Chest wall deformities
Key points
1:1000 M:F 80:20
1/3 Family history/from birth/connective tissue disorder or scoliosis
Most common is pectus excavatum - 2.6% of children, 88% of all chest wall deformities
Pathophysiology
Costal processes form on vertebra at week 5 gestation
Sternum develops from sternal bars - ribs contact them at weeks 7-9
Associations
Marfan syndrome
Ehlers-Danlos
Noonan syndrome
Can be acquired after surgery
Imaging
CT features:
Haller index = Transverse diameter divided by Anterior-posterior
>3.2 = severe Excavatum, predictor of need for surgery
<2 = moderate to severe carinatum, predictor for failure of brace
Also look for:
Sternal rotation
Cardiac displacement and compression and any
Associated bony abnormalities
Conservative management options (Pectus excavatum)
Vacuum bell
18 months, 3x per day 30 mins-1hour, follow up 3 years
Complications can be: Haematoma, petechia, paraesthesia
Silicone filling
Magnet device
Conservative management options (Pectus carinatum)
Pectus brace
14-16 hours per day, 18 months
Surgical management options
VATS assisted Nuss procedure can be done for symmetrical pectus excavatum
Nickel allergy test - Stainless steel bars can be bent to the patients shape, but titanium bars need to be pre-ordered. 1 or 2 bars can be inserted
Suture/wire bar to external chest wall fascia
Early complications: pneumothorax (most common), pleural effusion, hemothorax, pericarditis, pneumonia, wound infection, nickel allergy
Late complications:
Bar and stabiliser dislocation 5-15%, infection, skin erosion, late allergic reactions, recurrence of deformity
If requiring defibrillation with Nuss bar in situ - place pads anterior and posterior
Weak evidence for improvements in cardiac (RV) and respiratory function
May give moderate improvements in exercise tolerance
Ravitch procedure - can be used for both excavatum and carinatum
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
18 months, 3x per day 30 mins-1hour, follow up 3 years
Carinatum:
Pectus brace - 14-16 hours per day, 18 months
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
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
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
Vertically Expandable Prosthetic Titanium Rib (VEPTR)
Surgery only mild to moderate improvement
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:
Pre-pubertal skeletal reconstruction
Thoracic soft tissue reconstruction after puberty in one stage
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.