Sprengel Shoulder with Omovertebral Bone and Left Renal Agenesis in a Paediatric Patient: A Rare Case Report
Sprengel shoulder is a rare congenital anomaly caused by disrupted scapular descent during development. It is associated with hypoplastic or absent muscles such as the trapezius, sternocleidomastoid, and serratus anterior, leading to scapular winging. Cavendish grading classifies the severity as ver...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
JCDR Research and Publications Private Limited
2025-07-01
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| Series: | Journal of Clinical and Diagnostic Research |
| Subjects: | |
| Online Access: | https://jcdr.net/article_fulltext.asp?issn=0973-709x&year=2025&month=July&volume=19&issue=7&page=SD04-SD06&id=21261 |
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| Summary: | Sprengel shoulder is a rare congenital anomaly caused by disrupted scapular descent during development. It is associated with hypoplastic or absent muscles such as the trapezius, sternocleidomastoid, and serratus anterior, leading to scapular winging. Cavendish grading classifies the severity as very mild, mild, moderate, or severe. Mild cases may appear normal under clothing, while severe cases feature significant elevation, neck webbing, and brevicollis. One-third of cases have an omovertebral bone. Diagnosis is made through chest and shoulder radiography. Treatment is non-operative for mild cases, while moderate to severe cases require early surgical correction to prevent movement restriction. Sprengel shoulder is linked to conditions such as Klippel-Feil Syndrome (KFS), Poland syndrome, and VATER association, necessitating multidisciplinary evaluation. In this case report, a three-year-old girl presented with left shoulder elevation and restricted movement. Prenatal history revealed left renal agenesis. Examination showed an elevated left shoulder, restricted abduction, and apparent limb shortening. A typical Sprengel deformity with an omovertebral bone was diagnosed with the help of Magnetic Resonance Imaging (MRI). The Modified Excision and Reconstruction of Scapula (MEARS) procedure was planned, to improve mobility and quality of life for this patient. Additional evaluation was planned due to associated features such as a low hairline, vertebral fusion anomalies, and spina bifida, suggesting a possible genetic syndrome, most likely KFS. The rarity of Sprengel shoulder with an omovertebral bone and unilateral renal agenesis prompted this report. |
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| ISSN: | 2249-782X 0973-709X |