[Diagnostic difficulties in pediatric abdominal pain with potential appendicitis]. / Trudnosci diagnostyczne przyczyn bólów brzucha okolicy wyrostka robaczkowego u dzieci.

BACKGROUND: Pediatric abdominal pain is one of the most common symptom of children brought to attention of primary care physicians and pediatric surgeons. In many children clinical symptoms may be uncharacteristic and may lead to diagnostic difficulties. AIM: Clinical analysis of children with right lower quadrant abdominal pain suspected of appendicitis or children with periappendicular mass. MATERIALS AND METHODS: The histories of 14 children aged from 18 months to 17 years treated in Pediatric Clinic were analysed. All children were operated because of right lower quadrant abdominal pain or abdominal mass before admission to the Pediatric Clinic or during hospitalization in Pediatric Clinic. Intraoperatively in all children pathologies other than appendicitis were the cause of symptoms. RESULTS: The most often Crohn's disease were recognized (9 children), in 2 cases with concomitant other pathologies (fecal tumor of appendix in one case and with peritoneal abscess after perforation of intestinal wall). Sporadically the inflammation of the mesenterial lymph nodes caused by Yersinia infection suggested appendicitis. In one boy with ulcerative colitis, during exacerbation of the disease appendicitis complicated by rupture and peritonitis was observed. In 18-month old child with right lower quadrant abdominal mass invagination complicated by perforation of the ileum was recognized. In the case of 14-years old boy 6 months after appendectomy we observed mechanical intestinal obstruction complicated by perforation and peritonitis. Carcinoid of the appendix was the cause of abdominal pain in one child. CONCLUSIONS: 1. Appendicitis is the most frequent surgical etiology of the right lower quadrant pediatric abdominal pain. 2. Despite new diagnostic imagines there are no definite criteria to recognize appendicitis, in most cases physical examination and very carefull evaluation of abdominal pain are the most important. 3. All children with periappendipected of Crohn's disease. 4. All children with equivocal presentations of appendicitis and with normal appendix during operation should undergo further diagnostic evaluation.

Treatment of monostotic fibrous dysplasia with pamidronate.

INTRODUCTION: Fibrous dysplasia is a condition where fibrous tissue and bone with an altered structure replace normal bone. The craniofacial involvement is difficult to treat due to location, uncontrolled proliferation and compression of nerves. These result in facial asymmetry, pain, cranial nerve deficiencies, loss of vision or hearing, alterations in breathing, etc. The use of pamidronate, which inhibits the resorptive activity of osteoclasts may be an alternative to surgical intervention and may improve the results of treatment. AIM OF STUDY: To evaluate the treatment of monostotic craniofacial fibrous dysplasia with pamidronate. MATERIAL: A prospective pilot study was undertaken in 6 children with progressive fibrous dysplasia located in the mandible (3 pts), maxilla (2 pts) or cranial base (1 pt). Surgery consisted only of a biopsy and implantation of gentamycin sponge into the biopsy bed in 3 cases in which an infection was initially suspected. All patients received pamidronate infusions (1mg/kg i.v. for 3 days, every 4-6 months). The changes in bone density and extension of the disease were evaluated by serial orthopantomograms and CT scans. Follow up time was 9-24 months. RESULTS: Pain relief was achieved in all cases, decrease in swelling in 3, and stabilization in 3 cases. Radiological investigation revealed any progression but reduction in size and calcification of osteolytic lesions. The local bone density increased and there was no further spread of the disease in any of the patients. Inflammatory symptoms resolved in all cases. The only side effect was an increase in body temperature up to 38-40 degrees C. CONCLUSION: Pamidronate appears to be an effective and well-tolerated therapeutic option for patients with fibrous dysplasia. In the future, the combination of the metabolic blockage of the dysplasia by pamidronate and limited non-mutilating surgical interventions are possible.

[Ewing's sacroma / PNET--the possibility to assure local control]. / Miesak Ewinga / PNET -- mozliwosc uzyskania kontroli miejscowej.

UNLABELLED: Ewing's sarcoma and PNET are more and more frequently submitted to surgical treatment, as this method of local therapy seems to offer a better chance for cure. Which method surgery or radiotherapy (RTx) is more mutilating in childhood, should be carefully considered. The aim of the report is to review the possibility of local resection and reconstruction in various sites of the tumour. PATIENTS: 12 patients with Ewing's sarcoma / PNET were submitted to the site-specific surgical procedures and assessed for local control and function. All were pre-treated with CHT and continued that treatment after surgery. Four patients had fibular Ewing's sarcoma and all underwent subtotal fibulectomy. Long term complete remission (CR) was achieved in all. Two patients had Askin tumours; both developed pleural relapses distant to the primary tumour site. One patient had total scapulectomy (CR for 7 years). One patient had hemipelvectomy supplemented with RTx because of micro residue (CR for 10 years). Two patients were amputated: one for PNET of tibia invading nerves and vessels, another one for Ewing's sarcoma of femur. The first one developed pulmonary metastases and died one year later, the second one has been alive for 13 years but has badly accepted the prosthesis. Two patients with very extensive tumours of pelvis could never be operated on, they never achieved CR despite chemo- and radiotherapy and died. SUMMARY: Of 10 patients submitted to surgery 7 patients are alive and 4 have an excellent functional result. Of the 2 patients in whom the tumour was inoperable neither is alive. It seems justified to recommend surgical resection of Ewing's sarcoma / PNET; satisfying reconstruction is usually feasible. RTx seems to have value rather as a method supplementing minor failures of surgery than as the only local treatment.

[Intramuscular angiomyolipoma in children--own observations]. / Angiomyolipoma intramusculare u dzieci -- obserwacje wlasne.

UNLABELLED: Angiomyolipoma (AGML) is a rare benign tumour usually located in the kidneys, but it can occur also in other sites -- e.g. in the skeletal muscles. This location requires differentiation from sarcomas and angiomas. The aim of the study is to assess the diagnostic and therapeutic methods applied in the management of patients with AGML. PATIENTS: five patients were treated for intramuscular AGML (1998-2001). Tumour localisation was as follows: intermediate head of vastus muscle of the femur, lateral head, medial head, occipital muscles of the head, and the interosteal muscles of the metatarsum. All patients complained of severe, localized pain; the tumour mass was usually small and hardly palpable. The biopsy was preceded by ultrasound examination with Colour-Doppler option and CT examination with "angio" option. After the result of the pathological examination of the biopsy specimen was obtained, surgery was performed. One cm of healthy tissue around the tumour was the required margin of excision. This was obtained in all cases but one. Pain relief was obtained in all patients; in one, local relapse occurred. CONCLUSION: AGML is a rare neoplasm in extra renal locations. Its appearance seems typical, with severe pain and only small deformation of the muscle. In this sub-group of patients, AMGL is located frequently in the femoral muscles. USG and CT imaging may correctly suggest the character of the tumour; however, the extent of treatment, should be decided after the biopsy histopathological report is known. MRI seems unnecessary when good quality Doppler-sonography and CT are available.