Teenage girl with asymmetrical shoulder height
BMJ 2022; 376 doi: https://doi.org/10.1136/bmj-2019-067392 (Published 26 January 2022) Cite this as: BMJ 2022;376:e067392
- Kelechi C Eseonu, senior spinal fellow1,
- Uche Oduoza, specialty registrar2,
- J D Lucas, clinical lead3
- 1Guys and St Thomas’ NHS Trust, London, UK
- 2Trauma and Orthopaedics, Royal National Orthopaedic Hospital rotation, Stanmore, London, UK
- 3Paediatric Spinal Surgery, Evelina Children’s Hospital, Guys and St Thomas’ NHS Trust, London, UK
- Correspondence to: K C Eseonu Kelechi.eseonu{at}doctors.org.uk
A girl in her early teens presented to her general practitioner with a six month history of progressively worsening prominence of the right scapula, left shoulder tilting, and prominence of the left hip. She did not report pain or dysfunction.
The patient had no relevant family or medical history. Over the past year her height had increased rapidly. Menarche occurred six months before presentation.
The patient was concerned about her worsening body shape: she felt “slightly lopsided,” her clothes no longer fitted properly over her shoulders and hips, and she wanted to know the diagnosis and management options.
She was examined in her underwear, with a chaperone and her mother present. No café au lait spots, axillary freckles, hairy patches, or skin dimpling were seen on inspection of her trunk and lower back.
When standing, the patient’s right scapula and posterior ribs were prominent and her right shoulder was higher than the left (fig 1a). On forward bending, her right thoracic prominence was accentuated (fig 1b). Findings on neurological examination of the upper and lower limbs were normal.
Patient bending forwards—Adam’s forward bend test
The doctor ordered whole spine posteroanterior (fig 2) and lateral plain radiography and requested measurement of the Cobb angle for suspected scoliosis.
Posteroanterior radiograph of whole spine with Cobb angle 57°. On a posteroanterior view of the spine, tangents (white solid lines) are drawn along the superior endplate of the superior end vertebra and the inferior endplate of the inferior end vertebra. The Cobb angle is formed by the intersection of these two lines. The arrow shows the location of the iliac apophysis
Questions
1 What is the most likely diagnosis?
2 What are the potential differential diagnoses?
3 When would you refer a patient with scoliosis for specialist assessment?
Answers
1 What is the most likely diagnosis?
Adolescent idiopathic scoliosis—the most common form of juvenile scoliosis in patients with no other developmental delay or abnormal neurological findings.1 This spinal deformity has a lateral curvature, or Cobb angle, of >10°.1 Radiography showed a thoracic scoliosis with the right sided curve apex at T7. The Cobb angle measured 57° (fig 2).
Curves with a Cobb angle of <10° are not considered to indicate scoliosis and patients and family can be reassured.2
Overall, 1-3% of children aged 10-16 years have some degree of spinal curvature, but most do not require surgery.2
Girls are 10 times more likely to develop curves with a Cobb angle >30° than boys. The rate of back pain is increased in patients with adolescent idiopathic scoliosis—reportedly between 20% and 25%—compared with patients without spinal curvature.34
2 What are the potential differential diagnoses?
Other causes of scoliosis include congenital vertebral malformation, neuromuscular disorders, and syndromes resulting in developmental delay.5
Chest wall abnormalities, hairy patches, café au lait spots, axillary freckles, and skin dimpling over the lower back are possible signs of a neuromuscular cause of scoliosis. Definitive exclusion of underlying structural abnormalities requires whole spine magnetic resonance imaging.
3 When would you refer a patient with scoliosis for specialist assessment?
Patients younger than 13 years with a Cobb angle >10°
Patients aged 13-17 years with a Cobb angle >20°
Any scoliotic curve (Cobb angle >10°) in a patient younger than 18 years with an underlying condition (eg, neuromuscular condition, chromosomal or genetic abnormality).6
Before skeletal maturity, curves with a Cobb angle >25° continue to progress and require monitoring with serial whole spine radiography in a specialist clinic. Specialists grade skeletal maturity using a score called the Risser grading,2 related to fusion of the iliac crest apophysis (fig 2).
After skeletal maturity, curves with a Cobb angle <40° are less likely to progress. Thoracic curves with a Cobb angle >50° progress by 1-2° per year and lumbar curves with a Cobb angle >40° progress by 1-2° per year.57 Progressive scoliosis can lead to worsening deformity, cosmesis, and, in severe cases, cardiorespiratory compromise.8
Spinal bracing9 and physiotherapy10 can prevent progression into the surgical range (defined as a Cobb angle ≥ 50°) in skeletally immature children.11 Around 10% of adolescents with idiopathic scoliosis will need to be considered for surgery.12
A study of the natural history of adolescent idiopathic scoliosis found that untreated adolescent idiopathic scoliosis in patients with large thoracic curves might lead to increased back pain and pulmonary symptoms.13 Untreated patients are also at risk of substantial deformity, and the cosmetic aspect of this condition should not be underestimated.13
Patient outcome
The patient was referred to paediatric spinal orthopaedics. The diagnosis was confirmed after detailed neurological examination and whole spine magnetic resonance imaging to exclude intraspinal abnormalities.
After investigations and discussion with the multidisciplinary team, the patient underwent a posterior spinal instrumented correction and fusion. She recovered well (fig 3) and had an excellent outcome at two year follow-up.
Postoperative posteroanterior plain spinal radiograph
Learning points
Reassure patients and relatives that curves with Cobb angles <10° are normal variants of the spine.
Indication for referral depends on the magnitude of the curvature and the patient’s age and skeletal maturity.
Refer anxious patients and families to online resources such as www.sauk.org.uk.
Footnotes
Competing interests: The BMJ has judged that there are no disqualifying financial ties to commercial companies. The authors declare the following other interests: none.
Further details of The BMJ policy on financial interests are here: https://www.bmj.com/about-bmj/resources-authors/forms-policies-and-checklists/declaration-competing-interests.
Patient consent obtained. (Patient was 18 when she gave consent.)
Provenance and peer review: not commissioned; externally peer reviewed.
References
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