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R Bragheri

Publications and source records attributed to R Bragheri.

At least 19 recordsLinked to original sources
PubMed2003

Long-term follow-up in children treated for retroperitoneal malignant tumours.

Thirty-two long-term survivors after treatment with surgery, radiotherapy and chemotherapy for neuroblastoma (NRB) and Wilms' tumour (WT) were re-evaluated after a period of more than 10 years (mean 16 yr 1 mo, range 27 yr 2 mo/10 yr 5 mo). In addition to routine clinical and laboratory studies, all patients underwent auxometry, echocardiography, spinal X-ray, abdominal US and kidney radionuclide scans. Neither obvious physical signs nor cutaneous toxicity were noted at inspection. No dysmenorrhoea or sexual disorders were mentioned. Haematological data turned out to be all in the normal range, except for TIBC/UIBC ratio (out of range in 70 % of cases); gammaGt (62.5 %); A/G ratio (12.5 %); ALT (37.5 %). Auxometrical data were all in the normal range; in particular, results of mean weight and height were just slightly over the 50 degrees centile. Spinal X-rays revealed mild kyphotic deformities (68.9 %), pelvic obliquity and tilting (62.5 %), rotation of the vertebral bodies (34.3 %) and compensatory curves of the spine (21.8 %). No impairment of cardiac functions was noted at echocardiography. No alteration of the hepatic structure was detectable at US scans. Marked impairment of the renal function (20 % and 1.5 % residual function) was detected at radionuclide imaging in 2 NRB patients who had not undergone nephrectomy, with the kidney having almost disappeared in one. In conclusion, the study emphasises the need for careful and prolonged follow-up for the detection of sequelae than can appear even many years after the initial treatment, and the wisdom and the need for a continued search for less aggressive protocols; as long as the cure rate is not compromised, less aggressive protocols should aim for a satisfactory overall survival with a reduced incidence of sequelae and hence a better quality of life.

Adolescent↗
PubMed2001

Bilateral anterior pubic osteotomy in bladder exstrophy repair: report of increasing success.

Bilateral posterior iliac osteotomy is performed in most patients undergoing primary closure of an exstrophic bladder; the aims are to facilitate abdominal-wall closure, prevent postoperative wound dehiscene, and possibly, to achieve better urinary control in older age. A new technique, anterior pelvic osteotomy of the superior pubic ramus, seems to obtain tension-free symphysis approximation safely and quickly. We report our initial experience with this osteotomy. Five neonates, four males and one female from 1 to 4 days old, all underwent closure surgery for bladder exstrophy (BE) and subsequent bilateral osteotomy of the superior pubic ramus (SPRO). Postoperatively, Bryant's traction was applied. Tension-free, complete approximation of the symphysis and uncomplicated healing were achieved in all five cases without palsy of the obturator nerve or postoperative hemorrhage. Follow-up revealed partial rediastasis with a stable anterior pelvic ring. Tension-free closure and immobilization are important factors in both initial and subsequent closure of BE. Several osteotomy techniques are currently in use. SPRO presents numerous advantages, namely, ease and rapidity, minimal blood loss, and no requirement for an extra skin incision or need to turn the patient on the operating table. A certain degree of rediastasis with growth was subsequently observed: although undesirable, this complication is common to all osteotomy techniques. We believe that SPRO is a valid and uncomplicated method to facilitate BE closure.

Bladder Exstrophy↗
PubMed2001

Symptomatic unilateral renal angiomyolipoma in a child with tuberous sclerosis.

We report a case of unilateral renal angiomyolipoma in a 10-year-old boy with tuberous sclerosis. The case is worthy of attention because the symptoms occurred at an early age and because the lesion was unilateral, the latter confirmed at 11-year follow-up. The presentation and treatment are discussed, and the pertinent literature is reviewed. The case demonstrates that it is of clinical importance to monitor renal involvement in children with tuberous sclerosis.

Angiomyolipoma↗
PubMed2000

Abdominoscrotal hydrocele in childhood: is it really a rare entity?

OBJECTIVES: Abdominoscrotal hydrocele (ASH) is an apparently highly uncommon clinical entity especially in childhood, with only about 80 pediatric cases reported in the modern literature. METHODS: The incidence, diagnosis, and treatment of ASH are discussed with reference to 9 cases observed at our institution and to cases in the literature. RESULTS: Surgical correction was successful in all our cases and no hydrocele or hernia recurrence was registered. CONCLUSIONS: ASH incidence appears to be higher than reported; undescended testis is a frequent association and surgical correction through an inguinal approach is always possible and curative. Ligation of the processus vaginalis may prevent recurrence.

Abdomen↗
PubMed2000

Usefulness of ultrasonography in gastric foreign body retention.

Foreign-body ingestion occurs frequently in childhood. The diagnosis of ingestion is usually made by plain film radiography. Ultrasound is not routinely used in the investigation of gastric foreign bodies because of the limitations imposed by intestinal gas. This drawback can be overcome by distending the stomach with an anechoic liquid, which acts as an acoustic window. In a child who had ingested a coin, US performed after drinking tea was diagnostic and showed a hyperechoic image with an acoustic shadow inside the stomach. We believe that US is an appropriate technique for follow-up of gastric foreign bodies.

Child, Preschool↗
PubMed1999

Is bilateral congenital anorchia genetically determined?

Bilateral congenital anorchia (BCA) can be defined as complete absence of testicular tissue in a patient with male normal phenotype and karyotype. On the basis of familial occurrences of BCA a possible genetic aetiology has been hypothesised, i.e. mutations of the SRY gene which initiates the genetic cascade leading to testis development in mammals. The aim of the study is to assess this hypothesis. Eight boys affected by BCA have been studied; a normal monozygotic twin of one of the patients, a boy and a girl acted as controls. A normal 46, XY karyotype was detected in all patients; 3 had hypoplasia of the scrotum and 2 of the penis. Hormonal data were available for 5 patients: Prader's stimulation test to HCG showed in all lack of testosterone response, and 4 out of 5 had elevated FSH and LH levels. Complete absence of testicular tissue was confirmed in all by surgical exploration. DNA was sampled by Jeanpierre modified extraction method and amplification by polymerase chain reaction. The expected segment of 750 basepairs of the SRY gene, included between the two oligonucleotide primers Xes 10 and Xes 11, was found in all patients. SRY gene is present in our BCA patients as well as in normal boys, and therefore BCA does not seem related to an anomaly of the opening reading frame sequence of the SRY gene. Nevertheless, familial occurrences of BCA continue to suggest a genetic aetiology: further studies must therefore evaluate the possibility of punctiform mutations of the SRY gene, by direct sequentiation, and exclude abnormalities in the critical region DSS/AHC of the X chromosome, recently discovered as one of the loci involved in the differentiation of the male gonad.

Case-Control Studies↗
PubMed1999

Lipoblastoma and lipoblastomatosis in children.

Lipoblastoma and lipoblastomatosis are rare mesenchymal benign tumors of embryonal white fat. They are found primarily in children less than 5 years of age. This tumor presents in two forms: a localized well-circumscribed lesion (lipoblastoma), or a multicentric type (lipoblastomatosis). The authors presents three cases, two of them with a buttock lesion, the last with a paravertebral one. It is recommended a complete but conservative excision of the tumor because there is a natural tendency to involution, although in the first year of life a local reexcision for recurrence is sometimes described.

Abdominal Neoplasms↗
PubMed1994

The accuracy of magnetic resonance imaging and ultrasonography compared with surgical findings in the localization of the undescended testis.

OBJECTIVE: To determine the accuracy of ultrasonography (US) vs magnetic resonance imaging (MRI) in the detection of undescended nonpalpable testis. DESIGN AND SETTING: A clinical, radiological, and surgical study was undertaken in boys with undescended nonpalpable testes at Pavia and Milan (Italy) University Hospitals. PARTICIPANTS: Seventeen patients with undescended nonpalpable testes aged 10 months to 14.5 years who were examined between 1989 and 1993. A total of 22 nonpalpable testes were examined using US and MRI techniques. Twenty-one testes were explored surgically. RESULTS: The US located 13 (62%) of 21 testes, 12 of which were found at surgery while one (5%) proved to be false positive. Eight (38%) of 21 evaluations were negative; four (19%) were true-negative results. Of the 12 gonads correctly localized with US, two (17%) were located near the internal inguinal ring while 10 (83%) were within the inguinal canal. The MRI examination located 11 (52%) of 21 gonads, all found at surgery. Of the 10 negative results (48%), five (24%) were false negatives and five agenetic testes (24%) were not found surgically. Of the 11 testes correctly localized using MRI, four were located in the abdomen or just proximal to the internal inguinal ring and seven were in the inguinal canal. Five of the six high testes were correctly localized using US and MRI (two using US and four using MRI). CONCLUSIONS: Neither US nor MRI is currently sensitive enough to stand alone as a screening modality for a nonpalpable testis since the two techniques used separately gave a useful result in 16 (76%) of 21 testes. They appear to be complementary since their combined specificity compared with surgical findings was 95%. Since the localization of an undescended testis is important for planning surgical strategy, US should be used for screening evaluation, and if it is not successful, it should be followed (before any invasive diagnostic procedure) by MRI, which appears to be more reliable in the localization of undescended, high, and "functioning" testes.

Child↗
PubMed1994

Surgical treatment of necrotizing enterocolitis: when? how?

In 10 years (1981-1990) 28 out of 54 neonates (51.8%) with definite necrotizing enterocolitis (NEC) underwent surgery. Operation was performed at 13.5 +/- 8.8 (range 3-38) days of life, after 1.7 +/- 1.5 (range 1-6) days from the onset of symptoms. Aiming to perform laparotomy before the occurrence of perforation, surgery was liberally indicated in stage IIIa, according to Walsh-Kliegman. Explorative laparotomy (+peritoneal drainage in 2 cases) was performed in 4 patients with massive intestinal necrosis: all died within 3 days of surgery. In one neonate, only pneumatosis was present and resection was not considered mandatory. Intestinal resection and enterostomy was performed in 17 neonates, 5 of them with perforation; three developed an intestinal stenosis. Enterostomy was closed after 116.2 +/- 61.8 days (range 26-193); 11 patients (64.7%) are long-term survivors. Intestinal resection and primary anastomosis was performed in 6 babies, 3 of them with perforation. Postoperatively, 2 dehiscences and 1 stenosis were recorded, but all children survived. In our opinion, resection followed by primary anastomosis seems to be the most satisfactory surgical option.

Anastomosis, Surgical↗
PubMed1993

[Intestinal atresia: what is changed in the last 30 years].

The changes occurred in the management of intestinal atresia in the last 30 years are presented. On the basis of a series of 55 neonates (29 M/26 F; 14 premature; mean body weight 2640 g, range 1340-4100 g; 23 duodenal, 30 small bowel, 2 colon) four aspects of this malformation are discussed; epidemiology, diagnostics, therapy and prognosis. Epidemiology. 40% incidence reduction is detected; unchanged the relative incidence of Down neonates with duodenal atresia. Diagnostics. Prenatal US scan useful in 4 of 8 cases only; emphasized the usefulness of plain X-ray abdomen. Perioperative treatment. Dramatic impact of the artificial nutrition on the management of short bowel syndrome, with 7 cases (residual small bowel from 75 to 9 cm, mean 40 cm) successfully treated. Surgical technique. Confirmed the validity of the one-stage termino-terminal anastomosis. Prognosis. Significant improvement in the last three decades respectively with 50%, 87% and 93% of long-term survivors. In the first decade 6 over 8 deaths were related to the atresia, while in the last 10 years the only death recorded was related to an associated severe cardiac malformation.

Anastomosis, Surgical↗
PubMed1990

Turcot's syndrome with intestinal lymphoma in a child: an unusual case of triple tumor.

Turcot's syndrome, the association of brain tumor (usually glioblastoma, medullo-blastoma, or astrocytoma) and colonic polyps, is a very rare condition of which about 20 cases have been reported. It has been described only once previously with cancer in a third organ system. In this paper, we report a child affected with colonic polyposis and astrocytoma (i.e., Turcot's syndrome) associated with intestinal non-Hodgkin's lymphoma.

Adenomatous Polyposis Coli↗
PubMed1986

[Hypertrophic stenosis of the pylorus. Ultrasound and traditional diagnosis. Comparison of methods].

Hypertrophic pyloric stenosis (HPS) is the most frequent cause of abdominal surgery during the first months of life. A new diagnostic approach to this type of pathology is given by ultrasound examination which offers the opportunity to perform a precise study of pyloric muscle thickness, pyloric diameter width and pyloric muscle length. Ultrasound provides a quick diagnostic tool sparing radiation exposure to the patient. X-ray study is only to be reserved to the few cases in which clinical and ultrasound data are doubtful and--in all instances--to rule-out other possible causes of gastric outlet obstruction. We report 20 infants (14 males and 6 females) referred with clinical suspicion of HPS. This diagnosis has been confirmed by ultrasound in 12 cases, suspected in 1 and excluded in 7 cases. Upper gastrointestinal tract series confirmed the presence of HPS in 13 cases; discovered a huge gastroesophageal reflux in one and showed normal findings in 6 cases.

Female↗