Omphalocele

Omphalocele is a congenital condition characterized by an abdominal wall defect where the intestines, liver, and sometimes other organs remain outside the abdomen in a sac because of a defect in the development of the muscles of the abdominal wall. 

What is Omphalocele?

Omphalocele is a birth defect where the infant's intestines or other abdominal organs stick outside of the belly through the belly button (umbilicus). These organs are covered by a thin, nearly transparent sac that can easily be seen. The size of the omphalocele can vary, with some being small (involving only a portion of the intestines) and others being large (involving the majority of the abdominal organs).

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Difference Between Omphalocele and Gastroschisis

Omphalocele vs Gastroschisis

The terms omphalocele and gastroschisis often come up together due to their similarities, but they are distinct conditions.

  • Omphalocele: As mentioned, this condition involves organs protruding through the umbilicus and being covered by a sac.
  • Gastroschisis: This is another congenital defect where the baby's intestines are found outside of the baby's body, but here, there is no sac covering them and the defect is typically located to the right of the umbilicus.

The primary difference between omphalocele and gastroschisis lies in the presence of the sac and the location of the defect.

Omphalocele Gastroschisis

Both conditions require surgical intervention after birth, but the techniques and timing of the surgery might differ. Omphalocele often indicates a more complex situation due to the involvement of multiple organs and potentially associated abnormalities.


Causes of Omphalocele

The precise cause of omphalocele is not known, but it's believed to result from a combination of genetic and environmental factors. Certain genetic syndromes and chromosomal abnormalities are associated with an increased risk of omphalocele. Additionally, maternal factors such as obesity, smoking, and certain medications can increase the risk.


Omphalocele Radiology

Radiological imaging plays a crucial role in the diagnosis and management of omphalocele. Prenatal ultrasounds can often detect the condition, sometimes as early as the first trimester. These ultrasounds will show the protruding organs and Doppler studies can assess the blood flow to these organs.

After birth, further imaging such as X-rays and MRIs might be used to get detailed information about the organs involved and to plan for surgical intervention.


Treatment Options for Omphalocele

Initial Management

The initial management of a baby born with omphalocele focuses on protecting the exposed organs and preventing infections. The sac covering the organs is kept moist and protected with sterile dressings. The baby is usually placed in a warmer to maintain body temperature.

Surgical Intervention

The mainstay of treatment for omphalocele is surgical repair. The timing and method of surgery depend on the size of the omphalocele and the condition of the baby.

  • Primary Repair: For small omphaloceles, a primary repair might be possible. This involves surgically placing the organs back into the abdomen and closing the abdominal wall defect.
  • Staged Repair: Larger omphaloceles might require a staged approach. Initially, the organs are gradually moved back into the abdomen over several days or weeks using a device called a silo. Once the organs are back in the abdomen, the abdominal wall defect is repaired.

Postoperative Care

Postoperative care is crucial for recovery. Babies might need to stay in the neonatal intensive care unit (NICU) for a period, where they receive support for breathing, feeding, and infection prevention. Long-term follow-up is essential to monitor growth and development and to manage any complications.

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Complications and Prognosis

Complications

Complications from omphalocele can arise due to the condition itself or from surgical interventions. These include:

  • Infections: Due to the exposure of abdominal organs.
  • Respiratory Issues: Large omphaloceles can cause lung underdevelopment, leading to breathing problems.
  • Feeding Difficulties: Gastrointestinal issues might occur, necessitating specialized feeding plans.

Prognosis

The prognosis for babies with omphalocele depends on the size of the defect and the presence of associated anomalies. With advances in prenatal diagnosis, surgical techniques, and neonatal care, the survival rates for omphalocele have improved significantly. However, long-term outcomes can vary, and ongoing medical care may be needed.

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Frequently Asked Questions

1. What is an omphalocele?

An omphalocele is a birth defect in which the infant's intestines, liver, or other organs protrude outside the body through the belly button.

2. What is the difference between omphalocele and gastroschisis?

Omphalocele involves organs covered by a membrane, while in gastroschisis, the organs protrude without any protective covering.

3. How is omphalocele diagnosed?

Diagnosis is typically made during pregnancy through prenatal ultrasound, where the defect can be seen clearly.

4. What is the treatment for omphalocele?

Treatment involves surgery after birth to place the organs back inside the body, sometimes requiring multiple operations depending on the severity.

5. What are the complications of omphalocele?

Complications can include infection, breathing difficulties, and long-term digestive problems depending on the severity of the defect.

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