0 Diagnosis of anterior abdominal wall defects:
1. Determination of alpha fetoprotein and amniotic acetylcholinesterase in maternal blood
2. Fetal ultrasonography and/or MRI
Gastroschisis
0 The incidence is 2-4.9/10000 live births and is more common in males.
0 It is common in low birth weight and preterm infants.
0 Young maternal age and low socio-economic status are risk factors for aspirin, ibuprofen and pseudoephedrine used in the first trimester.
0 5-10 of the amniotic membrane. It is thought to occur as a result of rupture in weeks.
0 Gastroschisis is 2-5 times more common than omphoalocele. It is the most common abdominal wall defect.
Clinic
• There is a 2-5 cm diameter defect on the anterior abdominal wall, just to the right of the umbilical cord, and the stomach, small-large intestine are outside the abdomen. The liver is rarely outside the defect.
• Intestines are edematous and there is no sac on it. Intestines are covered with fibrin and their walls are thickened.
• Intestines are hardened, enlarged and thickened due to aseptic fetal peritonitis.
• The anterior abdominal wall muscles have completed their normal development.
• The navel is in its normal anatomical position (7-30%).
• Additional anomalies and chromosomal anomalies are rare.
• Although rare, the most common anomalies are related to the GIS. It is most often accompanied by jejunoileal anomalies.
• Jejunoileal malformations; jejunoileal atresia, Meckel's diverticulum and rotational anomalies.
• It is also a kind of ileal deformity and intestinal infarction known as intestinal malrotation, atresia, volvulus, "christmas tree" deformity.
• Cardiac and genitourinary system anomalies are less common and are generally not life-threatening.
• Gangrene and perforation may occur due to circulatory disorders.
• Hypoproteinemia and hypogammaglobulinemia due to plasma loss due to inflammation in the intestinal serosa are common.
Treatment:
• First of all, the intestines should be covered with a moist gas or bag, the baby should be warmed and liquid support should be provided.
• Decompression and antibiotherapy are performed with N/G.
• Surgery:
1. Primary repair
2. Create a ventral hernia and then repair it
3. Silastic silo
• The most important post-operative danger is respiratory failure, hypovolemia and sepsis.
Prognosis
• Prematurity, low birth weight, hypothermia and sepsis are effective on mortality.
• Since additional anomalies are rare, postoperative mortality is 5-15% (mean 7.7%).
Omphalocele (Exomphalos)
0 Frequency is 1-2.5/5000 live births.
0 It is common in low birth weight and preterm infants.
0 40% of cases have polyhydramnios.
0 10-12 of the midgut. It is caused by the inability to return from the extraembryonic coeloma to the abdominal cavity within weeks.
0 It is a transparent sac with an umbilical cord on it and filled with small intestine and/or liver.
Omphalocele (Exomphalos, Amniocele):
• Defects smaller than 4 cm in diameter are called umbilical cord hernia.
• In this case, there are only a few intestinal loops in the pouch.
• Small cord hernias that are left untreated are healed by gradually developing epithelialization, this is called "cutis navel".
Intact, Ruptured and Giant Omphalocele:
• An omphalocele is a solid sac with an umbilical cord on it and filled with intestines and liver.
• The outer surface of the omphalocele sac is formed by the amniotic membrane, and the inner surface is the peritoneum. Between these two membranes is a primitive mesenchymal tissue called Wharton's jelly.
• The diameter of this defect in the anterior abdominal wall may be between 4-12 cm.
• There are usually stomach, small and large intestine loops in the pouch.
In almost half of the cases, the liver is located in the sac.
• Regardless of the size of the defect, the abdominal muscles are normally developed and the rectus muscle at the edges of the defect is intact.
• Generally, the sac is intact at birth. Since the sac is avascular, it can rupture in a short time.
• The omphalocele sac may rupture in utero, during delivery or after delivery.
It has been reported that prenatal rupture develops in 10% to 1% of the cases.
Clinic:
• In 50 to 70% of omphalocele cases, visceral malformations accompany the picture.
• Musculoskeletal, gastrointestinal and genitourinary anomalies, especially heart, are common.
• The most common rotation anomalies among GIS anomalies are Meckel's diverticulum and patent omphalomesenteric duct.
• Cardiovascular anomalies are the most common. The frequency of cardiovascular anomaly explains the importance of performing echocardiography in fetuses with omphalocele.
• The two most common anomalies; Tetralogy of Fallot and atrial septal defect.
• May be a component of Beckwith-Wiedemann syndrome (EMG syndrome; Exomphalos, Macroglossia, Gigantism).
Other components; enlarged internal organs, hemihypertrophy and hypoglycemia.
• Pancreatic adenoma and nesidioblastosis
• Pentalogy of Cantrell: Upper midline omphalocele, anterior diaphragmatic hernia, sternal cleft, ectopia cordis, cardiac anomaly.
• Omphalocele is also common in Prune Belly syndrome.
• Chromosomal abnormalities such as trisomy 13-15, 16-18 and sometimes 21 are seen.
• Lung hypoplasia and lung anomalies are common in giant omphaloceles.
treatment
Additional anomalies should be carefully investigated.
N/G decompression and antibiotic therapy are performed.
The pouch is covered with damp and wet gauze.
Surgical:
• Primary repair
• Create a ventral hernia and then repair it
• Silastic silo
• Conservative treatment (epithelialization)
Those <4 cm are closed primarily, and those above 4 cm are closed gradually to increase the intra-abdominal pressure.
Prognosis
• When evaluated according to operated patients, the prognosis of omphalocele is worse than gastroschisis because of accompanying anomalies.
Omphalomesenteric Duct Residues
The omphalomesenteric duct provides a connection between the terminal ileum and the vitelline sac (yolk sac) in the embryological period.
In the intrauterine 16th week, it becomes obliterated together with the canal vessels and completely disappears after birth.
Due to the problems in the obliteration of the canal, some abnormal structures may develop after the baby is born.
The omphalomesenteric duct opening may be a band, sinus, or cyst.
There may be intestinal prolapse.
Intestinal obstruction may develop due to omphalomesenteric duct bands.
Patent omphalomesenteric duct: Both ends of the duct remain open (6%). From the navel comes the ileal content.
Meckel's diverticulum: The proximal (intestinal side) of the duct is not closed (80%).
Umbilical sinus: The distal (umbilical side) of the canal is not closed (2%). It can cause discharge from the navel.
Umbilical cyst: both ends of the canal are closed and a cystic structure has formed in the middle (2%).
Umbilical Granuloma
@ After the fall of the navel, a soft, pink-red mass of several mm in diameter can be seen in the umbilical pit, which is an umbilical granuloma.
@ It causes watering in the navel, swelling and redness of the surrounding skin.
@ Small granulomas are easily treated by cauterization with 1-2 times of silver nitrate application.
@ Large granulomas require excision and cauterization of the base.
Umbilical Polyp
@ After the navel falls, a cherry-red nodule-shaped red mass may form in the navel, which is an umbilical polyp.
@ The polyp often contains intestinal mucosa or rarely gastric mucosa as vitelline duct remnant.
@ It is often confused with umbilical granuloma.
@ When silver nitrate does not resolve with cauterization, it is understood to be an umbilical polyp and is surgically treated with excision.
Umbilical Hernia
@ 25% of infants in the first months of life have umbilical herni.
@ Umbilical hernias can be seen with thyroid dysgenesis, trisomies, Beckwith-Wiedemann syndrome and Hurler syndrome.
@ The majority of umbilical hernias close spontaneously by age 3. Most of the hernias that continue after the age of 4-5 close until puberty.
Complications such as incarceration (impression) and strangulation (circulatory disorder due to compression), perforation or evisceration can be seen in umbilical hernias. but it is very very rare.
Methods such as hernia ligation, money sticking and plaster sticking are useless, they can cause maceration of the skin, and most dangerously, they can prevent breathing with pressure, leading to asphyxia and death.
@ Over time, the majority of umbilical hernias close spontaneously with the development of the abdominal wall.
@ If there are no complications, it is expected until the age of 3 or school age.
@ The surgical method consists of closing the fascia defect with the Miller method by making a transverse incision under general anesthesia, without entering the abdomen.
Urakus Canal Remnants
@ The urachus duct is a structure that provides a connection between the allantois sac and the bladder at the beginning of the intrauterine period.
@ Urakal diverticulum: The proximal (bladder) side of the duct is open.
@ Urakal cyst: Both ends of the duct are closed, but a cystic structure has formed in the middle. It could be the cause of infection.
@ Urakal sinus: The distal (umbilical) side of the duct is open. It is the cause of discharge from the navel.
@Patent urakus: The urachal channel is completely open. There may be urine coming from the navel.
