Aim-To assess the value of contrast versus plain radiography in determining radio-opaque long line tip position in neonates. Methods-In a prospective study, plain radiography was performed after insertion of radio-opaque long lines. If the line tip was not visible on the plain film, a second film with contrast was obtained in an attempt to visualise the tip. Results-Sixty eight lines were inserted during the study period, 62 of which were included in the study. In 31, a second radiographic examination with contrast was necessary to determine position of the tip. In 29 of these, the line tip was clearly visualised with contrast. On two occasions, the line tip could not be seen because the contrast had filled the vein and obscured the tip from view. Eight of the lines that required a second radiograph with contrast were repositioned. Conclusion-Intravenous contrast should be routinely used in the assessment of long line position in the neonate. (Arch Dis Child Fetal Neonatal Ed 2001;84:F129-F130)
We report the rare presentation of Menkes disease with a congenital skull fracture, intracerebral bleeding, and seizures. The diagnosis was made at 3 months of age based on the characteristic features of the syndrome, by which time the child experienced uncontrollable seizures. Following progressive neurodegeneration, death occurred at 3 years of age. The prognosis in Menkes disease is dependent on early copper‐histidine therapy. Effective treatment has led to children surviving into adulthood. Diagnosing the syndrome during the neonatal period is difficult. There are no published reports of congenital skull fracture as a presenting sign of Menkes disease. It is concluded that Menkes disease should be considered in any child who presents with congenital skull fracture as early diagnosis and treatment significantly improve the outcome.
We report the rare presentation of Menkes disease with a congenital skull fracture, intracerebral bleeding, and seizures. The diagnosis was made at 3 months of age based on the characteristic features of the syndrome, by which time the child experienced uncontrollable seizures. Following progressive neurodegeneration, death occurred at 3 years of age. The prognosis in Menkes disease is dependent on early copper-histidine therapy. Effective treatment has led to children surviving into adulthood. Diagnosing the syndrome during the neonatal period is difficult. There are no published reports of congenital skull fracture as a presenting sign of Menkes disease. It is concluded that Menkes disease should be considered in any child who presents with congenital skull fracture as early diagnosis and treatment significantly improve the outcome.
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