[Congenital sternal cleft].
BACKGROUND
Congenital sternal cleft is a rare malformation. Little more than one hundred cases have been published, and rarely more than two cases are published in a single publication. We present five new cases that show the entire spectrum of defects.


METHODS
They were 3 males and 2 females. Age range between 1 day and 5 years of age. There were 2 supraxiphoid clefts (SC), 2 gladiolar defects and 1 xiphoid cleft. We evaluate the demographics, associated malformations, mode of treatments and results.


RESULTS
The SC were not associated to congenital herat malformations, but with a many other malformative spectrum (anterior cervical web, hemangiomatosis, CNS malformations, coloboma and pectus excavatum). The two females had SC and were operated on the 24th and 30th days of age. The first one developed a superior vena cava síndrome for a few days after surgery and a moderate pectus excavatum that did not required surgery. A midline cervical web was also surgically repaired at 10 years of age. The second patient with SC was treated with interferon alfa-2a and local infiltration of corticosteroid for treatment of hemangiomatosis. Both patients had excellent final results (follow up 11 and 5 years respectively). Gladiolar clefts were observed durign surgical repair of congenital Herat malformations (ventricular septal defect and partial anomalous of pulmonary venous draninage). They were repaired by direct closure of the defect. The patient with the xiphoid cleft had associaed a Cantrell pentalogy. He died 4 days after operation because of extreme prematurity and pulmonary vascular hypertenion.


CONCLUSIONS
1) We have observed five new cases of sternal cleft. 2) Each anatomical variety had their own mode of presentation, a different malformative spectrum and clinical evolution that are based on a distinct embryonic origin. 3) Excluding the xiphoid clefts, the long term surviving is 100%, but they are associated with a great variety of other congenital defects.
de Agustín Asensio
J C
JC
S. de Cirugía Pediátrica, Hospital Infantil Universitario Gregorio Marañón, Madrid. juandeagustin@mac.com
Greco Martínez
R
R
Sanz Villa
N
N
Hernández
E
E
Sánchez Paris
O
O
Vázquez-Estévez
J
J
D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D013249:Sternum / Q000002:abnormalities* / Q000601:surgery*