Spinal Neurenteric Cyst in Association with Klippel-Feil Syndrome: Case Report and Literature Review

Can, Anil; Rubio, Ellianne J. dos Santos; Jasperse, Bas; Verdijk, Robert M.; Harhangi, Biswadjiet S.

doi:10.1016/j.wneu.2015.03.015

Cited by 25 publications

(24 citation statements)

References 37 publications

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“…7,12 In 50% of cases, they are accompanied by vertebral abnormalities such as the Klippel-Feil syndrome, spinal defects, and spondylolisthesis. 7,13,14 In the case presented, it was associated with posterior cutaneous cervical fistula and hemivertebra at level C3-C4.…”

Section: Discussionmentioning

confidence: 82%

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Cervical Neurenteric Cyst: A Case Report

2019

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Objective Neurenteric cysts account for 0.7–1.3% of all spinal cord tumors. These rare lesions are composed of heterotopic endodermal tissue. Methods A 26-year-old woman with a 13-month history of severe cervicalgia and brachial paresthesia. Clinically she had mildbilateral brachial paresis (4/5), generalized hyperreflexia and a left Babinski Sign. Past medical history was significant for a cervical fistula closure when she was 1yearold. The superior somatosensory evoked potentials revealed medullary axonal damage with a left predominance. A cervical magnetic resonance imaging of the neck was performed showing a dorsal homogeneous cystic intradural extramedullary lesion with high signal intensity on T2. Computed tomography revealed a Klippel-Feil syndrome. Results A posterior laminectomy and surgical excision were performed without complications. Post-operative follow-up showed a complete recovery of arm strength. The histopathological report confirmed the preoperative diagnosis of neurenteric cyst. Most neurenteric cysts are located in the spine, mainly in a ventral position. A total of 95% of neurenteric cysts are found in the intradural/intramedullary compartment. Symptomatic neurenteric cysts typically appear in the second and third decades of life and are 1.5 to 3 times more common in men. In 50% of the cases, other vertebral malformations have also been reported. In this case, a congenital dorsal enteric cyst and a Klippel-Feil syndrome were observed. Conclusions The intraspinal neurenteric cysts are rare lesions that must be included in the differential diagnosis of a dorsal, intradural cystic structure. The diagnosis may be overlooked, especially in cases of chronic neck pain without neurological deficit. Level of evidence V; Expert Opinion.

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Section: Discussionmentioning

confidence: 82%

“…Computed tomography of the cervical spine can reveal evidence of bone lesions or associated vertebral abnormalities, as it did in our case. 7,13 The treatment of choice is complete surgical resection. The reported rate of recurrence is from 0 to 37% regardless of the level of the lesion.…”

Section: Discussionmentioning

confidence: 99%

Cervical Neurenteric Cyst: A Case Report

2019

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“…Интраспинальные кисты чаще встреча-ются в нижнешейном и верхнегрудном отделах по-звоночника. Типичная локализация -интрадураль-ные экстрамедуллярные кисты, преимущественно вентрального расположения [5]. Эндодермальные кисты при МРТ-исследовании обычно имеют изо-или гипоинтенсивный сигнал на Т1-взвешенных изображениях и гиперинтенсивный сигнал на изо-бражениях T2-режима, не накапливают контраст-ное вещество.…”

Section: Discussionunclassified

“…Дифферен-циальный диагноз необходимо проводить с арахно-идальными, эпидермальными и дермоидными ки-стами. Несмотря на доброкачественный характер, эндодермальные кисты могут рецидивировать, по-этому основной метод хирургического лечения -тотальное удаление кисты [5,6].…”

Section: Discussionunclassified

Spinal stroke in a pregnant female with an endodermal cyst of the cervical spinal cord (a case report and literature review)

et al. 2016

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“…[ 1 , 5 – 9 ] Clinically, most patients present with progressive focal pain at the level of spinal axis of the lesion with myelopathic signs, and radicular symptoms being most common and often depends on the location of the pathology. [ 3 , 4 , 6 , 10 – 13 ] The simultaneous occurrence of neurenteric cysts and tethered cord syndrome has been seen in clinical practice. [ 1 , 2 , 14 – 18 ] Magnetic resonance imaging (MRI) now offers clinician the golden opportunity of diagnosing these disorders preoperatively.…”

Section: Introductionmentioning

confidence: 99%

Thoracolumbar spinal neurenteric cyst with tethered cord syndrome and extreme cervical lordosis in a child

et al. 2018

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Rationale:Neurenteric cysts, are rare benign tumors of the central nervous system that are mostly located in the spinal cord and predominantly seen in male children although adult form of the disorder also occurs. The etiology and treatment of this disorder is still a matter of debate. Our case further throws more light on the pathogenesis and treatment of this disorder.Patient concerns:A 4-year-old boy presented with 5-month history of cervical lordosis and bilateral lower extremity pain that progressed to his abdomen and upper body. The pain was general, recurrent, non-persistent and progressive in nature with no paralysis. The pain was aggravated by trunk stretching and relieved when he assumed opisthotonos position so he preferred sleeping in this position at night.Diagnoses:Magnetic resonance imaging (MRI) revealed a cystic lesion at the thoracolumbar spine with tethering of spinal cord and cervical lordosis.Interventions:He was operated on successfully and the cervical lordosis and pain resolved.Outcomes:The child recovered well with no tumor recurrence and massive improvement of his life.Lessons:The gold standard treatment for this disorder is surgery although the precise surgical approach is still a matter of debate. We are of the view that surgical approach should be individualized and aim at total excision of the cyst.

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Spinal Neurenteric Cyst in Association with Klippel-Feil Syndrome: Case Report and Literature Review

Cited by 25 publications

References 37 publications

Cervical Neurenteric Cyst: A Case Report

Cervical Neurenteric Cyst: A Case Report

Spinal stroke in a pregnant female with an endodermal cyst of the cervical spinal cord (a case report and literature review)

Thoracolumbar spinal neurenteric cyst with tethered cord syndrome and extreme cervical lordosis in a child

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