Sternal malformations or anomalies
Anomalies of the sternum can be isolated as a single malformation or to be in association with a variety of syndromes involving other organ systems (24).
Sternal clefts are rare anomalies. Superior clefts are more frequent than inferior ones and isolated central clefts are extremely rare (2, 8). They are due to failure of fusion of the sternal bar (6). This failure can be complete or incomplete but complete sternal clefts is the least likely form (6, 8, 9). Sternal cleft could be diagnosed prenatally but isolated sternal cleft is often diagnosed as asymptomatic at birth (6, 11).
Another typical anomaly is the presence of double ossification centers in the manubrium with a cranio-caudal arrangement instead of a side by side position of the ossification centers. This is present in trisomy 21, monosomy X and diastrophic dysplasia among other conditions (20, 21, 22).
Other anomalies of the sternum associated with Monosomy X include short sternum, decreased ratio of sternal body to manubrium bowing of the mesosternum, mild pectus excavatum also, and postnatally: premature fusion of the manubrio-sternal junction, premature fusion of the mesosternum (21). Short sternum is can also be seen in trisomy 18 or after isotretinoin exposure (13, 24).
Defects of the lower part of the sternum and deficiency of the anterior diaphragm characterized certain anterior abdominal wall defects such as incomplete forms of Cantrell’s pentalogy (3).
At last, sternal anomalies exist in PHACES syndrome (facial Hemangioma, posterior fossa anomaly, arterial anomalies, aortic coarctation, eyes anomalies and sternum malformations) and in numerous other syndromes (17).
1 . The sternum. On http://theodora.com/anatomy/the_sternum.html (2007, October, 30th).
2 . Luthra S, Dhaliwal RS, Singh H. “ Sternal cleft-a natural absurdity or a surgical opportunity.” J Pediatr Surg. 2007 Mar; 42(3) : 582-4.
3 . Staboulidou I, Wustemann M, Schmidt P, Gunter HH, Scharf A.“ Increased fetal nuchal translucency as a predictor of Cantrell’s pentalogy—case report.” Z Geburtshilfe Neonatol. 2005 Dec; 209(6): 231-234.
4 . Boutiller B, Outrequin G. “ Sternum “ On http://www.anatomie-humaine.com/Sternum.html (2007, October, 30th ).
5 . Grevious MA, Fritz JP, Holterman MJ, Rodriguez P. “Trunk Embryology“ On http://emedicine.com/plastic/topic217.htm (2007, October, 30th).
6 . Yavuzer S, Kara M.” Primary repair of a sternal cleft in an infant with autogenous tissues.” Interactive Cardiovascular and Thoracic Surgery 2: 541-543 (2003).
7 . Stark P, Jaramillo D. ” CT of the sternum.” AJR 147 : 72-77, July 1986.
8 . Acastello E, Majluf R, Garrido P, Barbosa LM, Peredo A.” Sternal cleft : a surgical opportunity.” Journal of Pediatric Surgery, Vol 38, No 2 (February), 2003: pp178-183.
9 . Karakaya H, Sahin N, Arslan A, Trakya A.” Complete sternal cleft in adolescence.” The Internet Journal of Anesthesiology. 2003.Vol 2 Number 2.
10 . Twomey EL, Moore AM, Ein S, McAuliffe F, Seaward G, Yoo SJ.” Prenatal ultrasonography and neonatal imaging of complete cleft sternum : a case report.” Ultrasound in Obstetrics and Gynecology Vol 25, Issue 6, pp 599-601.
11 . Mahone PR, Sherer DM, D’Angio C, Woods JR Jr.” Prenatal ultrasonographic findings associated with wide clefting of the upper two thirds of the fetal sternum.” Am J Obstet Gynecol. 1992 Apr; 166(4) : 1219-21.
12 . Seeds JW, Cefalo RC, Lies SC, Koontz WL.“ Early prenatal sonographic appearance of rare thoraco-abdominal eventration.“ Prenat Diagn. 1984 Nov-Dec; 4(6) : 437-441.
13 . Rizzo R, Lammer EJ, Parano E, Pavone L, Argyle JC.” Limb reduction defects in humans associated with prenatal isotretinoin exposure.” Teratology. 1991 Dec; 44(6), 599-604.
14 . Yekeler E, Tunaci M, Dursun M, Acunas G.” Frequency of sternal variations and anomalies evaluated by MDCT.” AJR 2006; 186 : 956-960.
15 . Kohli V, Nooreyazdan S, Das BN, Kaul S, Singh J, Parmar V.” Surgical reconstruction for absence of sternum and pericardium in a newborn.” The Indian Journal of Pediatrics 2006; Vol 73, Issue 4, 367-368.
16 . Mc Cormick WF, Nichols MM.” Formation and maturation of the human sternum. Fetal period. ”Am J Forensic Med Pathol. 1981 Dec; 2(4): 323-328.
17 . Monnier D, Pelletier F, Aubin F, Puzenat E, Moulin T, Humbert P.” Syndrome PHACE (S) d’expression Incomplète : Hémangiome facial associé à une agénésie complète d’une artère carotide interne. ” Annales de Dermatologie et de Vénéréologie 2005, Vol 132, No 5, pp. 451-454.
18 . Rush WJ, Donnelly LF, Brody AS, Anton CG, Poe SA.” Missing sternal ossification center: Potential mimicker of disease in young children” Radiology 2002; 224: 120-123.
19 . Kriss VM. Down syndrome: imaging of multiorgan involvement. Clin Pediatr (Phila). 1999 Aug;38(8):441-9.— Edwards DK 3rd, Berry CC, Hilton SW. Trisomy 21 in newborn infants: chest radiographic diagnosis. Radiology. 1988 May;167(2):317-8.
20 . Horns JW, O"Loughlin BJ.Multiple manubrial ossification centers in mongolism. Am J Roentgenol Radium Ther Nucl Med. 1965 Feb;93:395-8 — Currarino G, Swanson GE. A developmental variant of ossification of the manubrium sterni in mongolism. Radiology. 1964 May;82:916.
21 . Mehta AV, Chidambaram B, Suchedina AA, Garrett AR. Radiologic abnormalities of the sternum in Turner"s syndrome.Chest. 1993 Dec;104(6):1795-9.
22 . Remes VM, Helenius IJ, Marttinen EJ. Manubrium sterni in patients with diastrophic dysplasia--radiological analysis of 50 patients. Pediatr Radiol. 2001 Aug;31(8):555-8. Currarino G. Double-layered manubrium sterni in young children with diastrophic dysplasia. Pediatr Radiol. 2000 Jun;30(6):404-9.
23 . O"Neal ML, Dwornik JJ, Ganey TM, Ogden JA.Postnatal development of the human sternum.J Pediatr Orthop. 1998 May-Jun;18(3):398-405
24 . Zalel Y, Lipitz S. The development of the fetal sternum : a cross sectionnal sonographic study. Ultrasound Obstet Gynecol 1999;13:187-190.