International Journal of Community Medicine and Public Health | December 2022 | Vol 9 | Issue 12 Page 1
International Journal of Community Medicine and Public Health
Das BK et al. Int J Community Med Public Health. 2022 Dec;9(12):xxx-xxx
http://www.ijcmph.com
pISSN 2394-6032 | eISSN 2394-6040
Case Report
Sprengel shoulder: a case report
Bipul Kumar Das
1
, Debajani Deka
2
*, Suyashi Sharma
3
INTRODUCTION
Sprengel shoulder is a rare deformity of shoulder girdle of
unknown etiology. Eulenburg in 1863, had first reported
this rare congenital anomaly of the scapula.
1
Sprengel
described it in 1891, and hence this deformity got its’
name.
2
Autosomal dominant inheritance has been
reported for this abnormality. Abnormal descent of the
scapula during 9
th
to 12
th
weeks of gestation leads to this
congenital anomaly.
3
It is a cosmetic deformity. Elevation
of scapula is the characteristic feature. A lump at the back
of the neck with restricted movements of the shoulder and
or arm are the presenting finding. The affected scapula is
rotated on its sagittal axis. The superior and the vertebral
border comes nearer to the medial line and to the axilla
respectively. Severity of this deformity varies widely
being almost invisible when covered with clothes to the
shoulder being elevated over 5 centimeters along with the
presence of webbing of neck. This congenital deformity
can be associated with other musculoskeletal
abnormalities such as Klippel-Feil syndrome, scoliosis
and rib anomalies.
4
In 30% of cases of Sprengel shoulder,
an omovertebral cartilaginous, fibrous or bony bar
connecting superomedial border of scapula to spine,
transverse process or lamina of cervical vertebra can be
seen and this causes restriction of movements around the
shoulder girdle.
7
ABSTRACT
Sprengel shoulder is a rare deformity of shoulder girdle of unknown etiology. Eulenburg in 1863, had first reported
this rare congenital anomaly of the scapula. Sprengel described it in 1891, and hence this deformity got its’ name.
Autosomal dominant inheritance has been reported for this abnormality. Abnormal descent of the scapula during 9
th
to
12
th
weeks of gestation leads to this congenital anomaly. It is a cosmetic deformity. Elevation of scapula is the
characteristic feature. A lump at the back of the neck with restricted movements of the shoulder and or arm are the
presenting finding. The affected scapula is rotated on its sagittal axis. The superior and the vertebral border comes
nearer to the medial line and to the axilla respectively. Severity of this deformity varies widely being almost invisible
when covered with clothes to the shoulder being elevated over 5 centimeters along with the presence of webbing of
neck. This congenital deformity can be associated with other musculoskeletal abnormalities such as Klippel-Feil
syndrome, scoliosis and rib anomalies. In 30% of cases of Sprengel shoulder, an omovertebral cartilaginous, fibrous
or bony bar connecting superomedial border of scapula to spine, transverse process or lamina of cervical vertebra can
be seen and this causes restriction of movements around the shoulder girdle. Surgical management is considered in
severe cases. Surgical management involves excision of the protruding portion of scapula and inferior translation of
scapula. This enhances shoulder functions as well as general appearance of the patient.
Keywords: Shoulder, Ribs, Spine, Scapula, Scliosis
1
Department of Pediatrics, All India Institute of Medical Sciences, Guwahati, Assam, India
2
Department of Anatomy, Gauhati Medical College and Hospital, Guwahati, Assam, India
3
Department of Anatomy, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
Received: 31 October 2022
Revised: 13 November 2022
Accepted: 15 November 2022
*Correspondence:
Dr. Debajani Deka,
E-mail: debajani.deka31@gmail.com
Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under
the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial
use, distribution, and reproduction in any medium, provided the original work is properly cited.
DOI: https://dx.doi.org/10.18203/2394-6040.ijcmph20223007