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  • 1
    ISSN: 1437-9813
    Keywords: Key words Sacrococcygeal teratoma ; Prognosis ; Functional impairment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract From 1976 to 1995, 23 children, 4 boys and 19 girls, were treated at our department for sacrococcygeal teratomas (SCT). Their records were analyzed retrospectively, considering age at operation, histopathology, recurrences, and long-term evolution. One died on the 1st day of life following tumor rupture with hemorrhagic shock without surgical intervention. All others were operated upon at a mean age of 4.2 days for those 19 (=82%) who were diagnosed in the neonatal period and whose histology proved benign. In the remaining 3 children, in whom tumor manifestation did not occur before 11 months, 13 months, and 10 years of age, respectively, histopathologic evaluation revealed 2 carcinomas and 1 yolk-sac tumor, and all 3 recurred. Overall, 5 patients died, the 1 mentioned above, 1 due to volvulus after laparotomy, and 1 from multiple associated congenital malformations. Two deaths were related to malignancy, whereby only 1 was a malignant teratoma diagnosed at the original operation. Eight children had recurrences, 2 were benign and 6 malignant, with 3 of the latter having been graded benign on histology of the primary tumor. Of the 18 surviving patients, 17 (93.5%) returned for clinical review following a standardized protocol. The average interval from the primary surgery was 12.3 years (range 3.5–22 years). Four had malignant tumors with a recurrence-free period of from 9 to 14 years; 5 (29.4%) had urinary or anorectal functional impairment. One child with a patulous anus presented with fecal soiling. Two reported nocturnal enuresis, 1 associated with perineal anesthesia. One had a neurogenic bladder with overflow voiding and bilateral third-degree vesicoureteral reflux. Second-degree reflux was found in the last patient. We conclude that follow-up after surgery for SCT should not only search for tumor recurrence but include the diagnosis and treatment of possible secondary urinary and/or fecal incontinence.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1437-9813
    Keywords: Key words Hirschsprung's disease ; Intestinal neuronal dysplasia ; Hirschsprung-associated intestinal neuronal dysplasia ; Pull-through operation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Between 1991 and 1993, 106 newly diagnosed cases of Hirschsprung's disease (HD) were prospectively analyzed for the association of HD and intestinal neuronal dysplasia (IND) at ten pediatric surgical departments in central Europe. Hirschsprung-associated IND (HaIND) was found in 40% of cases. IND was disseminated in one-third and localized in two-thirds of the patients. Initial clinical symptoms were related to the length of the aganglionic segment, but not to the presence of HaIND. An enterostomy performed in 72 cases (67.9%) was located in a segment of pathologically innervated bowel in 50% of all cases, but in 72% of cases of HaIND. The proximal margin of the resected bowel showed pathological innervation in 44% of cases. Supplemental biopsies from the intestine (apart from diagnostic suction biopsies and biopsies at the enterostomy site) led to the first identification or definition of length of associated IND in 17.9% of cases. Postoperatively, the presence of long-segment aganglionosis or associated IND implied a delay in the restoration of normal defecation. Persistent constipation was found in 40% of patients with associated disseminated IND at follow-up at 6 months, compared to 20.6% in patients with isolated HD. These children needed secondary interventions more often than patients with associated localized IND or isolated HD. HaIND thus has clinical implications for the postoperative course if IND is disseminated.
    Type of Medium: Electronic Resource
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