Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis

Learning from the differences

Joseph Gjolaj, Paul D. Sponseller, Suken A. Shah, Peter O. Newton, John M. Flynn, Philip R. Neubauer, Michelle C. Marks, Tracey P. Bastrom

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

STUDY DESIGN.: Retrospective case control study. OBJECTIVE.: To compare patients with Marfan syndrome and matched patients with adolescent idiopathic scoliosis (AIS) to illustrate the differences and identify areas for potential surgical improvement. SUMMARY OF BACKGROUND DATA.: Patients with Marfan syndrome commonly require spinal deformity surgery, but practice guidelines and results are not as established as those for patients with AIS. METHODS.: We matched 34 adolescents with Marfan syndrome with patients with AIS (ratio, 1:2) for age, sex, and degree of major deformity. Overall mean age was 14 ± 2 years and mean curves were 51° thoracic and 46° lumbar. Mean follow-up was 5.3 and 3.6 years, respectively. RESULTS.: The Marfan syndrome group had significantly more thoracolumbar kyphosis correction (9.5° vs. 0.1°, P = 0.05), significantly more levels fused (12 ± 2 vs. 9 ± 3, P ≤ 0.01), significantly more fusions to the pelvis (7 vs. 0, P = 0.01), and significantly more correction of sagittal imbalance (2.4 vs. -0.6 cm, P = 0.035). The Marfan syndrome group also had more intraoperative cerebrospinal fluid leaks (3 vs. 0, P = 0.01), significantly more instrumentation complications (3 vs. 1, P = 0.007), more reoperations for indications [such as fixation failure, distal degeneration, and spine fracture (9 vs. 0, P = 0.01)], and lower SRS-22 total (3.9 vs. 4.5, P = 0.01) and partial (P < 0.015) subscores. There were no significant differences between the groups in progression of unfused proximal thoracic curves, blood loss, neurological deficit, hospital stay, percent correction, or infection rate. CONCLUSION.: Patients with Marfan syndrome differ in several ways from those with AIS: they require more levels of surgical correction, more distal fusion, greater correction of sagittal balance, and more reoperations, and they have more intraoperative cerebrospinal fluid leaks and instrumentation-related complications. Knowledge of these differences is important for planning surgery.

Original languageEnglish (US)
Pages (from-to)1558-1565
Number of pages8
JournalSpine
Volume37
Issue number18
DOIs
StatePublished - Aug 15 2012
Externally publishedYes

Fingerprint

Marfan Syndrome
Scoliosis
Learning
Reoperation
Thorax
Kyphosis
Pelvis
Practice Guidelines
Case-Control Studies
Length of Stay
Spine
Infection

Keywords

  • complications
  • idiopathic scoliosis
  • Marfan syndrome
  • spinal instrumentation

ASJC Scopus subject areas

  • Clinical Neurology
  • Orthopedics and Sports Medicine

Cite this

Gjolaj, J., Sponseller, P. D., Shah, S. A., Newton, P. O., Flynn, J. M., Neubauer, P. R., ... Bastrom, T. P. (2012). Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis: Learning from the differences. Spine, 37(18), 1558-1565. https://doi.org/10.1097/BRS.0b013e3182541af3

Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis : Learning from the differences. / Gjolaj, Joseph; Sponseller, Paul D.; Shah, Suken A.; Newton, Peter O.; Flynn, John M.; Neubauer, Philip R.; Marks, Michelle C.; Bastrom, Tracey P.

In: Spine, Vol. 37, No. 18, 15.08.2012, p. 1558-1565.

Research output: Contribution to journalArticle

Gjolaj, J, Sponseller, PD, Shah, SA, Newton, PO, Flynn, JM, Neubauer, PR, Marks, MC & Bastrom, TP 2012, 'Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis: Learning from the differences', Spine, vol. 37, no. 18, pp. 1558-1565. https://doi.org/10.1097/BRS.0b013e3182541af3
Gjolaj, Joseph ; Sponseller, Paul D. ; Shah, Suken A. ; Newton, Peter O. ; Flynn, John M. ; Neubauer, Philip R. ; Marks, Michelle C. ; Bastrom, Tracey P. / Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis : Learning from the differences. In: Spine. 2012 ; Vol. 37, No. 18. pp. 1558-1565.
@article{96c7220ba4544c7b9e4f1eed9f463c33,
title = "Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis: Learning from the differences",
abstract = "STUDY DESIGN.: Retrospective case control study. OBJECTIVE.: To compare patients with Marfan syndrome and matched patients with adolescent idiopathic scoliosis (AIS) to illustrate the differences and identify areas for potential surgical improvement. SUMMARY OF BACKGROUND DATA.: Patients with Marfan syndrome commonly require spinal deformity surgery, but practice guidelines and results are not as established as those for patients with AIS. METHODS.: We matched 34 adolescents with Marfan syndrome with patients with AIS (ratio, 1:2) for age, sex, and degree of major deformity. Overall mean age was 14 ± 2 years and mean curves were 51° thoracic and 46° lumbar. Mean follow-up was 5.3 and 3.6 years, respectively. RESULTS.: The Marfan syndrome group had significantly more thoracolumbar kyphosis correction (9.5° vs. 0.1°, P = 0.05), significantly more levels fused (12 ± 2 vs. 9 ± 3, P ≤ 0.01), significantly more fusions to the pelvis (7 vs. 0, P = 0.01), and significantly more correction of sagittal imbalance (2.4 vs. -0.6 cm, P = 0.035). The Marfan syndrome group also had more intraoperative cerebrospinal fluid leaks (3 vs. 0, P = 0.01), significantly more instrumentation complications (3 vs. 1, P = 0.007), more reoperations for indications [such as fixation failure, distal degeneration, and spine fracture (9 vs. 0, P = 0.01)], and lower SRS-22 total (3.9 vs. 4.5, P = 0.01) and partial (P < 0.015) subscores. There were no significant differences between the groups in progression of unfused proximal thoracic curves, blood loss, neurological deficit, hospital stay, percent correction, or infection rate. CONCLUSION.: Patients with Marfan syndrome differ in several ways from those with AIS: they require more levels of surgical correction, more distal fusion, greater correction of sagittal balance, and more reoperations, and they have more intraoperative cerebrospinal fluid leaks and instrumentation-related complications. Knowledge of these differences is important for planning surgery.",
keywords = "complications, idiopathic scoliosis, Marfan syndrome, spinal instrumentation",
author = "Joseph Gjolaj and Sponseller, {Paul D.} and Shah, {Suken A.} and Newton, {Peter O.} and Flynn, {John M.} and Neubauer, {Philip R.} and Marks, {Michelle C.} and Bastrom, {Tracey P.}",
year = "2012",
month = "8",
day = "15",
doi = "10.1097/BRS.0b013e3182541af3",
language = "English (US)",
volume = "37",
pages = "1558--1565",
journal = "Spine",
issn = "0362-2436",
publisher = "Lippincott Williams and Wilkins",
number = "18",

}

TY - JOUR

T1 - Spinal deformity correction in Marfan syndrome versus adolescent idiopathic scoliosis

T2 - Learning from the differences

AU - Gjolaj, Joseph

AU - Sponseller, Paul D.

AU - Shah, Suken A.

AU - Newton, Peter O.

AU - Flynn, John M.

AU - Neubauer, Philip R.

AU - Marks, Michelle C.

AU - Bastrom, Tracey P.

PY - 2012/8/15

Y1 - 2012/8/15

N2 - STUDY DESIGN.: Retrospective case control study. OBJECTIVE.: To compare patients with Marfan syndrome and matched patients with adolescent idiopathic scoliosis (AIS) to illustrate the differences and identify areas for potential surgical improvement. SUMMARY OF BACKGROUND DATA.: Patients with Marfan syndrome commonly require spinal deformity surgery, but practice guidelines and results are not as established as those for patients with AIS. METHODS.: We matched 34 adolescents with Marfan syndrome with patients with AIS (ratio, 1:2) for age, sex, and degree of major deformity. Overall mean age was 14 ± 2 years and mean curves were 51° thoracic and 46° lumbar. Mean follow-up was 5.3 and 3.6 years, respectively. RESULTS.: The Marfan syndrome group had significantly more thoracolumbar kyphosis correction (9.5° vs. 0.1°, P = 0.05), significantly more levels fused (12 ± 2 vs. 9 ± 3, P ≤ 0.01), significantly more fusions to the pelvis (7 vs. 0, P = 0.01), and significantly more correction of sagittal imbalance (2.4 vs. -0.6 cm, P = 0.035). The Marfan syndrome group also had more intraoperative cerebrospinal fluid leaks (3 vs. 0, P = 0.01), significantly more instrumentation complications (3 vs. 1, P = 0.007), more reoperations for indications [such as fixation failure, distal degeneration, and spine fracture (9 vs. 0, P = 0.01)], and lower SRS-22 total (3.9 vs. 4.5, P = 0.01) and partial (P < 0.015) subscores. There were no significant differences between the groups in progression of unfused proximal thoracic curves, blood loss, neurological deficit, hospital stay, percent correction, or infection rate. CONCLUSION.: Patients with Marfan syndrome differ in several ways from those with AIS: they require more levels of surgical correction, more distal fusion, greater correction of sagittal balance, and more reoperations, and they have more intraoperative cerebrospinal fluid leaks and instrumentation-related complications. Knowledge of these differences is important for planning surgery.

AB - STUDY DESIGN.: Retrospective case control study. OBJECTIVE.: To compare patients with Marfan syndrome and matched patients with adolescent idiopathic scoliosis (AIS) to illustrate the differences and identify areas for potential surgical improvement. SUMMARY OF BACKGROUND DATA.: Patients with Marfan syndrome commonly require spinal deformity surgery, but practice guidelines and results are not as established as those for patients with AIS. METHODS.: We matched 34 adolescents with Marfan syndrome with patients with AIS (ratio, 1:2) for age, sex, and degree of major deformity. Overall mean age was 14 ± 2 years and mean curves were 51° thoracic and 46° lumbar. Mean follow-up was 5.3 and 3.6 years, respectively. RESULTS.: The Marfan syndrome group had significantly more thoracolumbar kyphosis correction (9.5° vs. 0.1°, P = 0.05), significantly more levels fused (12 ± 2 vs. 9 ± 3, P ≤ 0.01), significantly more fusions to the pelvis (7 vs. 0, P = 0.01), and significantly more correction of sagittal imbalance (2.4 vs. -0.6 cm, P = 0.035). The Marfan syndrome group also had more intraoperative cerebrospinal fluid leaks (3 vs. 0, P = 0.01), significantly more instrumentation complications (3 vs. 1, P = 0.007), more reoperations for indications [such as fixation failure, distal degeneration, and spine fracture (9 vs. 0, P = 0.01)], and lower SRS-22 total (3.9 vs. 4.5, P = 0.01) and partial (P < 0.015) subscores. There were no significant differences between the groups in progression of unfused proximal thoracic curves, blood loss, neurological deficit, hospital stay, percent correction, or infection rate. CONCLUSION.: Patients with Marfan syndrome differ in several ways from those with AIS: they require more levels of surgical correction, more distal fusion, greater correction of sagittal balance, and more reoperations, and they have more intraoperative cerebrospinal fluid leaks and instrumentation-related complications. Knowledge of these differences is important for planning surgery.

KW - complications

KW - idiopathic scoliosis

KW - Marfan syndrome

KW - spinal instrumentation

UR - http://www.scopus.com/inward/record.url?scp=84865647587&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84865647587&partnerID=8YFLogxK

U2 - 10.1097/BRS.0b013e3182541af3

DO - 10.1097/BRS.0b013e3182541af3

M3 - Article

VL - 37

SP - 1558

EP - 1565

JO - Spine

JF - Spine

SN - 0362-2436

IS - 18

ER -