Surgical treatment of the deformities of the long bones in severe osteogenesis imperfecta.

The authors report their experience in the surgical treatment of bone deformities in osteogenesis imperfecta, based upon 45 operations in 13 severely affected patients. Three different procedures were used. Fragmentation and temporary fixation by means of a wire does not guarantee a sufficient protection of the bone and shows a relapse of deformity at the lower limbs; fragmentation, realignment and intramedullary rods, technically easier, is indicated in patients who are near the end of osseous growth; the extensible Bailey-Dubow rod, being an even more complicated application, is absolutely indicated in the bone in growth. Functional recovery of the patients was satisfactory, and complications were scarse, with the exception of a significant incidence of diaphyseal atrophy.

Amtolmetin guacyl versus piroxicam in patients with osteoarthritis.

The efficacy and tolerability of amtolmetin guacyl (AMG), a new non-steroidal anti-inflammatory drug, were compared with piroxicam, in patients with osteoarthritis. In a randomized double-blind study patients with arthritis (n = 99) received either 600 mg AMG on an empty stomach or 20 mg of piroxicam on a full stomach, once daily for 30 days. All clinical parameters improved significantly with both drugs; there were no significant differences between the two treatments. Tolerability, assessed by the patients, was significantly better in the AMG group. In the piroxicam group nine of 50 patients withdrew because of side-effects (gastrointestinal) compared with two of 49 (nausea and headache) in the AMG group. There were three cases of perforation, ulcer and bleeding in the piroxicam group but no serious side-effects with AMG. Total numbers of side-effects were similar in the two groups, but epigastric and abdominal pain were more frequent and more intense with piroxicam. AMG was as effective as piroxicam in controlling the symptoms of osteoarthritis, but showed better gastrointestinal tolerability.

[A case of bilateral tibial agenesis with multiple malformations and an unusual face: a new syndrome?]. / Su di un caso di agenesia tibiale bilaterale con malformazioni multiple e faccia inusuale: nuova sindrome?

This paper deals with a patient affected with total bilateral tibial agenesis, multiple congenital deformities and a very unusual face. After a review of the literature this case has resembled interesting to point out because it is probably an autonomous nosologic form.

Corrective femoral osteotomy and telescopic nailing in osteogenesis imperfecta.

Six cases of osteogenesis imperfecta with femoral deformity treated by Bailey telescopic nailing are discussed. In 4 cases the operation was bilateral. The long-term results show that this nail lengthens without compromising longitudinal growth of the bone, while providing protection from fracture and recurrence of the deformity. Thinning of the diaphyseal cortex was a significant complication in 3 femurs out of 10. Although it is more difficult to apply, the telescopic nail is preferable to traditional nailing because of its better mechanical features which allow for early weightbearing and walking.

Neonatal mucolipidosis 2. The spontaneous evolution of early bone lesions and the effect of vitamin D treatment. Report of two cases.

Evolution of the early bone lesions in two children with mucolipidosis 2 was followed from birth. The progression of the bone changes did not differ from healing of rickets. Low levels of 1,25-(OH)2-D3 were found in one child and he was treated with vitamin D; resolution of the rachitic changes was more rapid than in the untreated child. It is suggested that in mucolipidosis 2 bone reacts to two independent factors, one controlling calcium metabolism, the other depending on the primary lysosomal enzyme defect. Since ricket-like features are not present in the other mucolipidoses or mucopolysaccharidoses, the defect of calcium metabolism seems to be related to the specific enzyme defect of mucolipidosis 2.

Congenital pseudarthrosis of the tibia associated with neurofibromatosis-1: treatment with Ilizarov's device.

We reexamined 21 patients with congenital pseudarthrosis of the leg (congenital pseudoarthrosis of the tibia; CPT) associated with neurofibromatosis-1 (NF-1), > or =2 years after the termination of treatment, for a statistical study of the results obtained by using Ilizarov's external fixator. Of the 21 tibias operated on, 17 consolidated after the first treatment, whereas four did not. Of the 17 consolidated tibias, four refractured and were retreated by using a variety of methods. Only one healed. At follow-up, which occurred > or =2 years after the removal of the fixator, the results were nine consolidations without deformities or with shortening <2 cm, five consolidations with axial deviation, and seven nonconsolidations. The statistically significant results were that (a) patients who were aged 5 years or older at operation had better results, and (b) the assembly II (resection of CPT stumps and their short-term compression possibly associated with corticotomy or epiphyseal distraction to correct limb discrepancy) gave better final results compared with the other device assemblies. We conclude that treatment with Ilizarov's fixator allows (a) a good percentage of healing over time (66.7%), especially in cases of normotrophic and cystic CPT; (b) further operations with or without the fixator to correct secondary or residual axial deviation; and (c) correction of limb discrepancy. This treatment avoids risking injury to the healthy contralateral leg. Additionally, for treatments that do not achieve satisfactory results, other treatment methods are not excluded. The CPT still remains a difficult problem for the orthopedic surgeon to solve.