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Sternal cleft with surgical repair and 3-year follow-up: case report and literature review

Published online by Cambridge University Press:  25 July 2025

Kevin GianarisOpen the ORCID record for Kevin Gianaris[Opens in a new window],
Amy Heincker and
Constantine Mavroudis
Kevin Gianaris*
Affiliation:
Indiana University School of Medicine, Indianapolis, IN, USA
Amy Heincker
Affiliation:
Peyton Manning Children’s Hospital, Indianapolis, IN, USA
Constantine Mavroudis
Affiliation:
Peyton Manning Children’s Hospital, Indianapolis, IN, USA
*
Corresponding author: Kevin Giarnari; Email: kgianaris16@yahoo.com
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Abstract

Sternal cleft is a rare congenital condition associated with unprotected viscera and respiratory failure. We present a case of a sternal cleft, absent right clavicle, and congenital diaphragmatic paresis in a neonate. The sternum was closed primarily, and the patient was taken back for plication of the diaphragm. At 3 years follow-up, the patient is free from complications.

Keywords

Congenital cardiac surgerysternal cleftsternal agenesis

Information

Type
Research Article
Information
Cardiology in the Young , Volume 35 , Issue 9 , September 2025, pp. 1940 - 1943
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Sternal cleft is a very rare congenital anomaly that is defined by a defect in the embryologic fusion of mesenchymal cells in the ventral midline, which is normally completed by the 10th week of gestation. Reference Torre, Rapuzzi, Carlucci, Pio and Jasonni1 It has an incidence of about 0.15% and is characterised as complete, superior incomplete, or inferior incomplete, depending on location and degree. Reference Klein, Kellner, Boemers and Mack-Detlefsen2 This defect is more commonly found in females and can be associated with other congenital anomalies such as pectus excavatum, maxillofacial defects, cardiovascular malformations, and syndromes like Cantrell Pentalogy Syndrome or PHACES. Reference Torre, Rapuzzi, Carlucci, Pio and Jasonni1,Reference Piro, Lena, Roggero, Stagnaro, Mattioli and Torre3 If it is not corrected, the mediastinal viscera are left unprotected. Patients may be asymptomatic, but there is a risk of dyspnoea, paradoxical chest movement, coughing, impaired oxygenation, and chest infections. Reference Sha, Deng and Ou4

Case

Patient is a female who presented at 2.5 months after a term, small for gestational age pregnancy with a sternal cleft, absent right clavicle, and congenital right diaphragmatic paresis with herniation. Physical exam demonstrated softness from the clavicular notch to the xiphoid and absence of a left clavicle with no haematomas or masses. CT imaging showed the absence of the sternum and the absence of a right clavicle with bulging of the anterior neck tissue and a duplicate superior vena cava. Echocardiography revealed normal heart function and structure and only a very small ventricular septal defect (Figure 5a). The patient received an operation to close the sternum primarily (Figures 1, 2, 3, 4). The patient was then taken back for a second surgery to create a right pleural cap 13 days after the initial operation due to continued oxygen requirements and inability to wean from the ventilator. The hospital course was complicated by fevers with positive cultures for Klebsiella that were treated with ceftriaxone while inpatient. The patient was discharged on no medications. On 3-year follow-up, the patient is asymptomatic. Echocardiology showed no new abnormalities and normal cardiac function without evidence of right or left atrial compression (Figure 5b).

Figure 1.

Operative photograph of a 2.5-month-old female with sternal cleft undergoing primary repair after general endotracheal anaesthesia. The congenitally separated sternal edges are highlighted. The area between the lines is comprised of cutaneous and subcutaneous tissue, which covers the pericardium and beating heart.

Figure 2.

Operative photograph shows the mediastinal incision and exposure after pericardial entry. The right pectoralis major muscle is being mobilised for the eventual primary mediastinal closure before primary sternal approximation.

Figure 3.

Operative photograph shows the 4 cm gap between the two sternal edges that have been sutured before the actual primary closure. The left side of the pericardium has been resected just medial to the left phrenic nerve, thereby preserving function. The heart was then shifted into the left pleural cavity to make room for the primary sternal closure. Central venous pressure was monitored during this time.

Figure 4.

Operative photograph shows the result of primary sternal closure before the bilaterally mobilised pectoralis major muscles were primarily reapproximated. The sternal closure was accomplished by elevating the sutures anteriorly while carefully approximating the sternal edges. The central venous pressure was continuously monitored without any signs of pressure elevation. The arterial pressure remained consistent with preoperative measurements. After the pectoralis major muscles were approximated, the subcutaneous tissue and skin were closed primarily.

Figure 5.

(a) Representative apical images from echocardiograms done before (Figure 5A) and 3 years after (Figure 5B) repair of the sternal cleft. No right atrial or ventricular compression is noted. Not shown on these still images, biventricular function was normal pre- and post-surgery. See text for further details. (b) Representative apical images from echocardiograms done before (Figure 5A) and 3 years after (Figure 5B) repair of the sternal cleft. No right atrial or ventricular compression is noted. Not shown on these still images, biventricular function was normal pre- and post-surgery. See text for further details.

Discussion

Sternal cleft presents a significant risk with unprotected mediastinal organs including infection, organ damage, and impaired air exchange and thus warrants surgical repair in most situations. Reference Torre, Rapuzzi, Carlucci, Pio and Jasonni1,Reference De Campos, Das-Neves-Pereira, Velhote and Jatene5–Reference Biswas, Chari, Khandelwal and Venkatramu8 To determine the best treatment modalities, we performed a literature review. We began the review by searching PubMed with the terms: “sternal cleft” and “bifid sternum.” We then conducted a forward and reverse search of references in studies to increase the results. Studies were included that introduced a unique case or series of cases of a sternal cleft. Cases could be isolated or associated with another condition. Only studies in English and describing human cases were included.

After performing the literature review, we present the possibilities for evaluation and treatment. Sternal cleft may be related to alcohol intake, HOX b gene abnormality, or riboflavin deficiency. Reference Torre, Rapuzzi, Carlucci, Pio and Jasonni1,Reference Forzano, Daubeney and White9 Evaluating the patient can begin prenatally. The defect can sometimes be identified prenatally on fetal ultrasound. Reference J., G., K. and P.10 There are currently no prenatal biomarkers. If the defect is found, caesarean delivery is recommended to minimise the risk of trauma to the sternal organs during delivery. Reference Sha, Deng and Ou4,Reference Twomey, Moore, Ein, McAuliffe, Seaward and Yoo11 Postnatally, sternal cleft can often be asymptomatic. However, a physical exam can reveal the defect. The preferred imaging modality initially is ultrasound. For further characterisation of the defect, CT scan or MRI can reveal the extent of the defect. There is no clear recommendation for which imaging modality is most preferred. However, imaging is recommended for surgical planning. Reference Torre, Rapuzzi, Carlucci, Pio and Jasonni1,Reference Khan, Amjad and Khan12

Once identified, the patient should be medically optimised and prepared for surgery, ideally from an inpatient setting. Reference Dòmini, Cupaioli, Rossi, Fakhro, Aquino and Chiesa13–Reference Alshomer, Aldaghri, Alohaideb, Aljehani, Murad and Hashem16 Surgical repair is recommended as soon as possible. The reasoning behind early repair is that the sternum is the most plastic at younger ages, and the risk of complications is minimised the younger it is performed. Reference De Campos, Das-Neves-Pereira, Velhote and Jatene5,Reference Khan, Amjad and Khan12,Reference Hazari, Mercer, Pawade and Hayes17,Reference Fokin18 As the patient ages, sternal cartilage ossifies and makes repair more difficult. Primary repair is the preferred technique because it preserves the native tissue and minimises the risk of infection that comes with a prosthesis in a newborn. Reference Torre, Rapuzzi, Carlucci, Pio and Jasonni1,Reference De Campos, Das-Neves-Pereira, Velhote and Jatene5,Reference De Campos, Das-Neves-Pereira, Velhote and Jatene5 However, if the defect is too large or there is a risk of cardiac compression, a bone graft may be used to create total closure. Reference Torre, Rapuzzi, Carlucci, Pio and Jasonni1,Reference De Campos, Das-Neves-Pereira, Velhote and Jatene5 Careful monitoring of central venous pressure and arterial pressure is recommended to identify the risks of organ or blood vessel compression that can come with sternal closure. Reference J., G., K. and P.10 Major complications that were also seen included retrosternal seroma, pneumothorax, stitch abscess, and pleural or pericardial tears during dissection. Reference J., G., K. and P.10 Chest tubes should be placed to remove excess blood and avoid effusions or tamponade. There is no definitive recommendation for follow-up, but generally, follow-up with a cardiologist or surgeon is recommended.

Conclusions

This case presents a successful sternal cleft repair in a neonate and provides imaging follow-up at 3 years post-op. Patients with sternal cleft are at increased risk of respiratory issues and mediastinal injury. Primary repair can prevent these complications and reduce morbidity for these patients. Complications to monitor for include blood vessel or organ compression, retrosternal seroma, pneumothorax, and pleural or pericardial tears.

Acknowledgements

Gabriel Paris from the Indiana University School of Medicine.

Financial support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Competing interests

None.

Ethical standards

Consent was given by the parents. Institutional Review Board approval is not applicable for this study.

Funding

This research project did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. The authors report no proprietary or commercial interest in any product mentioned or concept discussed in this article.

Disclosures

Authors have no relevant disclosures.

References

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Figure 0

Figure 1.Operative photograph of a 2.5-month-old female with sternal cleft undergoing primary repair after general endotracheal anaesthesia. The congenitally separated sternal edges are highlighted. The area between the lines is comprised of cutaneous and subcutaneous tissue, which covers the pericardium and beating heart.

Figure 1

Figure 2.Operative photograph shows the mediastinal incision and exposure after pericardial entry. The right pectoralis major muscle is being mobilised for the eventual primary mediastinal closure before primary sternal approximation.

Figure 2

Figure 3.Operative photograph shows the 4 cm gap between the two sternal edges that have been sutured before the actual primary closure. The left side of the pericardium has been resected just medial to the left phrenic nerve, thereby preserving function. The heart was then shifted into the left pleural cavity to make room for the primary sternal closure. Central venous pressure was monitored during this time.

Figure 3

Figure 4.Operative photograph shows the result of primary sternal closure before the bilaterally mobilised pectoralis major muscles were primarily reapproximated. The sternal closure was accomplished by elevating the sutures anteriorly while carefully approximating the sternal edges. The central venous pressure was continuously monitored without any signs of pressure elevation. The arterial pressure remained consistent with preoperative measurements. After the pectoralis major muscles were approximated, the subcutaneous tissue and skin were closed primarily.

Figure 4

Figure 5.(a) Representative apical images from echocardiograms done before (Figure 5A) and 3 years after (Figure 5B) repair of the sternal cleft. No right atrial or ventricular compression is noted. Not shown on these still images, biventricular function was normal pre- and post-surgery. See text for further details. (b) Representative apical images from echocardiograms done before (Figure 5A) and 3 years after (Figure 5B) repair of the sternal cleft. No right atrial or ventricular compression is noted. Not shown on these still images, biventricular function was normal pre- and post-surgery. See text for further details.