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Final ID: Poster #: CR-046

The natural history of pectus excavatum: An unusual illustration of deformity progression

Purpose or Case Report: Pectus excavatum is a deformity that affects 1-in-1000 children and causes 80% of all chest wall abnormalities. Although usually mild at a young age, it rapidly progresses during puberty requiring dedicated interval monitoring to assess the need for surgical intervention. This sudden acceleration is not without a physiological and psychological impact. Prior to surgery, the patient’s cardiopulmonary status is assessed. In addition, the patient’s psychological welfare should be monitored, given the known link of pectus excavatum with poor body image and social isolation.

The Haller Index is the standard measurement to assess the severity of the pectus deformity. A calculated index of > 3.25 is a generally accepted marker of disease and the need for surgical correction. This number represents a ratio between the distance from the posterior sternum to the anterior spine and the widest transverse diameter of the chest wall.

There has been an evolution of treatment strategies since this deformity was first recognized. In the 16th century medical management consisted of fresh air and breathing exercises; in the early 20th century, treatment swung to the other end of the spectrum with radical surgery involving excision of the anterior chest wall. Since then, surgical procedures have advanced to the current minimally invasive standard of care that incorporates the malleability of the chest wall using reconfiguration and bracing. Bilateral chest wall incisions are made and a subcutaneous substernal tunnel is created. With this procedure in mind, the pre-pubertal patients (11-14yrs) are the optimal age for surgical correction – providing the opportunity for the quickest recovery and excellent results.

This clinical case report is a unique look into the progression of pectus excavaum. The diagnosis was made by a radiologist through sequential abdominal CT scans performed to evaluate for recurrent disease for her Stage II Wilms Tumor. She was subsequently referred to a pediatric surgeon at the age of 12 for surveillance monitoring. Over the course of two years (2007-2009) the patient received biannually thoracic CT scans with progressively larger Haller Indices. Given the widening index and a new presentation of shortness of breath at the age of 14, the Nuss procedure was performed with an immediate improvement to the deformity. The bar remained in place for two years, and was successfully removed with only a mild pectus deformity remaining.

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Electronic Exhibits - Case Reports

Thoracic Imaging

Scientific Exhibits - Case Reports

More abstracts on this topic:
Diagnostic Performance of Ultra-Low (radiographic) Dose Chest CT in Pediatric Patients with Pectus Excavatum

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