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Thoracic Insufficiency Syndrome, Pediatric

Stamatia Alexiou, MD, Robert M. Campbell, Jr., MD and Oscar Henry Mayer, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Thoracic insufficiency syndrome (TIS) is the inability of the thorax to support normal respiration or lung growth.

  • Patients are skeletally immature with varied anatomic deformities th...

DIAGNOSIS

HISTORY

  • Prenatal ultrasound showing a hypoplastic thorax

  • Onset of clinical scoliosis

  • Onset of respiratory symptoms:

    • Relative exertional intolerance

    • Ineffective cough resulting in recurrent respir...

TREATMENT

GENERAL MEASURES

  • Bracing and halo-gravity traction can be used as a temporizing procedure and at best has been shown to control some forms of scoliosis or partially improve, but not correct, ...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • After TIS surgery, patients are followed with radiographs at regular intervals.

  • Pulmonary follow-up should include careful assessment for respiratory insufficiency...

ADDITIONAL READING

  • Campbell RM Jr. VEPTR: past experience and the future of VEPTR principles. Eur Spine J.  2013;22(Suppl 2):S106–S117. [View Abstract on OvidInsights]

  • Gadepalli SK, Hirschl RB, Ts...

CODES

ICD9

  • 756.9 Other and unspecified anomalies of musculoskeletal system

  • 756.3 Other anomalies of ribs and sternum

  • 756.4 Chondrodystrophy

  • 756.89 Other specified anomalies of muscle, tendon, fascia, and ...

FAQ

  • Q: What criteria need to be met before a patient can undergo VEPTR insertion?

  • A: The patient can be as young as 6 months and should not have reached skeletal maturity.

  • Q: What is the average length ...

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