González RY, Esquivel SL, Fleites GY

Language: Spanish
References: 9
Page: 1-8
PDF size: 356.38 Kb.

ABSTRACT

Introduction: Sprengel’s deformity is considered a rare congenital skeletal disorder characterized by scapula elevation and its lateral rotation, with peri-scapular muscles, hypoplasia and atrophy causing deformation in this anatomical region with limitation of movements. It can be associated with Klippel-Fail Syndrome. Imaging studies are essential for its diagnosis.
Objective: to present a pediatric patient with Sprengel's deformity associated with Klippel-Fail syndrome, in which case the use of imaging studies was essential for the diagnosis.
Case Report: a three-year-old pediatric female patient, with short, wide and winged neck, she had serious difficulties for performing shoulders movements. Physical examination showed pterygium and asymmetry of the shoulder girdle, due to the elevation of the left scapula. Cervical spine and chest radiography were requested along with multislice tomography, where a fusion of the cervical spine bodies and elevated left scapula were found, this finding confirm the association of Sprengel’s deformity with Klippel-Fail syndrome.
Conclusions: images studies have a vital importance to complete a correct diagnosis in patients who present scapular asymmetry and shortening or any deformity of the cervical spine. These types of studies allow demonstrating pathological findings in this anatomical region. Simple radiographic studies are the choice to start the study. Multislice tomography is an effective tool given the new reconstruction techniques which facilitate the anatomical interpretation on these deformities.

REFERENCES

1. Dhir R, Chin K, Lambert S. The congenital undescended scapula syndrome: Sprengel and the cleithrum: a case series and hypothesis. J Shoulder Elbow Surg [Internet]. 2018 [citado Feb 2020]; 27(2): 252-9. Disponible en: Disponible en: https://doi.org/10.1016/j.jse.2017.08.011

2. Pomares G, Journeau P, Seivert V, Mainard-Simard L. Deformación de Sprengel o elevación congénita de la escápula. Principios de los tratamientos quirúrgicos. EMC-Téc Quirúrgicas Ortop Traumatol [Internet]. 2014 [citado Ene 2020]; 6(4): 1-8. Disponible en: Disponible en: https://doi.org/10.1016/S2211-033X(14)69194-5

3. Mauck BM. Congenital anomalies of the trunk and upper extremity. En: Azar FM, Canale ST, Beaty JH. Campbell's operative orthopaedics. 13th ed. Philadelphia: Elsevier; 2017. p. 1161-74.

4. Navarro Vidaurri G, Domínguez Gasca LG, Domínguez Carrillo LG. Deformidad de Sprengel. Acta Méd [Internet]. 2017 [citado Ene 2020]; 15(3). Disponible en: Disponible en: https://www.medigraphic.com/pdfs/actmed/am-2017/am173o.pdf

5. Stelzer JW, Flores MA, Mohammad W, Esplin N, Mayl JJ, Wasyliw C. Klippel-Feil syndrome with Sprengel deformity and extensive upper extremity deformity: A case report and literature review. Case Rep Orthop [Internet]. 2018 [citado Ene 2020]; 2018. Disponible en: Disponible en: https://www.hindawi.com/journals/crior/2018/5796730/

6. Kamal YA. Sprengel deformity: An update on the surgical management. Pulsus J Surg Res [Internet]. 2018 [citado Feb 2020]; 2(2): 64-8. Disponible en: Disponible en: https://www.pulsus.com/scholarly-articles/sprengel-deformity-an-update-on-the-surgical-management.pdf

7. NORD: National Organization for Rare Disorders [Internet]. Danbury CT; c 1993 [actualizado 2017; citado 07/03/2020]. Disponible en: Disponible en: https://rarediseases.org/rare-diseases/sprengel-deformity/

8. Warner WC. Pediatric cervical spine. En: Azar FM, Canale ST, Beaty JH. Campbell's operative orthopaedics. 13th ed. Philadelphia: Elsevier ; 2017. p. 1857-96.

9. Mistovich RJ, Spiegel DA. Klippel-Feil Syndrome. Cap.700. En: Kliegman RM, Geme J, editors. Nelson textbook of pediatrics. 21th ed. Philadelphia: Elsevier; 2020. p. 3646-50.e1