Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patient

Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patient

Neurenteric cysts (NECs), also called enterogenous cysts or enterogenic cysts, are congenital malformative anomalies of endodermal origin that manifest with a variety of disorders, including spine anomalies. Neurenteric cysts are uncommon developmental disorders reported in 0.7%–1.3% of all spinal t...

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Main Authors: Vito Pavone, Andrea D. Praticò, Rosario Caltabiano, Giuseppe Barbagallo, Raffaele Falsaperla, Piero Pavone, Saveria Spadola, Martino Ruggieri
Format: Article
Language:English
Published: Elsevier 2017-09-01
Series:Journal of Pediatric Surgery Case Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2213576617301057
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spelling doaj-c428388e238540199e401058f23487272020-11-24T22:02:37ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662017-09-01241216Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patientVito Pavone0Andrea D. Praticò1Rosario Caltabiano2Giuseppe Barbagallo3Raffaele Falsaperla4Piero Pavone5Saveria Spadola6Martino Ruggieri7Orthopaedic Clinic, University of Catania, Azienda Ospedaliera Universitaria, Policlinico Vittorio Emanuele, Catania, ItalyDepartment of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, Italy; Department of Biomedical and Biotechnological Sciences, University of Catania, Catania, Italy; Corresponding author. Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, Italy.Department G.F. Ingrassia, Section of Anatomic Pathology, University of Catania, ItalyUnit of Neurosurgery, University Hospital “Policlinico-Vittorio Emanuele”, Catania, ItalyUnit of Pediatrics and Pediatric Emergency, University Hospital “Policlinico-Vittorio Emanuele”, Catania, ItalyUnit of Pediatrics and Pediatric Emergency, University Hospital “Policlinico-Vittorio Emanuele”, Catania, ItalyDepartment G.F. Ingrassia, Section of Anatomic Pathology, University of Catania, ItalyDepartment of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, ItalyNeurenteric cysts (NECs), also called enterogenous cysts or enterogenic cysts, are congenital malformative anomalies of endodermal origin that manifest with a variety of disorders, including spine anomalies. Neurenteric cysts are uncommon developmental disorders reported in 0.7%–1.3% of all spinal tumors. Klippel-Feil syndrome (KFS) defines a malformative spine disorder presenting with congenital fusion of cervical vertebrae and/or other parts of the spine. In patients with KFS, NECs are rarely reported; they may be silent for long periods of time, showing a slow progressive course or manifesting with an acute, severe neurological presentation or with fluctuating myelopathic symptoms. We report a young patient affected by KFS associated with a NEC which, in a short period of time, progressively caused myelopathic symptomatology. Surgical intervention resulted in resolution of the neurological signs. Keywords: Neurenteric cyst, Klippel-Feil syndrome, Intramedullary cysthttp://www.sciencedirect.com/science/article/pii/S2213576617301057
collection DOAJ
language English
format Article
sources DOAJ
author Vito Pavone
Andrea D. Praticò
Rosario Caltabiano
Giuseppe Barbagallo
Raffaele Falsaperla
Piero Pavone
Saveria Spadola
Martino Ruggieri
spellingShingle Vito Pavone
Andrea D. Praticò
Rosario Caltabiano
Giuseppe Barbagallo
Raffaele Falsaperla
Piero Pavone
Saveria Spadola
Martino Ruggieri
Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patient
Journal of Pediatric Surgery Case Reports
author_facet Vito Pavone
Andrea D. Praticò
Rosario Caltabiano
Giuseppe Barbagallo
Raffaele Falsaperla
Piero Pavone
Saveria Spadola
Martino Ruggieri
author_sort Vito Pavone
title Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patient
title_short Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patient
title_full Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patient
title_fullStr Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patient
title_full_unstemmed Cervical neurenteric cyst and Klippel-Feil syndrome: An abrupt onset of myelopathic signs in a young patient
title_sort cervical neurenteric cyst and klippel-feil syndrome: an abrupt onset of myelopathic signs in a young patient
publisher Elsevier
series Journal of Pediatric Surgery Case Reports
issn 2213-5766
publishDate 2017-09-01
description Neurenteric cysts (NECs), also called enterogenous cysts or enterogenic cysts, are congenital malformative anomalies of endodermal origin that manifest with a variety of disorders, including spine anomalies. Neurenteric cysts are uncommon developmental disorders reported in 0.7%–1.3% of all spinal tumors. Klippel-Feil syndrome (KFS) defines a malformative spine disorder presenting with congenital fusion of cervical vertebrae and/or other parts of the spine. In patients with KFS, NECs are rarely reported; they may be silent for long periods of time, showing a slow progressive course or manifesting with an acute, severe neurological presentation or with fluctuating myelopathic symptoms. We report a young patient affected by KFS associated with a NEC which, in a short period of time, progressively caused myelopathic symptomatology. Surgical intervention resulted in resolution of the neurological signs. Keywords: Neurenteric cyst, Klippel-Feil syndrome, Intramedullary cyst
url http://www.sciencedirect.com/science/article/pii/S2213576617301057
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