Search

Background Invasive aspergillosis (IA) is a serious problem in patients suffering from hematological malignancies. Surgical resection has been reported to improve disease control and patient survival. There are few reports describing the role of surgery in children with pulmonary IA. Procedure From October 1998 to September 2005, 21 patients fulfilled the inclusion criteria. Demographic and clinical data, as well as type and duration of antifungal therapy; surgery and related complications; time elapsing from surgery to resumption of chemotherapy were collected retrospectively through a specially designed form filled in by each investigator. Results Eleven males and 10 females, aged between 2 and 17 years underwent one or more surgical lung resections for diagnostic and therapeutic purposes. Surgical complications were reported in three patients. Two patients, who underwent a wedge resection and a lobectomy, respectively, had no fungal lesions detected at surgery. Seventeen of 20 patients with malignancy resumed chemotherapy after a median of 19 days from surgery, range 7–81, and 11 of them underwent hematopoietic stem cell transplantation after a median time of 60 days from surgery, range 19–110. After a median follow-up of 1.7 years, 12 patients are alive while 9 patients have died from progression of their underlying disease. Conclusions This study suggests that the combination of medical antifungal therapy and early surgical excision is a feasible and an effective strategy in pediatric patients with IA. In order to avoid unnecessary surgical procedures, we advise checking the response to antifungal therapy by chest-computed tomography immediately before the date of surgery. Pediatr Blood Cancer 2007;49:909–913. © 2006 Wiley-Liss, Inc.

A 14-yr-old White male was referred to the Urology Unit of the G. Gaslini Institute because of a post-traumatic urethral stenosis (arising from a bicycle accident) that, during the previous 18 months, had required repeated endoscopic urethral dilation manoeuvres at the patient's local hospital. Apart from the urethral stenosis, the patient had been in excellent health until 2 months before admission, when slowly progressive exertional dyspnoea, associated with nonproductive cough and right-sided posterior chest pain, developed. On admission, the patient appeared in good clinical condition. Decreased percussion and auscultatory sounds were noted over the middle and lower portions of the right hemithorax. A summary of the results of the blood tests performed on admission is shown in table 1⇓. View this table: Table 1 Results of blood tests performed on admission Chest radiography (fig. 1⇓) and high-resolution computed tomography (HRCT) (fig. 2⇓) were performed. Fig. 1.— Chest radiograph. Fig. 2.— High-resolution computed tomography of the chest at two different window settings: a) “parenchymal”; and b) “soft tissue”. Thoracocentesis was performed and 500 mL haemorrhagic pleural fluid aspirated. Pleural fluid analysis did not show any cytological changes suggesting malignancies, amylase levels were within the normal range and microbiological evaluation results were negative for bacteria, fungi, mycoplasmata, mycobacteria and viruses. Plasma d‐dimer levels were slightly elevated but ultrasonography of the deep venous system did not show signs of thrombosis in the legs, penis or pelvis. Echocardiographic evaluation did not demonstrate any right ventricular dysfunction. Ultrasonographic examinations showed that there were no abnormalities of the abdominal organs and no peritoneal effusion and ruled out the presence of testicular or thyroid tumours. Fibreoptic bronchoscopy was then performed and did not reveal any airway abnormalities. Bronchoalveolar lavage analysis was nondiagnostic (no siderocytes suggestive of pulmonary haemorrhage, acid-fast bacilli, bacteria, viruses, fungi or malignant cells were identified in the epithelial lining fluid or lavage fluid …

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.

Background/Purpose: Vesico-ureteric reflux (VUR) is a common problem in children with neuropathic bladder. Lesserdegree 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.

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.

Chromophobe renal cell carcinoma (CRCC) is a distinct variant of renal carcinoma generally affecting adults. We report a case of an unusual CRCC, arising in a male child affected by hypospadias. This case demonstrates that CRCC can occur in the pediatric patients and can be associated with genital tract anomalies such as Wilms tumor. Pediatr Blood Cancer 2008;50:413–415. © 2006 Wiley-Liss, Inc.

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.

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.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.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.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.

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.

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.

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.

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.

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.

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.

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.

Isolated oesophageal leiomyomatosis in children is very rare. The association between leiomyomatosis of oesophagus and female genital tract and an Alport like hematuric nephritis has been recognized as a distinct entity by Garcia-Torres and Guarner. Since then few other cases of this syndrome have been observed. Congenital cataracts and neurosensory deafness have been added to the clinical spectrum. Autosomal dominant inheritance is suggested by the familial cases. We report a patient who presents, as a new finding, leiomyomatosis of the rectum.

A protocol has been applied to all our patients consulting for nocturnal enuresis who also presented daytime wetting or urge to void, to rule out obstructive uropathies. Our protocol includes voiding cystourethrogram, IVP, cystometry and endoscopy. 64 females and 46 males, 4 to 14 year-old, were studied between 1980 and 1983. Obstructive urethral uropathy was ruled out in 37 females (58%) and 32 males (69%). The most common treatments in these patients were endoscopic resection (Bugbee) of posterior urethral valves in boys and resection of the distal ring in girls. All other associated diseases or malformations were treated according to their etiology. Results, analyzed through a questionnaire sent to parents, were good in 77% of the patients treated for obstruction. In conclusion we think that daytime wetting and urge to void are very frequently the emerging symptoms of a hidden uropathy, in most cases obstructing proximal or distal urethra. Surgical treatment can lead to a good cure rate.

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.

In spite of the progress in the field of surgical treatment of anorectal malformations, faecal incontinence is, in variable degrees, still an unpleasant and frequent postoperative sequela. Postoperative CT demonstrate the location of the pulled-through intestine, including whether it had been correctly placed through the levator ani and in the spincteric muscular complex. In our nine patients we discovered a correlation between the CT findings and the clinical picture. The cases of incontinence whether associated with sacral anomalies or not were characterized either by marked hypoplasia of musculature or by the pull-through having missed the sphincteric musculature. CT scans could be of use in planning further surgery.

The authors present the surgical technique for the correction of Hirschsprung disease following SOAVE's original description. A series of 270 cases observed in 20 years at the Pediatric Surgery Department of the "Instituto Giannina Gaslini" in Genova, Italy, are examined. These cases, all treated by surgery, were followed up in long term for more than one year both clinically and radiologically. Additionally 73 cases treated from 1977 on were also followed up with the aid of ano-rectal electro-manometry. The most recent diagnostic aspects are discussed, such as ano-manometry, and some conclusions are drawn about long term results.