[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.

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.

Mechanical complications related to indwelling central venous catheter in pediatric hematology/oncology patients.

Indwelling central venous catheters (CVC) are essential devices in the management of children with oncologic/hematologic diseases or following bone marrow transplantation. The authors report data on the mechanical complications observed in pediatric hematology/oncology patients, collected by a retrospective analysis of clinical records of 482 patients in whom 567 indwelling central venous catheters had been inserted from January 1992 to December 1998 at the G. Gaslini Institute. During the study period, 52 episodes of mechanical complications (9%) were observed: mechanical obstruction (24 episodes), catheter dislocation (13), problems related to catheter material (12), and accidental removal (3). In 25 cases removal and replacement of CVC was necessary for the treatment of complications, while medical treatment (thrombolytic-antithrombotic) was successful and well tolerated in 8. The study shows the importance of mechanical complications in children with indwelling CVC for hematologic or oncologic diseases. Moreover, the experience of administering a systemic low-dosage thrombolytic therapy demonstrates new prospects of reducing CVC replacement by restoring CVC viability.

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.

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.

Surgical treatment of scoliosis associated with myelomeningocele.

Twenty-nine patients (mean age 12 years) with severe thoracolumbar and lumbar scoliosis due to myelomeningocele were treated by spinal fusion (7 by posterior arthrodesis with instrumentation, 3 by anterior arthrodesis with instrumentation, 19 by combined anterior and posterior fusion with instrumentation). Fusion was extended to the sacrum in 15 patients. Mean period of follow-up was 6.2 years. The average Cobb angle changes were as follows: thoracic and thoracolumbar curves preoperatively 86 degrees to 45 degrees at follow-up (the final average curve correction was 47%); lumbar curves preoperatively 97 degrees to 48 degrees at follow-up (the final average curve correction was 50%). Average pelvis obliquity changed from 26 degrees to 13 degrees at follow-up with an average correction of 49%. The combined anterior and posterior instrumentation and fusion gave the best correction of deformity (the final average thoracic and thoracolumbar curve correction was 55%; the final average lumbar curve correction was 61%). Independent of the method of stabilization, post-operative wound infection was a serious problem (24%). The combined fusion-instrumentation method reduced the rate of pseudoarthrosis to 14%.

[Sepsis in children with malignant neoplasia, equipped with a Broviac-type venous catheter]. / Sepsi in bambini affetti da neoplasia maligna portatori di catetere venoso centrale tipo Broviac.

Indwelling central venous catheters obviate many problems in the care of children with malignancies, but they also are a well-known source of infection. We are reviewed the history of 584 Broviac catheters inserted from January 1984 to December 1991, in 475 children with cancer in order to assess the etiology of bacteremias, their association with neutropenia and their relationship with the presence of the catheters. The overall duration-time of the catheters, employed for blood tests, drug and blood infusions and parenteral nutrition, was 1-835 days (median 263, mean 186). Total catheter courses was 108.678 days. In this period 226 episodes of sepsis were observed in 180 patients: 157 in neutropenic patients and 69 in non neutropenic. Catheter related bacteremias were diagnosed in 65/226 episodes (29%): 23 (35%) were observed in neutropenic patients and 42 (65%) in non neutropenic (P < 0.005). Gram-positive pathogens were isolated in 28/65 (43%) episodes, Gram-negatives in 15/65 (23%), fungi in 9/65 (14%), and the remaining 13 (20%) were polymicrobial. In the last years we observed an increase of catheter related bacteremias due to Gram-negative rods no change was observed in pathogens causing catheters unrelated bacteremias. The high incidence of catheters related bacteremias in non neutropenic, non hospitalized patients, stress on the home-care of the catheters; a high level of suspicion of Gram-negative infections should be maintained in cancer patients with an indwelling central venous catheters.

[Treatment with Soave's technic and long-term results in 270 cases of Hirschsprung disease]. / Tratamiento y resultados a largo plazo de 270 casos de enfermedad de Hirschsprung con la técnica de Soave.

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.