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Case Reports
. 2008 Oct 15;146A(20):2688-90.
doi: 10.1002/ajmg.a.32515.

Congenital Lipomatous Overgrowth, Vascular Malformations, and Epidermal Nevi (CLOVE) Syndrome: CNS Malformations and Seizures May Be a Component of This Disorder

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Case Reports

Congenital Lipomatous Overgrowth, Vascular Malformations, and Epidermal Nevi (CLOVE) Syndrome: CNS Malformations and Seizures May Be a Component of This Disorder

Zoran S Gucev et al. Am J Med Genet A. .
Free PMC article

Abstract

A newborn girl was found to have a massive lymphatic truncal vascular malformation with overlying cutaneous venous anomaly associated with overgrown feet and splayed toes. These manifestations comprise the recently described CLOVE syndrome. She also had cranial asymmetry and developed generalized seizures, which were treated with anticonvulsants. Cranial CT showed encephalomalacia, widening of the ventricles and the sulci, hemimegalencephaly (predominantly white matter) and partial agenesis of corpus callosum. Review of the literature identified several other patients with CLOVE syndrome, some of whom were misdiagnosed as having Proteus syndrome, with strikingly similar manifestations. We conclude that CNS manifestations including hemimegalencephaly, dysgenesis of the corpus callosum, neuronal migration defects, and the consequent seizures, may be an rarely recognized manifestation of CLOVE syndrome.

Figures

Figure 1
Figure 1
Figure 1. A. The patient at 15 days of age, with a massive subcutaneous truncal overgrowth with overlying vascular malformation. Figure 1. B. The overgrowth of her right and left feet, respectively, with splaying of the digits. Figure 1. C. Plain radiographs of the feet show normal bony architecture, in contrast to the disorganized architecture of Proteus syndrome.
Figure 1
Figure 1
Figure 1. A. The patient at 15 days of age, with a massive subcutaneous truncal overgrowth with overlying vascular malformation. Figure 1. B. The overgrowth of her right and left feet, respectively, with splaying of the digits. Figure 1. C. Plain radiographs of the feet show normal bony architecture, in contrast to the disorganized architecture of Proteus syndrome.
Figure 1
Figure 1
Figure 1. A. The patient at 15 days of age, with a massive subcutaneous truncal overgrowth with overlying vascular malformation. Figure 1. B. The overgrowth of her right and left feet, respectively, with splaying of the digits. Figure 1. C. Plain radiographs of the feet show normal bony architecture, in contrast to the disorganized architecture of Proteus syndrome.
Figure 2
Figure 2
Her cranial CT shows encephalomalacia, enlarged ventricles and sulci, hemimegalencephaly (predominantly white matter), and partial agenesis of corpus callosum.

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