Name : Hamid reza
Family : Foroutan
Affiliation :Shiraz University of Medical Sciences, Pediatric surgery ward
Academic Degree : Prof. of Pediatric Surgery
Resident : Amir Hossein Hosseini
Gender : Male
Age : 1
A newborn baby with severe abdominal distension since first day of life. After evaluation patient was diagnosed as a new case of cystic fibrosis.
Cases at the most severe end of the spectrum may be diagnosed on prenatal ultrasonography with fetal ascites, polyhydramnios, bowel dilatation, intra-abdominal calcifications, and hydrops fetalis.
Discussion (Related Text)
Perforation of the intestine may occur in utero or shortly after birth. Frequently, the intestinal perforation seals naturally with relatively little meconium leakage into the peritoneal cavity. In some cases, with long-standing perforation, meconium peritonitis is more pronounced. Perforations occur most often as a complication of meconium ileus in infants with CF but are occasionally due to a meconium plug or in utero intestinal obstruction of another cause. At the other end are cases in which an intestinal perforation may seal spontaneously with only a minor meconium leak, so the event may never be detected except when meconium becomes calcified and is later discovered on radiographs of the abdomen. Alternatively, the clinical picture may be dominated by the signs of intestinal obstruction (as in meconium ileus) or chemical peritonitis. Characteristic clinical findings include abdominal distention, vomiting, and absence of stools. Treatment consists primarily of elimination of the intestinal obstruction and drainage of the peritoneal cavity.
Nelson Textbook of Pediatrics; Edition 19th