Intrauterine fistulation of perforated Meckel's diverticulum to the surface of the sac of an intact exomphalos minor.

The presence of Meckel's diverticulum in the exomphalos sac is a well known entity but the intrauterine perforation of the diverticulum is very rare. We report a case of intrauterine fistulation of Meckel's diverticulum to the surface of the intact sac of an exomphalos minor.

Management of hydatidosis in children. Twenty-one year experience.

PURPOSE: Hydatidosis is a serious problem in non-endemic countries due to the influx of immigrants from nations where preventive measures are inadequate. The aim of this retrospective study is to present our experience in the management of hydatidosis in children and to define the criteria for the most effective model of treatment. MATERIAL AND METHODS: Over a 21-year period (1985 - 2006), 150 children with pulmonary and abdominal hydatidosis (ECHINOCOCCUS CYSTICUS) were treated at our department. The anatomical location of the parasite was as follows: liver 82, lungs 59, spleen 4, mesentery 2, kidneys 2 and pelvic floor 1 case. Medical treatment with oral antihelminthic agents was given to 37 patients (45.1 %), with liver hydatidosis, 36 patients (61 %) with pulmonary hydatidosis and one patient with hydatid cyst of the spleen. The remaining 76 patients were submitted primarily to excision of the cyst or partial capsectomy. RESULTS: Medical treatment was ineffective in 16 patients (43.2 %) with liver hydatidosis, 11 patients (30.6 %) with pulmonary hydatidosis and one patient with hydatidosis of the spleen. All patients with pulmonary hydatidosis who failed to respond to medical treatment developed complications requiring surgical intervention. Of the 76 patients who were submitted to surgery initially, only 4 (5.3 %) presented with postoperative complications requiring reoperation. In all cases who responded inadequately to medical treatment or developed complications the cyst diameter exceeded 6 cm. The overall long-term results were good. CONCLUSIONS: a) Hydatid cysts with sizes exceeding 6 cm in diameter should not be treated medically; b) medical treatment seems to be more effective for pulmonary hydatidosis but failed medical treatment in these patients leads to complications with increased morbidity; c) large hydatid cysts should be treated surgically from the start.

Gastric fibrolipoma causing bleeding in a child.

Gastrointestinal lipomas are uncommon benign tumors usually occurring in the colon and rarely in the stomach. We report a case of a 10-year-old boy who presented with a two-week history of epigastric abdominal pain and several episodes of melena. Gastroscopy revealed a soft, elevated, broad based, polypoid lesion on the posterior wall, without superficial erosion or ulceration. One week later the patient was readmitted with melena and hematemesis, followed by a significant drop of hematocrit levels. A laparotomy was carried out and the mass was excised. Histological findings were consistent with a submucosal gastric fibrolipoma resected IN TOTO. The clinical presentation, diagnosis and management of this condition are discussed.

Amyand's hernia in premature neonates: report of two cases.

The finding of a normal or inflamed vermiform appendix within an inguinal hernia is termed Amyand's hernia. It is extremely rare in children, especially in infants and neonates. When it occurs, it is usually misdiagnosed as an irreducible or strangulated inguinal hernia, and the accurate diagnosis is made intraoperatively. We report two cases of Amyand's hernia in premature neonates. Both patients presented on admission with signs and symptoms indicating a strangulated right inguinal hernia, and the accurate diagnosis was made intraoperatively. One of them had progressed to local peritonitis. Appendicectomy and hernia repair were made at the same time through an inguinal transverse incision, and the postoperative course was uneventful in both. We point out the need to consider acute appendicitis in the differential diagnosis of strangulated right inguinal hernia.

Primary non-refluxing megaureter: need for conservative treatment.

PURPOSE: In the last two decades, many reports have confirmed the efficacy and safety of the conservative treatment of non-refluxing megaureter in asymptomatic patients and many cases of ureteral dilatation tend to resolve spontaneously. We report our experience on 108 patients with primary non-refluxing megaureter detected prenatally or diagnosed after birth and we discuss our results with long-term non surgical treatment. MATERIAL AND METHODS: All patients were evaluated by ultrasound (US), voiding cystourethrogram (VCUG) and MAG3 renography. Observation period ranged from 6-72 months (mean 29.1). RESULTS: Surgery was performed in 12 patients (11.1%) with severe hydroureteronephrosis. Complete resolution or significant improvement was noted in 80 cases (74%) and persisted in 16 cases (14.8%). In the group with spontaneous resolution the ureteral diameter was less than in patients without resolution. Megaureters grade 1 to 3 tended to resolve between 12 and 36 months of observation. CONCLUSION: Conservative management is the treatment of choice in primary non refluxing megaureter. The grade of hydroureteronephrosis is an important predictor factor and infants should be followed periodically with renal ultrasound and diuretic renography.

Diffuse intraabdominal desmoplastic small round cell tumor: a ten-year experience.

BACKGROUND: Intraabdominal desmoplastic small round cell tumors (IDSRCT) are rare in children and predominantly affect male adolescents and young adults. We present our experience in the management of five children with diffuse IDSRCT, managed with aggressive chemotherapy, surgery, radiotherapy and peripheral blood stem cell transplantation. MATERIAL AND METHODS: During the last decade five patients, four males and one female (mean age 9.6 years), with diffuse IDSRCT were managed in our department. The main symptoms were abdominal distention, vague abdominal pain, and vomiting. Three patients with inoperable tumor on admission were submitted initially to open biopsy followed by aggressive chemotherapy. Regression of the tumor was followed by a second laparotomy and radical excision of any macroscopically distinguishable masses, followed by chemotherapy. In the remaining two patients a debulking procedure was done initially, followed by chemotherapy. The accurate diagnosis of the disease was established by immunohistochemistry, additionally confirmed in the last two patients by molecular analysis. RESULTS: Three patients who had radical excision of the tumor and adjuvant chemotherapy had recurrence after two to six months. In the remaining two patients, recurrence was evident after two and eighteen months, respectively, following debulking. In addition, one patient with recurrence received radiotherapy and two others underwent peripheral blood stem cell transplantation. All but one patient died within three years from diagnosis. The last patient, who was submitted to a debulking procedure, is still alive eight months after the operation. CONCLUSIONS: Intrabdominal desmoplastic small round cell tumor is a highly aggressive malignancy with a very poor prognosis. Multiagent chemotherapy usually leads initially to a temporary regression of the tumor, but recurrence is the rule. Radical surgical excision, radiotherapy and peripheral blood stem cell transplantation does not seem to improve prognosis significantly. Despite all therapeutic modalities the outcome is dismal and surgical efforts can be considered only as palliative.