How not to do it: restrictive thoracic dystrophy after pectus excavatum repair

Abstract

The purpose of our paper is to call attention to acquired restrictive thoracic dystrophy (ARTD), an iatrogenic disease. The condition may occur following correction of pectus excavatum in young patients and is characterized by a reduced and restricted rib cage, usually with some recurrence of the deformity. The authors personal experience with five such patients includes analysis of their operative notes, radiographs, and long-term follow up. These, as well as literary, data indicate that the cause of ARTD is not that the operation is performed at an early age, but that inappropriate surgical technique was performed that may include radical chondrocostal resection, extirpation of growth centers, and suturing together of the perichondrial strips retrosternally, consequentially causing cartilaginous growth behind the sternum. A well-designed, conservative operation may be safely performed at any age, including in children less than 4 years old.

Diminished pulmonary function in pectus excavatum: from denying the problem to finding the mechanism

“…Around the time of Nuss' first report in 1997, there was a realization that open operation could be complicated by "acquired Jeune's syndrome" (9). This postoperative complication occurred in very young children (aged 3 to 5 years) and involved failure of the chest wall to grow following overly extensive resection of costal cartilages.…”

Contemporary management of recurrent pectus excavatum

“…Both age and surgical technique of primary repair are crucial factors implicated in recurrence. Previous investigators have suggested ineffective or extensive resection of the deformed costal-cartilages as a risk for recurrence following Ravitch operation [3,17,18]. In contrast, the main challenge of Nuss procedure, which exploits the strength of a convex steel bar against the deformed sternum, is to maintain the remodeling of the chest wall for a sufficient duration that the new contour is permanent.…”

Distraction Osteogenesis of Multiple Ribs for the Treatment of Acquired Thoracic Dystrophy

“…A hypoplastic, abnormally developed thorax has been described in patients after repair by the open Ravitch technique or other modified open repair techniques on young children. [3][4][5] The proposed etiology of this thoracic dystrophy is decreased longitudinal rib growth resulting from extensive resection of rib cartilage and injury to the costochondral junction in young patients during repair. 4,5 The disruption of the cartilage growth plate limits the growth and proper development of the chest wall.…”

“…[3][4][5] The proposed etiology of this thoracic dystrophy is decreased longitudinal rib growth resulting from extensive resection of rib cartilage and injury to the costochondral junction in young patients during repair. 4,5 The disruption of the cartilage growth plate limits the growth and proper development of the chest wall. This results in decreased thoracic volume and may cause restricted pulmonary function and cardiac compression.…”

“…This results in decreased thoracic volume and may cause restricted pulmonary function and cardiac compression. 3,5,6 The optimal treatment approach to increase thoracic volume by surgical intervention has been controversial and must accommodate chest wall expansion as children continue to grow. [7][8][9][10][11][12][13] Distraction osteogenesis, first presented by Codivilla in 1905 and revisited by Ilizarov starting in the 1950s, 14 describes the phenomenon in which separation and gentle traction of skeletal elements leads to metabolic activation and eventual bone formation between the separated skeletal elements.…”