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Background
Chest wall deformities are a group of congenital conditions that vary widely in presentation. They may occur alone or alongside other anomalies and genetic syndromes. Their impact can range from serious health risks in early life to cosmetic and psychosocial issues during adolescence. Management involves a combination of medical treatment, surgery, and genetic counseling. Surgical approaches are complex and influenced by factors such as the patient’s age, gender, timing of intervention, and choice of materials. This review focuses on the most common chest wall deformities encountered in general thoracic surgery, including pectus excavatum, pectus carinatum, sternal clefts, ectopia cordis, and the syndromes of Poland, Jeune, and Jarcho-Levin.
Epidemiology
Pectus Excavatum
Accounting for the majority (90%) of chest wall deformities, pectus excavatum is most commonly seen in individuals of White descent, affecting about 1 in every 400 births, with a notable male predominance (5:1). While it often occurs sporadically, up to 40% of cases have a positive family history, typically following an autosomal dominant inheritance pattern. It can also be part of genetic syndromes like Marfan and Noonan. Secondary cases may result from conditions like Poland syndrome. Most diagnoses occur by age two. Associated cardiac issues may include mitral valve prolapse, arrhythmias, and, less commonly, congenital heart defects. About two-thirds of affected individuals exhibit features of the MASS phenotype.
Pectus Carinatum
The second most prevalent chest wall anomaly, pectus carinatum is about 5–6 times less frequent than pectus excavatum and occurs in approximately 0.06% of live births, with a 4:1 male dominance. It may present as an isolated anomaly or within the context of genetic syndromes, most often Marfan or Noonan. A family history is present in around 25–30% of cases. Some individuals may also have related heart abnormalities or scoliosis (in up to 14%). The chondrogladiolar variant is the most common. Symptoms typically emerge or worsen during puberty, prompting medical evaluation.
Poland Syndrome
A rare condition seen in 1 in 20,000 to 30,000 births, Poland syndrome primarily affects males (2–3:1 ratio) and typically presents unilaterally, most often on the right side in males. While usually non-genetic, rare familial cases exist. If the anomaly appears on the left side, dextrocardia may also be present. The syndrome has been linked to other conditions including Moebius syndrome, Klippel-Feil syndrome, and various malignancies such as breast cancer and leukemia.
Sternal Clefts
Sternal clefts, representing about 0.15% of chest wall defects, occur more often in females (2:1). They can be categorized into superior (most common), complete, inferior, and sternal foramen types. These defects are sometimes associated with PHACES syndrome, midline fusion disorders, and chromosomal anomalies. An inferior sternal cleft is considered a key feature of Cantrell pentalogy, while superior clefts are usually isolated.
Ectopia Cordis
A highly rare anomaly, ectopia cordis occurs in about 1 in 5.5 to 7.9 million live births. It can present in thoracic, thoracoabdominal, or cervical forms. While it can appear on its own, it often coexists with congenital heart diseases, particularly tetralogy of Fallot, and midline defects such as omphalocele. Thoracoabdominal types are frequently linked with Cantrell pentalogy.
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
This rare skeletal disorder affects approximately 1 in 120,000 live births and is associated with high early mortality, especially within the first two years. The primary issue is restricted chest growth, impairing respiratory function. It may also involve abnormalities of the clavicle, limbs, pelvis, kidneys, liver, and pancreas.
Jarcho-Levin Syndrome
A rare congenital disorder involving spinal and rib malformations, Jarcho-Levin syndrome can be inherited in an autosomal recessive or dominant pattern. Spondylothoracic dysostosis, often linked with Puerto Rican ancestry, carries a high infant mortality rate (up to 100%), whereas spondylocostal dysostosis tends to have a better prognosis. It is associated with gene mutations in DLL3 and MESP2, and may also present features like a short trunk, facial anomalies, joint stiffness, hernias, urinary tract defects, and clubfoot.
Anatomy
Pathophysiology
Pectus Excavatum
This chest wall deformity involves a sunken appearance of the sternum due to an abnormal sternocostal joint structure, leading to a reduced anteroposterior chest diameter. The severity of the chest depression can compromise heart and lung function, often causing breathlessness and chest pain, particularly during exertion. It may also coexist with congenital heart defects, and its psychological impact—especially on body image—is significant.
Pectus Carinatum
This condition is characterized by an outward protrusion of the sternum, resulting from abnormal collagen alignment in the sternocostal cartilage. Although it typically doesn’t affect cardiopulmonary function, patients may experience respiratory discomfort due to a rigid chest wall and increased residual lung volume.
Poland Syndrome
Generally presenting as a cosmetic issue, Poland syndrome rarely leads to organ dysfunction. However, associated deformities like underdeveloped chest muscles or hand abnormalities can impair function—such as arm mobility or cause complications like lung herniation or flail chest. Surgical correction is advised in such cases, especially when quality of life or function is affected. Cosmetic concerns are particularly notable in adolescent girls due to breast asymmetry.
These defects involve a gap in the sternum, exposing mediastinal structures beneath a thin tissue covering. While some clefts may be harmless, others, particularly after surgery, can lead to complications like cardiac compression or abnormal breathing patterns.
Ectopia Cordis
This rare defect results from improper closure of the ventral body wall, causing the heart to develop outside the chest. Depending on the type—thoracic, thoracoabdominal, or cervical—the heart may be partially or fully exposed, sometimes only covered by a membrane or skin. The lack of protective structures and frequent association with other congenital defects severely impacts survival.
Jeune Syndrome
A genetic disorder affecting rib development, Jeune syndrome causes a narrow chest due to short ribs that don’t extend properly. This leads to restricted lung growth and reduced ventilation capacity, with respiratory issues worsening over time.
Jarcho-Levin Syndrome
This condition stems from defective signaling in somite development, leading to abnormal spine and rib formation. In spondylothoracic dysostosis, ribs are fused symmetrically along the spine, producing a “crab-like” appearance and a rigid chest wall that impairs breathing. Spondylocostal dysostosis presents with various rib anomalies that also contribute to spinal curvature and a restrictive chest, progressively compromising lung development.
Etiology
Pectus Excavatum: The precise cause of pectus excavatum is still unclear, though current theories suggest a defect in collagen metabolism leading to excess growth of sternal cartilage, ultimately causing inward displacement of the sternum. While typically an isolated condition, genetic links have been identified. It may occur as part of inherited syndromes—such as Marfan or Noonan (autosomal dominant), osteogenesis imperfecta (types I, III, IV; autosomal recessive), and X-linked connective tissue disorders. Even in non-syndromic cases, autosomal dominant inheritance patterns have been noted. A variant known as the MASS phenotype (involving mitral valve prolapse and other connective tissue features) is associated with FBN1 gene mutations.
Pectus Carinatum: Like pectus excavatum, this condition is believed to result from abnormal cartilage development. A mutation in the COL2A1 gene, which affects type II collagen, has been linked to the disorder. The inheritance patterns mirror those of pectus excavatum, often autosomal dominant with a possible familial background.
Poland Syndrome: This condition is thought to result from developmental disruption in the womb, rather than inherited genetics. The most supported theory is an interruption in embryonic blood flow—particularly affecting the subclavian or vertebral arteries—leading to underdevelopment of chest muscles and limbs. Another theory involves a defect in lateral plate mesoderm formation. Environmental factors, such as drug or tobacco exposure during pregnancy, may also play a role. Though usually non-genetic, familial cases do exist. Bilateral forms may suggest autosomal dominant inheritance, while associations with Mobius syndrome suggest a possible recessive or X-linked pattern.
Sternal Clefts: This rare anomaly results from the failure of ventral cell migration and fusion of sternal bars during weeks 6–9 of gestation. It may be linked to mutations in Hoxb genes. While most cases carry low genetic risk, some syndromic associations—such as Cantrell’s pentalogy (X-linked) and PHACE syndrome—have been observed.
Ectopia Cordis: This life-threatening defect arises from impaired ventral folding during early embryogenesis. One hypothesis suggests that early rupture of the chorionic or yolk sac may disrupt normal thoracic development. Genetic studies implicate reduced BMP2 expression in faulty morphogenesis. Though the exact inheritance pattern is unclear, this condition often coexists with major chromosomal anomalies such as Turner syndrome or trisomy 18.
Jeune Syndrome: This condition is inherited in an autosomal recessive pattern and is caused by mutations in genes involved in intraflagellar transport—especially IFT80 and DYNC2H1. These genes play a key role in cilia function. The condition typically carries a 25% chance of occurrence when both parents are carriers.
Jarcho-Levin Syndrome: The syndrome includes two major subtypes. Spondylocostal dysostosis is associated with mutations in DLL3 and MESP2, and may follow either dominant or recessive inheritance. Spondylothoracic dysostosis, on the other hand, is linked to autosomal recessive inheritance without involvement of the DLL3 gene. Mutations in PAX1 or PAX9 genes are also implicated. Genetic risk is dependent on whether one or both parents are carriers of the mutated genes.
Genetics
Prognostic Factors
Pectus Excavatum
Surgical correction—either through open techniques or the MARPE (Minimally Invasive Repair of Pectus Excavatum) method—has demonstrated high success rates, ranging from 86% to 98.1%. Patients typically show noticeable improvements in heart and lung function within 6 to 12 months post-surgery. Additionally, in about half of the cases, mitral valve prolapse associated with the condition resolves after surgical correction.
Pectus Carinatum: While minimally invasive approaches are still under evaluation, most surgical treatments follow principles from the modified Ravitch technique, yielding comparable results in aesthetics and symptom relief to pectus excavatum corrections. Non-surgical bracing can lead to visible improvements within 2–3 months, but treatment usually lasts 2 to 2.5 years. Despite its effectiveness, some patients find the brace uncomfortable or hard to tolerate.
Poland Syndrome: Outcomes vary depending on several factors such as the patient’s age, sex, severity of the deformity, presence of cancer, and the type of reconstructive surgery performed. These factors collectively influence the long-term prognosis.
Sternal Clefts: The outlook for patients with sternal clefts is influenced by the type and extent of the defect, the patient’s age, and any associated anomalies. Superior sternal clefts are often isolated and linked to a structurally normal heart, which supports a favorable prognosis. Early surgical repair—especially in newborns—can improve outcomes, although survival mainly hinges on other coexisting conditions.
Ectopia Cordis: This condition carries a generally poor prognosis. Thoracic presentations and cases associated with complex cardiac anomalies or Cantrell pentalogy have the highest mortality. In contrast, thoracoabdominal ectopia cordis may offer a slightly better prognosis due to a more favorable heart positioning.
Jeune Syndrome: Also known as asphyxiating thoracic dystrophy, this condition typically has a poor outcome, with mortality rates between 60% and 80%. However, some specialized centers report up to a 50% survival rate in milder cases following surgical intervention. Prognosis tends to improve with age.
Jarcho-Levin Syndrome: The outcome largely depends on the subtype. Spondylothoracic dysostosis is often fatal and unresponsive to surgical treatment, although some mild cases have shown survival rates up to 56% with intensive respiratory support. Spondylocostal dysostosis tends to have a more favorable prognosis, especially when treated with procedures like VEPTR (Vertical Expandable Prosthetic Titanium Rib). Both forms are associated with normal cognitive development, and prognosis improves if the child survives beyond six months of age.
Clinical History
Pectus Excavatum
Pectus Carinatum
Poland Syndrome
Sternal Clefts
Ectopia Cordis
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
Jarcho-Levin Syndrome
Physical Examination
Physical examination of chest wall deformities involves a visual inspection, palpation, and functional assessment. Clinicians assess for asymmetry, depression or protrusion of the sternum (as seen in pectus excavatum or carinatum), and associated rib abnormalities. The shape and motion of the chest during breathing are observed to detect any respiratory restriction. Palpation helps evaluate rigidity, tenderness, or gaps (in cases like sternal clefts). Postural assessment is important, especially for spinal alignment. Cardiopulmonary auscultation may reveal murmurs or diminished breath sounds, indicating internal compression. The Haller index may be calculated using imaging for severity grading, especially in pectus excavatum.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Pectus Deformities: Marfan syndrome is the most significant condition to consider, though a range of connective tissue disorders associated with chest wall abnormalities should be included in the differential.
Poland Syndrome
Differential Considerations: Distinguish from natural breast asymmetry in females, as well as pulmonary conditions such as Swyer-James syndrome and large pulmonary bullae, which can appear as a hyperlucent hemithorax on chest radiographs.
Sternal Clefts
Differential Diagnoses: Conditions involving congenital anomalies like limb-body wall complex, amniotic band syndrome, and Cantrell pentalogy should be considered.
Ectopia Cordis
Differential Diagnoses: Often considered alongside chromosomal disorders such as trisomy 18 and Turner syndrome due to overlapping structural anomalies.
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
Differential Diagnoses: Includes other skeletal dysplasias such as Ellis-Van Creveld syndrome, types I–IV of short rib polydactyly syndrome, Barnes syndrome, and Shwachman-Diamond syndrome.
Jarcho-Levin Syndrome
Differential Diagnoses: Should be differentiated from disorders like spondyloepiphyseal dysplasia, Morquio syndrome, chondrodysplasia, Klippel-Feil syndrome, and short rib polydactyly syndrome.
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Hormone Therapy
Immunotherapy
Hyperthermia
Photodynamic Therapy
Stem Cell Transplant
Targeted Therapy
Palliative Care
use-of-a-non-pharmacological-approach-for-treating-chest-wall-deformities
Pectus Excavatum
Physical Rehabilitation: Non-operative management primarily includes exercise-based physical therapy programs aimed at improving posture, respiratory function, and chest wall muscle strength.
Suction Devices: Some centers use external vacuum bell therapy, which applies negative pressure to elevate the sternum. While it offers a non-invasive alternative, long-term efficacy data is still limited.
Pectus Carinatum
Orthotic Bracing: In patients aged 10 to 15 years with chondrogladiolar-type deformities, compression bracing is often the first-line treatment. The brace is worn regularly and adjusted over time to correct the protrusion. This method is particularly effective before skeletal maturity and may avoid the need for surgery if initiated early and compliance is good.
Poland Syndrome: Although primarily surgically managed, non-surgical cosmetic options like autologous fat grafting may enhance contour and symmetry. These are used selectively, especially for improving soft tissue appearance in mild deformities or as a supplement to surgery.
Sternal Clefts: Observation is an option for asymptomatic patients with minor clefts and no cardiopulmonary compromise. These individuals are typically followed clinically without immediate intervention.
Ectopia Cordis: Non-surgical management is mostly supportive and palliative, including vital stabilization at birth. Surgery is the definitive treatment, but immediate neonatal care forms the non-operative cornerstone during initial stabilization.
Jeune Syndrome: Non-operative management includes intensive respiratory support, especially during early infancy. The goal is to manage thoracic insufficiency and optimize pulmonary function before considering surgical intervention.
Jarcho-Levin Syndrome: Like Jeune Syndrome, aggressive respiratory care is the mainstay of early non-surgical treatment. Ventilatory support may be required due to severe thoracic restriction and impaired lung development.
Role of Analgesics
Paracetamol (Acetaminophen): It is often used as baseline analgesic for mild to moderate pain.
Example: Oral/parenteral acetaminophen 15 mg/kg every 6 hours.
NSAIDs (e.g., Ibuprofen, Ketorolac, Diclofenac): It reduces inflammation and pain; may help minimize opioid requirement.
Example: IV Ketorolac 0.5 mg/kg every 6 hours (short-term use).
Role of Opioid Analgesics
Morphine: IV bolus or PCA (Patient-Controlled Analgesia) system.
Hydromorphone: Alternative to morphine with less histamine release.
Fentanyl: IV or transdermal patch in chronic cases or during procedures.
Role of Adjuvant Analgesics
Gabapentin or Pregabalin: Neuropathic component management, especially in chronic pain.
Ketamine (low dose): Used intraoperatively to reduce central sensitization and opioid use.
use-of-intervention-with-a-procedure-in-treating-chest-wall-deformities
Pectus Excavatum
Surgical Interventions:
Nuss Procedure (Minimally Invasive Repair of Pectus Excavatum – MIRPE): Insertion of a curved metal bar beneath the sternum to elevate the depressed chest. Bar remains for 2–3 years before removal.
Intervention Support: Use of rib stabilizers, thoracoscopic guidance to reduce complications.
Modified Ravitch Procedure: Open approach with resection of abnormal cartilage and sternal osteotomy.
Intervention Support: Retrosternal bone graft or bar for support and remodeling.
Plier Technique / Leonard Modification: Reserved for very severe deformities, may include muscle flap use.
Pectus Carinatum
Modified Ravitch Procedure: Resection of protruding cartilage and placement of a retrosternal bar if needed. Often includes wedge osteotomies for realignment.
Poland Syndrome
Stage 1 (Skeletal Reconstruction): Modified Ravitch or Nuss Procedure with rib graft or metal struts. Use of custom implants or 3D-molded prosthetics.
Stage 2 (Soft Tissue Reconstruction): Myocutaneous flaps (e.g., latissimus dorsi), autologous fat grafting. In females, breast implant reconstruction may be used.
use-of-phases-in-managing-chest-wall-deformities
Management of chest wall deformities typically follows a phased approach.
Phase 1 involves early assessment and non-operative management, including physical therapy, bracing (especially for pectus carinatum), and monitoring of growth and symptoms.
Phase 2 focuses on surgical intervention, indicated when deformities cause cardiopulmonary compromise, psychological distress, or fail conservative treatment. Procedures like the Nuss or Ravitch are selected based on the type and severity of deformity.
Phase 3 is the post-operative and rehabilitation phase, emphasizing pain control, physical rehabilitation, and bar removal if applicable. This phased approach ensures personalized, safe, and effective long-term outcomes.
Medication
Future Trends
Chest wall deformities are a group of congenital conditions that vary widely in presentation. They may occur alone or alongside other anomalies and genetic syndromes. Their impact can range from serious health risks in early life to cosmetic and psychosocial issues during adolescence. Management involves a combination of medical treatment, surgery, and genetic counseling. Surgical approaches are complex and influenced by factors such as the patient’s age, gender, timing of intervention, and choice of materials. This review focuses on the most common chest wall deformities encountered in general thoracic surgery, including pectus excavatum, pectus carinatum, sternal clefts, ectopia cordis, and the syndromes of Poland, Jeune, and Jarcho-Levin.
Pectus Excavatum
Accounting for the majority (90%) of chest wall deformities, pectus excavatum is most commonly seen in individuals of White descent, affecting about 1 in every 400 births, with a notable male predominance (5:1). While it often occurs sporadically, up to 40% of cases have a positive family history, typically following an autosomal dominant inheritance pattern. It can also be part of genetic syndromes like Marfan and Noonan. Secondary cases may result from conditions like Poland syndrome. Most diagnoses occur by age two. Associated cardiac issues may include mitral valve prolapse, arrhythmias, and, less commonly, congenital heart defects. About two-thirds of affected individuals exhibit features of the MASS phenotype.
Pectus Carinatum
The second most prevalent chest wall anomaly, pectus carinatum is about 5–6 times less frequent than pectus excavatum and occurs in approximately 0.06% of live births, with a 4:1 male dominance. It may present as an isolated anomaly or within the context of genetic syndromes, most often Marfan or Noonan. A family history is present in around 25–30% of cases. Some individuals may also have related heart abnormalities or scoliosis (in up to 14%). The chondrogladiolar variant is the most common. Symptoms typically emerge or worsen during puberty, prompting medical evaluation.
Poland Syndrome
A rare condition seen in 1 in 20,000 to 30,000 births, Poland syndrome primarily affects males (2–3:1 ratio) and typically presents unilaterally, most often on the right side in males. While usually non-genetic, rare familial cases exist. If the anomaly appears on the left side, dextrocardia may also be present. The syndrome has been linked to other conditions including Moebius syndrome, Klippel-Feil syndrome, and various malignancies such as breast cancer and leukemia.
Sternal Clefts
Sternal clefts, representing about 0.15% of chest wall defects, occur more often in females (2:1). They can be categorized into superior (most common), complete, inferior, and sternal foramen types. These defects are sometimes associated with PHACES syndrome, midline fusion disorders, and chromosomal anomalies. An inferior sternal cleft is considered a key feature of Cantrell pentalogy, while superior clefts are usually isolated.
Ectopia Cordis
A highly rare anomaly, ectopia cordis occurs in about 1 in 5.5 to 7.9 million live births. It can present in thoracic, thoracoabdominal, or cervical forms. While it can appear on its own, it often coexists with congenital heart diseases, particularly tetralogy of Fallot, and midline defects such as omphalocele. Thoracoabdominal types are frequently linked with Cantrell pentalogy.
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
This rare skeletal disorder affects approximately 1 in 120,000 live births and is associated with high early mortality, especially within the first two years. The primary issue is restricted chest growth, impairing respiratory function. It may also involve abnormalities of the clavicle, limbs, pelvis, kidneys, liver, and pancreas.
Jarcho-Levin Syndrome
A rare congenital disorder involving spinal and rib malformations, Jarcho-Levin syndrome can be inherited in an autosomal recessive or dominant pattern. Spondylothoracic dysostosis, often linked with Puerto Rican ancestry, carries a high infant mortality rate (up to 100%), whereas spondylocostal dysostosis tends to have a better prognosis. It is associated with gene mutations in DLL3 and MESP2, and may also present features like a short trunk, facial anomalies, joint stiffness, hernias, urinary tract defects, and clubfoot.
Pectus Excavatum
This chest wall deformity involves a sunken appearance of the sternum due to an abnormal sternocostal joint structure, leading to a reduced anteroposterior chest diameter. The severity of the chest depression can compromise heart and lung function, often causing breathlessness and chest pain, particularly during exertion. It may also coexist with congenital heart defects, and its psychological impact—especially on body image—is significant.
Pectus Carinatum
This condition is characterized by an outward protrusion of the sternum, resulting from abnormal collagen alignment in the sternocostal cartilage. Although it typically doesn’t affect cardiopulmonary function, patients may experience respiratory discomfort due to a rigid chest wall and increased residual lung volume.
Poland Syndrome
Generally presenting as a cosmetic issue, Poland syndrome rarely leads to organ dysfunction. However, associated deformities like underdeveloped chest muscles or hand abnormalities can impair function—such as arm mobility or cause complications like lung herniation or flail chest. Surgical correction is advised in such cases, especially when quality of life or function is affected. Cosmetic concerns are particularly notable in adolescent girls due to breast asymmetry.
These defects involve a gap in the sternum, exposing mediastinal structures beneath a thin tissue covering. While some clefts may be harmless, others, particularly after surgery, can lead to complications like cardiac compression or abnormal breathing patterns.
Ectopia Cordis
This rare defect results from improper closure of the ventral body wall, causing the heart to develop outside the chest. Depending on the type—thoracic, thoracoabdominal, or cervical—the heart may be partially or fully exposed, sometimes only covered by a membrane or skin. The lack of protective structures and frequent association with other congenital defects severely impacts survival.
Jeune Syndrome
A genetic disorder affecting rib development, Jeune syndrome causes a narrow chest due to short ribs that don’t extend properly. This leads to restricted lung growth and reduced ventilation capacity, with respiratory issues worsening over time.
Jarcho-Levin Syndrome
This condition stems from defective signaling in somite development, leading to abnormal spine and rib formation. In spondylothoracic dysostosis, ribs are fused symmetrically along the spine, producing a “crab-like” appearance and a rigid chest wall that impairs breathing. Spondylocostal dysostosis presents with various rib anomalies that also contribute to spinal curvature and a restrictive chest, progressively compromising lung development.
Pectus Excavatum: The precise cause of pectus excavatum is still unclear, though current theories suggest a defect in collagen metabolism leading to excess growth of sternal cartilage, ultimately causing inward displacement of the sternum. While typically an isolated condition, genetic links have been identified. It may occur as part of inherited syndromes—such as Marfan or Noonan (autosomal dominant), osteogenesis imperfecta (types I, III, IV; autosomal recessive), and X-linked connective tissue disorders. Even in non-syndromic cases, autosomal dominant inheritance patterns have been noted. A variant known as the MASS phenotype (involving mitral valve prolapse and other connective tissue features) is associated with FBN1 gene mutations.
Pectus Carinatum: Like pectus excavatum, this condition is believed to result from abnormal cartilage development. A mutation in the COL2A1 gene, which affects type II collagen, has been linked to the disorder. The inheritance patterns mirror those of pectus excavatum, often autosomal dominant with a possible familial background.
Poland Syndrome: This condition is thought to result from developmental disruption in the womb, rather than inherited genetics. The most supported theory is an interruption in embryonic blood flow—particularly affecting the subclavian or vertebral arteries—leading to underdevelopment of chest muscles and limbs. Another theory involves a defect in lateral plate mesoderm formation. Environmental factors, such as drug or tobacco exposure during pregnancy, may also play a role. Though usually non-genetic, familial cases do exist. Bilateral forms may suggest autosomal dominant inheritance, while associations with Mobius syndrome suggest a possible recessive or X-linked pattern.
Sternal Clefts: This rare anomaly results from the failure of ventral cell migration and fusion of sternal bars during weeks 6–9 of gestation. It may be linked to mutations in Hoxb genes. While most cases carry low genetic risk, some syndromic associations—such as Cantrell’s pentalogy (X-linked) and PHACE syndrome—have been observed.
Ectopia Cordis: This life-threatening defect arises from impaired ventral folding during early embryogenesis. One hypothesis suggests that early rupture of the chorionic or yolk sac may disrupt normal thoracic development. Genetic studies implicate reduced BMP2 expression in faulty morphogenesis. Though the exact inheritance pattern is unclear, this condition often coexists with major chromosomal anomalies such as Turner syndrome or trisomy 18.
Jeune Syndrome: This condition is inherited in an autosomal recessive pattern and is caused by mutations in genes involved in intraflagellar transport—especially IFT80 and DYNC2H1. These genes play a key role in cilia function. The condition typically carries a 25% chance of occurrence when both parents are carriers.
Jarcho-Levin Syndrome: The syndrome includes two major subtypes. Spondylocostal dysostosis is associated with mutations in DLL3 and MESP2, and may follow either dominant or recessive inheritance. Spondylothoracic dysostosis, on the other hand, is linked to autosomal recessive inheritance without involvement of the DLL3 gene. Mutations in PAX1 or PAX9 genes are also implicated. Genetic risk is dependent on whether one or both parents are carriers of the mutated genes.
Pectus Excavatum
Surgical correction—either through open techniques or the MARPE (Minimally Invasive Repair of Pectus Excavatum) method—has demonstrated high success rates, ranging from 86% to 98.1%. Patients typically show noticeable improvements in heart and lung function within 6 to 12 months post-surgery. Additionally, in about half of the cases, mitral valve prolapse associated with the condition resolves after surgical correction.
Pectus Carinatum: While minimally invasive approaches are still under evaluation, most surgical treatments follow principles from the modified Ravitch technique, yielding comparable results in aesthetics and symptom relief to pectus excavatum corrections. Non-surgical bracing can lead to visible improvements within 2–3 months, but treatment usually lasts 2 to 2.5 years. Despite its effectiveness, some patients find the brace uncomfortable or hard to tolerate.
Poland Syndrome: Outcomes vary depending on several factors such as the patient’s age, sex, severity of the deformity, presence of cancer, and the type of reconstructive surgery performed. These factors collectively influence the long-term prognosis.
Sternal Clefts: The outlook for patients with sternal clefts is influenced by the type and extent of the defect, the patient’s age, and any associated anomalies. Superior sternal clefts are often isolated and linked to a structurally normal heart, which supports a favorable prognosis. Early surgical repair—especially in newborns—can improve outcomes, although survival mainly hinges on other coexisting conditions.
Ectopia Cordis: This condition carries a generally poor prognosis. Thoracic presentations and cases associated with complex cardiac anomalies or Cantrell pentalogy have the highest mortality. In contrast, thoracoabdominal ectopia cordis may offer a slightly better prognosis due to a more favorable heart positioning.
Jeune Syndrome: Also known as asphyxiating thoracic dystrophy, this condition typically has a poor outcome, with mortality rates between 60% and 80%. However, some specialized centers report up to a 50% survival rate in milder cases following surgical intervention. Prognosis tends to improve with age.
Jarcho-Levin Syndrome: The outcome largely depends on the subtype. Spondylothoracic dysostosis is often fatal and unresponsive to surgical treatment, although some mild cases have shown survival rates up to 56% with intensive respiratory support. Spondylocostal dysostosis tends to have a more favorable prognosis, especially when treated with procedures like VEPTR (Vertical Expandable Prosthetic Titanium Rib). Both forms are associated with normal cognitive development, and prognosis improves if the child survives beyond six months of age.
Pectus Excavatum
Pectus Carinatum
Poland Syndrome
Sternal Clefts
Ectopia Cordis
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
Jarcho-Levin Syndrome
Physical examination of chest wall deformities involves a visual inspection, palpation, and functional assessment. Clinicians assess for asymmetry, depression or protrusion of the sternum (as seen in pectus excavatum or carinatum), and associated rib abnormalities. The shape and motion of the chest during breathing are observed to detect any respiratory restriction. Palpation helps evaluate rigidity, tenderness, or gaps (in cases like sternal clefts). Postural assessment is important, especially for spinal alignment. Cardiopulmonary auscultation may reveal murmurs or diminished breath sounds, indicating internal compression. The Haller index may be calculated using imaging for severity grading, especially in pectus excavatum.
Pectus Deformities: Marfan syndrome is the most significant condition to consider, though a range of connective tissue disorders associated with chest wall abnormalities should be included in the differential.
Poland Syndrome
Differential Considerations: Distinguish from natural breast asymmetry in females, as well as pulmonary conditions such as Swyer-James syndrome and large pulmonary bullae, which can appear as a hyperlucent hemithorax on chest radiographs.
Sternal Clefts
Differential Diagnoses: Conditions involving congenital anomalies like limb-body wall complex, amniotic band syndrome, and Cantrell pentalogy should be considered.
Ectopia Cordis
Differential Diagnoses: Often considered alongside chromosomal disorders such as trisomy 18 and Turner syndrome due to overlapping structural anomalies.
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
Differential Diagnoses: Includes other skeletal dysplasias such as Ellis-Van Creveld syndrome, types I–IV of short rib polydactyly syndrome, Barnes syndrome, and Shwachman-Diamond syndrome.
Jarcho-Levin Syndrome
Differential Diagnoses: Should be differentiated from disorders like spondyloepiphyseal dysplasia, Morquio syndrome, chondrodysplasia, Klippel-Feil syndrome, and short rib polydactyly syndrome.
Pediatrics, Cardiology
Pectus Excavatum
Physical Rehabilitation: Non-operative management primarily includes exercise-based physical therapy programs aimed at improving posture, respiratory function, and chest wall muscle strength.
Suction Devices: Some centers use external vacuum bell therapy, which applies negative pressure to elevate the sternum. While it offers a non-invasive alternative, long-term efficacy data is still limited.
Pectus Carinatum
Orthotic Bracing: In patients aged 10 to 15 years with chondrogladiolar-type deformities, compression bracing is often the first-line treatment. The brace is worn regularly and adjusted over time to correct the protrusion. This method is particularly effective before skeletal maturity and may avoid the need for surgery if initiated early and compliance is good.
Poland Syndrome: Although primarily surgically managed, non-surgical cosmetic options like autologous fat grafting may enhance contour and symmetry. These are used selectively, especially for improving soft tissue appearance in mild deformities or as a supplement to surgery.
Sternal Clefts: Observation is an option for asymptomatic patients with minor clefts and no cardiopulmonary compromise. These individuals are typically followed clinically without immediate intervention.
Ectopia Cordis: Non-surgical management is mostly supportive and palliative, including vital stabilization at birth. Surgery is the definitive treatment, but immediate neonatal care forms the non-operative cornerstone during initial stabilization.
Jeune Syndrome: Non-operative management includes intensive respiratory support, especially during early infancy. The goal is to manage thoracic insufficiency and optimize pulmonary function before considering surgical intervention.
Jarcho-Levin Syndrome: Like Jeune Syndrome, aggressive respiratory care is the mainstay of early non-surgical treatment. Ventilatory support may be required due to severe thoracic restriction and impaired lung development.
Pediatrics, Cardiology
Paracetamol (Acetaminophen): It is often used as baseline analgesic for mild to moderate pain.
Example: Oral/parenteral acetaminophen 15 mg/kg every 6 hours.
NSAIDs (e.g., Ibuprofen, Ketorolac, Diclofenac): It reduces inflammation and pain; may help minimize opioid requirement.
Example: IV Ketorolac 0.5 mg/kg every 6 hours (short-term use).
Pediatrics, Cardiology
Morphine: IV bolus or PCA (Patient-Controlled Analgesia) system.
Hydromorphone: Alternative to morphine with less histamine release.
Fentanyl: IV or transdermal patch in chronic cases or during procedures.
Pediatrics, Cardiology
Gabapentin or Pregabalin: Neuropathic component management, especially in chronic pain.
Ketamine (low dose): Used intraoperatively to reduce central sensitization and opioid use.
Pediatrics, Cardiology
Pectus Excavatum
Surgical Interventions:
Nuss Procedure (Minimally Invasive Repair of Pectus Excavatum – MIRPE): Insertion of a curved metal bar beneath the sternum to elevate the depressed chest. Bar remains for 2–3 years before removal.
Intervention Support: Use of rib stabilizers, thoracoscopic guidance to reduce complications.
Modified Ravitch Procedure: Open approach with resection of abnormal cartilage and sternal osteotomy.
Intervention Support: Retrosternal bone graft or bar for support and remodeling.
Plier Technique / Leonard Modification: Reserved for very severe deformities, may include muscle flap use.
Pectus Carinatum
Modified Ravitch Procedure: Resection of protruding cartilage and placement of a retrosternal bar if needed. Often includes wedge osteotomies for realignment.
Poland Syndrome
Stage 1 (Skeletal Reconstruction): Modified Ravitch or Nuss Procedure with rib graft or metal struts. Use of custom implants or 3D-molded prosthetics.
Stage 2 (Soft Tissue Reconstruction): Myocutaneous flaps (e.g., latissimus dorsi), autologous fat grafting. In females, breast implant reconstruction may be used.
Pediatrics, Cardiology
Management of chest wall deformities typically follows a phased approach.
Phase 1 involves early assessment and non-operative management, including physical therapy, bracing (especially for pectus carinatum), and monitoring of growth and symptoms.
Phase 2 focuses on surgical intervention, indicated when deformities cause cardiopulmonary compromise, psychological distress, or fail conservative treatment. Procedures like the Nuss or Ravitch are selected based on the type and severity of deformity.
Phase 3 is the post-operative and rehabilitation phase, emphasizing pain control, physical rehabilitation, and bar removal if applicable. This phased approach ensures personalized, safe, and effective long-term outcomes.
Chest wall deformities are a group of congenital conditions that vary widely in presentation. They may occur alone or alongside other anomalies and genetic syndromes. Their impact can range from serious health risks in early life to cosmetic and psychosocial issues during adolescence. Management involves a combination of medical treatment, surgery, and genetic counseling. Surgical approaches are complex and influenced by factors such as the patient’s age, gender, timing of intervention, and choice of materials. This review focuses on the most common chest wall deformities encountered in general thoracic surgery, including pectus excavatum, pectus carinatum, sternal clefts, ectopia cordis, and the syndromes of Poland, Jeune, and Jarcho-Levin.
Pectus Excavatum
Accounting for the majority (90%) of chest wall deformities, pectus excavatum is most commonly seen in individuals of White descent, affecting about 1 in every 400 births, with a notable male predominance (5:1). While it often occurs sporadically, up to 40% of cases have a positive family history, typically following an autosomal dominant inheritance pattern. It can also be part of genetic syndromes like Marfan and Noonan. Secondary cases may result from conditions like Poland syndrome. Most diagnoses occur by age two. Associated cardiac issues may include mitral valve prolapse, arrhythmias, and, less commonly, congenital heart defects. About two-thirds of affected individuals exhibit features of the MASS phenotype.
Pectus Carinatum
The second most prevalent chest wall anomaly, pectus carinatum is about 5–6 times less frequent than pectus excavatum and occurs in approximately 0.06% of live births, with a 4:1 male dominance. It may present as an isolated anomaly or within the context of genetic syndromes, most often Marfan or Noonan. A family history is present in around 25–30% of cases. Some individuals may also have related heart abnormalities or scoliosis (in up to 14%). The chondrogladiolar variant is the most common. Symptoms typically emerge or worsen during puberty, prompting medical evaluation.
Poland Syndrome
A rare condition seen in 1 in 20,000 to 30,000 births, Poland syndrome primarily affects males (2–3:1 ratio) and typically presents unilaterally, most often on the right side in males. While usually non-genetic, rare familial cases exist. If the anomaly appears on the left side, dextrocardia may also be present. The syndrome has been linked to other conditions including Moebius syndrome, Klippel-Feil syndrome, and various malignancies such as breast cancer and leukemia.
Sternal Clefts
Sternal clefts, representing about 0.15% of chest wall defects, occur more often in females (2:1). They can be categorized into superior (most common), complete, inferior, and sternal foramen types. These defects are sometimes associated with PHACES syndrome, midline fusion disorders, and chromosomal anomalies. An inferior sternal cleft is considered a key feature of Cantrell pentalogy, while superior clefts are usually isolated.
Ectopia Cordis
A highly rare anomaly, ectopia cordis occurs in about 1 in 5.5 to 7.9 million live births. It can present in thoracic, thoracoabdominal, or cervical forms. While it can appear on its own, it often coexists with congenital heart diseases, particularly tetralogy of Fallot, and midline defects such as omphalocele. Thoracoabdominal types are frequently linked with Cantrell pentalogy.
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
This rare skeletal disorder affects approximately 1 in 120,000 live births and is associated with high early mortality, especially within the first two years. The primary issue is restricted chest growth, impairing respiratory function. It may also involve abnormalities of the clavicle, limbs, pelvis, kidneys, liver, and pancreas.
Jarcho-Levin Syndrome
A rare congenital disorder involving spinal and rib malformations, Jarcho-Levin syndrome can be inherited in an autosomal recessive or dominant pattern. Spondylothoracic dysostosis, often linked with Puerto Rican ancestry, carries a high infant mortality rate (up to 100%), whereas spondylocostal dysostosis tends to have a better prognosis. It is associated with gene mutations in DLL3 and MESP2, and may also present features like a short trunk, facial anomalies, joint stiffness, hernias, urinary tract defects, and clubfoot.
Pectus Excavatum
This chest wall deformity involves a sunken appearance of the sternum due to an abnormal sternocostal joint structure, leading to a reduced anteroposterior chest diameter. The severity of the chest depression can compromise heart and lung function, often causing breathlessness and chest pain, particularly during exertion. It may also coexist with congenital heart defects, and its psychological impact—especially on body image—is significant.
Pectus Carinatum
This condition is characterized by an outward protrusion of the sternum, resulting from abnormal collagen alignment in the sternocostal cartilage. Although it typically doesn’t affect cardiopulmonary function, patients may experience respiratory discomfort due to a rigid chest wall and increased residual lung volume.
Poland Syndrome
Generally presenting as a cosmetic issue, Poland syndrome rarely leads to organ dysfunction. However, associated deformities like underdeveloped chest muscles or hand abnormalities can impair function—such as arm mobility or cause complications like lung herniation or flail chest. Surgical correction is advised in such cases, especially when quality of life or function is affected. Cosmetic concerns are particularly notable in adolescent girls due to breast asymmetry.
These defects involve a gap in the sternum, exposing mediastinal structures beneath a thin tissue covering. While some clefts may be harmless, others, particularly after surgery, can lead to complications like cardiac compression or abnormal breathing patterns.
Ectopia Cordis
This rare defect results from improper closure of the ventral body wall, causing the heart to develop outside the chest. Depending on the type—thoracic, thoracoabdominal, or cervical—the heart may be partially or fully exposed, sometimes only covered by a membrane or skin. The lack of protective structures and frequent association with other congenital defects severely impacts survival.
Jeune Syndrome
A genetic disorder affecting rib development, Jeune syndrome causes a narrow chest due to short ribs that don’t extend properly. This leads to restricted lung growth and reduced ventilation capacity, with respiratory issues worsening over time.
Jarcho-Levin Syndrome
This condition stems from defective signaling in somite development, leading to abnormal spine and rib formation. In spondylothoracic dysostosis, ribs are fused symmetrically along the spine, producing a “crab-like” appearance and a rigid chest wall that impairs breathing. Spondylocostal dysostosis presents with various rib anomalies that also contribute to spinal curvature and a restrictive chest, progressively compromising lung development.
Pectus Excavatum: The precise cause of pectus excavatum is still unclear, though current theories suggest a defect in collagen metabolism leading to excess growth of sternal cartilage, ultimately causing inward displacement of the sternum. While typically an isolated condition, genetic links have been identified. It may occur as part of inherited syndromes—such as Marfan or Noonan (autosomal dominant), osteogenesis imperfecta (types I, III, IV; autosomal recessive), and X-linked connective tissue disorders. Even in non-syndromic cases, autosomal dominant inheritance patterns have been noted. A variant known as the MASS phenotype (involving mitral valve prolapse and other connective tissue features) is associated with FBN1 gene mutations.
Pectus Carinatum: Like pectus excavatum, this condition is believed to result from abnormal cartilage development. A mutation in the COL2A1 gene, which affects type II collagen, has been linked to the disorder. The inheritance patterns mirror those of pectus excavatum, often autosomal dominant with a possible familial background.
Poland Syndrome: This condition is thought to result from developmental disruption in the womb, rather than inherited genetics. The most supported theory is an interruption in embryonic blood flow—particularly affecting the subclavian or vertebral arteries—leading to underdevelopment of chest muscles and limbs. Another theory involves a defect in lateral plate mesoderm formation. Environmental factors, such as drug or tobacco exposure during pregnancy, may also play a role. Though usually non-genetic, familial cases do exist. Bilateral forms may suggest autosomal dominant inheritance, while associations with Mobius syndrome suggest a possible recessive or X-linked pattern.
Sternal Clefts: This rare anomaly results from the failure of ventral cell migration and fusion of sternal bars during weeks 6–9 of gestation. It may be linked to mutations in Hoxb genes. While most cases carry low genetic risk, some syndromic associations—such as Cantrell’s pentalogy (X-linked) and PHACE syndrome—have been observed.
Ectopia Cordis: This life-threatening defect arises from impaired ventral folding during early embryogenesis. One hypothesis suggests that early rupture of the chorionic or yolk sac may disrupt normal thoracic development. Genetic studies implicate reduced BMP2 expression in faulty morphogenesis. Though the exact inheritance pattern is unclear, this condition often coexists with major chromosomal anomalies such as Turner syndrome or trisomy 18.
Jeune Syndrome: This condition is inherited in an autosomal recessive pattern and is caused by mutations in genes involved in intraflagellar transport—especially IFT80 and DYNC2H1. These genes play a key role in cilia function. The condition typically carries a 25% chance of occurrence when both parents are carriers.
Jarcho-Levin Syndrome: The syndrome includes two major subtypes. Spondylocostal dysostosis is associated with mutations in DLL3 and MESP2, and may follow either dominant or recessive inheritance. Spondylothoracic dysostosis, on the other hand, is linked to autosomal recessive inheritance without involvement of the DLL3 gene. Mutations in PAX1 or PAX9 genes are also implicated. Genetic risk is dependent on whether one or both parents are carriers of the mutated genes.
Pectus Excavatum
Surgical correction—either through open techniques or the MARPE (Minimally Invasive Repair of Pectus Excavatum) method—has demonstrated high success rates, ranging from 86% to 98.1%. Patients typically show noticeable improvements in heart and lung function within 6 to 12 months post-surgery. Additionally, in about half of the cases, mitral valve prolapse associated with the condition resolves after surgical correction.
Pectus Carinatum: While minimally invasive approaches are still under evaluation, most surgical treatments follow principles from the modified Ravitch technique, yielding comparable results in aesthetics and symptom relief to pectus excavatum corrections. Non-surgical bracing can lead to visible improvements within 2–3 months, but treatment usually lasts 2 to 2.5 years. Despite its effectiveness, some patients find the brace uncomfortable or hard to tolerate.
Poland Syndrome: Outcomes vary depending on several factors such as the patient’s age, sex, severity of the deformity, presence of cancer, and the type of reconstructive surgery performed. These factors collectively influence the long-term prognosis.
Sternal Clefts: The outlook for patients with sternal clefts is influenced by the type and extent of the defect, the patient’s age, and any associated anomalies. Superior sternal clefts are often isolated and linked to a structurally normal heart, which supports a favorable prognosis. Early surgical repair—especially in newborns—can improve outcomes, although survival mainly hinges on other coexisting conditions.
Ectopia Cordis: This condition carries a generally poor prognosis. Thoracic presentations and cases associated with complex cardiac anomalies or Cantrell pentalogy have the highest mortality. In contrast, thoracoabdominal ectopia cordis may offer a slightly better prognosis due to a more favorable heart positioning.
Jeune Syndrome: Also known as asphyxiating thoracic dystrophy, this condition typically has a poor outcome, with mortality rates between 60% and 80%. However, some specialized centers report up to a 50% survival rate in milder cases following surgical intervention. Prognosis tends to improve with age.
Jarcho-Levin Syndrome: The outcome largely depends on the subtype. Spondylothoracic dysostosis is often fatal and unresponsive to surgical treatment, although some mild cases have shown survival rates up to 56% with intensive respiratory support. Spondylocostal dysostosis tends to have a more favorable prognosis, especially when treated with procedures like VEPTR (Vertical Expandable Prosthetic Titanium Rib). Both forms are associated with normal cognitive development, and prognosis improves if the child survives beyond six months of age.
Pectus Excavatum
Pectus Carinatum
Poland Syndrome
Sternal Clefts
Ectopia Cordis
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
Jarcho-Levin Syndrome
Physical examination of chest wall deformities involves a visual inspection, palpation, and functional assessment. Clinicians assess for asymmetry, depression or protrusion of the sternum (as seen in pectus excavatum or carinatum), and associated rib abnormalities. The shape and motion of the chest during breathing are observed to detect any respiratory restriction. Palpation helps evaluate rigidity, tenderness, or gaps (in cases like sternal clefts). Postural assessment is important, especially for spinal alignment. Cardiopulmonary auscultation may reveal murmurs or diminished breath sounds, indicating internal compression. The Haller index may be calculated using imaging for severity grading, especially in pectus excavatum.
Pectus Deformities: Marfan syndrome is the most significant condition to consider, though a range of connective tissue disorders associated with chest wall abnormalities should be included in the differential.
Poland Syndrome
Differential Considerations: Distinguish from natural breast asymmetry in females, as well as pulmonary conditions such as Swyer-James syndrome and large pulmonary bullae, which can appear as a hyperlucent hemithorax on chest radiographs.
Sternal Clefts
Differential Diagnoses: Conditions involving congenital anomalies like limb-body wall complex, amniotic band syndrome, and Cantrell pentalogy should be considered.
Ectopia Cordis
Differential Diagnoses: Often considered alongside chromosomal disorders such as trisomy 18 and Turner syndrome due to overlapping structural anomalies.
Jeune Syndrome (Asphyxiating Thoracic Dystrophy)
Differential Diagnoses: Includes other skeletal dysplasias such as Ellis-Van Creveld syndrome, types I–IV of short rib polydactyly syndrome, Barnes syndrome, and Shwachman-Diamond syndrome.
Jarcho-Levin Syndrome
Differential Diagnoses: Should be differentiated from disorders like spondyloepiphyseal dysplasia, Morquio syndrome, chondrodysplasia, Klippel-Feil syndrome, and short rib polydactyly syndrome.
Pediatrics, Cardiology
Pectus Excavatum
Physical Rehabilitation: Non-operative management primarily includes exercise-based physical therapy programs aimed at improving posture, respiratory function, and chest wall muscle strength.
Suction Devices: Some centers use external vacuum bell therapy, which applies negative pressure to elevate the sternum. While it offers a non-invasive alternative, long-term efficacy data is still limited.
Pectus Carinatum
Orthotic Bracing: In patients aged 10 to 15 years with chondrogladiolar-type deformities, compression bracing is often the first-line treatment. The brace is worn regularly and adjusted over time to correct the protrusion. This method is particularly effective before skeletal maturity and may avoid the need for surgery if initiated early and compliance is good.
Poland Syndrome: Although primarily surgically managed, non-surgical cosmetic options like autologous fat grafting may enhance contour and symmetry. These are used selectively, especially for improving soft tissue appearance in mild deformities or as a supplement to surgery.
Sternal Clefts: Observation is an option for asymptomatic patients with minor clefts and no cardiopulmonary compromise. These individuals are typically followed clinically without immediate intervention.
Ectopia Cordis: Non-surgical management is mostly supportive and palliative, including vital stabilization at birth. Surgery is the definitive treatment, but immediate neonatal care forms the non-operative cornerstone during initial stabilization.
Jeune Syndrome: Non-operative management includes intensive respiratory support, especially during early infancy. The goal is to manage thoracic insufficiency and optimize pulmonary function before considering surgical intervention.
Jarcho-Levin Syndrome: Like Jeune Syndrome, aggressive respiratory care is the mainstay of early non-surgical treatment. Ventilatory support may be required due to severe thoracic restriction and impaired lung development.
Pediatrics, Cardiology
Paracetamol (Acetaminophen): It is often used as baseline analgesic for mild to moderate pain.
Example: Oral/parenteral acetaminophen 15 mg/kg every 6 hours.
NSAIDs (e.g., Ibuprofen, Ketorolac, Diclofenac): It reduces inflammation and pain; may help minimize opioid requirement.
Example: IV Ketorolac 0.5 mg/kg every 6 hours (short-term use).
Pediatrics, Cardiology
Morphine: IV bolus or PCA (Patient-Controlled Analgesia) system.
Hydromorphone: Alternative to morphine with less histamine release.
Fentanyl: IV or transdermal patch in chronic cases or during procedures.
Pediatrics, Cardiology
Gabapentin or Pregabalin: Neuropathic component management, especially in chronic pain.
Ketamine (low dose): Used intraoperatively to reduce central sensitization and opioid use.
Pediatrics, Cardiology
Pectus Excavatum
Surgical Interventions:
Nuss Procedure (Minimally Invasive Repair of Pectus Excavatum – MIRPE): Insertion of a curved metal bar beneath the sternum to elevate the depressed chest. Bar remains for 2–3 years before removal.
Intervention Support: Use of rib stabilizers, thoracoscopic guidance to reduce complications.
Modified Ravitch Procedure: Open approach with resection of abnormal cartilage and sternal osteotomy.
Intervention Support: Retrosternal bone graft or bar for support and remodeling.
Plier Technique / Leonard Modification: Reserved for very severe deformities, may include muscle flap use.
Pectus Carinatum
Modified Ravitch Procedure: Resection of protruding cartilage and placement of a retrosternal bar if needed. Often includes wedge osteotomies for realignment.
Poland Syndrome
Stage 1 (Skeletal Reconstruction): Modified Ravitch or Nuss Procedure with rib graft or metal struts. Use of custom implants or 3D-molded prosthetics.
Stage 2 (Soft Tissue Reconstruction): Myocutaneous flaps (e.g., latissimus dorsi), autologous fat grafting. In females, breast implant reconstruction may be used.
Pediatrics, Cardiology
Management of chest wall deformities typically follows a phased approach.
Phase 1 involves early assessment and non-operative management, including physical therapy, bracing (especially for pectus carinatum), and monitoring of growth and symptoms.
Phase 2 focuses on surgical intervention, indicated when deformities cause cardiopulmonary compromise, psychological distress, or fail conservative treatment. Procedures like the Nuss or Ravitch are selected based on the type and severity of deformity.
Phase 3 is the post-operative and rehabilitation phase, emphasizing pain control, physical rehabilitation, and bar removal if applicable. This phased approach ensures personalized, safe, and effective long-term outcomes.
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