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V Jasonni

Publications and source records attributed to V Jasonni.

At least 37 records · Page 2Linked to original sources
PubMed2002

Vesicoureteral reflux and ureteropelvic junction obstruction in children with horseshoe kidney: treatment and outcome.

PURPOSE: Horseshoe kidney is the most common renal fusion anomaly. We determined the treatment and outcome of vesicoureteral reflux and ureteropelvic junction obstruction in children with horseshoe kidney. MATERIALS AND METHODS: We reviewed the medical and radiological records of 52 consecutive children, including 32 boys and 20 girls, in whom horseshoe kidney was diagnosed at 2 children's hospitals during 1990 to 1999. Patient age at diagnosis was 1 day to 12 years (mean 3.9 years). In 2 children with horseshoe kidney neuropathic bladder was secondary to spina bifida and they were excluded from study. The diagnosis was made in all cases by abdominal ultrasound and confirmed by excretory urography or (99m)technetium-dimercaptosuccinic acid scan. Voiding cystourethrography was performed in 40 cases (80%). Patients were followed for 2 to 11 years (mean 4.2). RESULTS: Associated urological anomalies were identified in 26 patients (52%) with horseshoe kidney, including primary vesicoureteral reflux in 13, ureteropelvic junction obstruction in 12 and ectopic ureter in 1. Surgical intervention to correct the anomalies in 15 of the 26 children (58%) involved pyeloplasty in 8, ureteral reimplantation in 2, endoscopic treatment for vesicoureteral reflux in 2, ureterolithotomy in 1, upper pole heminephrectomy in 1 and valve fulguration in 1. No significant complications were observed in surgically treated patients. CONCLUSIONS: More than half of the patients with a clinically symptomatic horseshoe kidney have vesicoureteral reflux or ureteropelvic junction obstruction. Many patients with horseshoe kidney require surgical intervention for associated urological anomalies with good results.

Child↗
PubMed2001

Sacral development in anorectal malformations and in normal population.

BACKGROUND: The sacral ratio (SR) was proposed by Alberto Peña in 1995 as a reliable tool to evaluate sacral development in anorectal malformations (ARM). The SR is obtained by comparing sacrum size with fixed bony parameters of the pelvis. In previous studies, the average normal SR was calculated to be 0.74 for the anteroposterior view (APSR) and 0.77 for the lateral view (LSR). OBJECTIVES: (a) To evaluate the range of variability of the SR in a normal population with age as a possible variable factor. (b) To evaluate the reliability of the SR as a prognostic factor in patients with ARM. MATERIALS AND METHODS: We studied pelvic radiographs of 147 normal children in order to calculate a normal range of values for the SR. In 59 children with ARM, clinical parameters of faecal and urinary continence, spinal cord anomalies (as detected by MRI) and rectal innervation patterns (studied by enzymo-histochemistry) were correlated with SR values by Snedecor's 'F' test and Bravais-Pearson's 'r' correlation index. In 25 cases (15 children with ARM and 10 controls), multiple measurements of SR were obtained at different ages. RESULTS: Normal values for APSR ranged from 0.52 to 1.12, with an average of 0.74. Normal values for LSR ranged from 0.52 to 1.18, with an average of 0.75. Low SR values (below 0.52) correlated well with spinal cord anomalies and with unfavourable functional prognosis in children with ARM. APSR and LSR values increased with age in the normal population, while they decreased in patients with ARM. CONCLUSIONS: The SR has a wide range of normal values, and should be considered truly pathological when lower than 0.52, in either the AP or lateral views. Age has a significant influence on SR values.

Adolescent↗
PubMed2001

Medium-term results after cholecystectomy in patients younger than 10 years.

BACKGROUND: Gallbladder stones are a well-known and widely studied problem in children. Hematological disorders are the most common diseases that can cause cholelithiasis. However, in the last few years, the proportion of children with idiopathic cholelithiasis has increased 50%. Herein, we present a prospective study on laparoscopic cholecystectomy in a selected group of patients aged < 10 years. METHODS: Fifty-eight patients aged < 10 years underwent laparoscopic cholecystectomy for stones in the period 1992-99. The female/male ratio was 1.5, the mean age was 8 years (range, 2-10), and the mean weight was 30 kg. In all patients, parenteral nutrition, fasting state, and prolonged use of antibiotics had been suspended for > or = 6 months, and conservative treatment had been tried for > or = 12 months in the absence of symptoms of stone migration. All the patients were followed up after surgery: clinically at 1,6,12, and 36 months and by ultrasound at 1, 12, and 36 months. Liver function and hematological tests were performed in case of symptoms or if hemolytic disorders were the cause of stones. RESULTS: The mean operative time was 63 min (range, 30-120) in children undergoing cholecystectomy alone and 150 min in children undergoing associated splenectomy. There were no major complications or reoperations. Minor complications included bleeding from accessory cystic artery (n = 3) and insufflation of the omentum (n = 2). One case was converted to an open procedure due to technical problems. All the children were followed up and no complications were observed. CONCLUSION: We consider the laparoscopic approach the gold standard for cholecystectomy in children. This procedure does not have a complication rate any higher than open cholecystectomy, and patient follow-up is as good as that of open surgery. Previous abdominal surgery is not a contraindication to laparoscopy.

Child↗
PubMed2001

Solo-RBT: a new instrument for rectal suction biopsies in the diagnosis of Hirschsprung's disease.

BACKGROUND/PURPOSE: Preoperative histochemistry on rectal mucosal-submucosal specimens is the most important step in the diagnosis of Hirschsprung's disease and other dysganglionoses. Today, rectal mucosal-submucosal biopsy specimens are obtained by suction with the widely used tool first designed by Noblett in the late 1960s. The authors developed a new instrument, for which a patent has already been filed, for one-hand execution of rectal suction biopsies, which will make this operation easier and faster. METHODS: The authors modified the old tool but maintained the cutting method described by Noblett. The authors' innovative automatic tool (Solo-RBT) is based on the possibility of sequencing suction and cutting of the rectal mucosa just by pressing a "trigger." The trigger activates a plunger to obtain the vacuum necessary for the suction and a cutter for mucosal sampling. To perform a correct biopsy in children with different weight and in adults, Solo-RBT can be adjusted suitably to obtain automatically the vacuum required in each case. Additionally, this tool is provided with 2 different sizes of both capsules and cutters, and it can be disassembled completely for easy cleaning, maintenance, and sterilization. RESULTS: The instrument was tested clinically on 62 children (189 biopsies), and it proved to be versatile, atraumatic, and extremely handy, because it can be used by just 1 physician who only needs to press the trigger to obtain the biopsy. CONCLUSIONS: Solo-RBT not only maintains the benefits (atraumatic, easy to use, rapid) and the main characteristic (cutting system) of Noblett's tool but it also improves its features and eliminates most of its disadvantages. Therefore, Solo-RBT represents a significant improvement in the diagnosis of Hirschsprung's disease and other dysganglionoses.

Biopsy, Needle↗
PubMed2001

Pleuropulmonary blastoma.

We report a rare case of pleuropulmonary blastoma arising in a 3-year-old boy and involving the middle and upper lobes of the right lung. Radical resection of the mass was achieved by a bilobectomy. Microscopic examination of the histologic sections showed the typical findings of blastemal and mesenchymal areas with focal zones of rhabdomyoblastic and liposarcomatous differentiation. Monthly cycles of chemotherapy consisting of ifosfamide, vincristine and actinomycin D were given for 10 months after surgical resection. Our patient is presently alive and disease-free two years after diagnosis. As complete tumour ablation is essential to prevent local recurrence and allow any chance of survival, the favourable outcome of our patient is probably due to the radical resection of the neoplasm.

Antineoplastic Agents↗
PubMed2001

A new rapid acetylcholinesterase histochemical method for the intraoperative diagnosis of Hirschsprung's disease and intestinal neuronal dysplasia.

The most commonly used acetylcholinesterase (AChE) method for the diagnosis of Hirschsprung's disease (HD) and intestinal neuronal dysplasia (IND) was first introduced in 1964 by Morris Karnovsky and Logan Roots. This technique requires about 80 - 120 minutes incubation time and cannot be used for the intraoperative diagnosis of HD and IND. To avoid these limitations, in 1994 Kobayashi et al first proposed an accelerated modified method in two different versions, the first using diaminobenzydine (DAB) reagent, the second using 4-chloro-1-naphthol as final reagent. In the present study, we propose a new rapid variation of AChE staining which avoids the use of DAB and naphthol, notably toxic reagents, but follows the same acceleration principle of Kobayashi's technique. Our modified rapid AChE requires a total incubation time of only 8 minutes, which is compatible with intraoperative histochemical examination purposes. Intraoperative seromuscular or full-thickness intestinal biopsies were obtained from 92 children affected by intestinal dysganglionoses. The biopsies were frozen and cut in 15 microm cryostatic sections. Rapid AChE was performed with a special incubation medium using 3-amino-9 ethylcarbazole (AEC) as chromogenic substance. The two complementary histochemical techniques alpha-naphthylesterase (ANE) and lactate-dehydrogenase (LDH) were also used intraoperatively for the staining of ganglion cells. The diagnosis was confirmed postoperatively with conventional AChE Karnovsky technique, comparing the extensions of hyperganglionic, hypoganglionic and aganglionic segments in each studied case. The new rapid AChE modified method can identify ganglion cells and fibers using a dark brown precipitate. In all the cases studied, the intestinal innervation pattern identified with this modified technique was similar to that obtained with Karnovsky AchE. Seventy-eight HD, 8 isolated IND and 6 HD associated with an evident IND segment were diagnosed. This new rapid AChE histochemical technique avoids the use of DAB and naphthol, and can thus be considered safe for operators. Rapid AChE is a valid tool for both the evaluation of aganglionosis extension and for the identification of IND pattern during surgery. We recommend this very reliable method for the intraoperative diagnosis of HD and IND, in association with other enzymatic markers of ganglion cells (ANE or LDH). We propose the following diagnostic protocols: a) for preoperative histochemical study: conventional AChE plus LDH and NADPH-diaphorase; b) for intraoperative study: rapid AChE plus ANE.

Acetylcholinesterase↗
PubMed2000

Features and outcome of neuroblastoma detected before birth.

BACKGROUND/PURPOSE: The growing use of routine ultrasonography during pregnancy is leading to an increasing number of prenatally diagnosed neuroblastomas. Optimal strategy has not yet been defined for these patients, because knowledge on this particular neuroblastoma (NB) population is still limited. However, definite guidelines are needed to avoid inadequate treatment. The authors analyzed the cases of antenatally detected NB (ADNB) reported in the Italian Neuroblastoma Registry during the past 6 years to elucidate the features of this subset of NB. METHODS: The Italian Neuroblastoma Registry was reviewed for the period January 1993 to December 1998 to collect clinical, radiographic, surgical, and histopathological data on ADNB cases. NB stage was evaluated according to INSS criteria. All patients had undergone imaging (computed tomography or magnetic resonance imaging) of the primary tumor and bone marrow biopsy before surgical resection. RESULTS: Seventeen patients were identified. Primary tumour site was adrenal glands in 16 cases and retroperitoneal ganglia in 1. Stage distribution was stage I, 13 cases; stage II-A, 1 case; stage II-B, 1 case; stage IV-S, 2 cases. All cases underwent primary tumour resection. Mean age at surgery was 4 weeks. Resection of primary tumor was radical in 16 cases, partial in 1. All tumors were characterised by favourable histology according to Shimada classification. N-myc gene amplification was studied in 14 patients. N-myc amplification was detected only in a newborn with stage II-A NB, who died of massive bleeding 2 days after tumor resection. DNA index and 1p deletion were studied in 11 and 8 patients, respectively. Both diploidy and deletion of 1p were observed in a newborn who subsequently died of disease progression despite surgery, chemotherapy, and radiation therapy. Fourteen of 17 patients currently are alive and free of disease, and one with IV-S NB and short follow-up is alive with disease. CONCLUSIONS: Our data give evidence that in most cases infants with ADNB represent a subset of patients with excellent outcome. Aggressive treatment may not always be necessary. Infants with ADNB with unfavorable features should undergo early surgical excision, whereas patients with favourable features could be observed awaiting spontaneous regression of the mass, reserving delayed surgery for tumors that increase in size or do not regress.

Adrenal Gland Neoplasms↗
PubMed2000

Pathogenesis of Hirschsprung's disease.

Hirschsprung's disease is an inherited disorder showing incomplete penetrance and variable expressivity. Genetic mapping and mutation screening of candidate genes, together with the study of several natural and knockout animal models, clearly have shown the involvement of several different genes in the pathogenesis of Hirschsprung's disease. Among these genes, the RET proto-oncogene accounts for the highest proportion of both familial and sporadic cases, with a wide range of mutations scattered along its entire coding region. The low detection rate of RET mutations in Hirschsprung patients also led to different hypotheses, such as the existence of additional Hirschsprung genes. Different animal and human genetic studies have identified 6 Hirschsprung genes: RET proto-oncogene (RET), endothelin 3 (EDN3), endothelin B receptor gene (EDNRB), glial-cell-line-derived neurotrophic factor (GDNF), endothelin converting enzyme (ECE1), gene encoding the Sry-related transcription factor SOX10 (SOX10). Microenvironmental factors also can play a role in the pathogenesis of aganglionosis. The developmental process of the crest-derived progenitor cells is sensitive to the level of different molecules. The expression deficit of different factors (GDNF, NTN) in the hindgut, in the absence of genetic mutations, could determine a missed activation of the receptor system, causing enteric neuroblast migration arrest.

Child↗
PubMed2000

[Intractable diarrhea and neuroblastoma: report of a clinical case].

One of the typical presentations of neuroblastoma is intractable diarrhea or wdha (watery diarrhea, hypokalemia, achloridria). The case admitted to our Pediatric Surgery Department presented watery diarrhea due to VIP hyperincretion caused by a stage 1 neuroblastoma, whose ablation allowed a complete resolution of the clinical conditions. This case report can be useful in the discussion of the differential diagnosis of the most common clinical pictures.

Adrenal Gland Neoplasms↗
PubMed1999

Exclusion of the Sonic Hedgehog gene as responsible for Currarino syndrome and anorectal malformations with sacral hypodevelopment.

Anorectal malformations (ARMs) are common congenital anomalies that account for 1:4 digestive malformations. ARM patients show different degrees of sacral hypodevelopment while the hemisacrum is characteristic of the Currarino syndrome (CS). Cases of CS present an association of ARM, hemisacrum and presacral mass. A gene responsible for CS has recently been mapped in 7q36. Among the genes localized in this critical region, sonic hedgehog (SHH) was thought to represent a candidate gene for CS as well as for ARM with different levels of sacral hypodevelopment according to its role in the differentiation of midline mesoderm. By linkage analysis we confirmed the critical region in one large family with recurrence of CS. In addition, the screening of SHH in 7 CS and in 15 sporadic ARM patients with sacral hypodevelopment allowed us to exclude its role in the pathogenesis of these disorders.

Anal Canal↗
PubMed1999

Pancreatitis caused by duodenal duplication.

The authors present the investigations and surgical treatment of two cases of duodenal cystic duplication. Abdominal pain and gastroesophageal reflux were the most important symptoms and signs associated with an history of recurrent acute pancreatitis. Computed tomography scan, ultrasound examination, and cholangiography confirmed preoperatively the diagnosis, and a transduodenal surgical approach was carried out in both children. A simple marsupialization of the cyst was performed in the former, and a sphincterotomy with papillosphincteroplasty was associated in the latter. The diagnosis was confirmed by microscopy, and both the children are asymptomatic after a 14 and 18 months of follow-up. This report focuses on the importance of the cholangiopancreatography for every child presenting with recurrent, unexplained bouts of acute pancreatitis, and underlines the technical surgical aspects on the basis of the anatomic identification of the malformation.

Acute Disease↗
PubMed1999

Treatment of vesico-ureteric reflux in children with neuropathic bladder: a comparison of surgical and endoscopic correction.

BACKGROUND/PURPOSE: Vesico-ureteric reflux (VUR) is a common problem in children with neuropathic bladder. Lesser-degree VUR may be manageable by intermittent catheterization or by anticholinergics, but higher grades usually require surgical treatment. If left untreated, two thirds of such patients may experience deterioration of the upper renal tracts. The aim of this study was to compare the results of the STING (Subureteric Teflon Injection) technique with surgical ureteric reimplantation as treatment for VUR in neuropathic bladder. METHODS: From January 1981 to December 1996, 58 children with NB (81 ureters) were treated for VUR. STING and Cohen ureteroneocystotomy were performed in 40 and 41 ureters, respectively. Mean age was 4.5 years (STING) and 5.1 years (Cohen). RESULTS: Twenty-nine of 40 refluxing ureters (72.5%) were cured by STING, whereas Cohen eradicated reflux in 39 of 41 ureters (95.5%). No complications were observed in either group. All the ureters in which STING failed were treated successfully by Cohen ureteroneocystostomy. The 2 ureters still refluxing after surgical reimplantation were cured successfully by a single STING. The mean follow-up was 6.8 years in the Cohen group and 4.8 years in the STING group. During follow-up, no recurrence was observed in patients cured by open reimplantation. In the STING group, 2 previously cured ureters showed recurrence of VUR: both were treated successfully by a further STING. CONCLUSIONS: Open ureteral reimplantation is more effective than STING in correcting VUR in children with neuropathic bladder dysfunction. Nevertheless, the good success rate, the relative technical simplicity, outpatient nature, and rapid recovery point to STING as a safe and effective procedure for the initial treatment of VUR. Failure of STING does not preclude a successful open operation.

Adolescent↗
PubMed1999

Preoperative enzymo-histochemical diagnosis of dysganglionoses associated with anorectal malformations (ARM) with recto-vestibular and recto-perineal fistula.

The posterior sagittal anorectoplasty (PSARP) is widely recognized as the best technique available today for the surgical treatment of anorectal malformations (ARM). However, different retrospective studies on the functional results of PSARP in the treatment of ARM have shown different postoperative degrees of constipation. In particular, even in patients with normal sacrum, about 70% of operated ARM with vestibular fistula and about 50% with perineal fistula can be complicated by fecal constipation and pseudoincontinence. In order to identify preoperatively whether ARM patients present abnormal innervation patterns of rectal pouch and fistula (as reported by Holschneider et al [7]), we decided to study suction rectal biopsies performed by introducing SBT-100 rectal suction biopsy tool into the fistula at 6, 4, 3 and 2 cm from the meatus. To date, this approach has been adopted in 22 ARM cases (15 females and 7 males, age range 7 days-4 years), 6 of them with recto-vestibular fistula and 13 with recto-perineal fistula. Biopsies were frozen in isopentane at liquid nitrogen temperature and cryostatic sections were studied by acetylcholinesterase (AChE), succinic-dehydrogenase (SDH) and alpha-naphthyl-esterase (ANE) enzymo-histochemical techniques. The results concerning the innervation-type of fistula and proximal rectal pouch were confirmed by the biopsies obtained during PSARP. Our overall incidence of rectal innervation intrinsic disorders was 81.82%. In particular, all our cases of vestibular fistula presented associated dysganglionoses. The incidence of associated Hirschsprung's disease was high, corresponding to 18% of cases. Our results suggest that the high frequency of constipation in low forms of ARMS depends on primary intestinal neuronal malformations and it cannot be ascribed to a denervation secondary to rectal dissection and to PSARP procedure. We propose the introduction of this type of preoperative enzymo-histochemical diagnosis in ARM cases because it can select those patients with severe associated dysganglionoses. In our opinion, if this diagnosis is available preoperatively, PSARP can be performed without using abnormally innervated structures and reducing postoperative functional complications.

Anal Canal↗
PubMed1998

Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation.

A type I congenital cystic adenomatoid malformation (CCAM) in the left lower lobe was removed from a 11-year-old boy with a 3-month history of recurrent pneumonia. As incidental finding, a bronchioloalveolar carcinoma (BAC) was found in the lung parenchyma adjacent to the cyst. A left lower lobectomy was performed. At 18 months after surgery the patient is well and free of neoplastic disease. To the best of our knowledge, this association has not been reported previously in a pediatric patient. Malignancies complicating CCAM are rarely seen, but have been reported in adults. Including our case, eight cases of BAC and five cases of rhabdomyosarcoma (RMS) in association with CCAM have been reported so far. As CCAM can host metaplastic mucous cells, primitive mesenchymal cells and differentiated but poorly organized striated muscle fibers, it has been proposed that CCAM may act as a predisposing condition for oncogenesis. Our experience adds further support that CCAM can act as a premalignant lesion. Previous reports of both BAC and RMS in asymptomatic CCAM suggest prompt resection shortly after diagnosis.

Adenocarcinoma, Bronchiolo-Alveolar↗
PubMed1998

Pancreatitis caused by duodenal duplication.

Two cases of recurrent pancreatitis, due to duodenal duplication, are reported. The aim of this paper is to emphasise the role of endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic cholangiography (PTC) in the detection of associated pancreaticobiliary anomalies and in the planning of the correct surgical approach. The order of imaging in a child with recurrent pancreatitis should be US, barium meal and PTC. ERCP is often difficult to perform in children.

Child↗
PubMed1998

Lung resection in pediatric patients.

An evaluation of all pediatric patients with primary or secondary pulmonary disease operated upon from January 1993 to July 1996 by the same senior surgeon was carried out. The inclusion criterion was a lung resection in patients aged less than 14 years. Children were divided into two categories according to the neoplastic or non-neoplastic nature of their disease. In the first group a lobectomy was performed for primary lesions and wedge resection for secondary ones. In the second group lobar emphysema and cystic dysplasia were the major indications for lobectomy, while diagnostic wedge resections were performed for interstitial/infiltrative lesions. Several groups of techniques were identified according to the type of approach and the suture method. Video-assisted thoracoscopic surgery and a muscle-sparing approach were compared to classic posterolateral thoracotomy. The mechanical stapler-suturing method was compared to the manual suturing. Our results demonstrate the importance of mechanical suturing, particularly in decreasing anesthesia time and reducing the risk of dehiscence. The minimally invasive approach associated with mini-thoracotomy was particularly useful for patients with reduced oxygen saturation due to ventilatory and gas-exchange problems. The roles of staplers in lung parenchymal resection and minimally invasive procedures for improving the postoperative thoracic compliance of pediatric patients are stressed.

Adolescent↗