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New Therapeutic and Diagnostic Approach in Laparoschisis and Giant Omphalocele

Lazea Stefan, Vrinceanu Luminita, Mandrusca Ramona

Pediatric Surgery Department, Emergency Children Hospital Timisoara, Romania

 

Abstract

During the past decade, significant changes have occurred in the management of diagnosis and therapy of the new-born with gastroschisis and omphalocele. Prenatal ultrasonography and measurement of maternal serum alpha-protein have been found to be useful in identifying infants with an abdominal wall defect (17,19)

We report two babies treated in our clinic, one with a fetal gastroschisis and the other one with giant omphalocele, antenatally diagnosed by ultrasound and measurement of maternal serum alpha feto-protein. Both the mothers were advised to undergo a cesarean section at 37-38 week’s gestation at a university maternity but their options were different. One followed the obstetrician’s advice and the baby survived but the other one was borne by vaginally delivery, and inspite of all efforts, the baby could not be saved.

Key words: gastroschisis, omphalocele, antenatal diagnosis

 

Correspondence:

Lazea Stefan

Pediatric Surgery Department, Emergency Children Hospital,

Str. Iosif Nemoianu nr.2, Timisoara, Romania

 

Introduction

Prenatal diagnoses of gastroschisis and omphalocele requires adequate antenatal counseling depending upon the gestational age at diagnoses and association of other congenital anomalies (1,4,11). The medical team must discuss the various management options with the parents, which generally include pregnancy termination or delivery at a tertiary level center that has multimodality support available (2,7,16,20). Despite several decades of investigation and a vast array of new therapeutic approaches, the optimal therapeutic strategy for the newborn with a congenital defect of the abdominal wall remains undetermined. A unanimous management of the delivery has not been established. For small omphalocele and gastroschisis the baby can be delivered normally at term in a center with pediatric surgeons. Large omphalocele should be delivered by cesarean section at a higher level tertiary center (1,4,18).

Case Reports

Case 1

A 3100 grams female baby, the second borne delivered by cesarean section at 38 week’s gestation at a local hospital. The mother had history of polyhydramnios antenatally and she had undergone serial ultrasound studies once a month since the 20th weeks gestation after the fetus was diagnosed with gastroschisis. She was advised by her obstetrician to borne the child at a hospital with an intensive care nursery and experienced pediatric surgeons. She complied with the doctor’s advice and came to Timisoara. The baby was referred from maternity at the age of 30 minutes, in an infant transport incubator, with the exposed intestines covered with a wet wrap. At the Pediatric Surgical Intensive Care Unit, a naso-gastric tube was passed and was electively intubated as she developed respiratory distress syndrome . She was operated one hour after admission. The small intestine was returned easily to the abdomen so a primary repair of the gastroschisis was performed. During the operation and 6 hours postoperatively, the patient was intubated and mechanical ventilated. On the third post operative day, she was given parenteral nutrition and naso-gastric tube feeding was initiated slowly on the fourth post operative day. Unfortunately, on the 6th postoperative day ,the child developed abdominal distension, the gastric aspirate increased and the baby did not pass stool, even with enema. Abdominal radiography showed an airless right lower quadrant and a few dilated loops of bowel. We reopened the abdominal cavity in order to reevaluate the situation. No intestinal atresia was found but a couple of intestinal adhesions. We performed adhesiolysis and inserted a drain in the peritoneal cavity. For three more days she was fed by total parenteral nutrition. Once bowel function returned as evidenced by passing of a bowel movement, feedings via the naso-gastric tube were slowly initiated with Gesol, while intravenous feeds continued. Progresively the volume of the mother’s milk was increased over the next 3 days and the naso-gastric tube removed. The baby was discharged when she took all her feedings by mouth (mother’s milk) and she had an adequate weight gain.(3200 g)

Case 2

A 3000 g male baby was born vaginally at 38 week’s gestation at a regional maternity centre. The diagnosis of a giant omphalocele was suspected on an antenatal ultrasound study at 28 weeks gestation. The diagnosis was confirmed by repeated ultrasound examination and high levels of the maternal serum alpha-protein. The family was suggested to choose one of the next two options:-termination of the pregnancy, because a cardiac malformation was suspected to be associated; or delivery by cesarean section at 27-28 week’s gestation at a university maternity centre, close to a Pediatric Surgery Clinic Department. The mother refused both the options and the baby was borne vaginally at a regional hospital, 200km far from our hospital. It was a prolongation of labour followed by the rupture of the omphalocele membranes, partial evisceration and the contamination of the peritoneal cavity. The baby was transferred from this hospital in poor condition. The baby was diagnosed as Large omphalocele with ruptured membranes, partial evisceration, materno-fetal infection and hypothermia.

At the pediatric Surgical Intensive Care Unit, a naso-gastric tube was passed, antibiotics were instituted and hydration was begun intravenously. At about 4 hours after admission he was operated. Intraoperative examination revealed an abdominal wall defect, 8 cm in diameter, with partial rupture of the membranes and evisceration of the bowel ,the stomach and the right hepatic lobe. A primary surgical closure was impossible because it could result in increased intraabdominal pressure, so it was decided to use the Fufezan procedure-step1.We excised the borders of the ruptured membranes, restored two thirds of the viscera in the hypoplastic abdominal cavity, under a minimal pressure and sutured the borders of the remaining membranes to the border of the fascial defect.

During the operation and 36 hours after, the patient was intubated and mechanically ventilated.

Postoperatively, the recovery was good. The gastric aspirate diminished and the baby passed stool. Once the bowel function returned, the naso-gastric tube was removed.

After 7 days of life, the child’s condition started to deteriorate despite supportive measures. The baby had sepsis and the blood cultures grew Enterobacter. He died on the 13th day of hospitalisation due to Multiple Systemic Organ Failure. The parents did not give consent for postmortem examination.

Discussion

Gastroschisis and omphalocele are congenital defects of the abdominal wall that occur in approximately 1.75 to 2.5 in 10000 pregnancies (1,5,16). Gastroschisis occurs when there is evisceration of abdominal contents through a paraumbilical defect in the abdominal wall, usually to the right of the umbilical cord. It is less commonly associated with other abnormalities with the exception of intestinal atresia that occurs in about 10 percent cases. No genetic association exists and so, it has a better prognosis. On the other hand, omphalocele, a herniation of abdominal contents into the base of the umbilical cord, is more common and carries an increased risk for concomitant abnormalities and associated poor prognosis. However presence of major organ herniation such as liver and spleen makes the prognosis worse in both gastroschisis and omphalocele (7, 11, 15).

The diagnosis is usually made by antenatal ultrasound. The fetal abdominal cavity can be visualized on ultrasonography by 10 weeks following the mother’s last menstrual period. Typically, omphalocele and gastroschisis can be diagnosed around the 13 weeks gestation when the normal return of the fetal intestine to the abdominal cavity have already occurred (3).

On ultrasonography, an omphalocele has a smooth outline with an echogenic covering sac, from which the umbilical cord is seen to arise. In contrast, gastroschisis is less smoothly contoured, has no echogenic covering and is seen at some distance from the umbilical cord (7,20).

Maternal alpha-protein levels are significantly elevated over normal levels and is a useful diagnostic test. In many cases, the diagnosis is made by ultrasound following an elevated second trimester maternal serum AFP test. Nowadays, a special management of the fetus with abdominal wall defect is necessary (3, 7, 11).

Termination of the pregnancy might be offered to those mothers who have a fetus with a large omphalocele or a fetus with multiple congenital anomalies. Continuation of the pregnancy needs a close observation and serial fetal sonography for appropriate advice to the parents. The prognosis largely depends upon the size of the defect and the presence or absence of other birth defects (13, 14, 20). The open fetal surgery and fetoscopic fetal intervention can be performed, only for selected cases, but not in our country.

Delivery at a tertiary center might be indicated so that a multidisciplinary team can immediately evaluate and stabilize the baby. The mode of delivery as well as the postnatal treatment approach depend upon the size of the abdominal defect and the degree of liver involvement. If the defect is small and does not involve the liver, a vaginal delivery might be possible. In case of giant omphalocele cesarean delivery is usually required to avoid membrane rupture and liver trauma (13,14,21).

Fetal surgery is now available to selected infants diagnosed prenatally ,as an experimental procedure at a few research centers in the world. The future of the fetal surgery is yet to be determined. Its success will depend both on accurate identification of the fetus who has a poor prognosis and the demonstration of an increased survival with an in -utero surgey over conventional postnatal therapy for this subgroup of fetuses (3,14,18). Till than we have a long time, but we still believe that in the next few years, even in our country, with better medical education and possibility to antenatally detect major congenital malformations with appropriate management of the pregnancy in tertiary centers, we will be able to improve the prognosis of affected infants (4,19).

Conclusions

The benefits of prenatal diagnosis are as follows:

- Prenatal diagnosis determines the outcome of pregnancy

- It is helpful for couples to decide whether to continue the pregnancy

- It indicates possible complications that can arise at birth process

- Prenatal diagnosis is helpful for the management of the remaining weeks of pregnancy

- It prepares the couple for the birth of a child with an abnormality.

 

 

REFERENCES

1. Bawazir OA, Wong A, Sigalet DL: Absorbable mesh and skin flaps or grafts in the management of ruptured giant omphalocele. J Pediatr Surg 2003 May;

2. Cooney D: Defects of the Abdominal Wall. Pediatr Surg 1998; .

3. de Lorimier AA, Adzick NS, Harrison MR: Amnion inversion in the treatment of giant omphalocele. J Pediatr Surg 1991 Jul;

4. Dinatti LA, Meagher DP Jr, Martinez-Frontanilla LA: “Bucket handle” avulsion of intestine in gastroschisis. J Pediatr Surg 28(6):

5. Duffy PG: Bladder exstrophy. Semin Pediatr Surg 1996 May;

6. Dykes EH: Prenatal diagnosis and management of abdominal wall defects. Semin Pediatr Surg 1996 May;

7. Fok TF, Ng PC, Wong W, et al: High frequency oscillatory ventilation in infants with increased intra- abdominal pressure. Arch Dis Child Fetal Neonatal Ed 1997 Mar;

8. Langer JC: Gastroschisis and omphalocele. Semin Pediatr Surg 1996 May;

9. Molenaar JC: Cloacal exstrophy. Semin Pediatr Surg 1996 May;

10. Moore TC: Omphalomesenteric duct malformations. Semin Pediatr Surg 1996 May

11. Pacilli M, Spitz L, Kiely EM, et al: Staged repair of giant omphalocele in the neonatal period. J Pediatr Surg 2005 May;

12. Puligandla PS, Janvier A, Flageole H, et al: Routine cesarean delivery does not improve the outcome of infants with gastroschisis. J Pediatr Surg 2004 May;

13. Salihu HM, Boos R, Schmidt W: Omphalocele and gastrochisis. J Obstet Gynaecol 2002 Sep;

14. Sauter ER, Falterman KW, Arensman RM: Is primary repair of gastroschisis and omphalocele always the best operation? Am Surg 1991 Mar;

15. Suita S, Okamatsu T, Yamamoto T, et al: Changing Profile of Abdominal Wall Defects in Japan: Results of a National Survey. J Pediatr Surg 2000;

16. Suita S, Nagasaki A: Urachal remnants. Semin Pediatr Surg 1996 May

17. Tan KH, Kilby MD, Whittle MJ, et al: Congenital anterior abdominal wall defects in England and Wales 1987- 93: retrospective analysis of OPCS data. BMJ 1996 Oct 12

18. Vermeij-Keers C, Hartwig NG, van der Werff JF: Embryonic development of the ventral body wall and its congenital malformations. Semin Pediatr Surg 1996 May;

19. Wakhlu A, Wakhlu AK: The management of exomphalos. J Pediatr Surg 2000 Jan; 35(1):

20. Wheatley JM, Stephens FD, Hutson JM: Prune-belly syndrome: ongoing controversies regarding pathogenesis and management. Semin Pediatr Surg 1996 May;