The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.

We describe 13 patients with cerebral palsy and lordoscoliosis/hyperlordosis of the lumbar spine who underwent a posterior spinal fusion at a mean age of 14.5 years (10.8 to 17.4) to improve sitting posture and relieve pain. The mean follow-up was 3.3 years (2.2 to 6.2).

The mean pre-operative lumbar lordosis was 108^° (80 to 150^°) and was corrected to 62^° (43^° to 85^°); the mean thoracic kyphosis from 17^° (-23^° to 35^°) to 47^° (25^° to 65^°); the mean scoliosis from 82^° (0^° to 125^°) to 22^° (0^° to 40^°); the mean pelvic obliquity from 21^° (0^° to 38^°) to 3^° (0^° to 15^°); the mean sacral slope from 79^° (54^° to 90^°) to 50^° (31^° to 66^°). The mean pre-operative coronal imbalance was 5 cm (0 cm to 8.9 cm) and was corrected to 0.6 cm (0 to 3.2). The mean sagittal imbalance of -8 cm (-16 cm to 7.8 cm) was corrected to -1.6 cm (-4 cm to 2.5 cm). The mean operating time was 250 minutes (180 to 360 minutes) and intra-operative blood loss 0.8 of estimated blood volume (0.3 to 2 estimated blood volume). The mean intensive care and hospital stay were 3.5 days (2 to 8) and 14.5 days (10 to 27), respectively. Three patients lost a significant amount of blood intra-operatively and subsequently developed chest or urinary infections and superior mesenteric artery syndrome.

An increased pre-operative lumbar lordosis and sacral slope were associated with increased peri-operative morbidity: scoliosis and pelvic obliquity were not. A reduced lumbar lordosis and increased thoracic kyphosis correlated with better global sagittal balance at follow-up. All patients and their parents reported excellent surgical outcomes.

Lordoscoliosis and hyperlordosis are associated with significant morbidity in quadriplegic patients. They are rare deformities and their treatment is challenging. Sagittal imbalance is the major component: it can be corrected by posterior fusion of the spine with excellent functional results.

Cite this article: Bone Joint J 2014;96-B:800–6.

Aim:

To present 11 patients with quadriplegia who developed severe lordoscoliosis or hyperlordosis. This is a rare deformity in children with CP, treatment is challenging and there are less than 20 patients ever reported.