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Chest wall deformities
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Robert E. Kelly, Marcelo Martinez-Ferro, Horacio Abramson
The sternal cleft deformity is the least severe of the sternal abnormalities, which also include thoracic and thoracoabdominal ectopia cordis. A sternal cleft results from incomplete fusion of the sternal bars in the fetus. The heart is in an orthotopic position. While intrinsic cardiac anomalies are common in thoracoabdominal and thoracic ectopia cordis, they are rare in infants with a sternal cleft. Repair of the sternal cleft relieves the paradoxical motion often seen in this anomaly. The sternal cleft may be complete, but in most cases the inferior base of the sternum is fused. Repair is best achieved in the newborn period or within the first several months of life when the chest wall is most flexible and primary repair is tolerated by the infant.
Congenital thoracic deformities
Published in Prem Puri, Newborn Surgery, 2017
Konstantinos Papadakis, Robert C. Shamberger
Sternal clefts are malformations caused by the failure of fusion of the sternal elements. The etiology of sternal cleft deformity is unknown. There are studies on ventral body development in mice that indicate that impairment in Hoxb gene expression may be a possible factor.32 Cleft sternum may be complete or incomplete and results from failure of the mesenchymal plate fusion process at the eighth week of gestation. No familial predisposition has been described. Alcohol intake and methylcobalamine deficiency have been implicated. There is a slight female predominance. Sternal clefts can be associated with cervicofacial hemangiomas (vascular dysplasia) and PHACES syndrome. 33,34
PHACES syndrome with ectopia cordis and hemihypertrophy
Published in Baylor University Medical Center Proceedings, 2019
Jad Chokr, Bedros Taslakian, Gilbert Maroun, Gagandeep Choudhary
A female term infant was born to a 21-year-old gravida 1, para 0, aborta 0, nonsmoking mother after an uncomplicated pregnancy. The parents were nonconsanguineous and did not report any family history of congenital abnormalities. On physical examination, the infant had a complete sternal cleft, with a partially displaced heart outside the thoracic cavity, covered by a thin skin (Figures 1a–1c). Further examination revealed a faint facial hemangioma covering the right cheek (Figures 1a–1c). The remainder of her examination did not show any other abnormality. A plain chest radiograph showed dextrocardia and widely separated clavicles and anterior ribs (Figure 1d).