Omphalocele
(Omphalocele)
INTRODUCTION
Omphalocele or also called exomfalos occur when there is failure of the intestines back into the abdominal cavity in the 10th week of life in the womb. This failure also explains the high incidence of malrotation in omphalocele. Defect of the abdominal wall around the umbilicus is covered by a layer comprising a transparent layer on the outside of the amnion and peritoneum lining on the inside. Umbilical cord was found on top of the bag. Transparent layer has vascularization minimal so rapid necrosis and prone to infection.
Etiology
Embryology etiology of omphalocele is considered due to failure of fusion of cephalic fold of the abdominal wall, caudal, and lateral to the central defect that resulted in the inhibition of lateral wall and folds occur in the midline omphalocele low and epigastrium. Respectively due to impaired migration of cephalic and caudal folds. Epigastric omphalocele may be accompanied with a disability under the sternum, diaphragm and heart, whereas the caudal omphalocele may be accompanied by genitourinarius and gastrointestinal anomalies, which include ekstrofi cloaca (vesikointestinalis fissure). At the age of 10 weeks of fetal development will occur so that the lumen of the intestine from the abdomen ekstraperitoneum going into the abdominal cavity. When this process is inhibited, there would be pockets at the base of the umbilicus that contains the intestines, stomach and sometimes the liver. The walls are thin and consist of layers and layers of amnion peritonum the two nodes so that the bag looks from the outside
.
The cause of omphalocele
When the fetus grows and develops during pregnancy, there is a small open area in the abdominal wall to place through the umbilical cord that connects mother and fetus. When the fetus matures, the abdominal muscles should meet in the middle and grow together so that the wall that opens to close. Omphalocele occurs when the abdominal organs back into the abdominal cavity is not as it should.
Until the exact cause is unknown and not related to any dilakukam by his mother.
INCIDENCE
Abdominal wall defect was found in 1 in 5000 births. Most cases of omphalocele were found (30-70%) disetai
with other congenital abnormalities.
Table: The syndrome usually accompanies omphalocele
Pentalogi Cantrell (defects in the cephalic embryonic fold) | |
A. 2. 3. 4. 5. | Upper midline omphalocele Anterior diaphragmatic hernia Defective sternum Ektopia cardiac Defects in heart |
Lower midline syndrome (a defect in the caudal embryonic folds) | |
A. 2. 3. 4. 5. 6. | Ekstrofi vesika urinary or cloacal Imperforate anus Colonic atresia Fissure vesikointestinalis Sacral vertebral anomalies Meningomielokel |
Beckwith-Weidemann syndrome | |
A. 2. 3. 4. | Gigantism Makroglosis Omphalocele or umbilical hernia Pancreatic islet cell hyperplasia |
Trisomy Syndrome | |
A. 2. | Trisomy 13-15 Trisomy 16-18 |
Some other serious anomaly provides a particular challenge in the successful management of this problem.
Mortality / Morbidity
For over 30 years, the life of the baby with omphalocele increased from 6% in the 1960s to 90%. On the gain with abdominal wall defects susceptible to hypothermia, dehydration, sepsis, hypoglycemia. Because anesthesia and surgical techniques improved and the insufficient supply of good techniques for parenteral nutrition, the better the patient's life expectancy.
Omphalocele babies and abdominal wall defects ranging from 4-12 cm in Centar area, epigastric or hypogastric.
Omphalocele sac is usually intact, but there is the possibility of rupture in the uterus or because the birthing process.
SYMPTOMS
Omphalocele can be clearly seen because of abdominal contents protruding out through the navel (belly button) .
CLASSIFICATION
Small omphalocele: just seen protruding intestines
Large omphalocele: liver and spleen may come out
PROBLEMS IN omphalocele
- Infections, especially when the bag covering the ruptured organ
- The organs to be deprived of blood flow when twisted together so that it will damage the organ tissue
- The transparent coating has minimal vascularization so rapid necrosis and prone to infection.
DIAGNOSIS
- Diagnis omphalocele enough to see a defect in the umbilicus area covered by the thin transparent membrane. On the inside of the intestines can be seen, some liver, stomach and spleen may depend on a large defect.
- Fetal ultrasound in the second and third trimester of pregnancy
- Fetal echocardiogram for heart function
- X-Ray to see a picture of the organ tissue, bone, soft tissue, organ abnormalities to evacuate
- MSAFP (Maternal Serum Alpha Feto Protein)
Possible signs of structural abnormalities of the fetus.
Prognosis
Prognosis depends on:
A. Classification / size of the omphalocele
2. Disorders vaskulariasi the organ in question
3. presence of other congenital disorders
Total is the hope of improvement after surgery omphalocele, but nevertheless omphalocele is usually followed by other congenital disorders that how the child berantung improvement of the situation.
If the omphalocele is known before birth, state of the mother and child should always be monitored so that the situation is always healthy. Birth plans and the things that need to be done after birth should be regulated. The baby should be born in a place that has facilities for omphalocele surgical repair so as not to be moved around.
Parents should plan examination (screening) for the unborn child to find other congenital problems.
Some circumstances to consider:
o a ruptured omphalocele have a poor prognosis
o omphalocele with diameter of 5 cm or less in the term infant and primary closed generally have good prognosis.
Expectations after surgery
The defect is almost always can be improved by further surgery but the results depend on the magnitude of damage to organs and other congenital abnormalities that accompany.
COMPLICATIONS
- Infections
- Rupture
- Obstruction
- tissue necrosis
- Congenital abnormalities of the abdominal wall may be accompanied by other congenital abnormalities that worsen the prognosis
- Post operative:
Bleeding
Infection
Post surgical adhesion
Shock
Ileus paralysis
The risk of anesthesia in surgery omphalocele
A. Reaction anesthetic drugs
2. Impaired breathing during surgery
Risk of surgery
A. Bleeding
2. Infection
Additional risk in omphalocele repair surgery
A. Injury to the organ
2. Respiratory
3. Peritonitis
4. Temporary paralytic ileus
THERAPY
Prabedah preparation of patients with congenital abdominal wall defects should be directed to the prevention of hypothermia, hypovolemia, acidosis, and the threat of bacterial contamination in the intestinal vascular dieviserasi.
- The main purpose of restoring operations omphalocele sac into the abdominal cavity and close it.
- If the sac intact, surgery is not necessary, but must first deal with other congenital abnormalities.
- Replace the NGT to minimize bowel distension.
- Successful operation depends on whether or thoracic and abdominal cavity, the size of the defect.
- Do not open the bag when the operation can not be done. Treatment can be done by wrapping a layer of plastic wrap or viscera with "bowel bag" to prevent evaporation.
- When you better pack omphalocele but closing the abdominal cavity can not be done, there are 2 options:
1. Stage repair
2. Prosthetic patch repair
Goal to make the repair stage shaped like a silo protective ekstrabdominal (chimney) so that there is a gradual improvement opportunities from the bag and glue the second side of the gap with two layers of the abdominal wall parallel after the organs are wrapped gently inserted into the abdominal wall.
Prosthetic patch repair for the purpose of connecting the sides of the gap that separated the abdominal wall with synthetic material (such as polytetra-fluoroethylene) normally takes 5-7 days until the defect can be closed.
In some cases non-operative management is recommended in infants with severe congenital abnormalities or with a giant omphalocele.
Amniotic sac can be left to dry and form a "eschar". Vascularization will grow itself in layers of eschar , contracture of the wound and skin growth will close the defect. Typically will be formed and ventral hernia repair surgery is performed when the patient is stable.
Additional therapy after surgery include:
A. Oxygen
2. Mechanical Ventilators
3. Antibiotics
4. analgesic drugs
Follow-up
After surgery, NICU (Neonatal Intensive Care Unit) placed the baby in an incubator to keep warm and reduce the risk of infection.
REFERENCES
A. R. Wim de Jong & Sjamsuhidajat: omphalocele in Textbook of Surgery, EGC, 2003, p.520-21
2. Sabiston DC: BukuAjar Surgery, Section 2, EGC, 1994, p. 265-67
3. Schwartz, Shires, Spencer: omphalocele in Sari Core Principles of Surgery, EGC, 1995, p.567-68
4. Department of Surgery Faculty of Medicine / Hospital Dr. Cipto Mangunkusumo: Lecture set of Surgery Faculty of Medicine, 1994, p. 128-29
5. http:// www.emedicine / omphalo-gastroschisis / g.glasser / mohtm
6. http:// www.health system.virginia.edu / uvahealth / peds.digest / omphaloc. cfm
7. http:// www.brighamrad.harvard.edu/cases/bwh/hcache/30.fulhtml
8. http:// www.nlm.nih.gov/medlineplus/ency/article/002938.htm
9. http:// www.enwikipedia.org / wiki / omphaliocele
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