The outcome data of 132 patients treated at the Department of Pediatric Surgery in Mainz during the last 25 years were reviewed. Prenatal diagnosis of abdominal wall defects (AWDs) and associated malformations led to increasing selection of the patient population. The aim of primary closure of the abdominal wall can be achieved more frequently in gastroschisis (GS) than omphalocele (OC), while the postoperative course is more complicated and of longer duration in GS. Delayed or secondary closure extended the hospitalization period but had no negative effects on the outcome. Reoperations or planned secondary operations were performed in 23 patients with GS and 14 with OC. Early mortality was 15/55 for OC and 21/77 for GS over the period of 25 years. In recent years, a drastic reduction in mortality has occurred, and mortality is now mainly due to additional malformations. Further development and quality of life are not significantly reduced after survival of an isolated AWD. Malpositioning of parenchymatous organs after closure of AWDs has to be considered during pregnancy and abdominal operations.
We evaluated in this study the value of preoperative examinations of anal atresia with an optimised MRI strategy. 13 patients with anorectal anomalies, most of them younger than one year, underwent an MRI examination of the pelvis. 10 of these patients were operated afterwards. Compared with the situs shown by the operation the results of the MRI examination were very exact in all cases in respect of the level of atresia and the development of the striated muscle complex. Fistulas could be completely described in most cases. MRI demonstrated additional lesions in some patients which were of great importance for planning the further treatment of the patients. As a consequence MRI can give valid information on anal atresia for planning the operative treatment.
A 14 years old boy with Kirner's deformity is demonstrated. He has the typical radial and volar deviation of the terminal phalanges of the 5th finger. Nature and inheritance of the disease are discussed. We assume inborn ossification disturbances. Most authors recommend to avoid any therapy. But we feel that nowadays it is possible to use plaster splints in young children or to do a corrective operation in older ones with good success.
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