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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), Case 1: Spine radiographs at the age of 1 year and 14 years, respectively.
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Figure 1: (A, B), Case 1: Spine radiographs at the age of 1 year and 14 years, respectively.

Mentions: Case 1 (female, born in 1973). The first patient treated at the authors' Division had multiple distal congenital contractures with a progressive, right convex, thoracolumbar scoliosis. She had already undergone 4 operations for bilateral clubfoot deformity before the age of 4; she could walk only in domestic environment and the clinical history showed compromised respiratory capacity. At the age of 1, the radiographic evaluation of the spine had shown a 20° right convex scoliotic curve with mild lumbar hyperlordosis and pelvic obliquity of 5°; no congenital vertebral anomalies had been seen. By the age of 14 and with a Risser index of 4, despite the full-time brace, scoliosis had evolved rapidly into a structured and stiff deformity with a Cobb angle of 70° and multiple vertebral fusions near the curve's apex: she presented severe lumbar hyperlordosis of 103°, thoracic kyphosis of 49° and pelvic obliquity had increased to 9° (Figure 1A and 1B). In 1987, at the age of 14, a spinal arthrodesis was performed from T4 to L4 according to the Harrington-Luque technique. Postoperatively, the Cobb angle was 27°, lumbar lordosis 59°, pelvic obliquity 3° and thoracic kyphosis 20°. During surgery no complications were encountered; blood transfusion was performed in the immediate perioperative period. A cast orthosis was applied for the subsequent 6 months followed by a full-time treatment with Lyonnaise brace for 1 year. The patient was clinically reviewed for 9 years after surgery. At follow-up, no long-term complications were observed, correction was stable and, at the age of 23, the above mentioned values were practically unchanged (Figure 2A and 2B). Although the walking capacity had remained the same, the patient referred a significant improvement in the physical and emotional state, with a satisfactory quality of life both indoors and outdoors.


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), Case 1: Spine radiographs at the age of 1 year and 14 years, respectively.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: (A, B), Case 1: Spine radiographs at the age of 1 year and 14 years, respectively.
Mentions: Case 1 (female, born in 1973). The first patient treated at the authors' Division had multiple distal congenital contractures with a progressive, right convex, thoracolumbar scoliosis. She had already undergone 4 operations for bilateral clubfoot deformity before the age of 4; she could walk only in domestic environment and the clinical history showed compromised respiratory capacity. At the age of 1, the radiographic evaluation of the spine had shown a 20° right convex scoliotic curve with mild lumbar hyperlordosis and pelvic obliquity of 5°; no congenital vertebral anomalies had been seen. By the age of 14 and with a Risser index of 4, despite the full-time brace, scoliosis had evolved rapidly into a structured and stiff deformity with a Cobb angle of 70° and multiple vertebral fusions near the curve's apex: she presented severe lumbar hyperlordosis of 103°, thoracic kyphosis of 49° and pelvic obliquity had increased to 9° (Figure 1A and 1B). In 1987, at the age of 14, a spinal arthrodesis was performed from T4 to L4 according to the Harrington-Luque technique. Postoperatively, the Cobb angle was 27°, lumbar lordosis 59°, pelvic obliquity 3° and thoracic kyphosis 20°. During surgery no complications were encountered; blood transfusion was performed in the immediate perioperative period. A cast orthosis was applied for the subsequent 6 months followed by a full-time treatment with Lyonnaise brace for 1 year. The patient was clinically reviewed for 9 years after surgery. At follow-up, no long-term complications were observed, correction was stable and, at the age of 23, the above mentioned values were practically unchanged (Figure 2A and 2B). Although the walking capacity had remained the same, the patient referred a significant improvement in the physical and emotional state, with a satisfactory quality of life both indoors and outdoors.

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