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Sternal reconstruction by extracellular matrix: a rare case of phaces syndrome
Francesco Molinaro1, Alfredo Garzi2, Elisa Cerchia3
1Division of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Italy.
Insights
Surgical repair of congenital sternal defects, like those in PHACES syndrome, can be successfully achieved using extracellular matrix tissue. This innovative approach offers excellent cosmetic and functional outcomes for pediatric patients.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Biomaterials in Medicine
Background:
- Congenital sternal defects result from failed midline development and fusion.
- Surgical correction is crucial during infancy for functional reasons.
- Chest wall reconstruction has historically presented complex challenges.
Purpose of the Study:
- To report a successful sternal reconstruction in a rare case of PHACES syndrome.
- To evaluate the use of an extracellular matrix (ECM) tissue matrix for chest defect closure.
- To promote ECM use in pediatric chest wall reconstruction.
Main Methods:
- Sternal reconstruction and thoracic defect closure using ECM Biologic tissue matrix.
- Surgical intervention in a pediatric patient with PHACES syndrome.
- Utilizing the maneuverability and plasticity of ECM for complex reconstruction.
Main Results:
- Successful sternal reconstruction and complete closure of the thoracic defect.
- Achieved good compliance and optimal cosmetic results.
- Demonstrated positive outcomes with ECM tissue matrix implantation.
Conclusions:
- Extracellular matrix is a viable option for complex pediatric chest wall reconstruction.
- ECM offers advantages such as maneuverability, plasticity, and tolerability.
- The use of ECM allows for growth with the child, ensuring optimal long-term results.
Abstract:
Congenital defects of the sternum are rare and due to a failure of midline development and fusion of the sternal bones. Surgical correction of a sternal cleft should be preferred during infancy for functional reasons. Chest wall reconstruction represented a complex problem in the last decades. We report our successful outcome of sternal reconstruction in a rare case of PHACES syndrome, in which the patient was submitted to reconstruction of the sternum and complete closure of the thoracic defect by the employ of an extracellular matrix XCM Biologic tissue matrix. We promote the use of extracellular matrix in surgical reconstruction of chest defects for its maneuverability, plasticity, tolerability and the possibility of growing with the children's chest getting a good compliance and optimal cosmetic results.
