Pediatric surgery

Pediatric surgery lectureslecture No. 22018

Dr.OSAMA AL-MUSHHADANY MRCS(ENGLAND) IRAQI BAORD PEDIATRIC SURGERY Assistant prof. Nineveh medical college

Congenital abdominal wall defects.

1-Omphalocele 2-Gastroschiasis 3-Prune belly Syndrome

Omphalocele

Abdominal wall defect at umbilicus with covering sac Frequently associated with other anomalies Giant omphaloceles: It contain liver, small bowel, large bowel, and stomach .

Gastroschisis

Abdominal wall defect to right of umbilicus with no covering over intestines

Embryology and etiology:-

An omphalocele occurs if the intestines fail to return to the abdominal cavity. Varying amounts of bowel may be contained within the omphalocele sac. Other intraabdominal viscera including liver, bladder, stomach, ovary, and testis can also be found within the sac. The sac consists of the covering layers of the umbilical cord, which include amnion, Wharton’s jelly, and peritoneum. The umbilical cord is attached to the sac itself.

The etiology of gastroschisis is less clear. One theory suggests that gastroschisis results from failure of the mesoderm to form in the anterior abdominal wall. A second theory posits that failure of the lateral folds to fuse in the midline leaves a defect to the right of umbilicus

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 cleft

Gastroschisis 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)

Prenatal Diagnosis

Elevated maternal serum alpha fetoprotein Ultrasound help to diagnose omphalocele and gastrosciasis and detect some of the associated anomalies. 1-detection of bowel loops freely floating in the amniotic fluid and a defect in the abdominal wall to the right of a normal umbilical cord are diagnostic of gastroschiasis. 2-detection of herniated bowel covered by sac with umbilical cord attached the top of this mass are diagnostic of omphalocele.

Ultrasound finding

Omphalocele
Gastroschisis

What is the mode of delivery in case of omphalocele?

If the Omphalocele is larger than 5 cm delivary should be through cesarean section (CS ),while if less than 5 cm could be with normal vaginal delivary In case of gastroschiasis better to deliver by CS because vaginal delivary increase the risk of bowel injury and infection .

Resuscitation of neonate with omohalocele

Early search for associated anomalies is important in all neonates should undergo echocardiography, and ultrasonography for renal evaluation. Blood sample for genetic evaluation.


Initial 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) Antibioyic cover Vitamine K

Treatment option for omphalocele:-

It depend on the size of the defect, the baby's gestational age and the presence of other associated anomalies. 1-Immediate primary closure:- Defect that are less than 1.5 cm in diamter are referred to as hernia of the cord and repaired easily shortly after birth . Larger defect(medium size omphalocele) but still but still easy to close without high increase in the intraabdomial pressure can also closed shortly after birth.


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

3-Delayed staged closure:-

Children with large omphalcele managed by excising the covering sac and mobilizing skin flap to cover the exposed viscera leaving large ventral hernia that could be closed after 1-2 years



4-Non-surgical treatment of omphalocele:“paint” membrane with betadine to Thicken the memberane and later surgery. After 2-3 years surgery done .Note:-rupture of the sac may occurs antenataly or during delivary or postnataly and this serious complication need emergency management like gastroschiasis to prevent sepsis .

Initial Management of gastroschiasis:-

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(200 cc /kg/24hr) Invistigation(U/S of renal system,Echocardiograghy and Blood group) Vitamine K

Gastroschisis .

Surgery is an emergency operation due to exposure of intestine to the environment 1-Primary closure is attempted with the same principles of omphalocele closure. 2-May require silo with slow return of intestine into small abdominal cavity to Maintain good perfusion and prevent ischemia. 3-Delayed staged closure:-mobilizing skin flap to cover the exposed viscera leaving large ventral hernia that could be closed after 1-2 years

No role for non-surgical management. Feeding difficulties are main post-operativer problem The patient is at risk for adhesions throughout life

Prognosis?

Prune Belly Syndrome
Characterized by: deficiency of abdominal wall musculature a dilated, non-obstructed urinary tract bilateral cryptorchidism talipes equinovarus and hip dislocation Incidence is 1/35-50,000 >95% occur in males

Thank you

1-what is the diagnosis?2-what are the differences
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