Staged surgical treatment of brachioplexopathy in an adolescent with Klippel-Feil syndrome: a rare clinical case and literature review
https://doi.org/10.14531/ss2021.1.6-13
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.
About the Authors
E. V. Petrova
H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery
64/68 Parkovaya str., Pushkin, St. Petersburg, 198412, Russia
Russian Federation
Russian Federation
MD, PhD, senior researcher, Department of Arthrogryposis
O. E. Agranovich
H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery
64/68 Parkovaya str., Pushkin, St. Petersburg, 198412, Russia
Russian Federation
Russian Federation
DMSc, Head of the Department of Arthrogryposis
M. V. Savina
H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery
64/68 Parkovaya str., Pushkin, St. Petersburg, 198412, Russia
Russian Federation
Russian Federation
MD, PhD, Head of the Laboratory of Physiological and Biomechanical Research
E. L. Gabbasova
H. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery
64/68 Parkovaya str., Pushkin, St. Petersburg, 198412, Russia
Russian Federation
Russian Federation
neurologis, Department of Arthrogryposis
V. P. Snishchuk
Leningrad Regional Children’s Clinical Hospital
6 Komsomola str., St. Petersburg, 195009, Russia
Russian Federation
Russian Federation
neurosurgeon, chief external expert for pediatric neurosurgery of the Leningrad Region Healthcare Committee
A. Yu. Mushkin
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,
Russian Federation
Russian Federation
DMSc, Prof., chief researcher, Head of Clinic of Pediatric Surgery and Orthopedics, Head of the Scientific and Clinical Centre for Spinal Pathology; Professor of the Department of traumatology and orthopedics
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Review
For citations:
Petrova E.V., Agranovich O.E., Savina M.V., Gabbasova E.L., Snishchuk V.P., Mushkin A.Yu. Staged surgical treatment of brachioplexopathy in an adolescent with Klippel-Feil syndrome: a rare clinical case and literature review. Russian Journal of Spine Surgery (Khirurgiya Pozvonochnika). 2021;18(1):6-13. https://doi.org/10.14531/ss2021.1.6-13
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