GGC Paediatric Guidelines
Pectus excavatum (PEX) is a congenital chest wall abnormality presenting in childhood, with a male: female ratio of 4:1. The severity of the abnormality has traditionally been quantified by cross sectional imaging techniques using the Haller index (where an index greater than 3.25 was considered moderate to severe), 1 and other indices such as the correction index that may be more useful in asymmetrical cases. The Haller Index is useful, but its value has been challenged since its first publication, with indications that there may be age or gender related differences, however an index greater than 2.7 is indicative of severity. 2 Therefore not only cross sectional imaging but symptomology, both physiological and psychological, will aid in decision making towards surgical repair. Surgical intervention for PEX is well established in the form of the Nuss and Ravitch procedures. 3
Cardio-respiratory investigation & management of adolescents with pectus excavatum
Objectives
We have developed this multidisciplinary team (MDT) guideline to rationalise the cardiorespiratory investigation and management of patients presenting with marked pectus excavatum. In particular to determine which physiological factors may influence surgical repair, in particular cardiorespiratory function and any degree of impairment.
Scope
This guideline brings together the multidisciplinary team involved in the care of teenagers with pectus excavatum: chest wall deformity team, including paediatric surgical team and specialist physiotherapists; paediatric cardiology; radiology and paediatric respiratory team.
Audience
This guideline is intended for member of the MDT caring for children with pectus excavatum and to ensure appropriate investigations are undertaken in a timely manner to optimise patient care.
The impact of a severe PEX on the underlying heart structures, in particular the anteriorly placed right ventricle (RV), have been documented in adults. 4 Transthoracic echocardiography (TTE) is used to ensure normal cardiac anatomy, in particular the absence of mitral or tricuspid valve prolapse,5 but more importantly to assess for evidence of RV compression. Unlike the left ventricle (LV), assessing right ventricular function is challenging by TTE and therefore other modalities, for example, cardiac MRI provide detailed information regarding both cardiac anatomy and biventricular function. 6
The Scottish National Chest Wall Service sees between 100-165 new patients per year, with over 35% having pectus excavatum. Over the past 3 years since the service was commissioned, between 12-13 patients have been listed for a Nuss operation each year. The decision to operate takes into account psychological factors, severity of defect and objective markers of severity such as cardiopulmonary exercise testing (CPET) and CT scans. The limitations of the CT scan that does not give any measure of physiological impact of the pectus defect, have led to the development of the outlined pathway for patients being considered for surgery, either due to severity, symptomatology or CPET results.
* Follow up as per paediatric cardiology consultant advice
* If no evidence of RV compression on echo – CT or a non-functional, non contrast MRI of the chest (rather than formal Cardiac MR with volumes) can also be used for surgical planning.
*** If evidence of RV compression pre op, patient will receive cardiology follow up post NUSS bar removal – this may be with paediatric or adult cardiology and will be a new referral from the surgical team