Congenital Sternal Cleft With Absence of Anterior Pericardium

The authors present a case of a full-term female neonate, weighing 3.1 kg, who was diagnosed postnatally with a partial sternal cleft. Cardiac evaluation demonstrated a PDA and PFO. CT and US confirmed 2-3 gap between the costal cartilages. Clinical exam was otherwise unremarkable. The patient underwent primary repair of the cleft at 5 days of age.

The xyphoid process was present inferiorly with the defect superiorly. After a midline incision, there was a notable absence of pericardium anteriorly. The thymus was resected, and the PDA was ligated. The inferior sternal portion was divided, and the edges were excised in order to realign with the remaining costal cartilages. Extensive pectoralis muscle flaps were created bilaterally, and the mediastinal pleura was opened widely on each side. The chest wall was aggressively mobilized off the diaphragm. Interrupted 2-0 Vicryl sutures were placed to bring the costal edges together in the midline, followed by pectoralis muscle reapproximation.

She was extubated successfully the next day and discharged home on postoperative day three. At one month follow-up, her sternum has been healing well without any clinical concerns.

Overall, sternal clefts are a rare diagnosis and a cardiac evaluation is necessary for complete workup. The authors advocate for early repair of such defects in order to allow primary closure in a tension-free manner. This can be accomplished by performing extensive chest wall mobilization so that attachments to the diaphragm are divided, both pleural spaces are opened widely, and extensive pectoralis muscle flaps are raised. Intracardiac pressure monitoring is recommended during chest wall reapproximation and closure.

References

1. Karamustafaoglu Y, Yanik F, Yoruk Y, Basaran U. Congenital superior sternal cleft repair using primary closure. Balkan Med J. 219 Mar;36(2):141-142.
2. Torre M, Rapuzzi G, Carlucci M, Pio L, Jasonni V. Phenotypic spectrum and management of sternal cleft: literature review and presentation of a new series. Eur J Cardiothorac Surg. 2012;41:4-9.
3. Semlacher R, Nuri M. Successful management of absent sternum in an infant using porcine acellular dermal matrix. Arch Plast Surg. 2019 Sep;46(5):470-474.
4. Das D, Dutta N, Pandey P, Basu A, Sharma MK, Koley R, et al. Complete sternal cleft with tetralogy of Fallot. Ann Pediatr Cardiol. 2020 Jan-Mar;13(1):81-83.

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