflux barrier, poor esophageal function and eventually, duodenogastric reflux. This study aims at examining the upper gastrointestinal functional status in a cohort of patients with both EA and DA. METHODS A retrospective survey of patients treated for EA and DA between 1965 and 2012 was conducted. Clinical charts, office visits, imaging, upper gastrointestinal endoscopy and esophageal pH metry/impedance were used to assess the long-term condition of the esophagus, the presence of gastroesophageal reflux disease (GERD) and/or the need for fundoplication. RESULTS Twenty out of 581 patients treated for EA had associated DA. Ten/twenty children survived; 1 had primary esophageal replacement. With a median follow-up of 9 years, 8/9 had complicated outcomes and 5 still suffered digestive ailments: 2 GER; 1 eosinophilic esophagitis; 1 nodular gastritis, and 1 wrap herniation. A total of 10 procedures were performed: 8 fundoplications, 1 esophagogastric dissociation and 1 replacement with colon. DISCUSSION The association of EA with DA involves a poor upper digestive function with high risks of GERD and fundoplication failure. The lifelong synergistic play of esophageal, gastric and duodenal dysfunctions in these patients prompts long-term follow-up, and eventually active treatment.

Complete Ureteral Duplication: Outcome of Different Surgical Approaches.

INTRODUCTION The surgical management of complete ureteral duplication anomalies is not consensual. OBJECTIVE To characterize the pediatric population who underwent surgery for complete ureteral duplication and assess the outcomes of different approaches. MATERIAL AND METHODS Clinical records from patients treated between January 2008 and June 2014 were retrospectively reviewed. Epidemiology, diagnosis, clinical manifestations and surgical procedures were collected and analysed. Ureteral units were divided into two groups: A, with ureterocele; and B, without it. RESULTS Forty-one ureteral units from 32 patients with complete duplication underwent surgery. In group A (n = 18), the selected primary procedure was: ureterocele punction (12); ureter reimplantation (3); pyelopyelostomy (2); heminephrectomy (1). A reintervention was required in 3 of the 12 units submitted to punction: heminephrectomy (1), ureteroureterostomy (1), and ureteric reimplantation (1). In group B (n = 23), STING was performed in 10 units, ureteric reimplantation in 3, pyelopyelostomy in 3, ureteroureterostomy in 1, and heminephrectomy in 6; two cases required reintervention. DISCUSSION A conservative primary approach was favoured in cases with ureterocele and/or reflux in hemisystems worth preserving (53.7%); it was effective per se in 75% (n = 9/12) units in group A and 80% (n = 8/10) in group B. An ablative primary procedure was adopted in 17% (n = 7/41) cases, 5.6% of group A (n = 1/18) and 26.1% of group B (n = 6/23). CONCLUSIONS A conservative approach is effective as a primary and isolated procedure in the majority of cases with ureterocele or vesicoureteral reflux. Further studies are needed to establish the advantages over primary invasive or ablative approaches.

Neonatal splenic necrosis not related to wandering spleen

Splenic necrosis is extremely rare in neonates, the cases recorded so far being secondary to torsion of wandering organs. A newborn with an abdominal mass who underwent exchange transfusions through an umbilical catheter is presented here. Comprehensive investigation led to the suspicion of enteric duplication, but a splenic necrosis with no features of wandering spleen was found at laparotomy. The pathogenesis and preoperative diagnostic work-up of splenic necrosis are emphasised.