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Pediatric Reports
Volume 9
Issue 2
10.4081/pr.2017.7211
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Case Report

Adams-Oliver Syndrome with Unusual Central Nervous System Findings and an Extrahepatic Portosystemic Shunt

by
Carlos Pérez-García
1,*,
Yolanda Ruíz Martín
1,
Alejandra Aguado del Hoyo
2,
Carlos Marín Rodríguez
2 and
Minia Campos Domínguez
1
1
Department of Radiology, General University Hospital Gregorio Marañón, Madrid, Spain
2
Department of Pediatric Radiology, Mother and Child Hospital Gregorio Marañón, Madrid, Spain
3
Department of Pediatric Dermatology, Mother and Child Hospital Gregorio Marañón, Madrid, Spain
*
Author to whom correspondence should be addressed.
Pediatr. Rep. 2017, 9(2), 7211; https://doi.org/10.4081/pr.2017.7211
Submission received: 5 May 2017 / Revised: 5 May 2017 / Accepted: 5 June 2017 / Published: 26 June 2017

Abstract

We report a case of a premature neonate girl with scalp and skull defects and brachydactyly of the feet consistent with an Adams-Oliver syndrome (AOS). The patient had central nervous system abnormalities, such as periventricular calcifications, hypoplastic corpus callosum, and bilateral hemispheric corticosubcortical hemorrhagic lesions. A muscular ventricular septal defect and a portosystemic shunt were diagnosed. To our knowledge, this is the first report of congenital supratentorial grey-white matter junction lesions without dural sinus thrombosis in association with AOS. Some of these lesions may be secondary to birth trauma (given the skull defect) whilst others have a watershed location, perhaps as further evidence of vascular disruption and decreased perfusion during critical periods of fetal brain development as the previously proposed pathogenesis of this syndrome.
Keywords: Adams-Oliver Syndrome; Periventricular calcifications; Portosystemic-shunt; Pediatric Neuroradiology Adams-Oliver Syndrome; Periventricular calcifications; Portosystemic-shunt; Pediatric Neuroradiology

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MDPI and ACS Style

Pérez-García, C.; Martín, Y.R.; del Hoyo, A.A.; Rodríguez, C.M.; Domínguez, M.C. Adams-Oliver Syndrome with Unusual Central Nervous System Findings and an Extrahepatic Portosystemic Shunt. Pediatr. Rep. 2017, 9, 7211. https://doi.org/10.4081/pr.2017.7211

AMA Style

Pérez-García C, Martín YR, del Hoyo AA, Rodríguez CM, Domínguez MC. Adams-Oliver Syndrome with Unusual Central Nervous System Findings and an Extrahepatic Portosystemic Shunt. Pediatric Reports. 2017; 9(2):7211. https://doi.org/10.4081/pr.2017.7211

Chicago/Turabian Style

Pérez-García, Carlos, Yolanda Ruíz Martín, Alejandra Aguado del Hoyo, Carlos Marín Rodríguez, and Minia Campos Domínguez. 2017. "Adams-Oliver Syndrome with Unusual Central Nervous System Findings and an Extrahepatic Portosystemic Shunt" Pediatric Reports 9, no. 2: 7211. https://doi.org/10.4081/pr.2017.7211

APA Style

Pérez-García, C., Martín, Y. R., del Hoyo, A. A., Rodríguez, C. M., & Domínguez, M. C. (2017). Adams-Oliver Syndrome with Unusual Central Nervous System Findings and an Extrahepatic Portosystemic Shunt. Pediatric Reports, 9(2), 7211. https://doi.org/10.4081/pr.2017.7211

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