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Surgical treatment of scoliosis in a rare disease: arthrogryposis.

Greggi T, Martikos K, Pipitone E, Lolli F, Vommaro F, Maredi E, Cervellati S, Silvestre MD - Scoliosis (2010)

Bottom Line: Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days.Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.

View Article: PubMed Central - HTML - PubMed

Affiliation: Spine Surgery Division, Rizzoli Orthopaedic Institute, Bologna, Italy. tiziana.greggi@ior.it.

ABSTRACT

Background: The reported incidence of scoliosis in arthrogryposis varies from 30% to 67% and, in most cases, the curves progress rapidly and become stiff from early age.The authors report six cases of scoliosis in arthrogryposis to assess the role of surgical treatment.

Methods: Six cases (3 males, 3 females; mean age at surgery 13.2 years) with arthrogryposis multiplex congenita associated with the characteristic amyoplasia were reviewed: they were operated on for scoliosis at the authors' Spine Surgery Department between 1987 and 2008.Surgery was performed using the Harrington-Luque instrumentation (2 cases), the Luque system (1), a hybrid segmental technique with hooks and screws (1) and spinal anchoring with pedicle screws (2).

Results: The patients were clinically and radiologically reviewed at a mean follow-up of 4.2 years, ± 2.7 (range, 1 to 9 years). Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days. Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.

Conclusions: The experience acquired with the present case series leads the authors to assert that prompt action should be taken when treating such aggressive forms of scoliosis. In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.

No MeSH data available.


Related in: MedlinePlus

(A, B, C, D), Case 5: Postoperative radiographs, anteroposterior (A) and laterolateral (B) view and clinical picture at a 2-year follow-up (C and D).
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Figure 9: (A, B, C, D), Case 5: Postoperative radiographs, anteroposterior (A) and laterolateral (B) view and clinical picture at a 2-year follow-up (C and D).

Mentions: Case 5 (male, born in 1995, patient with congenital multiple arthrogryposis). In pediatric age he had been operated on twice for congenital clubfoot deformity according to the Codivilla technique and twice for non - traumatic bilateral knee dislocation. At the age of 9, the radiographic evaluation of the spine showed a stiff and structured large thoracolumbar curve of 84° with pelvic obliquity of 5°, while the Risser sign was still 0. Despite the full-time brace treatment since the age of 10, the deformity continued to progress until reaching the following values at the age of 12: thoracolumbar curve with Cobb angle of 100°, pelvic inclination 18°, lumbar lordosis 82° and thoracic kyphosis 73°, with a Risser sign of 1 (Figure 8A, B, C and 8D). The patient was of low stature and overweight with a BMI of 29.5; in addition, a notable restrictive pulmonary deficit was observed and confirmed by the patient's lung capacity (FVC = 54%; FEV1 = 65%). In 2007, posterior instrumented arthrodesis was performed from T4 to L2 using only pedicle screws and achieving a partial correction of the deformity values (thoracolumbar curve 65°, pelvic obliquity 8°, lumbar lordosis 61° and thoracic kyphosis 38°) (Figure 9A and 9B). A Lyonnaise brace was applied for 2 months after surgery. At a 2-year follow-up, no long term complications were observed; radiographs revealed a solid fusion and no sign of mobilization; the young boy maintained his walking capacity in community (Figure 9C and 9D).


Surgical treatment of scoliosis in a rare disease: arthrogryposis.

Greggi T, Martikos K, Pipitone E, Lolli F, Vommaro F, Maredi E, Cervellati S, Silvestre MD - Scoliosis (2010)

(A, B, C, D), Case 5: Postoperative radiographs, anteroposterior (A) and laterolateral (B) view and clinical picture at a 2-year follow-up (C and D).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2991290&req=5

Figure 9: (A, B, C, D), Case 5: Postoperative radiographs, anteroposterior (A) and laterolateral (B) view and clinical picture at a 2-year follow-up (C and D).
Mentions: Case 5 (male, born in 1995, patient with congenital multiple arthrogryposis). In pediatric age he had been operated on twice for congenital clubfoot deformity according to the Codivilla technique and twice for non - traumatic bilateral knee dislocation. At the age of 9, the radiographic evaluation of the spine showed a stiff and structured large thoracolumbar curve of 84° with pelvic obliquity of 5°, while the Risser sign was still 0. Despite the full-time brace treatment since the age of 10, the deformity continued to progress until reaching the following values at the age of 12: thoracolumbar curve with Cobb angle of 100°, pelvic inclination 18°, lumbar lordosis 82° and thoracic kyphosis 73°, with a Risser sign of 1 (Figure 8A, B, C and 8D). The patient was of low stature and overweight with a BMI of 29.5; in addition, a notable restrictive pulmonary deficit was observed and confirmed by the patient's lung capacity (FVC = 54%; FEV1 = 65%). In 2007, posterior instrumented arthrodesis was performed from T4 to L2 using only pedicle screws and achieving a partial correction of the deformity values (thoracolumbar curve 65°, pelvic obliquity 8°, lumbar lordosis 61° and thoracic kyphosis 38°) (Figure 9A and 9B). A Lyonnaise brace was applied for 2 months after surgery. At a 2-year follow-up, no long term complications were observed; radiographs revealed a solid fusion and no sign of mobilization; the young boy maintained his walking capacity in community (Figure 9C and 9D).

Bottom Line: Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days.Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.

View Article: PubMed Central - HTML - PubMed

Affiliation: Spine Surgery Division, Rizzoli Orthopaedic Institute, Bologna, Italy. tiziana.greggi@ior.it.

ABSTRACT

Background: The reported incidence of scoliosis in arthrogryposis varies from 30% to 67% and, in most cases, the curves progress rapidly and become stiff from early age.The authors report six cases of scoliosis in arthrogryposis to assess the role of surgical treatment.

Methods: Six cases (3 males, 3 females; mean age at surgery 13.2 years) with arthrogryposis multiplex congenita associated with the characteristic amyoplasia were reviewed: they were operated on for scoliosis at the authors' Spine Surgery Department between 1987 and 2008.Surgery was performed using the Harrington-Luque instrumentation (2 cases), the Luque system (1), a hybrid segmental technique with hooks and screws (1) and spinal anchoring with pedicle screws (2).

Results: The patients were clinically and radiologically reviewed at a mean follow-up of 4.2 years, ± 2.7 (range, 1 to 9 years). Three minor postoperative complications were encountered; a long-term pulmonary complication was seen in one case after reintervention and was successfully resolved after 10 days. Surgery was successful in the other 5 cases, where solid arthrodesis was achieved and no significant curve progression was observed at follow-up.

Conclusions: The experience acquired with the present case series leads the authors to assert that prompt action should be taken when treating such aggressive forms of scoliosis. In case of mild spinal deformities in arthrogryposis, brace treatment should be attempted, the evolution of the curves being unpredictable; however, when the curve exceeds 40° and presents with marked hyperkyphosis, hyperlordosis or pelvic obliquity, surgery should not be delayed.

No MeSH data available.


Related in: MedlinePlus