Friday 2 July 2010
Case 12 : Sternal cleft
Definition: Congenital sternal cleft is a rare disease, and primary repair in the neonatal period is its best management.
Congenital sternal cleft is a rare disorder in which there is a gap in the midline of the anterior chest wall between the two halves of the sternum.
Typically, the contour of the mediastinal structures can be seen beneath the skin. It is rare and the exact incidence is not known.
It results from failure of fusion of the two lateral mesodermal sternal bars by 8 weeks of gestation.
Most cases are diagnosed shortly after birth and are reported only rarely in adults.
Sternal clefts may be classified as follows:
(1) complete cleft sternum without additional malformations
or with ectopia cordis, large vessel, facial, and
(2) superior sternal clefts without additional malformations or with anomalies as in cases of total clefts;
(3) inferior sternal clefts with additional severe malformations (Cantrell’s syndrome)
(4) sternal clefts within the scope of a total ventral cleft.
Inferior sternal cleft usually occurs in Cantrell pentalogy, which also includes an omphalocelelike abdominal defect, a crescent-shaped anterior diaphragmatic defect, and a hole in the pericardium, which allows pericardial-peritoneal communication.
Cantrell pentalogy is also often associated with various intra-cardiac anomalies, such as atrial or ventricular septal defect, tetralogy of Fallot, and ventricular diverticulum.
enterogenous cyst (17806026)
The demonstration of the inferior sternal cleft using three-dimensional reconstruction: a case report. Cudi Tuncer M, Ufuk Aluçlu M, Karabulut O, Ulkü R, Savaş Hatipoğlu E, Nazaroğlu H. Rom J Morphol Embryol. 2009;50(3):513-6. PMID: 19690785 [Free]
Sternal cleft associated with enterogenous cyst treated during the newborn period. Luis AL, López Gutierrez JC, Fernández A, Avila LF, Encinas JL, Andrés AM, Hernández F, Nistal M, Tovar JA. Eur J Pediatr Surg. 2007 Aug;17(4):275-7. PMID: 17806026
Complete congenital sternal cleft associated with pectus excavatum. Sarper A, Oz N, Arslan G, Demircan A. Tex Heart Inst J. 2002;29(3):206-9.PMID: 12224725 [Free]