Staged surgical treatment of brachioplexopathy in an adolescent with Klippel-Feil syndrome: a rare clinical case and literature review-Reference-Cited by-同舟云学术

Staged surgical treatment of brachioplexopathy in an adolescent with Klippel-Feil syndrome: a rare clinical case and literature review

Published:2021-04-06 Issue:1 Volume:18 Page:6-13
ISSN:2313-1497
Container-title:Hirurgiâ pozvonočnika (Spine Surgery)
language:
Short-container-title:Hir. pozvonoč.

Author:

Petrova E. V.¹^ORCID,Agranovich O. E.¹^ORCID,Savina M. V.¹^ORCID,Gabbasova E. L.¹^ORCID,Snishchuk V. P.²^ORCID,Mushkin A. Yu.³^ORCID

Affiliation:

1. H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery 64/68 Parkovaya str., Pushkin, St. Petersburg, 198412, Russia

2. Leningrad Regional Children’s Clinical Hospital 6 Komsomola str., St. Petersburg, 195009, Russia

3. St. Petersburg Research Institute of Phthisiopulmonology; Pavlov First Saint Petersburg State Medical University 32 Politekhnicheskaya str., St. Petersburg, 194064, Russia; 6–8 L’va Tolstogo str., St. Petersburg, 197022, Russia,

Abstract

Klippel-Feil syndrome is a congenital malformation, the leading component of which is a violation of segmentation of the cervical vertebral bodies. The syndrome can be combined with other skeletal anomalies: skull asymmetry, scoliosis, high shoulder blades, and cervical ribs. Treatment of the syndrome is usually symptomatic; indications for surgical treatment are progressive neurological disorders and persistent pain syndrome, which usually develop due to instability of unblocked segments, or neurogenic pain. A clinical case of treatment of a 17-year-old patient with Klippel-Feil syndrome who developed a picture of severe upper limb monoparesis during three years due to compression of the brachial plexus associated with cervical ribs is presented. Decompression of the brachial plexus was performed, which led to rapid relief of pain syndrome and gradual partial regression of motor disorders. Due to incomplete restoration of the gripping function, tendon-muscle plasty of the right hand was performed, which significantly improved the possibility of self-care. The results of radiation and staged neurophysiological studies are described, as well as a review of the literature on the Klippel-Feil syndrome.

Publisher

Association of Spine Surgeons

Subject

Anesthesiology and Pain Medicine,Orthopedics and Sports Medicine,Surgery

Reference20 articles.

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