Bone Abstracts

Background: Osteogenesis imperfecta (OI) is a rare skeletal disorder characterised by bone fragility usually due to inherited mutations in the genes for type 1 collagen. Children with OI present with numerous physical manifestations due to ligamentous laxity and bone fragility (1).

Presenting problem: Poor posture and associated back pain is a common problem for many children with mild to moderate OI. Hypermobility can result in muscle imbalance which may lead to excessive lengthening/weakening of the abdominal muscles and tightening of the iliopsoas and spinal erector muscle. Consequent is an increase in the natural lordosis of the lumbar spine which can result in spondylolisthesis/ spondylolysis (2). This poster describes the case of a 14 year old with a diagnosis of OI type 1. In 2014 she presented with a lumbar sacral spondylolysis which deteriorated gradually over a 2 year period with resultant back pain and potential surgical intervention.

Clinical management: Good posture, dependent on the balance of the skeleton and optimal symmetrical alignment, is an active and dynamic process which underpins functional activities (3, 4). A posterior tilt wedge cushion was provided, used in conjunction with a standard classroom chair and foot rest. Positioning was checked for optimal alignment by the occupational therapist and monitored daily by teaching support staff.

Goals of provision:
• Maintain skeletal alignment in sitting

• Provide a stable base of support to promote function

• Increase tolerance of optimal sitting posture

• Decrease pain and fatigue

• Correct skeletal deformity in sitting

Outcome: Six months following initial provision the patient attended a follow up outpatient appointment. Back pain had resolved and X-ray showed an improvement in the spondylolysis. No further spinal follow up was required.

Discussion: Evidence on the effectiveness of posture cushions in the OI population is scant. This case study suggests the use of a posterior wedge cushion to improve a lordotic posture and consequent spondylolysis can also reduce the incidence and severity of back pain in children mildly to moderately affected by OI. The need for surgical intervention may also be avoided.

Disclosure: The authors declared no competing interests.