Children with spina bifida develop a wide variety of congenital and acquired orthopedic deformities. Among these are hip deformities such as contracture, subluxation, or dislocation. Patients may also have problems with the knee joint, such as knee flexion or extension contracture, knee valgus deformity, or late knee instability and pain. In addition, rotational deformities of the lower extremities, either internal or external torsion, are common as well. This paper will review both the overall orthopedic care of a patient with spina bifida and provide a focused review of the diagnosis and management of the above deformities. In addition, this paper will review the incidence, etiology, classification, and prognosis of spina bifida. The use of gait analysis and orthoses will be covered as well. The forthcoming Part II will cover foot and ankle deformities in spina bifida.

Both congenital and acquired orthopaedic deformities are common in patients with spina bifida. Examples of congenital deformities, which are present at birth, include clubfoot and vertical talus. Acquired developmental deformities are related to the level of neurologic involvement and include calcaneus and cavovarus. Orthopaedic deformities may also result from postoperative tethered cord syndrome. The previously published Part I reviewed the overall orthopaedic care of a patient with spina bifida, with a focused review of hip, knee, and rotational deformities. This paper will cover foot and ankle deformities associated with spina bifida, including clubfoot, equinus, vertical talus, calcaneus and calcaneovalgus, ankle and hindfoot valgus, and cavovarus. In addition, this paper will address the issues surrounding skin breakdown in patients with spina bifida.

In 1994, we presented a series of 52 Ortolani-positive hips (group 1) with a success rate of reduction of 85%. Now, our protocol for treating Ortolani-positive hips has evolved to include serial orthopaedic office-based ultrasound in all patients and use of a hip abduction orthosis in hips remaining unstable after 3 weeks in a Pavlik harness. Three previous studies at major centers reported successful reduction in only 63% to 71%. In group 2, our current success rate of 93% exceeds that previously reported by us and is significantly greater than these 3 previous studies (all P < 0.006). Furthermore, our current treatment module has decreased our rate of open reduction from 10% in group 1 to 5% in group 2. LEVEL OF RELEVANCE: Therapeutic study, level 3.