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Pectus Conditions


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What is Pectus Deformity?

Pectus deformity refers to a group of conditions that cause malformation of the chest. Pectus excavatum and carinatum, also called funnel and pigeon chest respectively, are the most common of these. In these two conditions, the breastbone (Sternum) is either sunken or elevated above the level of the ribs, creating a visual defect in the middle of the chest. A third, less common variant, known as pectus arcuatum occurs when there is a deformity at the join between the upper and lower parts of the breastbone, creating a step-like appearance.

Pectus deformities occur in 1 to 400-1500 children, with males affected more than females. A small proportion of individuals will present with a pectus deformity as part of a wider connective tissue condition, the most common of these being Marfan’s Syndrome. Some patients may also receive additional diagnoses such as scoliosis, and kyphosis.

The video below provides more information and is from the Pectus Engagement Event held at the Royal College of Surgeons of England on February 2nd, 2023. At this event, clinicians, MPs, Journalists, commissioner’s, patients, and their families discussed the important issues facing those suffering from pectus deformity and launched the most recent guidelines for optimal management.

The PowerPoint below provides additional information, delivered by Consultant Thoracic Surgeon, Mr Ian Hunt.

 

 

Effects of Pectus Conditions

There are varying degrees of pectus deformity which impact individuals in different ways. Current research is being undertaken into the wide-ranging pathophysiological effects of pectus deformities. The following non-exhaustive list includes some of the symptoms reported by patients.

Physical effects 

  • Chest pain
  • Difficulty breathing
  • Episodes of wheezing and coughing
  • Feeling dizzy or faint
  • Increased fatigue and weakness
  • Decreased exercise tolerance
  • Irregular heartbeat (arrythmia)
  • Difficulty swallowing (dysphagia)

Psychological / emotional effects

  • Low self esteem
  • Anxiety and depression

It is recognised that a pectus deformity can cause profound psychological and emotional issues for patients, especially for children as they are growing up and the deformity becomes more visible. It is encouraged to discuss these concerns with a GP or thoracic surgeon to explore treatment options. 

 

What are the treatments available?

There are several treatment options for pectus deformity, ranging from non-surgical options such as psychological support, physiotherapy, bracing and vacuum bell therapy to minimally invasive surgical corrective options such as Nuss, PectusUP or modified Ravitch, otherwise open Ravitch. Cosmetic based implant procedures can also be considered.

 

Conservative options

Clinical Psychology

Pectus deformities can have a significant impact on body image and quality of life in both adults and children. It is therefore important to acknowledge that support from specialists in psychology may, for some individuals, form an important element of the treatment required with or without other conservative or surgical treatments. This service can be accessed via your GP.

Vacuum Bell Therapy

Vacuum bell therapy is designed for mild forms of pectus excavatum (funnel chest). A suction cup is placed on the sunken area of the chest and a patient activated vacuum device is triggered to gradually correct the deformity.  The video below illustrates an example fitting of the vacuum bell therapy system.

→ Vacuum Bell Therapy

Bracing

Bracing techniques are most used often used for pectus carinatum (pigeon chest). Gradual pressure is applied to the protruding breastbone and ribs, which overtime corrects the deformity of the chest wall. This form of treatment often depends on patient age and the maturity of the skeleton. The animation below illustrates an example fitting of the bracing system.

→ Bracing

Physiotherapy

Physiotherapy with a tailored exercise prescription is a recommended adjunct to surgical and non-surgical treatment options for improved functional outcome.

Please note:
The type of device used should be discussed with your thoracic or paediatric surgeon to ensure they meet health and safety standards. As with all interventions, there are risks and benefits associated with conservative treatments. The main risks include damage to skin through abrasion from the devices as well as treatment failure. The benefits include possible correction of the deformity without more invasive surgical intervention. We encourage you to discuss these with your surgeon.

 

Surgical options

Surgical options can be considered for several reasons which range from cosmetic to functional with impaired exercise tolerance. The following extra tests may be needed:

  • Computerised Tomography (CT) Scan or Magnetic resonance Imaging (MRI). This is to assess the shape of the chest in detail and work out the Haller index.
  • Electrocardiogram (ECG) and Echocardiogram. This is to check the function of the heart.
  • Cardiopulmonary exercise test (CPET)
  • Lung function tests
  • Marfan’s Risk Calculator

Several surgical techniques (all under general anaesthetic) are available:

The Ravitch or Modified Ravitch procedures

Indication: Asymmetrical Pectus Excavatum or Pectus Carinatum

The Ravitch procedure involves cutting away the cartilages causing the pectus deformity. The breastbone position is then changed (brought forward or pushed back). Sometimes, surgeons may place a small metal bar or mesh to provide additional support. More commonly the patient’s own muscles are used to provide the support.

The video below illustrates the operation (each surgeon may perform a slightly different version of the procedure; this would be discussed in clinic). Please note the video contains graphic images, and parental supervision is advised for viewers under the age of 18.

→ The Ravitch or Modified Ravitch procedures

The Nuss Procedure

Indication: Pectus Excavatum

The Nuss procedure relies on the flexibility of the chest. 2 small cuts are made on the side of the chest. A curved titanium bar is put in to push the breastbone forwards. The bar then stays in place to support the new shape for 2 to 3 years before being removed in a second operation. Sometimes 2 or 3 bars are needed. The bar is put in under the skin and behind the breastbone. A small camera is used to guide the bar as it is put in the chest. The ‘u‘ shaped bar is flipped to an ‘n’ position to push the breastbone forward. No cartilage is removed or cut. This procedure is less invasive than the Ravitch procedure.

A second operation is needed to remove the bar(s). The Nuss procedure is less effective at correcting asymmetrical deformities, which are likely to still be present after the procedure. The animation below illustrates an example of the Nuss procedure (your surgeon may perform a slightly different version of the procedure; this would be discussed with you in clinic).

→ Nuss Procedure

Pectus-Up

Indication: Pectus Excavatum

Pectus-Up also known as Taulinoplasty is a relatively newer technique that uses a plate-like implant or prosthesis and a sternal tractor screw. Together they are used to raise the sternum to the desired position. The video below illustrates the surgery (each surgeon may perform a slightly different version of the procedure; this would be discussed in clinic).

Please note the video contains graphic images, and parental supervision is advised for viewers under the age of 18.

→ Pectus Up 

Anatomical Implant Procedure

Indication: Pectus Excavatum

For pectus excavatum with normal exercise tolerance, pectus implants can be used as an alternative to more invasive procedures. It involves inserting an implant into a pectus excavatum defect, which sits under the skin. The video below demonstrates the operation. (each surgeon may perform a slightly different version of the procedure; this would be discussed in clinic).

Please note the video contains graphic images, and parental supervision is advised for viewers under the age of 18.

→ Anatomical Implant Procedure 

It is important to note that the availability of each of these procedures, particularly Pectus-Up and anatomical implants vary depending on the local health service provider.

It is important to note that the availability of each of these procedures, particularly Pectus-Up and anatomical implants vary depending on the local health service provider. 

 

 

What are the benefits and risks of having surgery?

What are the benefits of having surgery?

All operations aim to improve the appearance of your chest and in some cases reduce the physical symptoms caused by the deformity. This has both psychological and possible physiological benefits. There is ongoing research which supports that surgical intervention may improve the function of the heart and lungs (and overall quality of life).

What are the risks of surgery?

All operations are considered major surgery and can involve a long period of recovery and rehabilitation. As with all surgery there are several risks involved when seeking surgical correction of a pectus deformity. You should discuss these with your surgeon. Complications following surgery are rare but can be serious.

Below is a guide to some of the possible risks:

Common risks

  • Wound infection
  • Redness and thickening of the scar.
  • Collapsed lung – treated with a chest drain.
  • Collection of fluids under the skin or around the lung – may require a chest drain.

Less common risks

  • Movement of inserted bars in Nuss or PectusUp
  • Movement of the chest back to its pre-surgical shape
  • Bleeding from heart and blood vessels – may require a blood transfusion.
  • Death because of major bleeding or reactions to the anaesthetic.

Will I have pain after the operation?

Pain is expected in all the surgical options, but particularly in the Nuss, Ravitch and PectusUP procedures. Your surgeon and anaesthetist should discuss methods of pain control with you, especially if you have any concerns.

Immediately after surgery, you will be started on strong pain control which will be reduced as your pain improves. Often Nuss patients will have an epidural as a method of pain control. The severe pain following surgery should subside after a few days. You can expect to have some pain and discomfort for a few weeks or months after the surgery.

 

What is recovery like?

The average stay in hospital is about 5 days, with a shorter stay for the anatomical implant. You will be discharged from hospital once you are well enough. You will usually need to meet these criteria:

  •  Chest drains removed
  •  Able to walk on your own and upstairs
  •  Pain is well controlled
  •  Bowels working
  • Support from friends and family at home

Once you go home you will need to continue painkillers, which can be reduced as your pain improves. You should not suddenly stop taking pain relief. If you require more tablets your GP can prescribe these. You can gradually return to activity as you feel able, however you may experience increased tiredness at first, this will improve. It can take up to a month to feel comfortable to lay on your side, and a few months to a year to return to activities that involve heavy pushing or lifting.

The video below may help to answer other questions you may have about the surgery and recovery, although detailed information specific to your case will be provided by your surgical team.

→ Pectus Excavatum Q&A Dr Shyam Kolvekar 

 

What is the referral pathway for treatment in the Republic of Ireland?

Patients seeking treatment in the Republic of Ireland (ROI) can be referred by their GP or other clinicians. All surgery is publicly funded. Conservative treatment options such as vacuum bell therapy and bracing are not publicly funded for adults and will need to be sourced privately. Vacuum bell therapy is publicly funded for children.

The following list provides more information on surgeons/centres and the available treatment options: 

Adults
Prof. Karen Redmond 
Mater Misericordiae University Hospital, Dublin.
Treatments offered: NUSS, PectusUP, Anatomik Modelling Implant, Ravitch for PE/PC Slipping Rib/Rib Flare deformities. 
PA Tel: +353 1 8032162 
PA Email: ALeavy@mater.ie
Fax: +353 1 8034048 

Adults and Children
Prof. Mark Redmond, Mr Jonathan McGuinness, Mr Lars Nölke
Children's Health Ireland, Crumlin Hospital, Dublin
Treatments offered: Vacuum Bell therapy, Pilegaard Procedure, Ravitch for PE/PC deformities.

Private options
Pectus Check  
Treatments offered: Vacuum bell therapy, bracing, NUSS, PectusUP, Anatomik Modelling Implant, Ravitch for PE/PC/Slipping Rib/Rib Flare deformities. 
PA Tel: +353 85 2562200
PA Email: info@sshi.ie
Fax: +353 1 5563415 (GDPR Compliant)

Beacon Hospital, Dublin 
Treatments offered: bracing for PC deformities.

 

What is the referral pathway for treatment in Wales?

The referral pathway in Wales will depend on the pectus deformity.

Pectus Carinatum (pigeon chest)

For pectus carinatum a GP can refer to Consultant Thoracic Surgeon, Mr Vasileios Valtzoglou. Following an initial outpatient assessment the most appropriate treatment pathway will be decided. If the decision is to proceed with surgery this will be carried out via the NHS. Conservative options which include brace therapy will need to be funded privately, however follow up to monitor progress can be arranged via the NHS by contacting the team’s secretary on the number below.

Pectus Excavatum (funnel chest)

For pectus excavatum, a GP can refer to Consultant Thoracic Surgeon Miss Malgorzata Kornaszewska. Similarly, following an initial outpatient assessment, a discussion will decide the most appropriate treatment pathway. If the decision is to proceed with surgery this again be carried out via the NHS. Conservative options which include vacuum bell therapy will need to be funded privately, however follow up to monitor progress can be arranged via the NHS by contacting the team’s secretary on the number below.

Both Consultant Surgeons are based at University Hospitals of Wales in Cardiff. The department can be contacted via their secretary on 02920743284

 

What is the referral pathway for treatment in England?

For patients in England,  a referral can be sent to a thoracic surgeon or for children, too a paediatric centre with a pectus service. This referral can be made by any clinician but will most often be your GP. Not all hospitals are able to assess patients for pectus care so your referral may be sent on to another unit.

The pectus care assessment unit may see you and undertake several investigations before discussing your case in a national multidisciplinary meeting for pectus conditions. This will decide on the best treatment pathway for your specific case.

If you go forward with surgery, the operation will be at one of several pectus surgical centres in England. These are St. Bartholomew’s Hospital in London, South Tees Hospitals NHS Foundation Trust in Middlesbrough and Alderhey Children's Hospital in Liverpool. After the operation your care will be split between the surgery centre and the local pectus care assessment unit.

A simple flowchart is shown below.

For further information about the NHS England referral criteria, pathway, and rationale for commissioning, please see the commissioning statement here:

   → Commissioning Statement

 

What is the referral pathway for treatment in Scotland?

The referral pathway in Scotland will depend on whether you are an adult or under children’s services (16 years and under) and covers the full assessment and treatment of anterior chest wall deformities that include pectus excavatum, pectus carinatum and other complex variations such as pectus arcuatum . Referrals can be made by your GPs or any other healthcare team.

Adults

Adult patients in Scotland can be referred to the Department of Cardiothoracic Surgery at the following hospitals:

Golden Jubilee National Hospital: All surgeons

Royal Infirmary of Edinburgh: Mr Malcolm Will, Consultant Thoracic Surgeon       

Aberdeen Royal Infirmary: Mr Mohammed Khalil, Consultant Thoracic Surgeon

All three centres provide the Ravitch and NUSS procedures with surgery publicly funded with no constraints. Clinical psychology is available as part of treatment in selected cases. Conservative therapies including vacuum bell therapy and bracing are not publicly funded for adults in Scotland.

Teenage patients under adult services may be referred to paediatric services (see below) where conservative treatments are publicly funded.

Children and Adolescents

Children and adolescent patients (16 years and under) in Scotland can be referred to Mr Carl Davis or Mr James Andrews, Consultant Paediatric Surgeons at the Royal Hospital for Children in Glasgow.

The service provides non-invasive treatment options such as dynamic bracing for pectus carinatum, vacuum bell for pectus excavatum and physiotherapy for the management of posture and breathing. Surgical options are reserved for those unsuitable for or not responding to the above. The Modified Ravitch procedure is the surgical treatment for pectus carinatum and arcuatum and the NUSS procedure is used for pectus excavatum. 

 

What is the referral pathway in Northern Ireland?

Treatment of patients with pectus deformity in Northern Ireland is performed by the Department of Cardiothoracic Surgery at the Royal Victoria Hospital in Belfast. GPs and other clinicians can refer to the department where patients will be assessed to discuss treatment options. Conservative treatment options including bracing and vacuum bell therapy can be discussed but these are currently only available privately. Surgical intervention is fully funded by the NHS and the service currently provides the Ravitch procedure.

 

Support Groups

If you are a patient or family member and would like to join a pectus community for further support, please visit the Pectus Support Group UK Facebook Group here: 

 → Support Group

Here is a link to the Marfan’s Trust website in the event that you have received a Marfan’s Syndrome diagnosis as part of your work up for Pectus Deformity.

 → Marfan's Trust 

 

Academic Initiatives to Advance Pectus Care

RESTORE Trial

We have designed the first RCT to compare how surgery may affect the health of those with severe Pectus Excavatum (PE). We will ask 200 people over 12 years old, with severe PE, and who have symptoms to take part. We will use questionnaires to measure physical function, quality of life, psychological wellbeing and use an exercise test called a cardiopulmonary exercise test (CPET) to measure how well peoples' heart and lungs work. We will also look at whether surgery represents value for money for the NHS and for patients. People who agree to take part will be randomised; each group will have 100 people. 

For further information on the RESTORE Trial please click here: 

https://www.fundingawards.nihr.ac.uk/award/NIHR158749

 

 

Pectus Care Guidelines 

The following link outlines the best practice consensus guidelines from the joint specialist societies on the treatment of patients with pectus abnormalities.

Pectus Care Guidelines