Thoracogenic Spinal Deformity: A Rare Cause of Early Onset Scoliosis

International Congress on Early Onset ScoliosisNovember 19 & 20, 2015

A. Noelle Larson, MD, Sarah Eby, BS, Tricia St. Hilaire, MPH, Michael Glotzbecker, MD, John Smith, MD, Klane White, Children’s Spine Study Group

• Congenital diaphragmatic hernia repair • Scoliosis in 13-30% (Russell, 2014;

Jancelewicz,2010 )

• Scoliosis following chest wall resection • Superior to 6th rib: progression odds ratio

10.6 (Glotzbecker, 2013)

• Progressive scoliosis; convexity toward resection

(Kawakami, 1994)

• Congenital heart defect repair• Curves >20° in 8.5% (Reckles, 1975)

Introduction

Evaluate patients with severe scoliosis following surgery for congenital conditions

Purpose

• Multicenter database; 2002-2014• 2381 patients prospectively enrolled

• 41 patients with thoracogenic scoliosis (1.7%)

• Interventions• 14 observed• 10 braced• 1 definitive fusion • 16 treated with VEPTR

Methods

Diagnosis Non-operative

VEPTR

Previous cardiac surgery 5 5

Tracheoesophageal fistula 1 3

Congenital diaphragmatic hernia

5 3

Spinal cord tumor 6 2

Radiation 1 0

Pulmonary hypoplasia 1 2

Chest wall tumor 3 3

Table: Number of subjects with primary underlying diagnosis; one subject in VEPTR group with pulmonary hypoplasia also had congenital diaphragmatic hernia, the other also had previous cardiac surgery.

ResultsDiagnoses

Non-operative

VEPTR

Total 23 16

Males 15 6

Females 8 10

Mean age at initial visit (years)

5.5 (3.5) 6.6 (4.5)

Mean follow-up (years) 2.9 (2.4) 3.8 (3.1)

Table: Values given as mean (standard deviation).

ResultsDemographics

Non-operative

VEPTR

Cobb angle

Initial visit 33° (12.7°) 65° (20.7°)

Follow-up visit 26° (14.5°) 50° (15.7°)

Table 2: Pre- and post-treatment. Values given as mean (standard deviation).

ResultsCurve Magnitude

• VEPTR group• Brachial plexus palsy (1)

• Resolved with revision of rib hooks• Spine infection (2)• Hardware failure (1)• Device migration (1)

• Non-operative group – none

ResultsComplications

A B

Figure 1. Scoliosis following tracheoesophageal fistula repair. A) Initial curve; Cobb angle = 72°, 13.2 years old; B) Following treatment with VEPTR; Cobb angle = 49°, 15.8 years old.

ResultsCase Example

• Heterogeneous sample• In many cases, information lacking regarding

index surgery

Limitations

• Scoliosis can develop following thoracotomy, other pediatric surgical procedures

• Represents <2% of EOS pts in our registry• Need prospective study to determine risk

factors for development of scoliosis• Future work

• Develop / implement preventative measures

Conclusions

Selected references

• Glotzbecker, M. P., Gold, M., Puder, M., & Hresko, M. T. (2013). Scoliosis after chest wall resection. Journal of Children's Orthopaedics, 7(4), 301–307.

• Kawakami, N., Winter, R. B., Lonstein, J. E., & Denis, F. (1994). Scoliosis secondary to rib resection. Journal of Spinal Disorders, 7(6), 522–527.

• Russell, K. W., Barnhart, D. C., Rollins, M. D., Hedlund, G., & Scaife, E. R. (2014). Journal of Pediatric Surgery. Journal of Pediatric Surgery, 49(6), 886–889.

• Jancelewicz, T., Vu, L. T., Keller, R. L., Bratton, B., Lee, H., Farmer, D., et al. (2010). Long-term surgical outcomes in congenital diaphragmatic hernia: observations from a single institution. Journal of Pediatric Surgery, 45(1), 155–160.

• Reckles, L. N., Peterson, H. A., Weidman, W. H., & Bianco, A. J. (1975). The association of scoliosis and congenital heart defects. The Journal of Bone and Joint Surgery. American Volume, 57(4), 449–455.