1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-2-95-a 10.15844/pedneurbriefs-2-12-10 Vascular Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Transposition Of Great Vessels And Mobius Syndrome http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 12 1988 01 08 2016 2 12 95 95 Copyright: © 1988 The Author(s) 1988 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Möbius syndrome and transposition of the great vessels

The vascular theory of embryopathogenesis for Mobius syndrome is proposed in a case report of a 3-month-old boy from the University of New Mexico School of Medicine, Albuquerque, NM.

 Vascular Theory Mobius Syndrome Muscular Tissue

The vascular theory of embryopathogenesis for Mobius syndrome is proposed in a case report of a 3-month-old boy from the University of New Mexico School of Medicine, Albuquerque, NM. At birth, he had bilateral facial and abducens palsies, left acheiria (congenital absence of the hand), and transposition of the aorta and pulmonary artery. He was lethargic, cyanotic, and in respiratory distress, and he expired after an arterial switch procedure with closure of a septal defect. The authors cite 2 reports of Mobius syndrome with cardiac anomalies, both presenting with dextrocardia and the Poland anomaly (unilateral hypoplasia or absence of pectoral muscles, nipple, and upper limb). An intrapartum insult during the fourth to seventh week of gestation is consistent with the vascular theory of embryopathogenesis. [1]

COMMENT. Congenital facial diplegia and abducens palsy, Mobius syndrome, has been explained as either a primary hypoplasia of cranial nerve nuclei or a primary deficiency of the muscles derived from the first two branchial arches. A dysgenesis of both neural and muscular tissue has been proposed in some cases. In the above case report, the concomitant occurrence of the vascular anomaly supports the theory of impaired cranial nerve nuclear development due to interruption of vascular supply at or around the sixth intrauterine week.

 Raroque HG Jr Hershewe GL Snyder RD Möbius syndrome and transposition of the great vessels Neurology 1988 Dec 38 12 1894 5 10.1212/WNL.38.12.1894 3194069