Zeitschrift für Kinderchirurgie : organ der Deutschen, der Schweizerischen und der Osterreichischen Gesellschaft für Kinderchirurgie = Surgery in infancy and childhood
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The enzyme histotopochemical characteristics of 18 cases with ultrashort Hirschsprung's disease - investigated during the past 20 years - are analysed. The aganglionic segment, having an extension of 1-3 cm, is characterised by an increase of acetylcholinesterase activity in parasympathetic nerve fibres of the muscularis mucosae. A similar increase of acetylcholinesterase in the nerve fibres of the lamina propria mucosae - as characteristic in Hirschsprung's disease - is usually absent. ⋯ Ultrashort Hirschsprung is a rare disease, although it is observed more often than total aganglionosis of the colon (Zuelzer-Wilson syndrome). The sex ratio, being 5 male male: 1 female, is within the range of Hirschsprung's disease. Hence, enzyme histotopochemical means enable substantiation of ultrashort Hirschsprung's disease as a special form of an aganglionosis of the distal rectum.
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Case Reports
[Scheuthauer-Marie-Sainton syndrome. Differential diagnosis and treatment of clavicular deformity].
The genesis and clinical characteristics of Scheuthauer-Marie-Sainton's syndrome (dysostosis cleido-cranialis), of congenital pseudoarthrosis of the clavicula of post-traumatic clavicular pseudoarthrosis in childhood determine the differential diagnostic considerations of pseudoarthrosis in children in cases which are initially not clear. The therapy is, however, independent of the cause of the disease: Resection of the pseudoarthrotic bone, filling of the defect via a cortico-spongious block and stabilisation via plate osteosynthesis.
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Biography Historical Article
[Johannes Fatio: his life, works and terrible end (farewell lecture 26 January 1984)].
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Urachal cysts are rare and usually as asymptomatic until they become infected. The route the infection follows is uncertain. A very unusual case is reported of a newborn female afflicted with a posteriorly ruptured urachal cyst causing incapsulated peritonitis, in which we obtained a visualization of the cyst during fistulography
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Forty children with severe head injury were studies retrospectively. All were admitted to the medical center within 6 hours after injury. Seventeen had Glasgow Coma Scales of 3 to 4 and 23 scales of 5 to 7. ⋯ In contrast, survivors usually had patent basal cisterns on CT, normal coagulation data or only moderate CC and slight to rarely moderate intracranial hypertension. It is concluded that by using the proposed criteria, early assessment of severity and prediction of outcome after severe paediatric head injury is possible. In contrast to the Glasgow Coma Scale these criteria are applicable and retain predictive power also in children who receive early and intensive ICP-lowering therapy.