Chest wall deformities

Key points

Spectrum of Chest Wall Deformities

Pectus Excavatum

Definition

Pectus Excavatum (PE), or “funnel chest,” is a congenital deformity characterised by posterior depression of the sternum and adjacent costal cartilages

Incidence and Associations

Occurs in 1:1000 live births

One-third have a positive family history

Associated conditions:

• Connective tissue disorders: Marfan, Ehlers-Danlos, Noonan

• Poland syndrome, scoliosis

Higher recurrence risk in syndromic cases

Aetiology

Failure of proper development and alignment of costal cartilage and sternum.

Multifactorial: Genetic predisposition + abnormal growth

Pathophysiology

Posterior displacement of the lower sternum compresses the heart and lungs.

Leads to:

• Cardiac:  displacement/rotation/compression → reduced stroke volume during exertion.

• Pulmonary restriction → mild restrictive lung disease (rarely obstructive)

Clinical Features

• Cardiac: Chest pain, palpitations, arrhythmias, dyspnoea on exertion

• Respiratory: Dyspnoea, exercise intolerance, atelectasis

• Psychosocial: Body image concerns, anxiety, depression

  
  

Physical Examination

Type: Symmetrical vs. asymmetrical; localised (cup-shaped) vs. diffuse (saucer-shaped)

Assess:

• Features of connective tissue disorders

• Spine for scoliosis

• Cardiac and respiratory systems

Investigations

Severity Indices

Haller Index

• Definition: Ratio of transverse diameter to AP diameter at max sternal depression

• Moderate to severe carinatum, predictor for failure of brace: <2

• Normal: ~2.5

• Mild: 2.5–3.2

• Severe: ≥3.25 (commonly used surgical threshold).

Limitation: Ignores asymmetry and limited use in sternal rotation

Solution: Correction Index (>28% significant)

Also look for:

Sternal rotation

Cardiac displacement and compression

Associated bony abnormalities

Management of Pectus Excavatum

Non-Surgical:

Exercise and posture training

Psychological support

Vacuum Bell Therapy:

• Silicone suction cup creates negative pressure

• Used in mild/moderate, compressible deformities

• Regimen: 1–2 hrs daily for ≥8 months.

• Contraindications: Severe mixed deformity, bleeding disorders, cardiac anomalies. Certain dermatological conditions

• Complications can be: Haematoma, petechia, paraesthesia

Alternatives are:

• Silicone filling

• Magnet device

Surgical

Nuss Procedure (MIRPE)

Ideal age: 11–14 years

Indication: Symmetrical severe PE with symptoms

Technique:

• Small bilateral thoracic incisions.

• Retro-sternal tunnel created under thoracoscopic guidance

• Tape pulled along the tunneller

• Bar inserted and rotated to lift sternum

• Stabilisers prevent migration

Materials: Stainless steel (contains nickel) or titanium (MRI safe, for allergy cases)

Pain control: Cryoablation of intercostal nerves. Epidural, Paravertebral block

Complications

• Cardiac or pericardial injury - If requiring defibrillation with Nuss bar in situ - place pads anterior and posterior

• Pneumothorax, infection

• Bar displacement, allergic reaction

• Chronic pain (10–20%)

  
  

Outcones

• Elective bar removal: 2–3 years post-op

• Early removal if: Infection, migration, allergic reaction, refractory pain

• Weak evidence for improvements in cardiac (RV) and respiratory function

• May give moderate improvements in exercise tolerance

Alternative Surgical Approaches

Modified Ravitch: Open resection and sternal osteotomy

Abramson technique: Reverse Nuss for Pectus Carinatum

Hybrid repairs for redo cases

Pectus Carinatum

Features:

Anterior protrusion of the sternum and costal cartilages

Often associated with scoliosis and connective tissue disorders

Management:

Dynamic compression bracing (first-line): 8 hrs/day for ~8 months.

Surgical: Ravitch or Abramson procedure

Ravitch procedure:

Open

Midline incision in males

Submammary in females

Reflect recti

Parasternal chondrotomy

Watch for internal mammaries

Can do second osteotomy

Trans-sternal bar +/-additional struts for excavatum (not necessary for carinatum

Pectus less invasive extra-pleural repair (PLIER) needed No chest drain

If the patient also requires scoliosis surgery, they should be done separately, it does not matter which order

Conservative managment and operations are no longer funded by the NHS, and must be funded privately by the patient or with an individual funding request

Standard scenario

Patient with chest wall deformity

Concerns:

1. Functional (Cardiorespiratory)

2. Possible association/underlying condition

3. Cosmetic/psychological

History

Cardiorespiratory - Exercise tolerance

Psychological symptoms

Previous neonatal thoracic surgery

Co-morbidities

Examination

Deformity, symmetry, compressibility if carinatum defect

Spinal examination

Cardiac - displaced apex, murmur of mitral prolapse

Marfanoid features

Investigations

Echo

Spirometry

Not usually funded on NHS, however if in a setting where it is (e.g. Successful individual funding request, private practice, local trust arrangement)

Proceed to:

Clinical photography and CT scan

3D scanning

Management:

Conservative to start

Excavatum: Vacuum bell

Carinatum: Brace

Operative:

Excavatum:

VATS assisted Nuss

Nickel allergy testing first

If allergic - need pre-formed titanium bar

Carinatum:

Ravitch procedure - Parasternal chondrotomy

Other conditions associated with chest wall deformities

Thoracic Insufficiency Syndrome (TIS)

A group of disorders defined by the “inability of the thorax to support normal respiration or lung growth” (Robert Campbell)

Includes Jeune and Jarcho-Levin syndromes

Pathophysiology

Normal lung growth needs:

• 3D thoracic expansion

• Spine and ribs growing synchronously

TIS occurs with congenital rib fusions, scoliosis, rib agenesis

Clinical Features

Progressive respiratory failure

Restrictive lung disease

Poor exercise tolerance, recurrent infections

Management

VEPTR (Vertical Expandable Prosthetic Titanium Rib):

·        Expands thorax, corrects scoliosis

·        Requires staged expansions every 6–12 months

Alternative: Growth-friendly spinal rods

Goal: Delay definitive fusion until skeletal maturity

Jarcho-Levin Syndrome

Severe anomalies of the vertebral column and ribs resulting in a shortened thoracic cavity

Jeune Syndrome (Congenital Asphyxiating Thoracic Dystrophy)

1 in 100, 000 liver births

Autosomal recessive chondroplasia - Intraflagellar transport 80 (IFT80) mutations

Small rigid thorax - horizontal ribs

Pulmonary hypoplasia, renal abnormalities, long bone shortening, polydactyly

CT chest

Surgical options:

Median sternotomy - methacrylate cement inbetween

Lateral thoracic expansion:

Divide ribs 4-7 starting anterior and alternating posterior - then join 2 ribs together

Divide ribs 3 + 8 in middle and leave open

Sternal clefts

A cleft in the upper aspect of the sternum is not associated with any syndromes

Upper sternal defects paradoxically deepen with inspiration and protrude with expiration

Lower sternal clefts are associated with Pentalogy of Cantrell

PHACES (Posterior fossa malformations, facial Hemangiomas, Arterial, Cardiac, and Eye anomalies, Sternal cleft, and Supraumbilical raphe syndrome)

Complete sternal clefts are the rarest, with complete separation of the sternum from the manubrium to the xiphoid

Repair:

In first few weeks of life - primary suture closure

If older than 1 year - needs bone/cartilage graft

Poland syndrome

1 in 10,000 -100,000 live births

Vascular accident (subclavian) or mesodermal plate disruption.

Congenital absence or underdevelopment of the sternocostal part of the pectoralis major, and sometimes the pectoralis minor, usually affecting one side of the body

Additional associated features:

• Hand abnormalities in 66% of cases, with symbrachydactyly being the most common, involving absence of middle phalanges

• Absence of hair in the axilla on the affected side

• Abnormal or absent breast and areolar development in approximately 50% of cases

• Missing or underdeveloped ribs may also be present

Physical examination should assess the size and strength of the serratus anterior, latissimus dorsi, and trapezius muscles, with particular attention to the latissimus dorsi, especially if surgery is planned

CT or MRI

Surgery:

Rib reconstruction

Female breast reconstruction with lat dorsi flap + implants

Surgical intervention in Poland Syndrome is generally considered for psychological, cosmetic, or functional reasons and is recommended after puberty

The objectives of surgery include improving chest wall symmetry, creating the anterior axillary fold, and reconstructing the hand if necessary

Surgery is only indicated in children if they have cardiorespiratory compromise

It is performed in two stages:

1. Pre-pubertal skeletal reconstruction

2. Thoracic soft tissue reconstruction after puberty in one stage

Page edited by Prof. Ashok Daya Ram MBBS, FRCS, FRCPS, FEBPS, FRCS (Paed Surgery), Consultant Paediatric and Neonatal Surgeon, Norfolk and Norwich University Hospital, Norwich, UK. October 2025

References

SPRINT Paediatric Surgery teaching series

Nolan, Heather, et al. "Chest Wall Deformities." Pediatric Surgery NaT, American Pediatric Surgical Association, 2021. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829074/all/Chest_Wall_Deformities.

González, Raquel, et al. "Pectus Carinatum." Pediatric Surgery NaT, American Pediatric Surgical Association, 2022. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829076/all/Pectus_Carinatum.

Chandler, Nicole, et al. "Pectus Excavatum." Pediatric Surgery NaT, American Pediatric Surgical Association, 2022. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829075/all/Pectus_Excavatum.

González, Raquel, et al. "Poland Syndrome." Pediatric Surgery NaT, American Pediatric Surgical Association, 2021. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829077/all/Poland_Syndrome.

Berg, Patrick, et al. "Sternal Cleft." Pediatric Surgery NaT, American Pediatric Surgical Association, 2021. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829602/all/Sternal_Cleft.

Berg, Patrick, et al. "Thoracic Dystrophy." Pediatric Surgery NaT, American Pediatric Surgical Association, 2021. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829078/all/Thoracic_Dystrophy.