A 6-year-old male came to clinic for well child care who had severe spastic cerebral palsy. Overall he was doing well with his multiple therapies and was mobile with assistance for part of the day. His father was concerned that his back seemed to be curving more when he walked with his walker and when sitting in his wheelchair. He felt this was getting worse recently. His son was non-verbal but the father didn’t feel that there was any pain or discomfort. He also didn’t notice any skin changes from chair positioning. The father had not asked the child’s physical therapists if they had any concerns.
The pertinent physical exam showed a non-communicative male with spastic cerebral palsy. He sat preferentially leaning to the left in his chair. With lying on the table, the physician noticed him leaning more to the left, and anteriorly no chest wall asymmetry was noted. When prone there was no specific spinal curvature noted, and there was normal alignment of the scapula and shoulders. It was difficult for him to lie flat because his hips which were held in a flexed and abducted position. No skin breakdown was noted and his bilateral ankle-foot orthosis fit properly. The diagnosis of spastic cerebral palsy was again conformed. The physician wasn’t entirely sure what the problem was but felt that the abnormal hip flexion was probably causing malpositioning. She was not sure if there was actual scoliosis but his underlying condition increased its possibility. The patient had not seen his orthopaedic physician recently and was re-referred. A hip dysplasia was diagnosed and surgery was recommended in the near future. His wheelchair was also modified for better positioning.
Alignment of the spine is measured from a plumbline dropped from C7 vertebrae as the line of reference. Lordosis is anterior curving, kyphosis is posterior curving and scoliosis is lateral curving relative to this line. Scoliosis has a large differential diagnosis with neuromuscular and spinal abnormalities being the most common as groups. Adolescent idiopathic scoliosis is the most common type of idiopathic scoliosis and the most common type of scoliosis (as a distinct entity) overall. By mid- to late- adolescence, 2-3% will have a 10-degree angle or more. The Cobb angle measures the degree of curvature and its determination can be seen here.
Treatment for scoliosis consists of 3 O’s – observation, orthosis (bracing) and operation.
The differential diagnosis of scoliosis includes:
- Compensatory – because of unequal leg length discrepencies
- Posture – ceases with repositioning or bending over
- Structural – persists with bending over
- Arnold-Chiari malformation
- Cerebral palsy
- Down syndrome
- Ehler-Dahlos syndrome
- Friedrich’s ataxia
- Klippel-Feil syndrome
- Muscular dystrophy
- Prader-Willi syndrome
- Spinal abscess
- Spinal cord trauma
- Spinal tumor
- Spinal muscular atrophy
- Spinal deformity
- Bone tumor
- Congenital scoliosis
- Spina bifida
- Juvenile rheumatoid arthritis
- Osteogenesis imperfecta
- Spinal irradiation (post)
- Spinal trauma
Questions for Further Discussion
1. How is congenital scoliosis different from other forms of scoliosis?
2. What criteria are used for determining treatment using orthosis or surgical operations?
- Disease: Scoliosis
- Specialty: Orthopaedic Surgery and Sports Medicine
- Age: School Ager
To Learn More
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Illingworth RS. Common Symptoms of Disease in Children. Blackwell Scientific Publications: Oxford. 1988:255-256.
Dobbs MD. Neuromuscular Scoliosis.Medscape.
Available from the Internet at http://emedicine.medscape.com/article/1266097-overview#a0102 (rev. 4/27/2010, cited 6/4/12).
Letts RM. Congenital Spinal Deformity. Medscape.
Available from the Internet at http://emedicine.medscape.com/article/1260442-overview (rev. 2/7/2012, cited 6/4/12).
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
25. Quality patient care and assisting patients in dealing with system complexities is advocated.
26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital