[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.

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.

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.

Vesical continence in bilateral ectopic single ureters.

PURPOSE: We explore the possibility of gaining suitable vesical capacity and continence in bilateral single ureters without having to resort to urinary diversion or bladder neck reconstruction. MATERIALS AND METHODS: In the last 10 years we treated 6 girls with bilateral ectopic ureters, and 2 with a single kidney and urethral ectopic ureter. Both ureters were ectopic in the urethra in 3 cases, and 1 ureter was inserted in the urethra and 1 in the bladder neck in the other 3. Preoperative examinations included ultrasonography, cystography, excretory urography and endoscopy in all cases, and magnetic resonance imaging in 2. Patient age at intervention ranged from 1 month to 8 years. RESULTS: Cross-trigonal reimplantation of the ectopic ureters through a correctly positioned neomeatus was performed in 7 cases. In 2 patients it was impossible to reach the ureteral insertion from inside the bladder (1 ureter in 1 and both ureters in 1), and so the distal tract of these ureters was resected extravesically as close as possible to the urethra. One patient with a single kidney also had an anorectal malformation and a rectovaginal fistula. She had pelvic renal ectopia and the ureter was inserted beneath the urogenital diaphragm causing minimal bladder capacity. Therefore, a sigmoid neobladder with appendicostomy was constructed according to the Mitrofanoff principle. A Young-Dees bladder neck plasty was done in 2 patients at the same time as ureteral reimplantation and 3 years later in 1. Followup urodynamics revealed more than acceptable continence. Bladder capacity was insufficient in only 1 polyuric patient. CONCLUSIONS: A bladder with bilateral ureteral ectopia is not necessarily useless, and the majority of patients can achieve normal bladder function and capacity along with normal transurethral voiding and satisfactory continence.

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.

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.

Follicular cell carcinoma of the thyroid in a child after bone marrow transplantation for acute lymphoblastic leukemia.

Follicular cell carcinoma (FCC) of the thyroid is rarely found during childhood. We report a 12 1/2-year-old girl with acute lymphoblastic leukemia, who developed rapidly growing FCC of the thyroid, 3 years after bone marrow transplantation. The role of chemotherapy in the induction of such secondary tumors after transplantation is discussed, and a proposal for the approach to these patients is suggested.

[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.

[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.

[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.

[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.

Intraabdominal calcifications in the newborn: an unusual case with anorectal malformation and other anomalies.

Intraabdominal calcifications are an unusual radiographic finding in the newborn and can easily be misinterpreted as meconium peritonitis. The authors refer to a patient with anorectal malformation (ARM), intraluminal calcifications and other anomalies. Careful differentiation of the type of intraabdominal calcifications can suggest the possibility of other initially unsuspected anomalies.

New concepts in preoperative imaging of anorectal malformation. New concepts in imaging of ARM.

In this study of 14 patients with anorectal anomalies CT and MRI were employed for preoperative assessment. The use of a pressure enhanced water soluble enema via the colostomy proved to be an extremely efficient method for showing a fistula. MRI studies were enhanced by the use of vaseline oil and in one case this technique was used prior to surgery to provide important information by injecting through a perineal fistula. CT and axial MRI proved to be more valuable than sagittal MRI which is only useful for the length of the atretic segment. The authors consider that a combined approach using pressure enhanced water soluble enema and MRI will provide the most valuable preoperative information to plan a successful operative approach and enable an accurate prognostic evaluation of continence in these difficult and complex patients.

[Blocked transcolostomy colonography in the identification of anorectal malformations]. / La colongrafia transcolostomica sbarrata nell'identificazione della malformazione anorettale.

The study of anorectal malformations has considerably improved over the last years also thanks to the use of such diagnostic techniques as CT and MR imaging, which allow a better knowledge of perineal region anatomy and the demonstration of the development of sphincteric structures. The most important diagnostic phase for the identification of anorectal malformations consists in the detection of rectal fistulas. The authors report on their experience with barred water-soluble contrast enema in the study of anorectal anomalies. Over 2 years, 23 patients with anorectal malformations (10 males and 13 females) previously submitted to colostomy, were studied with this method. Fistulas were detected in 21 cases; in the extant 2 patients (both females affected with Down syndrome), no fistulas were detected, not even at surgery, which was posterior sagittal anorectoplasty according to Peña-De Vries. In our experience, the most frequent anomalies were recto-bulbo-urethral fistula in males and low recto-vaginal fistula in females. The authors suggest the use of this method as a routine diagnostic examination, because of its proven reliability.

[Computed tomography vs. magnetic resonance in the diagnosis of anorectal anomalies]. / Tomografia Computerizzata vs Risonanza Magnetica nella diagnostica delle anomalie ano-rettali.

Anorectal malformations include a variety of anatomical anomalies which are often difficult to assess. Thirty-four patients (age range: 1 month-19 years) were studied over a 4-year period, 14 of them preoperatively and 20 postoperatively. In addition to inverted lateral plain films, a barred Iopamiro enema was performed in 13 of 14 cases by a Foley's catheter via colostomy. Endocolic pressure could thus be increased, which allowed the detection of rectal fistulas in 11 of 13 cases. Preoperative CT was performed in 9 cases, and MRI in 5. Both CT and axial MR scans provided accurate images of sphincteric muscles: they were well developed in 7 patients and poorly developed in 6; in 1 case the muscles were not identified. Sagittal MR scans only demonstrated the distance between blind pouch and perineum. Sagittal MRI also provided images of the spine (1 patient exhibited tethered cord). In the postoperative management, except for severe sacral anomalies, miscentered puborectal muscle and hypoplastic musculature were observed as the most frequent causes of fecal incontinence in patients treated with abdomino-perineal pull-through of the colon. To select the cases who needed reoperation, most frequently secondary PSARP, 17 patients were studied by CT and 7 by MRI; both techniques were employed in 4 cases. In 6 patients good development of sphincteric musculature and neo-anorectum correctly positioned within were observed. In 4 cases the neo-anorectum was correctly positioned, but sphincteric muscles were too hypoplastic to allow good continence; in 10 patients with incontinence, the neo-anorectum was seen outside sphincteric musculature: in 2 cases the latter was well developed (1 patient had had severe surgical damage to levator ani), while in 8 it appeared to be poorly developed. In our experience, postoperative CT allowed a correct evaluation to be made, whereas MRI failed to yield additional information.