A newborn is found to have a defect in the central abdomen at bih. All of the following features are suggestive of Omphalocele as a diagnosis except that
Correct Answer: The defect is open
Description: Omphalocele- larger defect, covered by a membrane, Higher association with anomalies, bowel usually normal.CONGENITAL OMPHALOCELEAn omphalocele is a herniation or protrusion of the abdominal contents into the base of the umbilical cord. In contrast to the more common umbilical hernia, the sac is covered with peritoneum without overlying skin. Immediate surgical repair, before infection, has taken place and before the tissues have been damaged by drying (saline-soaked sterile dressings should be applied immediately) or by rupture of the sac, is essential for survival.The majority ([?]75%) of infants with omphalocele have associated congenital anomalies/syndromes, including Beckwith-Wiedemann syndrome (omphalocele, macrosomia, hypoglycemia), and other chromosomal (29%, including trisomies 13 and 18) and nonchromosomal (45%) multiple and isolated congenital anomalies (musculoskeletal, 24%; urogenital, 20%; cardiovascular, 15%; and central nervous system, 9%). The survival rate is approximately 80% overall, but in infants with isolated omphalocele, the survival rate is >90%.Ref: Nelson Textbook of Pediatrics; 20th edition; Page no: 890
Category:
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