Combination of the spinal hernia, sprengel’s deformity, and situs viscerum inversus

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Background. The combination of Sprengel’s deformity with various types of spinal hernias and internal organ development anomalies, such as situs viscerum inversus (inverse or mirror location of internal organs), is a rare case. The etiology of these malformations is unknown, but their combination may suggest a common pathogenesis and cause.

The work aimed to review the literature and describe a clinical case of a patient with multiple malformations of the spine and spinal cord along with unilateral Sprengel’s deformity and situs viscerum inversus.

Materials and methods. A 4-year-old patient was diagnosed with myelomeningoradiculocele of the lumbosacral spine, malformation of the spine by the segmentation impairment type of C7–Th1, Th1–Th2, and Th2–Th3 vertebrae, non-closure of the arches of the С5–Т2 and Т12–S5 vertebrae, S-shaped congenital scoliosis, condition after surgical removal of myelomeningocele of the lumbosacral spine, concrescence and hypoplasia of ribs 1, 2, and 3 on the right, severe right-sided Sprengel’s deformity, and situs viscerum inversus.

Conclusions. The case description of concomitant malformations contributes to clinical material accumulation and further research in determining the factors of etiopathogenesis. Understanding the processes of pathoembryogenesis in combined malformations of the musculoskeletal system allows early suspicion and identification of latent deformities.

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About the authors

Stanislav V. Ivanov

G.I. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery

Author for correspondence.
ORCID iD: 0000-0002-2187-3973

Candidate of Medical Sciences, Head of Department Nо. 5 (Department of Cerebral palsy
and Center Spina Bifida), G.I. Turner National Medical Research Center for Сhildren's Orthopedics and Trauma Surgery

Russian Federation, 196603, Saint-Petersburg, Pushkin, Russian Federation

Dmitry S. Zharkov

G.I. Turner National Medical Research Center for Сhildren’s Orthopedics and Trauma Surgery

ORCID iD: 0000-0002-8027-1593
Russian Federation, 196603, Saint-Petersburg, Pushkin, Russian Federation


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Supplementary files

Supplementary Files
1. Fig. 1. Patient A.: A, B, C, D - the child's appearance, range of motion in the shoulder joints; D, E - the appearance of the feet before the start of the cast.

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2. Fig. 2. Patient A .: A, B - spiral computed tomography of the upper shoulder girdle, cervical and thoracic spine.

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3. Fig. 3. Patient A .: A, B - the result of treatment of paralytic equino-cavo-varus deformity of the feet. The appearance of the feet after 3 months. after treatment.

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СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
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