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The Pectus Care Guidelines: Best Practice Consensus Guidelines From the Joint Specialist Societies SCTS/MF/CWIG/BOA/BAPS for the Treatment of Patients With Pectus Abnormalities

Thursday, July 18, 2024

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Source

Source Name: European Journal of Cardio-Thoracic Surgery

Author(s)

Joel Dunning, Clare Burdett, Anne Child, Carl Davies, Deborah Eastwood, Tim Goodacre, Frank-Martin Haecker, Simon Kendall, Shyam Kolvekar, Lisa MacMahon, Sean Marven, Sarah Murray, Babu Naidu, Bejal Pandya, Karen Redmond, Aman Coonar

Pectus defects are a group of congenital conditions found in approximately 1 in 250 people, where the sternum is depressed back toward the spine (excavatum), protrudes forward (carinatum) or, more rarely, is a mixture of both (arcuatum or mixed defects). For the majority of patients, it is well tolerated, but some patients are affected psychologically, physiologically or both. 

The deformity becomes apparent at a young age due to the growth of the ribs and the cartilage that links them to the sternum. The majority of defects are mild and are well tolerated, i.e. they do not affect activity and do not cause psychological harm. However, some young people develop lower self-esteem and depression, causing them to withdraw from activities, such as swimming and dancing, and from interactions that might “expose” them, such as sleepovers, dating, going to the beach, and wearing fashionable clothing. This psychological harm occurs at a crucial time during their physical and social development. A small number of patients have more extreme depression of their sternum that impedes their physiological reserve, which can occur when engaging in strenuous exercise, such as running, but can also limit moderate activity, such as walking and climbing stairs. The effects can be so extreme that symptoms occur at rest or cause life-threatening compression of the major blood vessels and organs. The group of patients with physiological impairment typically also suffer from low self-esteem and depression. 

This paper summarizes the current evidence for the different treatment strategies for this condition, including supportive care, psychological support and nonsurgical techniques including bracing and vacuum bell therapy. The authors also consider surgical techniques including the Ravitch procedure, the Nuss procedure (minimally invasive repair of pectus excavatum), pectus implants, and other rare procedures such as Pectus Up. 

For the majority of patients, supportive care is sufficient, but for a minority, a combination of the other techniques may be considered. 

This paper also outlines best practice guidance for the delivery of such therapies, including standardized assessment, consent to treatment, audit, quality assurance, and long-term support. 

All the interventions have risks and benefits that the patient, parents, and clinicians need to carefully consider and discuss when deciding on the most appropriate course. The authors hope this evidence review of “Best Practice for Pectus” will make a significant contribution to those considerations and help all involved—from patients to national policy makers—to deliver the best possible care. 

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