The Institute of Chest Wall Surgery
Reviewed by Dr. Juan Luo
Dr. Luo specializes in chest wall deformities. She has treated nearly 2,000 patients and performed over 800 corrective surgeries, with a focus on achieving both functional and aesthetic outcomes. She is highly skilled in scar management, employing unique techniques to optimize healing and appearance.
Sternal cleft is a rare congenital chest wall deformity. However, it is very important that both families and medical professionals clearly understand its causes, symptoms, risks, and modern surgical solutions, as the early diagnosis and timely management affect long-term outcomes.
What Is a Sternal Cleft?
Sternal cleft, also called sternal fissure or bifid sternum, is a rare congenital malformation caused by partial or total failure of sternal fusion[1]. It presents as a sternal cleft or defect in the upper chest wall, often with exposed intramediastinal tissue and paradoxical thoracic movements.
From an epidemiological perspective, the condition is exceptionally rare, with an incidence rate estimated at approximately 2 in 100,000 live births[2]. It accounts for less than 0.15% of all chest wall malformations[3]. While it affects both genders, some literature suggests a slight female predominance[2].
Sternal cleft can be classified as complete or incomplete, with incomplete forms further subdivided into superior or inferior. Any of these forms may appear as a solitary finding or be accompanied by additional congenital malformations[4]. However, isolated sternal clefts are uncommon, and most cases are accompanied by other conditions.
A. Superior Sternal Cleft: This is the most common form, where the fusion failure occurs at the top of the sternum (manubrium). It creates a V-shaped or U-shaped defect on the sternum.
B. Inferior Sternal Cleft: A much rarer presentation where the lower portion of the sternum fails to fuse. This specific type is frequently accompanied by thoracoabdominal anomalies(e.g., Pentalogy of Cantrell).
C. Complete (Total) Sternal Cleft: In this type, the sternum is completely split from the manubrium to the xiphoid process (the lowest end of the sternum), forming a full-length gap down the center of the chest.
What Causes a Sternal Cleft and How Does It Present?
Causes of Sternal Cleft
Sternal cleft is a congenital condition that arises during embryonic development. Its exact etiology remains unclear, but the pathogenesis is primarily associated with abnormalities in sternal development.
The sternum forms from bilateral mesenchymal bands, which normally develop into sternal plates and fuse at the midline around the 7th to 10th week of gestation[5,6]. Any disruption or abnormality during this fusion process may result in a sternal cleft.
In simple terms, a sternal cleft results from incomplete or partial fusion of the two sternal plates during embryonic development.
Common Symptoms of Sternal Cleft
Typical clinical manifestations of sternal cleft include paradoxical breathing, cyanosis, and recurrent respiratory infections, with some patients developing significant dyspnea in severe cases.
However, specific sternal cleft symptoms can be more complex depending on the classification:
Superior sternal cleft may occur in isolation or in association with other anomalies. When isolated, it may present only with respiratory or circulatory abnormalities; when associated with additional defects, the clinical presentation becomes more complex.
Inferior sternal cleft is often accompanied by Pentalogy of Cantrell, which is characterized by a sternal defect, partial pericardial defect, anterior diaphragmatic defect, supraumbilical
Complete sternal cleft frequently coexists with other malformations, such as absence or deficiency of the pectoral muscles, pericardial agenesis, and various visceral anomalies. It may also be accompanied by cardiac ectopia, which can severely impair cardiac function.
Does It Worsen With Age?
A common concern among parents is whether the cleft will enlarge or compress internal organs as the child grows. Though the defect doesn't "worsen" in the sense of further separation, leaving a congenital sternal cleft untreated can result in:
Decreased protection for the heart and lungs
Diminished capacity for chest expansion.
Increased respiratory complications
Cosmetic complaints that tend to get worse with increasing age
Most importantly, delayed surgery is more technically difficult as the chest becomes rigid. For these reasons, medical experts usually recommend early evaluation and surgical planning.
How Is Sternal Cleft Diagnosed?
In most cases, the clinician recognizes the condition in the first newborn assessment due to the physical signs, which are usually clear and unmistakable.
Physical examination at birth often reveals several important characteristics, such as a longitudinal depression or groove in the midline of the pectoral region, abnormal chest wall movement during the respiratory cycle, and the absence of the rigid breastbone upon palpation. These features raise suspicions that initiate further diagnostic workup.
After the initial assessment, further examinations are required to confirm the presence and severity of a sternal cleft. Common evaluations include imaging studies such as chest CT scans and 3D reconstruction, as well as cardiac assessments like echocardiography and angiography.
Cardiac assessment is primarily performed because the sternal cleft is often accompanied by various types of cardiac malformations, making comprehensive screening for related anomalies essential.Fetal sonographic diagnosis of sternal cleft is possible, but less practiced[8].
Surgical Treatment Options and Best Timing
It is widely recognized by the international medical community that surgical intervention is the only definitive treatment for sternal clefts. The primary goal of the operation is to restore the integrity and stability of the thoracic cage, protect the mediastinal organs from injury, and reestablish normal respiratory motion, while also improving the chest wall appearance.
Early surgical intervention for sternal cleft is recommended.
Surgical repair is generally recommended in neonates or young infants. At this stage, the bony structures are soft and highly malleable, making it the optimal time for sternal cleft correction [2,8,9].
Surgical Techniques
1. Direct Suturing
Direct closure is suitable for patients with a small gap between the residual bones on both sides of the sternal cleft, as suturing does not exert significant pressure on the mediastinal structures.
Some authors advocate completing the repair within the neonatal period (preferably before 3 months of age), to enable primary closure of the defect[9].
2. Chest Wall Reconstruction
In cases involving larger defects or more rigid sternum, chest wall reconstruction surgery is required.
This approach typically involves using the patient’s own ribs to reconstruct the sternal defect, followed by repairing the rib defect with MatrixRIBs.
Surgical planning must consider factors such as patient age, sternal cleft type and extent, and presence of other deformities.
Treatment at ICWS
ICWS is a professional diagnosis and treatment center for chest wall disorders and the world's largest center for chest wall deformity correction.
With over 15 years of experience in chest wall condition research, our team has successfully performed more than 10,000 chest wall surgeries for patients from all over the world.
We encourage parents of infants diagnosed with sternal cleft to contact us at ICWS as early as possible. Early evaluation leads to lower risk and fewer complications during surgery, assuring a lifetime of improved quality of life.
References:
[1] Zamfir, C., et al. (2014). Sternal cleft – a rare congenital malformation. Journal of Pediatric Surgery Case Reports. https://www.sciencedirect.com/science/article/pii/S2213576614000153
[2] De Macedo, J. P. C., De Araujo, P. H. X. N., De Campos, J. R. M., & Pêgo-Fernandes, P. M. (2024b). Sternal cleft: new options for reconstruction. Journal of Thoracic Disease, 16(2), 1724–1729. https://doi.org/10.21037/jtd-23-645
[3] Pediatric Surgery NAT. (n.d.). Sternal cleft. https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829602/all/Sternal_Cleft
[4] Hinchcliff, K. M., Xue, Y., & Wong, G. B. (2021b). Reconstruction of congenital sternal cleft. Annals of Plastic Surgery, 86(5S), S418–S421. https://doi.org/10.1097/sap.0000000000002724
[5] Altalib, A. A., Miao, K. H., & Menezes, R. G. (2023, July 24). Anatomy, thorax, sternum. StatPearls - NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK541141/
[6] The Fetus. (n.d.). Sternum. https://thefetus.net/content/sternum
[7] Sana, M. K., & Rentea, R. M. (2023, May 29). Pentalogy of Cantrell. StatPearls - NCBI Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK558948/
[8] Gebremariam, D. S., Miruts, A., & Desta, K. G. (2023). Isolated V-Shaped sternal cleft — a rare chest wall malformation. Pediatric Health Medicine and Therapeutics, Volume 14, 81–87. https://doi.org/10.2147/phmt.s397462
[9] Alshomer, F., Aldaghri, F., Alohaideb, N., Aljehani, R., Murad, M. A., & Hashem, F. (2017). Reconstruction of congenital sternal clefts: Surgical experience and literature review. Plastic and Reconstructive Surgery – Global Open, 5(11), e1567. https://doi.org/10.1097/GOX.0000000000001567
