Atopic dermatitis and congenital defects, disease, and mortality.
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This study comprised 7 patients with longitudinal anomalies of the knee and leg classified in accordance with the 'classification of congenital limb deficiencies' introduced by Frantz and O'Rahilly. The anomalies were paraxial hemimelia of the fibula (4 instances), paraxial hemimelia of the tibia (6 instances). In the absence of the proximal tibia, therapy consisted of knee reconstruction according to Brown. In the absence of the distal part of the tibia a proximal tibiofibular synostosis was performed. In one instance corrective osteotomy of the tibia was required in the absence of the fibula. Exarticulation according to Syme was resorted in order to cope with an incorrigible abnormal position of the foot and for leg length inequality. The therapy should aim at giving the child walking ability at a normal time. Exarticulation has to be preferred to amputation in the treatment of these patients.
Unrecognized spontaneous cerebrospinal fluid (CSF) otorrhea led to recurrent bacterial meningitis in three children. The underlying cause of the spontaneous CSF otorrhea was proved to be a congenital cystic dilatation of the cochlear aqueduct and Mondini dysplasia of the temporal bone. The CSF leak recurred in all patients after an initial surgical attempts to close the defects through a tympanotomy. A suboccipital approach was used successfully to obliterate the CSF fistulas in two children; a translabyrinthine approach was used in the other. A search of the literature revealed that Mondini dysplasia is a congenital anomaly that can commonly cause spontaneous CSF otorrhea in children. The authors discuss the management of this uncommon entity on the basis of a literature review and their own experience.
The author presents data on the prevalence of birth defects in the Czech Republic during the period from 1990-1996. The paper contains also data on results of prenatal diagnosis of birth defects in the Czech Republic during the same period and indicates achievements in secondary prevention of abnormal phenotypes, which contributed in a major way to the reduced incidence of some types of inborn defects in the population.
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