Pectus Excavatum (Cobbler's Chest, Sunken Chest, Funnel Chest)

Clinical Features:

• Anomaly that arises from posterior depression (sunken appearance) of the sternum.
• May compress the heart against the spine, deviating the heart to the left.

Complications:

• Cardiac - apparent cardiomegaly, cardiac compromise, mitral valve prolapse
• Respiratory - lung basal collapse, respiratory compromise, restrictive airway disease
• Skeletal - back pain, chest pain
• Psychosocial - cosmetic issues

Epidemiology

• Pectus excavatum occurs in approximately 1 in 150 to 1000 births.
• There is a male predominance (male-to-female ratio of 3:1).
• Family members affected in 35% to 45% of cases.[1][2]

Associations:

• Isolated anomaly (most commonly)
• Diaphragm abnormalities
• Familial (~37% have 1st degree relative)
• Fetal alcohol syndrome
• Homocystinuria
• Intra uterine pressure
• Marfan syndrome
• Noonan syndrome
• Prematurity
• Rickets
• Spinal muscular atrophy

Chest Xray Findings:

• May mimic cardiomegaly.
• Displacement of the pulmonary vascularity & obscured right cardiac border resemble a right middle lobe process.
• Posterior ribs are horizontal.
• Anterior ribs have a more vertical course.

Management - Haller Index Calculation:

• The Haller index can be used based on CT scan measurements.
• The Haller index is the ratio between the horizontal distance of the inside of the ribcage & the shortest distance between the vertebrae & sternum.[3]
• A Haller index over 3.25 is often defined as severe.[4]

Management - Surgery:

• Surgical correction - relief of restrictive airway disease & for cosmesis.
• Techniques include - Magnetic mini-mover procedure, Nuss procedure, Ravitch technique, & Vacuum bell technique.

References:

* [1] - Andre Hebra. "eMedicine — Pectus Excavatum". [link]
* [2] - "Pectus Excavatum: Frequently Asked Questions: Surgery: UI Health Topics". Harold M. Burkhart and Joan Ricks-McGillin. [link]
* [3] - Haller JA, Kramer SS, Lietman SA (1987). "Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report". J. Pediatr. Surg. 22 (10): 904–6.
* [4] - Jeannette Diana-Zerpa; Nancy Thacz Browne; Laura M. Flanigan; Carmel A. McComiskey; Pam Pieper (2006). Nursing Care of the Pediatric Surgical Patient (Browne, Nursing Care of the Pediatric Surgical Patient). Sudbury, Mass: Jones & Bartlett Publishers. p. 253. ISBN 0-7637-4052-7.
* Shamberger RC (1996). "Congenital chest wall deformities". Curr Probl Surg 33 (6): 469–542.

Image Credit (Top): Chest Xray by Dr Donna D'Souza on RadPod (cc)

Image Credit (Top): Scar Post Surgery by Jordan Hackworth on Flickr (cc)

Tags: Back Pain - Chest Pain - Funnel Chest - Homocystinuria - Marfan Syndrome - Mitral Valve Prolapse - Noonan Syndrome - Pectus Excavatum - Prematurity - Rickets - Spinal Muscular Atrophy - Surgery
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