Dr.Osama Esmaeel Hudder MRCS(England) Iraqi Board Pediatric Surgery
Congenital abdominal wall defects.1-Omphalocele 2-Umbilical cord hernia 2-Gastroschiasis 3-Prune belly Syndrome
Omphalocele
Abdominal wall defect at umbilicus with covering (sac may rupture) Frequently associated with other anomalies Giant omphaloceles: It contain liver,small bowel, large bowel, and stomach .Omphalocele Associated Anomalies
Chromosomal abnormalities (50%) Trisomies 13, 18, 21 Congenital heart disease (50%) Neural tube defects (40%) Beckwith-Wiedemann syndrome hyperinsulinism, visceromegaly, macroglossia, hepatorenal tumors, cloacal extrophy Pentalogy of Cantre omphalocele, ectopia cordis, anterior diaphragmatic hernia, intracardiac defect, sternal cleftPrenatal Diagnosis
Elevated maternal serum alpha fetoprotein Ultrasound Omphalocele GastroschisisWhat is the mode of delivery in case of omphalocele?
If the Omphalocele is larger than 5 cm delivary should be through CS ,while if less than 5 cm could be with normal vaginal delivary
Initial Management
Early management aimed at maintaining circulation to bowel and preventing infection while stabilizing infant (temperature/fluids) : Cover the defect with sterile dressing soaked in warm saline to prevent fluid loss Incubator Nasogastric decompression Urinary catheter for drainge IV fluids with glucose(150cc /kg/24hr) Invistigation(U/S of renal system,Echocardiograghy,Blood glucose level,Blood group) Vitamine KSurgical Treatment
The operation is not an emergency unlike gastroschiasis. There are three approuch to manage omphalocele:- 1-Primary surgical Closure. Under general anasthesia with endotreacheal tube,we excise the sac and return the viscera to the abdominal cavity and close the defect.we have to avoid high increase in intraabdominal pressure.and some times we have to close the skin only or to do release inscion or to use special type of mesh. Our aim is to do Primary Surgical Closure: Success dependent on size of the defect and size of the abdomen.2-Staged Closure: When the Abdominal cavity is small,we start with Gradual reduction of the contents into the abdominal cavity using an extra-abdominal extension of the peritoneal cavity (termed a silo) and using gentle pressure. Usually requires 1-3 weeks, after which the defect is then primarily closed.
Silo closure
Omphalocele
3-Non-surgical treatment of omphalocele:“paint” membrane with betadine to Thicken the memberane and later surgery. After 2-3 years surgery done .Gastroschisis
Abdominal wall defect to right of umbilicus with no covering over intestinesGastroschisis Rarely associated with other anomalies
But, it may associated with gastrointestinal problems (25%) Including atresia, volvulus, stenosis Loss of bowel secondary to ischemia Compromised bowel function Rarely will have significant infarction of most of small bowel (i.e. lethal)