IntroductionThe myelomeningocele (MMC) is one of the dysraphic disorders of the neural arch. Pathogenically it is caused by the absence of proliferation of neuroectodermal cells after migration is finished. The occult form without neurologic deficit is estimated to have incidence of up to 1%, while a spina bifida aperta cystica with everted myelon and complete paraplegia occurs in 0.005% of live births. With the help of postnatal closure of the sac and the early commencement of hydrocephalus therapy, the life expectancy of patients with myelomeningocele has nearly reached the level of the normal population [3,24]. While therapy during infancy concentrates on taking care of the cerebral and renal function, during growth and adolescence, more and more problems concerning the skeletal system arise, caused by the wish for maximum mobility and improved nursing aspects such as lying in supine position and sitting without support. The clinical appearance is dominated by the spastic or paralytic neurological lesion at different levels. In addition, the increasing spinal deformity in combination with trophic skin and soft tissue disturbances leads to secondary problems, i.e. decubital ulcers and contractures of hips and limbs. Kyphosis appears in 12-28% of the cases, with neurologic damage at the thoracic level [2,15,24, 25]. The gibbus, however, develops usually between L2 and L5 [4].
Problems of the kyphotic spineMyelomeningocele at the thoracic level is in most cases accompanied by other malformations of the neural arch. They can aggravate each other, if for example during progressive kyphosis the dura is pulled caudally by a tethered Abstract The progression of kyphosis in myelomeningocele is independent of skeletal growth and requires early operative correction and stabilization to prevent a loss of sitting ability. In severe cases, only vertebrectomy makes it possible to achieve correction, stability and skin-closure without tension. In 14 patients with myelomeningocele gibbus, kyphectomy was performed, removing two vertebral bodies on average. The average kyphosis angle decreased from 128°to 81°, enabling most of the patients to participate again in social life by restoring wheelchair mobility. Nevertheless, a significantly higher complication rate was found compared to other correctional operations, lengthening the average hospital stay to 41 days. Special problems arose from trophic disorders of the skin and soft tissue and from the dystrophic muscles below the level of neural malfunction. In three cases, kyphosis reappeared cranial to the fused segments, requiring ventral stabilization. With respect to increasing kyphosis angle, an early intervention should be aimed at. A secondary operation can be necessary, if surgery is performed without taking care of the growth potential.