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Background Incontinentia pigmenti (IP) is a rare X-linked dominant disorder that involves ectodermal tissues of multiple systems. Previous reports are few in Taiwan. To contribute toward better understanding of IP, we describe and discuss the clinical features of cases that were diagnosed in a medical center during the past 12 years. Methods The medical records of all patients with IP between July 1995 and June 2007 were reviewed retrospectively. The demographics, physical findings, pathology reports, molecular study reports, eosinophil counts and outcome were recorded. Results A total of 4 patients, 3 female and 1 male neonate, who met the criteria for the diagnosis of IP were enrolled. Among these cases, 3 were not diagnosed with IP at initial presentation but were regarded to have infectious diseases. A definite family history of 3 consecutive generations was proved not only by clinical manifestations but also by molecular study in 1 patient. The patient also had retinal and vitreous body hemorrhage, which rapidly progressed to retinal detachment of the right eye in 2 months. Another patient presenting with stage III hyperpigmentation at birth had an extremely rare finding of left foot deformity. The male patient had unilateral and localized vesicular lesions over his left thigh. Conclusion Diagnosis of IP is difficult in the neonatal period. Referral to experienced specialists is necessary. Multiple clinical characteristics of IP and rapid progression of ophthalmologic manifestations can be demonstrated through our study. Furthermore, 3 of the 4 cases in our study are the very first reports in Taiwan.

We report the case of a newborn who suffered right atrial laceration during delivery. To our knowledge, there has been no previous report of blunt injury causing right atrial laceration with hemopericardium complicated by cardiac tamponade in the perinatal period.

Background: Central venous catheterization is an important procedure for infant patients for a number of different purposes, including nutritional support, surgical operation, hemodynamic monitoring, and multiple lines for critical care medications. Subclavian vein catheterization (SVC) is one of the central vein catheterization techniques. SVC can be performed from 4 different locations: right supraclavicular (RSC), left supraclavicular (LSC), right infraclavicular (RIC), and left infraclavicular (LIC). The purpose of this study was to evaluate the relative effectiveness and complication risks of these 4 SVC locations in infants. Methods: In our pediatric intensive care unit, which is part of a tertiary medical center, a well-trained fellow doctor performed the following catheterizations: 21 RSC, 24 LSC, 24 RIC, and 22 LIC, for a total of 91 SVC operations in infants. The patients were placed in the Trendelenburg position. The site of puncture was decided by the operator. Statistical significance was analyzed according to Fisher's exact test and 2-sample t test. Results: The overall success rate was 90.1% (82 out of 91 operations). No statistically significant differences were noted among these 4 groups, either in the success or complication rate. There were 6 cases of arterial puncture (5 supraclavicular and 1 infraclavicular, p = 0.09), 2 cases of pneumothorax (1 RSC and 1 RIC), and 2 cases of malpositioned catheter (1 RSC and 1 RIC). There was no mortality. Conclusion: In our study, we found that there was no statistically significant difference among the 4 SVC locations in effectiveness of operation or in risk of complication. There was a tendency to damage the subclavian arteries through the supraclavicular route. (J Chin Med Assoc 2006;69(4):153-156)