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Intracranial Tumor Presenting As Raymond Syndrome In A Pediatric Patient

Tomas-Esteban Danica ¹

Dizon April ² Ang Lourdes ² Reyes Karen ²

1 Resident, Rizal Medical Center, Pasig City, Philippines

2 Consultant, Rizal Medical Center, Pasig City, Philippines

Abstract

This is a case of a pediatric patient who presented with a sudden onset of right abducens nerve palsy with contralateral hemiplegia with no facial paralysis. With the constellation of symptoms aided by the presence of enhancing pontine mass on magnetic resonance imaging, the presence of diffuse intrinsic pontine glioma (DIPG) was presumed to have caused the findings consistent with the common type of Raymond syndrome.

Author Contributions

Received Oct 26, 2021 Accepted Nov 07, 2021 Published Nov 12, 2021

Copyright© 2021 Tomas-Esteban Danica, et al.
License
This work is licensed under a Creative Commons Attribution 4.0 International License. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Competing interests

The authors have declared that no competing interests exist.

Funding Interests:

Citation:

Tomas-Esteban Danica, Dizon April, Ang Lourdes, Reyes Karen et al. (2021) Intracranial Tumor Presenting As Raymond Syndrome In A Pediatric Patient Journal of Ophthalmic Science. - 2(4):18-21

DOI 10.14302/issn.2470-0436.jos-21-4003

Introduction

Raymond syndrome is one of the ocular motor clinical syndromes that is mainly characterized by ipsilateral abducens nerve palsy and contralateral hemiparesis1 or hemiplegia.2 The lesion in these cases affects the ventromedial pons where the abducens nerve fascicle and nondecussated corticofacial and corticospinal tract are located.1There are two syndrome types depending on the structures involved: 1. classic type, involving the ipsilateral abducens fascicle, undecussated corticofacial and corticospinal fibers, and 2. common type, caused by a lesion involving the ipsilateral abducens fascicle and non-decussated corticospinal while sparing the corticofacial fibers.3

The objective of this paper is to report a case of a pediatric patient with medial deviation of the right eye associated with left sided hemiplegia, diagnosed as a case of Raymond Syndrome (common type), which is an extremely rare neurologic condition among pediatric patients.

Discussion

An injury to the ventral pons results to either Raymond and Millard-Gubler syndromes; each can be differentiated generally by the affectation to the facial nerve with Raymond presenting as alternating ipsilateral abducens weakness with contralateral hemiplegia while Millard-Gubler manifesting with ipsilateral facial paresis. However, with the advancement in cranial imaging, neuroanatomical correlation has been widely explored and it was found out that contralateral central facial paresis may also occur at the pontine base as what is seen in the classic type of Raymond syndrome. 3

In general, few cases of Raymond syndrome had been reported 49, mainly of the common type and less of the classic type3. Furthermore, pure Raymond syndrome is an extremely rare neurologic disorder as many structures present along the abducens nerve fascicle may be affected.4

Raymond syndrome is a posterior circulatory stroke syndrome11 mostly affecting adults, however, may also be seen in children. An abducens nerve paresis in a young child raises the suspicion for Diffuse Intrinsic Pontine Glioma (DIPG) since it is regarded as a highly sensitive predictor 7 and magnetic resonance imaging is usually diagnostic.8 DIPG, histologically characterized as astrocytoma, is a fatal brain tumor, accounting for 10-20% in affected pediatric population.10. In this case, Raymond Syndrome brought about by DIPG was a primary consideration on the basis of the presentation of sudden onset of sixth nerve palsy in a young patient supported by the typical radiologic findings consistent with glioma. Furthermore, surgical debulking is a challenge because of its eloquent brainstem location, making radiation therapy the only proven treatment in prolonging progression-free survival.5

Journal of Ophthalmic Science