[Gastric fibroid polyp in a 4-month-old girl with Costello syndrome]. / Polipo fibroide gastrico in sindrome di Costello.

We describe the case of a 4-month-old girl with a gastric fibroid polyp. This was an occasional radiographic finding, confirmed by sonography and computerized tomography. This very rare benign tumor was surgically removed. The diagnosis of Costello syndrome was based on clinical appearance. This is the first report of a gastric fibroid polyp in Costello syndrome, a genetic disease with a high tumor frequency.

Intravascular endothelial proliferations in children.

Six children who had benign cutaneous vascular lesions characterized by papillary or solid proliferation of the endothelial cells were seen in a 16-year period. In five of them, the lesions had the histologic features of the entity originally described by Masson and recently reported by Clearkin and Enzinger as intravascular papillary endothelial hyperplasia. In one of the five, the lesion appeared in the pure form within large vascular spaces, whereas in the remaining four it appeared as a very limited focal change in a hematoma. The authors agree with others that this lesion represents a peculiar variant of an organizing thrombus. The histologic features of the lesion in the sixth patient were compatible with the so-called "intravenous atypical vascular proliferation," another pseudomalignant vascular disorder recently described by Rosai and Akerman.

Caspofungin associated with liposomal amphotericin B or voriconazole for treatment of refractory fungal pneumonia in children with acute leukaemia or undergoing allogeneic bone marrow transplant.

Caspofungin, in association with other antifungal drugs, was administered as rescue therapy in two cases of documented and one case of possible invasive fungal infection in children with acute leukaemia or undergoing allogeneic bone marrow transplant. The combined therapy was well-tolerated and seemed to be effective in all three patients. A combination antifungal therapy including caspofungin could represent an effective therapy for children with invasive mycoses refractory to single-agent antifungal therapy.

Adenocarcinoma of the rectosigmoid colon in a 10-yr-old child.

The authors describe a case of adenocarcinoma of the rectum in a 10-yr-old boy. The clinical picture, the surgical technique, and the histology are described, an reference is made to the small number of similar cases appearing in the world literature.

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.

Total colectomy and straight ileo-anal soave endorectal pull-through: personal experience with 42 cases.

During the period from 1974 to June 2000 we used the straight ileo-anal Soave pull-through to treat 42 patients (24 affected by total colonic aganglionosis [TCA], 10 with ulcerative colitis and 8 with familial polyposis). The aim of this paper is to show that this operation, associated with total colectomy, is highly recommended, causing a lower number of complications when compared to the various "reservoir" techniques. The mean age of the 24 patients with TCA at the time of the pull-through was 2.8 years; in the ulcerative colitis group, it was 14.3 years and in the familial polyposis group 27.2 years. We always used an ileo-anal deferred anastomosis and never performed temporary loop-diverting ileostomy at the time of the pull-through. In the TCA patients we had no immediate or long-term serious post-operative complications: ileal adaptation, after a frequency of 10 - 12 liquid stools a day, showed a gradual, constant and in some cases amazing improvement in all children. Two years after surgery, the mean stool frequency was 3.6 per 24 hours with no significant differences between the 3 main groups; only 4 children still presented with occasional soiling. After pull-through, all children showed normal growth curves in the long term. There was no malabsorption, no serious electrolyte imbalance, no perianal excoriation, no strictures or intestinal obstruction; their quality of life was considered more than satisfactory by the children's families. We have no direct experience with the various ileal "reservoir" techniques for ulcerative colitis and ileal polyposis nor with colon-sparing operations for TCA; as reported in the literature, all these surgical procedures seem to have a higher number of complications such as pelvic sepsis, pouchitis, enterocolitis, etc. compared with our series; we therefore confirm that total colectomy with the straight ileo-anal Soave pull-through is our treatment of choice, as it is simpler to perform and has fewer short- and long-term complications.

Fetal ovarian cysts: prenatal diagnosis and management. Report of two cases and review of literature.

The Authors report two cases of antenatally diagnosed fetal ovarian cysts. In the first case the cysts underwent spontaneous resolution. In the second case the newborn was submitted to adnexectomy for cyst torsion. A review of the literature is reported.

[Congenital hepatic fibrosis]. / La fibrosi epatica congenita.

According to their experience and to a review of the most important papers in the field, the Authors examine the clinical, morphological, diagnostical and therapeutical aspects of congenital hepatic fibrosis. The initial sign is almost always hepatosplenomegaly and diagnosis can be achieved by means of clinical, anamnestic, histological and ecotomographic findings. Relationships among congenital hepatic fibrosis, infantile polycystic disease of the kidney and liver and Caroli's disease are stressed.

[Bacterial, mycotic and protozoal cholangitis in childhood]. / Le colangiti batteriche, micotiche, protozoarie in età pediatrica.

The Authors present the cases of cholangitis in childhood observed in the Institute of Infectious diseases of Genoa from 1973 to 1982. Cholangitis rarely starts by alone, whereas it appears usually in association with hepatic underlying disease. In childhood the symptomatological triad (fever, jaundice, pain) is infrequent: only jaundice is a common sign of disease. Polymicrobial aetiology of cholangitis is very usual, but Streptococci and E. coli are the aetiological agents most frequently observed; sometimes also anaerobic organsimee, fungi and protozoa cause cholangitis.

[Antireflux plastic repair of the cologastric anastomosis in a female patient operated on by retrosternal esophagocoloplasty]. / Plastica antireflusso dell'anastomosi colongastrica in paziente operata con esofago-colon-plastica retrosternale.

Esophageal atresia without fistula is a rather uncommon malformation (about 8% of all esophageal atresias); its surgical correction is sometimes difficult because of the long gap between the two pouches. When a delayed anastomosis cannot be performed, it is necessary to perform a colon interposition. In one patient operated with retrosternal esophageal-colon-plasty a second operation was needed to correct a symptomatic reflux causing dysphasia and severe aspiration pneumonia. The distal end of the colon was tapered around a chest tube; then a submucosal gastric tunnel was prepared, and the tapered colon was pulled through it and anastomosed to the gastric lumen. The child showed no evidence of gastrocolic reflux in the three years following the operation and is today healthy. Radiologic and scintigraphic examinations, performed ten months after the operation, showed the good canalization of the interposed colon and the continence of the antireflux anastomosis.

[Mesenteric cystic lymphangioma: a case report]. / Linfangioma cistico mesenterico: descrizione di un caso.

Intraabdominal lymphangiomas are rare. A five-year-old girl with abdominal lymphangiomas was successful treated by excision of the mass and resection of short segment of small bowel. We present a case report.

[Axial computed tomography in anorectal malformations: a pre- and postoperative indication?]. / Tomografia axial computerizada en las malformaciones anorectales: una indicacion pre y postoperatoria?

Ten patients with anorectal malformations were studied by preoperative CT: well developed sphincteric muscular structures (puborectal sling of levator ani, muscular striated complex and external sphincter) were present in six patients. In three patients muscular structures were poorly developed (case 2a with rectovaginal fistula, case 5a with rectocloacal fistula and case 7a with prostatic fistula). In one case with bladder fistula, the sphincteric musculature was not identified at all. Eleven patients were postoperative studied by pelvic CT: Two of 11 cases had a good sphincteric control. The other nine patients were incontinent. In three of the nine incontinent patients, the colon was appropriately place, but puborectal muscle and sphincteric complex were hypoplastic. In the other six cases CT showed a pulled through colon out of the sphincteric structures. Four of them presented major bony sacral abnormalities and/or lesion of the sphincteric muscle structures. The remaining two patients with an evident pulled-through colon out of a normally developed sphincteric region and without sacral anomalies, were selected for a secondary PSARP procedure. In one of these two patients the operation brought about a complete resolution of the fecal incontinence. The pelvic CT is a useful examination in the anorectal malformation for the preoperative prognostic evaluation of continence, while postoperative CT is essential in selecting those incontinent patients who need a secondary PSARP for the treatment of fecal incontinence.

[An association between anorectal malformations and Down's syndrome]. / Associazione tra malformazioni anorettali e sindrome di Down.

Down's syndrome is the most frequent chromosomal anomaly in humans and sometimes is associated with anorectal anomalies. The anorectal malformations include many varieties of anatomical anomalies, which are often difficult to evaluate. The Authors believe preoperative CT or MRI of the pelvis, together with other clinical and radiological examination to be a valid mean in the preoperative prognostic evaluation. In this study they analysed the association of anorectal malformations and Down's syndrome and the absence of a genito-urinary or perineal fistula.

[Sacrococcygeal teratomas: diagnosis and treatment]. / Teratomi sacro-coccigei: diagnosi e trattamento.

The Authors present ten cases of sacro-coccygeal teratomas observed during the last ten years at the G. Gaslini Children Institute, Genova. Two cases was diagnosed in the pre-natal period. Diagnostic methods, histologic aspect and surgical treatments are discussed.

[Apple peel small bowel: surgical correction and post-operative treatment]. / Apple peel small bowel: correzione chirurgica e trattamento post-operatorio.

The authors describe 6 cases of Apple Peel Small Bowel observed in the last ten year experience in Institute G. Gaslini of Genoa, Italy. They make a precision on early diagnosis and on surgical treatment, which is original, and also on post-operatory medical treatment. The importance of making an optimal nutritional support with total and partial parenteral nutrition for long time is of great significance to have a better outcome and psycho-physical development in such patients.

[Unusual pelvic pathology treated with posterior sagittal anorectoplasty]. / Patologia pelvica rara trattata secondo i principi della PSARP.

Posterior sagittal approach is very useful for the correction of anorectal malformations, but it can be used also for the treatment of pelvic or perineal masses. The authors present 3 patients affected one by a perineal hamartoma, one by a rectal duplication and one by a retrorectal cystic teratoma. All the patients were treated following the PSARP technique, using the Penã's bipolar electro-stimulator just in order to respect all the muscles of the anorectal sphincters. Using this procedure the authors were able to preserve the full anorectal continence in all the 3 patients.