Oct 11, 2010

obstuesday | fetal gastrochisis

Many weeks ago, a major storm of uncertainty was brewing wildly within the department over the mode of delivery for an unfortunate baby with gastroschisis at gestation 36weeks, borderline prematurity.

Decision was made after discussion between the head of department with the paediatric surgeon. Much anxiety came from the referral center which failed to read the vital line in the nicely paragraphed conclusion after the detail scan which clearly stated ‘no contraindication for vaginal delivery’, and misinformed about the prior knowledge by the paediatric surgical team. In summary, it was a breakdown of communication due to misinformation.

With the termination of the pregnancy by which I meant delivery, much anxiety was terminated as well.

It struck me as a trainee how little I knew about fetal gastroschisis. Therefore, I looked it up.

What is gastroschisis?
- Inherited congenital abdominal wall defect
- Intestines and sometimes other organs develop outside fetal abdomen through opening in abdominal wall

Gastroschisis also known as?
- Paraomphalocele, Laparoschisis, Abdominoschisis

How to differentiate gastroschisis from omphalocele?
- Omphalocele involved the cords, and organs enveloped by visceral peritoneum

Embryological maldevelopment of gastroschisis?
- Failure of fusion of lateral body folds from ventral to midline
- At fourth week of development
- Bowel herniates through rectus muscle, lying in the right of umbilicus

What is the prevalence of gastroschisis?
- 1 in 10,000
- Male more than female

What is the sonographic features of gastroschisis?
- bowel outside of abdomen

What is the risk factors of gastroschisis?
- young maternal age
- low parity
- maternal smoking, drug abuse
- maternal urinary tract infection, sexually transmitted disease

What is the genetic inheritance of gastroschisis?
- usually autosomal recessive
- can be also sporadic mutation and autosomal dominant

What is the ideal mode of delivery for fetal gastroschisis?
- no difference in neonatal outcome between vaginal and abdominal delivery
- abdominal delivery only by obstetric indication
- may opt for abdominal delivery in the event of lack of resources

What is the ideal timing of delivery?
- no consensus, but ideally at term or with evidence of lung maturity through amniotic sampling

What is the prognosis of gastroschisis?
- 90% survival

What factors badly affect the prognosis of gastroschisis?
- prematurity
- presence of other congenital anomaly
- in utero infection

What is the most common accompanying congenital anomaly?
- atresia of intestine with obstruction
- other malformations are rare

What is the expected neonatal management for baby postdelivery?

At birth, the exposed intestines will be carefully wrapped to prevent heat loss and dehydration and the newborn baby will be transferred immediately to the Children’s Hospital. One option is to bring the baby to the operating room soon after birth and attempt to return all the intestines inside the abdomen. This is called a one-stage or primary repair. In some cases, this is not possible since the abdominal cavity is “too small” relative to the amount of intestines that need to be placed inside. In these cases, we construct a “silo” that we sew to the abdominal wall. The excess bowel is left in the silo and each day a little bit is pushed inside until the silo can be removed. This is called a staged repair. More recently, another option is the placement of a “spring-loaded silo” that can be placed at the bedside without general anesthesia. The definitive closure in the operating room takes place a few days later.



gastroschisis

3 comments:

Gypsy Rose said...

Nice, concise and informative. My grandbaby was just diagnosed with gastroschisis- due in Feb. More young women need to know the risks of pregnancy at early age.

pilocarpine said...

i hope everything goes well with your grandbaby...

Raeanne said...

I had a baby with gastroschisis in Nov 2008. I don't fit into the demographic of someone who would usually have a baby with this condition, and neither do many of the other gastroschisis parents I have met since our experience, which leads me to question what is actually causing it.
Unfortunately in the US and other countries around the world (especially Australia and the UK), this defect is on the rise, and the statistic is no longer 1 in 10,000; according to the latest US CDC report it is now 1 in 2,229. There is also a new support organization that has developed since our experience with our daughter called Avery's Angels Gastroschisis Foundation, and while many/most members are located in the US, the organization wishes to be a global support for families affected by this birth defect. More information can be found at www.averysangels.org