Ovarian masses in children.

We evaluated the outcome of children with ovarian mass operated on at our Center over an 8-year period. Thirty-four girls aged 1 day to 17 years were included in the study. Mean duration of follow-up was 39.5 months. Eighteen had a nonneoplastic mass and 16 a neoplastic mass, eight of which were malignant. Patients with a malignant tumor underwent adnexectomy of the affected side and appendectomy, without removal of the uterus or the other ovary and without partial omentectomy; only the one girl with bilateral malignant disease had bilateral adnexectomy. Five of the eight patients with malignant disease received chemotherapy. All patients are alive with no evidence of disease. Pediatric ovarian masses are rare but have a relatively high rate of malignancy. They differ from adult malignant tumors in many aspects. Conservative surgery should be applied to preserve fertility and combined, if necessary, with aggressive chemotherapy. A good prognosis may be expected in most cases, even with progressive disease.

Open lung biopsy--successful diagnostic tool with therapeutic implication in the critically ill paediatric population.

Open lung biopsy (OLB) is an important diagnostic tool in children with immune deficiency and/or chronic lung disease with diffuse pulmonary compromise. These patients require a tailored therapeutic approach owing to their fragile status and the side effects of unnecessary or inadequate treatment. Twenty-six patients of mean age 5.6 y underwent 41 open lung biopsies in our centre between 1991 and 1995. Seventeen (65%) were immunocompromised (including 13 with malignancy) and 9 had other lung diseases. The biopsies were diagnostic in 25 patients (96%), and complete clinical-pathological correlation was found in 11 (42%). A specific infectious aetiology was detected in nine patients (35%), all of them immunocompromised. Therapeutic changes were instituted on the basis of the biopsy findings in 18 patients (69%). Two patients had postoperative complications (prolonged pleural leak). We conclude that OLB is a safe diagnostic procedure even in the critically ill child and should be employed without hesitation when conventional methods fail to provide a definitive diagnosis to help redirect therapy.

Splenic "regeneration" after partial splenectomy for Gaucher disease: histological features.

Partial splenectomy for Gaucher disease is often followed by reenlargement of the splenic remnant. It remains unclear if this process is due to tissue regeneration or to continued deposition of glucocerebroside in the reticuloendothelial system or both. We compared the splenic architecture before and after reenlargement in three cases of failed repeated partial splenectomy after two, six and five years. Using the number of lymphoid follicles per hundred low power fields (LF/LPF) as an arbitrary index, we found that prior to the first operation 18, 20 and 27 lymphoid follicles were present per one hundred low power fields, while at the second operation, the corresponding rates were 11, 15 and 17; in control spleens, an average of 712.5 lymphoid follicles were present in one hundred low power fields. The difference in the LF/LPF ratio before and after reenlargement, led us to speculate that splenic re-enlargement in Gaucher disease is mainly the result of the continued deposition of the glucocerebroside in the reticuloendothelial system of the splenic remnant, though some degree of true regeneration as well cannot be completely ruled out. These findings are compared with animal studies and results for partial splenectomy on humans, performed for trauma. Further studies in patients with Gaucher disease are warranted to better define the underlying mechanism of splenic reenlargement.

A stercoraceous ulcer of the colon in neglected Hirschsprung's disease.

A large, nonspecific, chronic ulcer was found in the sigmoid colon of a 13-year-old child with neglected, undiagnosed Hirschsprung's disease (HD). There is no known association between HD and colonic ulcers, suggesting that the ulcer was a true stercoraceous ulcer of the colon and not an intrinsic defect of the aganglionotic bowel.

Xanthogranulomatous pyelonephritis mimicking malignant disease: is preservation of the kidney possible?

Xanthogranulomatous pyelonephritis (XGP) is an uncommon form of pyelonephritis rarely seen in children. It is characterized by destruction of the renal parenchyma and invasion of adjacent tissues, mimicking renal tumors. Preoperative diagnosis is very difficult. Two children with XGP are presented. One underwent nephrectomy and the other drainage of a renal abscess with kidney preservation. Although surgery is considered the only effective treatment, a high index of suspicion and renal biopsy may prevent radical nephrectomy.

Vertical partial splenectomy for epidermoid cyst.

Benign lesions of the spleen usually can be surgically removed by partial splenectomy. When the lesion is longitudinal and occupies most or the entire length of the spleen, partial splenectomy using the common surgical technique, based on the distribution of the organ's blood supply, is impossible. A new approach for "vertical" partial splenectomy for epidermoid cyst is described, using splenic artery occlusion and straight suture needles.