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Notizen: Abstract The marked advantages and merit of pre-term and particularly pre-labor (PTPL) cesarean section (C-section) in the avoidance, and indeed, virtual elimination of severely disabling gastroschisis (GS) complications in infants diagnosed prior to birth by ultrasound has unfortunately remained controversial in the 10 to 12 years since it was first reported and strongly recommended by numerous authors. During this period, GS has remained one of the four major causes of the short-gut syndrome (SGS) in infancy and childhood and a major cause of prolonged, costly, complicated, and hazardous neonatal intensive care unit stays with requirements for total parenteral nutrition (TPN). The most serious and frequent complications of GS in infants born without PTPL C-section are the occurrence of the “peel”, which greatly enlarges and rigidifies the eviscerated gut, and of “complicated GS” (intestinal atresia/s, stenosis, necrosis, perforations) (CGS). The “peel” occurs in 100% of these cases and CGS in approximately 20%. “Peel” enlargement and rigidification of eviscerated intestine in the presence of a reduced peritoneal cavity causes great difficulty in covering the eviscerated, enlarged, and rigidified gut with abdominal wall, skin, a prosthesis, etc., and frequently produces gut ischemia from excessive pressure, which may lead to necrotizing enterocolitis (NEC) and SGS as well as prolonged hospital stays. The presence of a “peel” greatly complicates the hazards of dealing with cases of CGS, as resection and anastomosis are virtually impossible in the presence of a “peel.” The authors report personal experience with 77 cases of GS dating as far back as 1951; 44 of the infants were born after the onset of labor by vaginal or C-section delivery and all had some degree of “peel” formation. Of 320 cases from the literature (including some of the cases reported here), 61 (19.1%) involved CGS. Of the 33 cases born PT, and especially PL, there were no cases of “peel” and only 1 case of CGS (3.0%). This infant had a single atresia associated with a very small (1 cm) defect in the abdominal wall and no labor-induced “peel,” which was easily and successfully repaired by resection and anastomosis. The 6.4-fold reduction in the occurrence of CGS by PTPL C-section (3.0% vs 19.1%) was statistically significant by the chi-square test (P 〈 0.05), as was the 100% elimination of the disabling “peel.” If the single case of CGS associated with a very small defect and no labor or labor-associated “peel” is eliminated, the incidence of CGS in the remaining PTPL group of 32 cases falls to 0 (0% versus 19.1%, P 〈 0.007). PT and especially PL C-section may be expected to virtually eliminate “peel” formation and CGS and to remove GS as one of the four major causes of SGS. The findings of this report that PT labor prior to PT C-section may result in both “peel” formation and CGS further solidifies the role of labor in the production of both the “peel” and the equally disabling CGS. Failure to appreciate the central role of labor in GS complications has doubtless contributed to the persistent controversy concerning the value and importance of PTPL C-section for gastroschisis diagnosed in utero. The pediatric surgeon has an important responsibility with the obstetrician to monitor the possible occurrence of occult labor in the waning weeks of pregnancy and be prepared to do a prompt C-section if it occurs and there is adequate lung maturity. The achievement of “peel”- and CGS-free gut would greatly facilitate the use of the new Bianchi technique of gut reduction without anesthesia. The combination of the use of epidural anesthesia for the elective PTPL C-section with the Bianchi approach would spare both mother and baby any untoward effects of general anesthesia and present the potential for massive reductions in hospital costs with minimal patient manipulation and disturbance. For infants born with labor-associated “peel,” re-evaluation of the suitability and effectiveness of surgical “peel” decortication from involved gut is strongly urged.

Notizen: Abstract Extraneural metastases of intracranial germinomas, although infrequent, are associated with a generally poor prognosis despite the high radiosensitivity of localized primary tumors. Ventriculoperitoneal shunts have been implicated in facilitating metastatic spread of primary intracranial germinomas. We present a case of a successfully irradiated suprasellar germinoma recurring after 13 months as an intra-abdominal yolk-sac tumor in a young man. The tumor was eradicated with a combination of systemic chemotherapy and local irradiation, with no residual viable tumor cells confirmed at final surgical extirpation. The role of cerebrospinal fluid (CSF) shunts in metastases, mixed germ-cell tumor histology, and tumor markers in recurrences as well as radiation doses and volumes for treating primary tumors are discussed. Systemic chemotherapy may be utilized as prophylaxis against shunt metastases when CSF drainage is necessary.

Notizen: Abstract  Extraneural metastases of intracranial germinomas, although infrequent, are associated with a generally poor prognosis despite the high radiosensitivity of localized primary tumors. Ventriculoperitoneal shunts have been implicated in facilitating metastatic spread of primary intracranial germinomas. We present a case of a successfully irradiated suprasellar germinoma recurring after 13 months as an intra-abdominal yolk-sac tumor in a young man. The tumor was eradicated with a combination of systemic chemotherapy and local irradiation, with no residual viable tumor cells confirmed at final surgical extirpation. The role of cerebrospinal fluid (CSF) shunts in metastases, mixed germ-cell tumor histology, and tumor markers in recurrences as well as radiation doses and volumes for treating primary tumors are discussed. Systemic chemotherapy may be utilized as prophylaxis against shunt metastases when CSF drainage is necessary.