0000000000362330
AUTHOR
S. Hofmann-v. Kap-herr
The clinical relevance of non-classified dysganglionoses and implications for a new grading system
In addition to the classified types of dysganglionosis, certain non-classified dysganglionoses (NCD) (types 1-4) were introduced by Meier-Ruge in 1992. Clinical data on these conditions are limited. Among 134 children with intestinal dysganglionoses (ID) treated from 1979 to 1999, 12 were identified to have a NCD. Their clinical course is presented. The existence of mild ID (type 1) is difficult to demonstrate. Current definitions and data on clinical relevance are not convincing. An indication for surgical treatment is not present. Isolated hypogenesis of the submucous plexus (SMP) (type 2, n = 8) is clinically a more severe kind of intestinal neuronal dysplasia type B and often requires e…
The diagnostic significance of cholescintigraphy and ultrasound examination in cholestatic syndromes in infancy
Thirty-three neonates and infants with cholestatic syndromes of various etiologies were evaluated by cholescintigraphy and ultrasound examinations. The results of these two diagnostic procedures were compared with the final diagnosis as confirmed by liver biopsy and/or laparotomy and the clinica follow-up of the infants. Fourteen patients had an obstructive cholangiopathy (11 biliary atresia, 2 choledochal cysts, 1 congenital choledochal stenosis), 13 had neonatal hepatitis, and 6 had cholestasis of another etiology. Typical ultrasonic and/or scintigraphic findings confirmed or excluded the need for surgical exploration in 28 patients. Thus, for must infants with cholestatic syndromes it is…
Complex long-segment intestinal dysganglionosis.
A case is reported with aganglionosis of the rectum, sigma, and descending colon; dysganglionosis with heterotopic ganglionic cells in the muscularis propria of the hypoganglionic transverse colon; and extreme hypoganglionosis (without detection of ganglionic cells) of the ascending colon and distal ileum. The ileum showed a transition zone with hypoganglionosis and intestinal neuronal dysplasia (IND) type B. As to the etiology of such complex intestinal innervation defects, pre- and perinatal perfusion deficits must be considered because their localization seems to be linked to the vascular anatomy of the colon. Early diagnosis may be difficult, causing a delay in operative treatment and m…
Die Bedeutung der Thoraxverletzung beim multitraumatisierten Kind
Anhand von zwei Studien wird der Stellenwert der Thoraxverletzung beim multitraumatisierten Kind dargelegt. Die Zahl der Thoraxverletzten im Rahmen des Multitraumas hat um 8% auf 34% zugenommen. Schwerere Einzelverletzungen werden trotz Ruckganges des Multitraumas beobachtet. Thoraxverletzungen treten als zentrales Trauma auf; deshalb weist die Extremitatenperipherie nur selten zusatzliche Verletzungen auf. Die altersabhangig unterschiedlichen Blutvolumina, der besondere Flussigkeitsstoffwechsel, die verschiedenen Elastizitatsperioden des Brustkorbes und altersabhangige Lungenvolumina mussen bei der Beurteilung solcher Verletzungen genau beachtet werden.
Diagnostik und operative Therapie von Schilddrüsenerkrankungen im Kindesalter
Schilddrusenerkrankungen im Kindesalter fuhren nur selten zu einer operativen Therapie und erfordern dann ein differenziertes operatives Vorgehen und eine gute interdisziplinare Zusammenarbeit von Padiatern, Kinderchirurgen und Nuklearmedizinern, insbesondere in der Nachsorge. Dadurch lassen sich funktionell und prognostisch sehr gute Ergebnisse erzielen. Das Ausmas der Radikalitat beim kleinen, intrakapsularen papillaren Karzinom (low risk) ist weiterhin in Diskussion.