INTRODUCTION:

Gastroschisis is a congenital anterior abdominal wall defect characterized by evisceration of intra-abdominal organs without covering membrane through a defect in form of cleft on the right side of umbilical cord. It means “cleft belly” in the Ancient Greek language and was first defined by Calder in 16th century in the medical literature. There were no reports of survivors until Watkins closed the defect on a baby with gastroschisis in 1943 (1, 2).³ In cases with gastroschisis the localization and development of the umbilicus remains normal. However, due to failure of the right omphalomesenteric artery a full-thickness anterior abdominal wall defect arises in this area, thus abdominal organs herniate through this defect (3). In our case, the diagnosis of gastroschisis is established at the prenatal fourth month by the detection of right paraumbilical defect.⁴

Gastroschisis occurs early during pregnancy when the muscles that make up the baby’s abdominal wall do not form correctly. A hole occurs which allows the intestines and other organs to extend outside of the body, usually to the right side of belly button. Because the intestines are not covered in a protective sac and are exposed to the amniotic fluid, the bowel can become irritated, causing it to shorten, twist, or swell.⁵

RISK FACTORS:

The factor that increases the risk of gastroschisis is as follows:

· Young mothers<20

· Folic acid deficiency

· Hypoxia

· Salicylates, acetaminophen, ibuprofen and pseudophedrine consumption during pregnancy.

· Marijuana, cocaine and alcohol consumption during pregnancy.

· High risk pregnancy such as those complicated by infection or anything that contributing to low birth weight can increase the incidence of gastroschisis which is more frequent in newborn that are small for gastational age.⁶

· Younger age: teenage mothers were more likely to have a baby with gastroschisis than older mothers, and White teenagers had higher rates than Black or African-American teenagers.⁶

· Alcohol and tobacco: women who consumed alcohol or were a smoker were more likely to have a baby with gastroschisis.

INCIDENCE AND ETIOLOGY:

Gastroschisis occure in about 1 in 11,000 babies. The malformation is slightly more frequent in male than female. The male to female ratio is 1.5:1. It has been reported that the incidence of gastroschisis has increase in recent years.⁷

Gastroschisis as a stand alone congental defect is usually inherited in an autosomal recessive manner. It may begin as a sporadic mutation, can be associate with nongenetic congenital disorder but has also been observed to be autosomal dominant.

PATHOPHYSIOLOGY:

Gastroschisis occur due to herniation (rupture) at the base of the umbilical cord, which allows variable amount of intestine to herniate out (pouch out) into the amniotic fluid. This event can take place antenatal (before birth) or perinatal (around the time of birth).

Failure of mesoderm to form in the body wall

Rupture of the amnion around the umbilical ring with subsequent herniation of bowel

Abnormal involution of the right umbilical vein leading to weakening of the body wall and gut herniation^.

Disruption of the right vitelline (yolk sac) artery with subsequent body wall damage and gut herniation^.

Abnormal folding of the body wall results in a ventral body wall defect through which the gut herniates

Failure to incorporate the yolk sac and related vitelline structures into the yolk sac⁸

CLINICAL MANIFESTATION:

· The abdominal wall defect is fairly uniform in size (<5cm) and locaton (to the right of umbilical cord)

· The amount of inflammation, edema and turgor of the intestine, as the size of abdominal cavity, may vary and determine whether reduction of the extruded intestine and closure of the of the bowel so it may be difficult to determine if associated intestinal Atresia is present.⁹

· Intestinal dysfunction may take as long 4-6 weeks to resolve.

DIAGNOSTIC EVALUATION:

Gastroschisis can be diagnosed during pregnancy or after the baby is born. During Pregnancy

During pregnancy, there are screening tests (prenatal tests) to check for birth defects and other conditions. Gastroschisis might result in an abnormal result on a blood or serum screening test or it might be seen during an ultrasound (which creates pictures of the body).¹⁰

After the Baby is born

Gastroschisis is immediately seen at birth

OTHER:

· Polyhydroamnios occur in association with fetal gastroschisis and intestinal atresia.

· Elevation of maternal serum alpha- fetoprotein is associated with gastroschisis.

· An elevated MSAFP level is an indication for ultrasonography to determine if structural abnormalities are present in the fetus. Infants with gastrochisis can be identified by prenatal ultrasonography. When gastroschisis is identified antenatally, serial ultrasonography is performed to identify impending threats to the intestine.¹¹

· Amniocentesis is used to monitor lung maturity and determine when to induce labor.

MANAGEMENT:

Soon after the baby is born, surgery will be needed to place the abdominal organs inside the baby’s body and repair the defect.¹²

If the gastroschisis defect is small (only some of the intestine is outside of the belly), it is usually treated with surgery soon after birth to put the organs back into the belly and close the opening. If the gastroschisis defect is large (many organs outside of the belly), the repair might done slowly, in stages. The exposed organs might be covered with a special material and slowly moved back into the belly. After all of the organs have been put back in the belly, the opening is closed.

Babies with gastroschisis often need other treatments as well, including receiving nutrients through an IV line, antibiotics to prevent infection, and careful attention to control their body temperature.

A. MEDICAL MANAGEMENT:

· The gastroschisis should be carefully wrapped in pads soaked n saline so that the herniated intestine does not dry out.

· A nasogastric tube is put to remove air and decompress the intestine.

· Respiratory distress in a neonate with gastroschisis may respond to gastrc decompression, although endotracheal intubation may still be needed.

· Fluid, electrolyte, and heat losses must be minimized and corrected. Because of significant ongoing fluid losses with an open abdominal wall defect, administer IV fluid (20ml/kg, lactated ringer solution or normal saline), followed by 10% dextrose and normal saline solution at 2-3 times the baby’s maintenance fluid requirement.¹³ This will also help compensate for postoperative fluid space fluid loses.

· The baby should be placed under a radiant heater.

· A urinary catheter should be inserted to monitor urine output and asses the efficacy of fluid resuscitation.

· A rectal examination should be performed to dilate the anal canal. Reduction of the herniated viscera is facilitated by evacuating meconium from the sigmoid colon.

· Broad spectrum antibiotics are administered to prevent contamination of the peritoneal cavity.

· A central venous line may be placed intra operatively to provide parenteral nutrition, thereby minimizing catabolic protein loss during the period of gastrointestinal dysfunction.

B. SURGICAL CARE:

Soon after the baby is born, surgery will be needed to place the abdominal organs inside the baby's body and repair the abdominal wall. Even after the repair, infants with gastroschisis can have problems with feeding, digestion of food, and absorption of nutrients. Silastic sheet are sutured to full thickness of the extended abdominal wall defect and closed over the eviscerated intestine, whose reduction is facilitated by stretching the abdominal musculature, emptying the stomach and bladder, and manually evacuating the colon. The general procedure for gastroschisis is to simply tuck the protruding organ back into the opening and apply a belly band pressure until the wound healing occurs.¹⁴ New advances have been pioneered in repairing the protruding bowel by placing a protective “silo” around the intestine outside the abdomen and then slowly pressing the herniated intestine into the abdominal cavity. This new procedure allows the bowel to return to its intended shaped and location without further traumatizing the infant’s viscera with undue intestinal pressure. The main cause for lengthy recovery period in patient is the time taken for the infant’s bowel function to return to normal.

C. NURSING MANAGEMENT:

· Thermoregulation is critical because significant heat loss can occur through the exposed intestine.¹⁵

· Use warmers and monitor the child’s temperature.

· Maintain asepsis. (during any procedure, hygiene maintenance.)

· Minimize movement of the infant.

· Assess for circulatory compromise, obstruction, sepsis: monitor temperature, pulse, capillary refill time, skin colour, changes in the respiratory pattern and heart rate.

· Observe for respiratory distress.

· Fluid-volume management: monitor intake and output, daily weights, assess fontanels and maintain IV line.

· Maintain NG tube for decompression, monitor bowel sounds and stools, measure abdominal girth.

· Maintain parenteral nutrition to sustain growth.

· Provide emotional support for parents (loss of the “perfect child”.)

· Encourage parents for provision of care.

POST-OP NURSING CARE:

· Maintaining fluid and electrolyte balance

· TPN via central venous catheter to provide nutrition while bowel rests and heals

· Progress to oral feedings once bowel motility occurs

· Prevent Infection (IV antibiotics.)

· Assess for Complications.

· Educate parents.

PROGNOSIS:

Current advance in surgery techniques and intensive care management of neonates the survival rate of 90%, in adequate setting. Prenatal diagnosis either through ultrasonography or any other method available, allow the mother to be refered to an adequate centre, where a caesarean section or induced natural birth can be done before term. Soon after birth, surgery is perform on the newborn. The morbidity is closely related to the presence of other malformation and complication of the wound or intestine. Patents frequently require more than one surgery.

Difference between gastrochisis and omphalocele:

FEATURES	GASTROCHISIS	OMPHALOCELE
Defect	Open	Membrane covered
Defect size	2-5cm	2-15 cm
Umbilical cord	Left of defect	Centre of membrane
Bowel	Inflammed	Normal
Anomalies	10%	60% associated condition


GASTROCHISIS	OMPHALOCOELE

DISCUSSION:

Gastroschisis remains as one of the congenital abdominal wall defect. Anterior abdominal wall muscles develop from muscle cells migrating from myotomes into the somatic mesoderm, which appears in the end of the 2nd week of intrauterine life.¹⁶ There are various theories established to explain the embryopathogenesis related to the appearance of the anomaly. These theories contain possible pathologies such as a vascular accident associated with right umbilical vein or right omphalomesenteric artery, a rupture occurring on the bottom of umbilical cord during the physiologic herniation of intestines.¹⁷ No matter which of them occurred, the primer cause for the appearance of the anomaly is the inconsistency of the interaction between ectoderm and mesoderm which is normally supposed to compose anterior abdominal wall in the end of the 3rd week of intrauterine development. Gastroschisis, also known as “laparoschisis”, is encountered in one of 10000 newborns. The incidence does not vary concerning race or gender. However, it is indicated that being a mother in a very young age rises the risk of gastroschisis. Another aspect regarding the disease is that for mother to be a smoker. It is shown that smoking increases the risk of encountering anterior abdominal wall defect by 2.1 times .¹⁸Werler et al. reported taking pseudoephedrine increases the risk of gastroschisis by 3 times, while salicylate and acetaminophen by 11 times. The defect on anterior abdominal wall is a 2-4 cm sized full-thickness abdominal wall defect located on mostly right, rarely left lateral side of umbilical cord. The location of umbilical cord remains unchanged. Frequently intestines and sometimes stomach, colon herniate towards the outside of abdomen. In some cases; bladder, uterus, tubes even testicles and overs may also eviscerate thorough the defect. If liver occasionally is located outside of the defect, this indicates poor prognosis.

Recent findings:

The incidence of gastroschisis has increased dramatically over the past 20 years, leading to a renewed interest in causation. The widespread availability of maternal screening and ultrasound results in very high rates of prenatal diagnosis, which enables evaluation of the optimal timing and mode of delivery.¹⁹ The preferred method of surgical closure continues to be an issue of debate among pediatric surgeons, whereas postsurgical treatment seeks to expedite the initiation and progression of enteral feeding and minimize complications. A small subset of babies with complex gastroschisis leading to intestinal failure benefit from the knowledge and expertise of dedicated interdisciplinary teams, which seek to bring novel therapies and improved clinical outcomes.

REFERENCES:

1. Williams LJ, Kucik JE, Alverson CJ, Olney RS, Correa A. Epidemiology of gastroschisis in metropolitan Atlanta, 1968 through 2000. Birth Defects Res A. 2005; 73:177-83.

2. Bird TM, Robbins JM, Druschel C, Cleves MA, Yang S, Hobbs CA, and the National Birth Defects Prevention Study (2009). Demographic and environmental risk factors for gastroschisis and omphalocele in the National Birth Defects Prevention Study. J Pediatr Surg, 44:1546-1551.

3. Feldkamp ML, Reefhuis J, Kucik J, Krikov S, Wilson A, Moore CA, Carey JC, Botto LD and the National Birth Defects Prevention Study. Case-control study of self reported genitourinary infections and risk of gastroschisis: findings from the national birth defects prevention study, 1997-2003. BMJ. 2008 Jun 21; 336(7658): 1420-3.

4. Başak E. Doğumsal karın duvarı defektleri tanı ve tedavisi. 2013 April. Available from: URL: http://www.medikalakademi.com.tr/ dogumsal-karin-duvari-defektleri-tani-ve-tedavisi/.

5. Bosnalı O, Gülçin N, Celayir AC et al. Karın ön duvarı defektierinde sağkalım oranlarını etkileyen faktörler. Zeynep Kamil Tıp Bülteni 2013; 44(1):43-7.

6. Hoyme HE, Jones MC, Jones KL. Gastroschisis: Abdominal wall distruption secondary to early gestational interruption of the omphalomesenteric artery. Sem Perinatol 1983; 7: 294-8.

7. Sharma Rimple, Essentials of Pediatrics Nursing, jaypee Brothers Medical Publishers (P) LTD, 1^st edition, page no 71-76.

8. Ghai OP, Essential Pediatrics, CBS Publishers and Distributors Pvt Ltd, Seventth edition, Page no 34-41.

9. Başaklar C. Bebek ve çocukların cerrahi ve ürolojik hastalıkları. 1. Baskı. Ankara: Palme Yayıncılık; 2007.p. 1053-86.

10. Roeper PJ, Harris J, Lee G, Secular rates and correlares for gastrochisis in California (1968-1977). Teratology 1987; 35: 203-10.

11. IGNOU Article, Pediatric Nursing, page no 77-83.

12. Wilson Hockenberry, Nursing care of Infant and Children, 17 edition, page no 62-65.

13. Martinez-Frias MLi Prieto SL, Zaplana J. Epidemiological study of gastroschisis and omphalocele in Spain. Teratology 1984; 29:337-82.

14. Haddow JE, Polamaki GE, Holman MS. Young maternal age and smoking during pregnancy as risk factors for gastroschisis. Teratology 1993; 47: 225-8.

15. Werler MM, Mitchell AA, Shapiro S. First trimester maternal medication use in relation to gastroschisis. Teratology 1992; 45: 361-7.

16. De Lorenzo M, Yazbeck S, Ducharme JC. Gastroschisis: a 15-year experience. J Pediatr Surg. 1987; 24: 343-5.

17. https://www.americanjournalofsurgery.com/article/0002-9610(58) 90471-9/pdf

18. Datta Parul, Pediatric Nursing, 2^nd Edition, Page no 16-24.

19. Parker SE, Mai CT, Canfield MA, Rickard R, Wang Y, Meyer RE, et al; for the National Birth Defects Prevention Network. Updated national birth prevalence estimates for selected birth defects in the United States, 2004-2006. Birth Defects Res A Clin Mol Teratol. 2010; 88(12): 1008-16.

Received on 20.08.2020 Modified on 29.09.2020

Int. J. Nur. Edu. and Research. 2021; 9(1):108-112.

DOI: 10.5958/2454-2660.2021.00027.2