Klippel-Feil syndrome accompanied by partial cleft of the cervical spine: a not-so-unusual combination?

Vieira, Vítor Lopes Galvão; Bertholdo, Débora

doi:10.1590/0100-3984.2017.0123

Cited by 3 publications

(1 citation statement)

References 4 publications

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“…When the split cord malformation is partial, it is called partial diastematomyelia (spinal cord cleft), seen associated with Klippel–Feil syndrome. 23 Split cord malformations include a wide spectrum of spinal abnormalities, which may range from simple diastematomyelia to complete duplication of the spinal column with two hemicords.…”

Section: Discussionmentioning

confidence: 99%

Spinal Dysraphisms: A New Anatomical–Clinicoradiological Classification

Chellathurai

Kathirvelu

Mukkada

et al. 2021

Indian J Radiol Imaging

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Background Spinal dysraphisms refer to the congenital abnormalities of the spine and spinal cord due to aberrations in the processes of gastrulation, primary neurulation, and secondary neurulation. Embryology of many complex spinal dysraphisms are yet poorly understood and there is no agreeable anatomical–clinicoradiological classification with inclusion of recently documented and complex spinal dysraphisms. Aims and Objectives The main objective of this study was to review the imaging features of spinal dysraphisms with a better understanding of embryological abnormalities and propose a new classification inclusive of all complex and unusual dysraphisms based on anatomical and clinicoradiological correlation. Materials and Methods This was a retrospective single institutional observational study of 391 cases of spinal dysraphism for 10 years in our institution. Of 391 cases included in the study, 204 were males and 187 were females. Also, 123 cases belonged to the 0–6 months age group, 38 cases belonged to the 7–12 months age group, 156 belonged to the 1–5 years age group, 39 cases belonged to the 6–10 years age group, and 35 cases belonged to 10–20 years age group. Results An anatomical–clinicoradiological analysis of cases yielded a high proportion of cases of spinal lipomas, including lipomyeloceles and lipomyelomeningoceles (31.3%) and posterior myelomeningocele (14.2%). Anterior myelocoele (0.2%), sacral chordoma(0.2%), and intrasacral meningocele (0.2%) formed the least proportion of cases. A new classification was proposed based on the analysis of acquired data. Conclusion A structured approach in imaging spinal dysraphism is necessary for imaging evaluation in recent years. The proposed new classification based on clinicoradiological correlation and anatomic location is inclusive of unusual and complex dysraphisms.

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Section: Discussionmentioning

confidence: 99%

Spinal Dysraphisms: A New Anatomical–Clinicoradiological Classification

Chellathurai

Kathirvelu

Mukkada

et al. 2021

Indian J Radiol Imaging

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Klippel-Feil syndrome cases from Slovakia

Hukeľová

Krošláková

2021

International Journal of Paleopathology

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High Cervical Partial Posterior Cord Cleft in a Case of Klippel-Feil Syndrome

Arora¹,

Ahluwalia²,

Mittal³

et al. 2021

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Posterior high cervical spinal cord cleft has been rarely reported in association with Klippel - Feil syndrome (KFS). Its presence may have prognostic value in long term neurological outcome. If there is segmentation anomaly of two or more vertebrae during embryonic life mainly involving the cervical vertebrae, it can result in Klippel - Feil syndrome. It is a rare anomaly and is present with the incidence of 1 : 42000 births.1 During 2nd to 8th week of embryonic life, the segmentation of the mesodermal somites of the spine in the cervical region fails and leads to cervical spine synostosis which is also known as KFS. Although most commonly the KFS patients present with the symptoms of short length of the neck, hairline posteriorly is low and the mobility in the upper part of the spine is restricted. It is also sometimes associated with other congenital anomalies like Sprengel’s deformity, hemivertebra, basilar impression, cleft palate, and many more rare anomalies.2 Most commonly the patients having this syndrome show restriction of the motion in the neck region.3

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Klippel-Feil syndrome accompanied by partial cleft of the cervical spine: a not-so-unusual combination?

Cited by 3 publications

References 4 publications

Spinal Dysraphisms: A New Anatomical–Clinicoradiological Classification

Spinal Dysraphisms: A New Anatomical–Clinicoradiological Classification

Klippel-Feil syndrome cases from Slovakia

High Cervical Partial Posterior Cord Cleft in a Case of Klippel-Feil Syndrome

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